Pediatric Secrets E-Book

Richard A. Polin; Mark F. Ditmar

BOOK

Pediatric Secrets E-Book

Richard A. Polin | Mark F. Ditmar

(2015)

Additional Information

Book Details

ISBN: 978-0-323-31580-7
Edition: 6
Language: English
Pages: 752
Subjects: Paediatric medicine
Medical study & revision guides & reference material

Abstract

Portable and high yield, Pediatric Secrets is perfect for use in clinical rotations, exam prep, or as a handy clinical reference. Drs. Richard A. Polin and Mark F. Ditmar present the essential questions and answers to help you better meet the challenges you face every day, while updated chapters highlight the latest standards in pediatric care. A bestselling volume in the Secret Series®, its Q&A format, helpful lists and tables, mnemonics, and informal tone combine to make reference fast, easy, and enjoyable.

"Key Points" boxes and a highly detailed index allow you to find information quickly and easily.
Unique Q&A format expedites learning and retention.
Top 100 Secrets section combines the top 100, high-yield facts into one chapter, providing a concise overview of the latest issues in pediatrics.
High-yield content is ideal for exam preparation.

Provides the current standards of care for pediatric students and practitioners.
Updated chapters reflect the latest advances in pediatrics.
Medicine eBook is accessible on a variety of devices.

Section Title	Page	Action	Price
Front Cover	Cover
Inside Front Cover	ES2
Pediatric Secrets	iii
Copyright	iv
Preface	v
Contributors	vi
Contents	x
Top 100 secrets	1
Chapter 1: Adolescent Medicine	7
clinical issues	7
1. What are the three leading causes of mortality in adolescents?	7
2. How common is dating violence among adolescents?	7
3. Which sports cause the greatest number of concussions in teenagers?	7
4. Which diagnoses require mandatory disclosure regardless of confidentiality?	7
5. How does the ``HEADS´´ mnemonic assist in adolescent interviewing?	7
6. When does sexual orientation usually emerge?	8
7. What characterizes gender identity, gender expression, and gender dysphoria?	8
8. What health disparities are particular to LGBTQ youth?	8
9. How may social media impact adolescent behavior?	8
10. What is cyberbullying?	8
11. Which teenagers <18 years can give consent for their medical care?	8
Eating disorders	9
12. How is the diagnosis of anorexia nervosa made?	9
13. What are signs of anorexia nervosa on physical examination?	9
14. What are the differential diagnoses that one must consider when evaluating a patient with anorexia nervosa?	9
15. What are good and bad prognosticators for recovery from anorexia?	9
16. Why are adolescent girls with anorexia nervosa at risk for low bone mineral density?	9
17. What are the clinical differences between males and females with anorexia nervosa?	9
18. What electrolyte disturbances occur in patients with severe anorexia nervosa and what are the potential clinical effects?	10
19. What causes sudden death in patients with anorexia nervosa?	10
20. What are indications for hospital admission for a patient with anorexia nervosa?	10
21. What are the medical complications of bulimia nervosa?	10
22. An 11-year-old with weight loss due to avoidance of food because of its sensory characteristics has what condition?	11
23. What is the primary biochemical feature of the refeeding syndrome?	11
24. Name the three features that constitute the ``female athlete triad.´´	11
Menstrual disorders	12
25. What is the median age of menarche in the United States?	12
26. How do you define a normal menstrual cycle?	12
27. What is the physiology of a normal menstrual cycle?	12
28. What is the difference between primary and secondary amenorrhea?	12
29. What is the value of a progesterone challenge test in a patient with amenorrhea?	12
30. What are some of the causes of amenorrhea in adolescents?	12
31. How do you define the different types of ``rrhagias´´?	12
32. What is the differential diagnosis of heavy menstrual bleeding?	12
33. You see an 18-year-old female who comes to your office complaining of 10 days of heavy menstrual bleeding, including s ...	14
34. What are the two key clinical features that determine the management of abnormal uterine bleeding?	14
35. How would you treat a patient with heavy menstrual bleeding?	14
36. You see a 16-year-old overweight female who reports having irregular periods, acne, and having to remove hair on her u ...	14
37. How common is dysmenorrhea?	14
38. Does dysmenorrhea occur more commonly in early or late adolescence?	14
39. What is the difference between primary and secondary dysmenorrhea?	14
40. What two classes of medications are most commonly used for dysmenorrhea?	15
41. What is a common cause of chronic pelvic pain in adolescents without a history of pelvic inflammatory disease (PID)?	15
42. What is the peak age for ovarian torsion?	15
43. In what setting should ectopic pregnancy be suspected?	15
Obesity	16
44. What is the body mass index (BMI)?	16
45. How predictive is early childhood obesity of later adolescent obesity?	16
46. What are some of the health risk factors related to obesity?	16
47. What variety of factors may contribute to obesity?	17
48. What features on physical examination are particularly important in the evaluation of the obese patient?	17
49. How does sleep affect weight?	17
50. What are the diagnostic criteria for the metabolic syndrome?	17
51. Why is a short obese 11-year-old of more clinical concern than a tall obese 11-year-old?	17
52. What are some key points when discussing weight reduction counseling and management with a teenager?	18
53. What are the indications for bariatric surgery in adolescents?	18
Sexual development	18
54. What is Tanner staging for boys?	18
55. What is the normal progression of sexual development and growth for boys during puberty?	19
56. What are the ranges of normal in the stages of pubertal development in girls?	19
57. What is the normal progression of sexual development and growth for girls during puberty?	20
58. Has the age of menarche declined in the United States during the century?	20
59. When do boys develop the ability to reproduce?	20
60. How is delayed puberty defined?	20
61. Why should the sense of smell be tested in a teenager with delayed puberty?	20
62. What is the most common cause of delayed puberty?	20
63. What features suggest constitutional delay of puberty?	21
64. Which laboratory tests should you consider in a boy or girl with delayed puberty?	21
65. What is the most common cause of primary gonadal failure in boys?	21
66. Can puberty be safely accelerated?	21
67. How do you evaluate a breast lump noted by a teenage girl on self-examination?	21
68. Should breast self-examination be taught and emphasized for all teenage girls?	22
Sexually transmitted infections	22
69. How does the prevalence of sexually transmitted infections (STIs) in adolescents compare with that of adults?	22
70. What is the best way to screen for STIs?	22
71. What is the most common STI in sexually active adolescent females?	23
72. How should we screen for STIs in adolescent females?	23
73. How should we screen for STIs in adolescent males?	23
74. Are pelvic examinations with specula always required to obtain specimens for STI diagnosis in teenagers?	23
75. Which STI is most closely linked to cervical cancer?	23
76. What are the manifestations of HPV infection?	23
77. When are Pap smears indicated in teenagers?	24
78. Describe the appearance of condylomata acuminata	24
79. What is the natural history of genital warts?	24
80. What is the typical presentation of chlamydial genital infections in both female and male teenagers?	24
81. What is the typical appearance of N. gonorrhoeae on Gram stain?	25
82. What are the minimal criteria for the diagnosis of PID?	25
83. What additional criteria support the diagnosis of PID?	25
84. Which adolescents with PID should be hospitalized for intravenous antibiotics?	25
85. What are the common causative pathogens for PID?	26
86. A sexually active 17-year-old girl with adnexal and RUQ tenderness probably has what condition?	26
87. What are the sequelae of PID?	26
88. How are the genital ulcer syndromes differentiated?	26
89. What are risk factors for the acquisition of genital ulcer disease?	27
90. What are the main differences between HSV-1 and HSV-2?	27
91. How do recurrent episodes of genital herpes simplex infections compare with the primary episode?	27
92. How are the three most common causes of postpubertal vaginitis clinically distinguished?	28
93. How does the vaginal pH help determine the cause of a vaginal discharge?	28
94. How does evaluation of the vaginal discharge help identify the etiology?	28
95. How is trichomoniasis diagnosed?	28
96. If a patient is receiving a standard treatment for a trichomonal infection, why should alcohol be avoided?	29
97. What are ``clue cells	29
98. What is the etiology of bacterial vaginosis?	29
99. What are the criteria for the diagnosis of bacterial vaginosis?	29
100. What is expedited partner therapy?	29
101. What are CDC treatment recommendations for common STIs?	29
Substance abuse	30
102. What are the categories of abused drugs?	30
103. What is the CRAFFT screen?	31
104. What are characteristic physical signs of illicit drug use?	31
105. Should an adolescent be screened for drug abuse without his or her consent?	32
106. How long do illicit drugs remain detectable in urine specimens?	32
107. What is the genetic predisposition for alcoholism?	33
108. What are other individual risk factors for alcohol abuse?	33
109. Which type of substance abuse is more common in younger adolescents than older adolescents?	33
110. What is the leading cause of fatality related to inhalant abuse?	33
111. What are the toxicities of chronic marijuana use?	33
112. Is marijuana a ``gateway´´ drug?	33
113. What performance-enhancing drugs are used by teenagers?	33
114. What are the potential side effects of anabolic steroids?	34
115. When does cigarette smoking begin?	34
116. What are the main reasons that cigarette smoking begins?	34
117. What are the risks of chewing tobacco?	34
118. What are the risks of E-cigarette use among adolescents?	34
119. What are the 5 ``As´´ of smoking cessation counseling?	35
120. Are tattoos a tip-off to high-risk behaviors?	35
Teenage male disorders	35
121. How common is gynecomastia in teenage boys?	35
122. Why does gynecomastia occur so commonly in young teenage boys?	35
123. Which boys with gynecomastia warrant further evaluation?	35
124. What are the clinical manifestations of testicular torsion?	36
125. When is testicular torsion likely to occur?	36
126. How is testicular torsion diagnosed?	36
127. How is testicular torsion treated?	36
128. If complete testicular torsion has occurred, how long is it before irreversible changes develop?	36
129. How is testicular torsion clinically differentiated from other causes of the acute painful scrotum?	36
130. How does the Prehn sign help distinguish between epididymitis and testicular torsion?	37
131. What is the most frequent solid cancer in older adolescent males?	37
132. What is the significance of a varicocele in a teenager?	37
133. Which varicoceles warrant surgical intervention?	37
134. On which side do varicoceles more commonly occur?	37
135. What is the difference between phimosis and paraphimosis?	37
136. What are pearly penile papules and should a teen worry about them?	37
Teenage pregnancy and contraception	38
137. What are trends in teenage pregnancy in the United States?	38
138. What factors make it more likely that a teenager will become pregnant?	38
139. If a teenager has been pregnant once, how likely is she to become pregnant again during her teenage years?	38
140. What are the risks for infants of teenage mothers?	38
141. How soon after conception will a urine pregnancy test become positive?	38
142. Which contraceptive methods are appropriate for adolescents?	39
143. What are contraindications to the use of estrogen-containing contraceptive methods?	39
144. How likely are teenagers to use contraception?	39
145. Is a pelvic examination mandatory before starting a patient on contraception?	40
146. What oral treatment is most commonly used for emergency postcoital contraception?	40
Teenage suicide	40
147. What is the main predictor of suicidal ideation in teenagers?	40
148. How often do adolescents attempt suicide in the United States?	40
149. Who are more likely to attempt suicide, males or females?	40
150. Which adolescents are at increased risk for suicide?	41
151. Is a 16-year-old female able to give consent for treatment of a sexually transmitted infection (STI) in most states?	41.e1
152. What drugs are associated with gynecomastia?	41.e1
153. What is the Maudsley Method for treating anorexia nervosa?	41.e1
154. If a teenager has infectious mononucleosis (IM), how likely is she to develop chronic fatigue syndrome (CFS)?	41.e1
155. What is binge eating disorder?	41.e1
156. A teenage girl develops migratory polyarthritis, fever, and scattered petechial lesions several days before menses. ...	41.e1
157. How is the diagnosis of PID definitively made?	41.e1
158. What are the two subtypes of anorexia nervosa?	41.e2
159. What hormonal abnormalities may be seen in anorexia nervosa?	41.e2
160. How is the diagnosis of bulimia nervosa made?	41.e2
161. What modalities are used to treat eating disorders?	41.e2
162. How should a very anemic teenager with abnormal uterine bleeding and orthostatic signs be managed?	41.e2
163. How effective is treatment for bacterial vaginosis?	41.e3
164. Why do more ovarian torsions occur on the right than the left?	41.e3
165. Is ultrasonography typically diagnostic in the setting of ovarian torsion?	41.e3
Chapter 2: Behavior and Development	42
Attention-deficit/hyperactivity disorder	42
1. What are the characteristics of attention-deficit/hyperactivity disorder (ADHD)?	42
2. How common is ADHD?	42
3. Are boys or girls more likely to be diagnosed with ADHD?	42
4. Is there a genetic predisposition to ADHD?	42
5. What conditions can mimic ADHD?	42
6. Is there a definitive diagnostic test for ADHD?	43
7. How should ADHD be treated?	43
8. What are the best medications for treating ADHD?	43
9. Is a positive response to stimulant medication diagnostic of ADHD?	43
10. Is an electrocardiogram (ECG) required before beginning patients on stimulant medication for ADHD?	43
11. How young is ``too young´´ to diagnose ADHD and prescribe stimulant medications?	43
12. What are the risks for adolescents with ADHD?	44
13. Does sugar or food additives make children hyperactive?	44
14. Are complementary or alternative medicine (CAM) therapies beneficial for ADHD?	44
15. Do children with ADHD become teenagers and adults with ADHD?	44
Autism	45
16. What is the DSM-5?	45
17. How did classifications change for disorders of autism in the DSM-5?	45
18. What are the two essential features of autism?	45
19. Which behaviors of children should arouse suspicion of possible autism?	45
20. When should screening be done for autism?	45
21. What studies should be considered in the evaluation of a child with suspected autism?	46
22. What accounts for the apparent increase in autism in the United States?	46
23. Do vaccines cause autism?	46
24. Does early intervention and/or therapy improve the outcome in children with autism?	46
Behavior problems	47
25. What are the most common types of behavior problems in children?	47
26. How much do babies normally cry each day?	47
27. What is infantile colic?	47
28. What causes colic?	47
29. Are there any treatments that are useful for colic?	47
30. What evaluations should be done for the excessively crying infant?	47
31. How should children be punished?	48
32. How valid is the proverb ``spare the rod and spoil the child´´ as a defense for corporal punishment?	48
33. Is physical injury a concern in children with head banging?	48
34. What is the difference between a ``blue´´ breath-holding spell and a ``white´´ breath-holding spell?	48
35. When should a diagnosis of seizure disorder be considered rather than a breath-holding spell?	48
36. Does treatment with iron decrease the frequency of breath-holding spells?	49
37. When does prolonged thumb-sucking warrant intervention?	49
38. What treatments are used for thumb-sucking?	49
39. When should ``toilet training´´ be started?	49
40. Are girls or boys toilet trained earlier?	49
Cranial disorders	49
41. How many fontanels are present at birth?	49
42. When does the anterior fontanel close?	49
43. Which conditions are most commonly associated with premature or delayed closure of the fontanel?	50
44. When is an anterior fontanel too big?	50
45. What are the types of primary craniosynostosis?	50
46. What is the most common type of primary craniosynostosis?	50
47. What causes craniosynostosis?	51
48. What is positional or deformational plagiocephaly?	51
49. How is positional plagiocephaly differentiated from plagiocephaly caused by craniosynostosis?	51
50. What conditions are associated with skull softening?	51
51. What is the significance of craniotabes?	52
52. What evaluations should be done in a child with microcephaly?	52
53. What are the three main general causes of macrocephaly?	52
Dental development and disorders	53
54. When do primary and permanent teeth erupt?	53
55. What is the significance of natal teeth?	53
56. How common is the congenital absence of teeth?	53
57. What are mesiodentes?	53
58. What is the significance of an infant presenting with a single central upper tooth?	53
59. What is a ranula?	54
60. Where are Epstein pearls located?	54
61. What is the most common chronic disease of childhood?	54
62. What are milk-bottle caries?	54
63. How does fluoride minimize the development of dental caries?	54
64. What is fluorosis?	55
65. How long should fluoride supplementation be continued?	55
66. How effective are dental sealants for preventing cavities?	55
67. How common is gingivitis in children?	55
68. What is the largest health-related expense before adulthood for normally developing children?	55
69. What causes halitosis is children?	55
70. Pacifiers: friend or foe?	56
Developmental assessment	56
71. What aspects of development are typically monitored?	56
72. What are primitive reflexes?	56
73. What three primitive reflexes, if persistent beyond 4 to 6 months, can interfere with the development of the ability ...	56
74. At what age do children develop handedness?	56
75. What percentage of children are left-handed?	56
76. Are there ethnic differences in development in the first year of life?	57
77. What are the major developmental landmarks for motor skills during the first 2 years of life?	57
78. How valuable is the timing of crawling as a marker of development?	57
79. What are the most common causes of gross motor delay?	57
80. What are major red flags that a childs development is abnormal?	57
81. What features suggest a possible metabolic cause for disordered development?	58
82. Do infant walkers promote physical strength or development of the lower extremities?	58
83. Do twins develop at a rate that is comparable to infants of single birth?	58
84. Do premature infants develop at the same rate as term infants?	58
85. When can an infant smell?	59
86. What are the best measures of cognitive development?	59
87. What do the stages of play tell us about a child's development?	59
88. What can one learn about a child's developmental level with regard to the use of a crayon?	59
89. What is the value of the Goodenough-Harris drawing test?	60
90. What are key physical exam features in the evaluation of a child with possible developmental delay?	60
91. In infants with global developmental delay, what are the likely causes?	60
92. What factors increase the likelihood of finding a potentially progressive disease in patients with global delay?	60
Language development and disorders	60
93. What are average times for the development of expressive, receptive, and visual language milestones?	60
94. What are signs of significantly delayed receptive and expressive speech warranting evaluation?	61
95. Do deaf infants babble?	61
96. At what age does a child's speech become intelligible?	61
97. What are the most common causes of so-called delayed speech?	61
98. What risk factors make hearing loss more likely in a newborn or young infant?	61
99. What causes flat tympanograms?	62
100. A toddler with a bifid uvula and hypernasal speech most likely has what condition?	62
101. When is stuttering abnormal?	63
102. What advice should be given to parents of a child who stutters?	63
103. Which infants with ``tongue tie´´ should have surgical correction?	63
Mental retardation/intellectual disability	64
104. Why is the term mental retardation being changed?	64
105. How is intellectual disability defined?	64
106. How is intelligence classified with IQ scores?	64
107. What features can indicate cognitive problems in infants and young children?	64
108. Worldwide, what is the most common preventable cause of intellectual disability?	65
Psychiatric disorders	65
109. What is the prevalence of childhood psychiatric disorders?	65
110. What items constitute the ``11 Action Signs´´?	65
111. If a parent has an affective disorder, what is the likelihood that an offspring will have similar problems?	66
112. How does mania differ in children and adolescents?	66
113. What ritualistic behaviors are common in children with obsessive-compulsive disorder?	66
114. What are the differences between child-onset OCD and adult-onset OCD?	66
115. What distinguishes a conduct disorder from an oppositional defiant disorder?	67
116. What are common symptoms of depression in children and adolescents?	67
117. How is major depressive disorder in children diagnosed?	67
118. What are treatments for major depressive disorder in children and adolescents?	67
119. How likely is it that a depressed teenager will be a depressed adult?	67
120. What are types of anxiety disorders in children?	67
121. Which is preferable for children with anxiety disorders, cognitive behavioral therapy (CBT) or medication?	68
122. What characterizes bipolar disorder?	68
Psychosocial family issues	68
123. How likely is it that children in the United States will experience the separation or divorce of their parents?	68
124. How do children of different ages vary in their response to parental divorce?	68
125. What factors are central to a good outcome after a divorce?	68
126. What is the ``vulnerable child syndrome´´?	69
127. How does the cognitive understanding of death evolve?	69
128. Should adopted children be informed of their adoption?	69
129. How common is domestic violence?	69
130. Who are ``latchkey´´ children?	69
131. What are the effects of heavy television watching in young children?	70
School problems	70
132. How is ``learning disability´´ defined?	70
133. What distinguishes dyslexia, dyscalculia, and dysgraphia?	70
134. What are clues that a school-age child may have dyslexia?	70
135. How are the two types of school avoidance behaviors distinguished?	71
136. How much of a problem are bullies?	71
Sleep problems	71
137. What is the average daily sleep requirement by age?	71
138. Why is the supine sleeping position recommended for infants?	71
139. When do infants begin to sleep through the night?	71
140. What advice to parents may minimize the problem of night waking?	71
141. How common are sleep problems in elementary school-age children?	72
142. What are parasomnias?	72
143. At what age do sleepwalking and sleeptalking occur?	72
144. What is the difference between nightmares and night terrors?	72
145. What recommendation should be given to a parent whose child is having night terrors?	72
Visual development and disorders	72
146. How well does a newborn see?	72
147. Do babies make tears?	72
148. At what age does an infant's eye color assume its permanent color?	73
149. A 2-week-old infant with intermittent eye discharge and clear conjunctiva has what likely diagnosis?	73
150. What are the valves of Rosenmüller and Hasner?	73
151. What is normal visual acuity for children?	73
152. When do binocular fixation and depth perception develop in children?	73
153. How does refractive capacity vary with age?	74
154. How are the degrees of blindness classified?	74
155. What is strabismus?	74
156. A 2-month-old baby is noted to have eyes that appear to turn outward rather than looking forward. Is this strabismus?	74
157. Name the types of childhood strabismus	75
158. What separates pseudostrabismus from true strabismus?	75
159. What is amblyopia?	76
160. What are the causes of amblyopia?	76
161. Which treatments are effective for amblyopia?	76
162. What is the red reflex test?	76
163. Why are early diagnosis and treatment critical for patients with congenital cataracts?	76
164. What is ectopia lentis?	76
165. What diseases may present with a white pupil?	76
166. How common are unequally sized pupils?	77
167. Is heterochromia normal?	77
168. When are children aware of color differences?	77
169. How is color blindness inherited?	77
170. What are the comorbid disorders commonly seen with ADHD?	77.e1
171. When is masturbation in a child considered pathologic?	77.e1
172. Does participation in day care during infancy and the toddler years have negative effects on cognitive development?	77.e1
173. In addition to learning disabilities, what factors may contribute to academic underachievement?	77.e1
Chapter 3: Cardiology	78
Clinical issues	78
1. Is cardiac pathology the most common cause of chest pain in children?	78
2. What is the clinical distinction between costochondritis and Tietze syndrome?	78
3. What are potential red flags that increase the likelihood of a cardiac cause for chest pain?	78
4. A child with sharp, stabbing, very localized chest pain that occurs at rest and resolves completely without associated...	78
5. What is the significance of mitral valve prolapse (MVP)?	79
6. What connective tissue diseases may be associated with MVP?	79
7. What are the common types of vascular rings and slings?	79
8. What evaluations are commonly done if a vascular ring is suspected?	80
9. Describe four categories of cardiomyopathy in children	80
10. What mineral is added to hyperalimentation fluids to prevent a potential cardiomyopathy?	81
11. What are the cardiac causes of sudden cardiac death in children and adolescents?	81
12. What historical features may identify the patient who is at risk for sudden death?	81
13. What features in the preparticipation sports physical examination identify patients at risk for sudden death?	81
14. Should an electrocardiogram (ECG) be included in the preparticipation screening of young athletes?	81
15. Name five disorders in which a screening ECG might identify a subject at risk for sudden death	82
16. What is the likely diagnosis in a 10-year-old little leaguer who develops sudden cardiac arrest after being struck in...	82
17. In which patients is syncope more likely to be of a cardiac nature?	82
18. What arrhythmias may be associated with syncope?	82
19. What are the most common clinical signs of coarctation of the aorta (Fig.3-2) in older children?	83
20. How much does peak exercise affect cardiac output?	83
21. What cardiac lesions can lead to thrombosis and stroke?	83
22. What are two of the more common neuromuscular diseases in which a cardiac consultation is routinely recommended?	84
23. Why are chemotherapeutic agents that use arsenic of cardiac concern?	84
Congenital heart disease	84
24. What prenatal maternal factors may be associated with cardiac disease in the neonate?	84
25. In a cyanotic newborn, what test can help distinguish pulmonary disease from cyanotic congenital heart disease (CHD)?	84
26. Which congenital heart lesions commonly appear with cyanosis during the newborn period?	85
27. In the patient with suspected heart disease, what bony abnormalities seen on a chest radiograph increase the likelihoo ...	85
28. How do pulmonary vascular markings on a chest radiograph help in the differential diagnosis of a cyanotic newborn with ...	85
29. What ECG findings suggest specific congenital heart conditions?	85
30. What chest radiograph findings (Fig.3-3) are considered characteristic for various CHDs?	86
31. What are the common ductal-dependent cardiac lesions?	86
32. What types of CHDs are associated with the right aortic arch?	86
33. Name 5 different types of left ventricular outflow tract stenosis	86
34. Which genetic syndromes are most commonly associated with CHD?	86
35. Which infants with CHD should be evaluated for other anomalies?	87
36. Describe the clinical manifestations of a large patent ductus arteriosus (PDA)	87
37. How commonly do PDAs occur in premature infants?	87
38. Is a ``to-and-fro´´ murmur a good description for the heart murmur of a PDA?	87
39. How can you explain a PaO2 of more than 400mm Hg in a blood sample from an umbilical catheter in a newborn with transp ...	88
40. How do the presenting symptoms of ventricular septal defect (VSD) and atrial septal defect (ASD) differ?	88
41. What is the primary concern of the pediatric cardiologist if a child with a large VSD is lost to follow-up and comes ...	88
42. What are some of the common presenting symptoms in older children with primary pulmonary hypertension?	88
43. What examination features are suggestive of pulmonary hypertension?	88
44. What is the anomaly in Ebstein anomaly?	88
45. What are the four structural abnormalities of tetralogy of Fallot?	88
46. What occurs during a ``Tet spell´´?	88
47. Name two conditions in which the murmur has disappeared or diminished in intensity and yet the patient is actually worse	89
48. After what age does a presumed peripheral pulmonic branch stenosis murmur deserve more detailed study?	89
49. What is the role of pulse oximetry in screening for complex congenital heart disease (CCHD) in asymptomatic infants i...	89
50. What is the AAP screening protocol for CCHD using pulse oximetry?	89
51. Which ductal-dependent lesions are the AAPs primary targets for screening with the use of pulse oximetry?	90
52. What should parents be told about the risk for recurrence of common heart defects?	90
53. Can you think of a ``handy´´ way to remember the congenital cyanotic heart diseases?	90
Congestive heart failure	90
54. Identify the clinical signs and symptoms associated with CHF in children.	90
55. How is heart size assessed in older children?	90
56. In infancy, how does the likely cause of CHF vary by age?	90
57. What are the typical ages for the presentation of CHF with CHD?	92
58. If a patient develops CHF and cardiomegaly during the newborn period, but no heart murmur is heard, what is the diffe...	92
59. If a patient develops CHF and cardiomegaly after the newborn period, but no murmur is heard, what is the differential...	92
Electrocardiograms and arrhythmias	92
60. How does the ECG of a term infant differ from that of the older child?	92
61. What are the characteristic features of the ECG of a premature infant?	92
62. Describe the ECG abnormalities associated with potassium and calcium imbalances	92
63. What is the difference between a QT interval and a corrected QT interval (QTc)?	93
64. What causes a prolonged QT interval?	93
65. What ECG features are found in the long QT syndromes?	94
66. What characterizes torsades de pointes?	94
67. When should amiodarone not be used as the first-line therapy in patients with ventricular tachycardia?	94
68. What are the ECG findings in patients with complete heart block?	95
69. How abnormal are premature atrial contractions?	95
70. How does SVT in children differ from physiologic sinus tachycardia?	95
71. When are isolated premature ventricular contractions (PVCs) usually benign in the otherwise healthy school-aged child?	95
72. Name the two most common mechanisms of SVT	95
73. What are the clinical settings in which SVT may occur?	95
74. What are some of the causes of a wide QRS complex?	95
75. What vagal maneuvers are used to treat paroxysmal SVT in children?	96
76. In addition to vagal maneuvers, what treatments are used acutely for managing SVT?	96
77. Why should an electrographic tracing (preferably with multiple leads) be carried out while administering intravenous ...	96
78. In what settings should the dose of adenosine be modified for a suspected cardiac arrhythmia?	96
79. Which children are candidates for transcatheter ablation techniques for SVT?	96
80. What is the lethal arrhythmia of WPW syndrome?	97
81. How is WPW syndrome diagnosed on the baseline ECG?	97
Infectious and inflammatory disorders	97
82. How many blood cultures should be obtained in patients suspected of bacterial endocarditis?	97
83. Why might properly collected blood cultures be negative in the setting of clinically suspected bacterial endocarditis?	97
84. When is antibiotic prophylaxis for a dental procedure recommended?	98
85. How reliable is the echocardiogram for diagnosing bacterial endocarditis (BE)?	98
86. When should myocarditis be suspected?	98
87. What conditions are associated with the development of myocarditis?	98
88. A child visiting from South America presents with symptoms including unilateral eye swelling and new-onset acute CHF....	99
89. What are the common clinical signs and symptoms of pericarditis?	99
90. What is the position of comfort in the patient with pericarditis?	99
91. What is Kawasaki disease?	99
92. What are the principal diagnostic criteria for Kawasaki disease?	99
93. What makes incomplete (or atypical) Kawasaki disease incomplete (or atypical)?	99
94. Which diagnostic manifestation of Kawasaki disease is most commonly absent?	99
95. What laboratory tests are often abnormal in the first 7 to 10 days of the Kawasaki disease?	100
96. What is the typical age of children with Kawasaki disease?	100
97. Why should all children with Kawasaki disease receive intravenous immunoglobulin (IVIG) therapy?	100
98. Is aspirin therapy of benefit for children with Kawasaki disease?	100
99. What is the likelihood of a patient developing coronary artery pathology with and without treatment for Kawasaki disease?	101
Pharmacology	101
100. How long before oral digoxin begins to work?	101
101. A child with WPW syndrome is given digoxin to prevent SVT. Why is the pediatric cardiologist concerned?	101
102. When should indomethacin be administered to newborns with a PDA?	101
103. What are the side effects of indomethacin in the neonate?	101
104. What are the contraindications for indomethacin therapy?	102
105. What are the indications for prostaglandin E1 (PGE1) in the neonate?	102
106. What are the major side effects of PGE1?	102
107. How do α, β, and dopaminergic receptors differ?	102
108. How do relative receptor effects differ by drug type?	102
109. How are emergency infusions for cardiovascular support prepared?	102
Physical examination	103
110. What causes the first heart sound?	103
111. What causes the second heart sound?	103
112. In what settings can an abnormal second heart sound be auscultated?	103
113. What is the difference between pulsus alternans and pulsus paradoxus?	103
114. How is pulsus paradoxus measured?	103
115. What is the differential diagnosis for a systolic murmur in each auscultatory area?	103
116. What are the most common innocent murmurs?	103
117. What is the effect of sitting up on the typical innocent murmur?	103
118. What features are suggestive of a pathologic murmur?	105
119. If a murmur is detected, what other factors suggest that the murmur is pathologic?	105
Surgery	105
120. What are shunt operations?	105
121. Name the major shunt operations (Fig.3-13) for CHD.	105
122. What is the purpose of the Fontan procedure?	106
123. What are the most common rhythm disturbances after the Fontan procedure?	106
124. In what type of cardiac surgery is the complication of protein-losing enteropathy more common?	106
125. What are some of the reasons to surgically close a VSD?	106
126. What are the indications for closure of an atrial septal defect?	107
127. What is the typical timing for the three operations for children with HLHS?	107
128. What are long-term survival rates for children who undergo surgery for HLHS?	107
129. What is the long-term prognosis for heart transplantation during infancy and childhood?	107
130. A 5-year-old girl, 2 weeks after an uncomplicated repair of a secundum atrial septal defect, presents with fever, re...	107
131. What is the etiology of postoperative hypertension following repair of coarctation of the aorta?	107
132. A 5-year-old boy, 6 days after an uncomplicated surgical repair of a coarctation of the aorta, presents with respira...	107
133. Can a patient with heart disease simultaneously be polycythemic and iron deficient?	108.e1
134. What syndrome should come to mind for the patient with pulmonary stenosis, liver disease, and hypercholesterolemia?	108.e1
135. What is the difference between isotonic and isometric exercise?	108.e1
136. What are some of the genetic diseases associated with pulmonary valve stenosis and/or pulmonary artery branch stenosis?	108.e1
137. You are seeing a newborn with a heart murmur and the parents tell you that their obstetrician had obtained a fetal c...	108.e1
138. What is the typical ventricular heart rate in newborns with atrial flutter?	108.e1
139. What are the chances of an infant having complete heart block if the infants mother has systemic lupus erythematosus...	108.e1
140. What conditions should be considered in the differential diagnosis of Kawasaki disease?	108.e1
141. Is palpation for femoral pulses a reliable screening tool for coarctation of the aorta in infants and older children?	108.e2
142. Where is the best place to position your stethoscope to hear an aortic ejection click?	108.e2
143. What is postcoarctectomy syndrome?	108.e2
Chapter 4: Dermatology	109
Acne	109
1. When is acne most likely to develop?	109
2. When are pimples precocious?	109
3. Which skin structure is involved in acne pathogenesis?	109
4. What are the four key factors in acne pathogenesis?	109
5. Are blackheads caused by dirty skin?	109
6. What is the difference between neonatal acne and infantile acne?	109
7. Which disorders resemble neonatal and infantile acne?	110
8. Is an infant with acne more likely to be a teenager with acne?	110
9. Which factors exacerbate acne?	110
10. What are the most severe forms of acne?	110
11. What is the therapeutic approach to acne?	111
12. When is the use of oral isotretinoin indicated in teenagers with acne?	111
13. What serious side effects may be associated with systemic minocycline therapy for acne?	111
14. Which combination of acne products will cause a yellow-orange skin and hair discoloration?	111
15. What other topical acne products should not be used in combination?	112
16. What color will your red towel become after you wipe your BPO-covered face with it?	112
Clinical issues	112
17. What skin findings in the midline lumbosacral region are suggestive of occult spinal dysraphism?	112
18. What is the significance of accessory tragi?	113
19. What conditions cause ringlike rashes on the skin?	113
20. What is the appearance and natural history of molluscum contagiosum?	113
21. What is the best way to eradicate molluscum contagiosum?	113
22. What are the common causes of acute urticaria in children?	113
23. What is the characteristic clinical picture of erythema nodosum?	114
24. What, technically, are warts?	114
25. How are plantar warts distinguished clinically from calluses?	114
26. How can common warts be treated?	114
27. An 8-year-old has a hard, nontender, freely mobile nodule of the neck with a slightly bluish hue of the skin. What is...	115
28. What is the ``teeter-totter sign´´?	115
29. What are the most common causes of lumps and bumps in the skin of children?	115
30. Why is a pyogenic granuloma neither pyogenic nor a granuloma?	115
31. What condition is classically diagnosed by the Darier sign?	115
32. What disorder can present as ``freckles´´ associated with hives?	116
33. What is impetigo?	116
34. Is topical or systemic therapy better for impetigo?	116
35. What dermatologic sign starts from a scratch?	116
36. Does a geographic tongue occur as a result of global travel?	117
37. What diseases are associated with a strawberry tongue?	117
38. What should parents look for in a sunscreen label?	117
Eczematous disorders	117
39. What is the difference between eczema and atopic dermatitis?	117
40. What is the usual distribution of rash in atopic dermatitis?	118
41. Describe the five key battle plans to treat atopic dermatitis	118
42. Why is there a black box warning on topical calcineurin inhibitors?	118
43. Why shouldnt fluorinated (halogenated) and other potent topical steroids be used on the face?	119
44. Is there a genetic basis for atopic dermatitis?	119
45. Are there consistent immunologic alterations in children with atopic dermatitis?	119
46. What is the role of filaggrin mutations in the development of atopic dermatitis?	119
47. What other skin conditions mimic atopic dermatitis?	119
48. What is the ``atopic march´´?	120
49. What features help differentiate seborrheic from atopic dermatitis during infancy?	120
50. How should parents cope with cradle cap?	120
51. What condition causes bumps on the cheeks, upper arms, and thighs?	120
52. What are the causes of irritant contact diaper rash?	120
53. What features of diaper rash suggest more sinister diseases?	120
54. Are topical steroid/antifungal preparations useful for treating children with diaper dermatitis?	121
55. Which dietary deficiencies may be associated with an eczematous dermatitis?	121
56. What are the two main types of contact dermatitis?	121
57. What type of agents can cause allergic contact dermatitis in children?	121
58. When does the rash in poison ivy appear relative to exposure?	121
59. Are the vesicles in poison ivy contagious?	121
60. What is the ``id´´ reaction?	121
61. How does the vehicle used in a dermatologic preparation affect therapy?	122
Fungal infections	122
62. What are useful methods for diagnosing tinea infections?	122
63. How does one differentiate between irritant diaper dermatitis and candidal diaper dermatitis?	122
64. Does a scaly scalp and swollen glands qualify a patient to initiate griseofulvin?	123
65. Why is it necessary to culture for tinea capitis?	123
66. How can a culture be obtained if fungal culture medium is not available in the office?	123
67. What are the clinical presentations of tinea capitis?	123
68. How should children with tinea capitis be treated?	123
69. How should children who are receiving systemic medication for tinea capitis be monitored?	124
70. What is a kerion?	124
71. What puts the ``versicolor´´ in tinea versicolor?	124
72. How is tinea versicolor treated?	124
73. Which rashes resemble tinea pedis (athletes foot) in children?	125
Hair and nail abnormalities	125
74. How fast does hair grow?	125
75. On what parts of the skin is hair not normally found?	125
76. What causes sparse or absent hair in children?	125
77. Which kind of alopecia simply requires a change in hairstyle as the treatment?	125
78. How can alopecia areata be differentiated clinically from tinea capitis?	125
79. What are reported as poor prognostic indicators for recovery of hair in patients with alopecia areata?	126
80. What are treatments for alopecia areata?	126
81. Are most hairs growing or resting?	126
82. You are evaluating a healthy 4-year-old with fine, sparse hair who has never had a haircut. What condition do you sus...	126
83. What is the likely diagnosis in a child who develops diffuse hair loss 3 months after major surgery?	126
84. What puzzling cause of asymmetric hair loss in a child will sometimes cause an intern to pull his or her hair out?	127
85. What causes green hair?	127
86. How should ingrown toenails be managed?	127
87. Which pathogens are responsible for paronychia?	127
88. A healthy 7-year-old child who develops progressive yellowing and increasing friability of all nails over a period of...	127
89. What nail change may follow hand, foot, and mouth disease (HFMD) several weeks after the other hand and foot changes ...	127
Infestations	127
90. How do lice differ?	127
91. What are the clinical findings of head lice infestation?	128
92. How is the diagnosis of head lice made?	128
93. What types of treatment are available for head lice?	128
94. What precautions should be taken before prescribing malathion 0.5% lotion (Ovide) for head lice?	129
95. Should parents nit pick?	129
96. How is a skin scraping for scabies or ``scabies prep´´ done?	129
97. What treatment eliminates the scabies babies?	129
98. Which areas of the body are more commonly involved in scabies in younger children compared with adults?	130
99. In what conditions is the ``breakfast, lunch, and dinner´´ sign noted?	130
100. Will getting a new mattress ensure that the bed bugs wont bite?	130
Neonatal conditions	130
101. What are the most common birthmarks?	130
102. How should pustular lesions be evaluated in the newborn period?	131
103. What is the differential diagnosis of vesicles or pustules in the newborn?	131
104. What is the medical significance of cutis marmorata?	131
105. A healthy infant with scattered reddish nodules on the back skin most likely has what condition?	132
106. What should the family of a newborn with a yellow, hairless patch with a cobblestone texture be advised to do?	132
107. What syndromes are associated with aplasia cutis congenita?	132
108. Describe the appearance and distribution of transient neonatal pustular melanosis.	133
109. Is erythema toxicum neonatorum really toxic?	134
110. For academic (and billing) purposes, is it possible to be more scientific about the diagnosis of ``prickly heat´´?	134
Papulosquamous disorders	134
111. What diseases are associated with the Koebner reaction?	134
112. What is the typical pattern of lesions in childhood psoriasis?	135
113. What percentage of children with psoriasis have nail involvement?	135
114. A skin scale that easily bleeds on removal is characteristic of what condition?	136
115. How is the increased prevalence of childhood obesity associated with psoriasis?	136
116. What are treatment modalities for psoriasis?	136
117. What are the eight Ps of lichen planus?	136
118. How is pityriasis rosea distinguished from secondary syphilis?	136
119. What is the treatment for pityriasis rosea?	136
120. What is the likely diagnosis for a 5-year-old who presents with a linear array of recently acquired pink to hypopigm...	137
Photodermatology	137
121. Why is limiting excessive sun exposure in children important?	137
122. Why should indoor tanning be addressed and discouraged in children?	137
123. What are good strategies for protection against sun exposure?	137
124. How is the SPF of a sunscreen determined?	137
125. Should sunscreens be avoided in infants?	137
126. Do we risk developing vitamin D deficiency by using sun protection?	138
127. Which ``lime´´ disease is not transmitted by ticks?	138
128. Which conditions are associated with marked sun sensitivity?	138
129. What is the appearance of polymorphous light eruption?	138
130. Is a child with sun sensitivity protected by sitting behind a window?	138
Pigmentation disorders	139
131. What disorders of childhood are associated with areas of hypopigmentation?	139
132. Is treatment helpful for children with postinflammatory hypopigmentation?	139
133. What treatments are available for vitiligo?	139
134. What conditions are associated with congenital depigmentation of the skin?	139
135. What is the likely diagnosis if a patient taking trimethoprim-sulfamethoxazole develops a single erythematous, sharp...	140
136. Why are Spitz nevi and malignant melanoma often confused?	140
137. In children with pigmented nevi, what factors increase the risk of melanoma?	140
138. How do the ABCs of pediatric melanoma differ from adult melanoma?	140
Vascular birthmarks	140
139. How are vascular birthmarks classified?	140
140. What is the natural history of untreated infantile hemangiomas?	141
141. What are the major goals of the management of infantile hemangiomas?	141
142. What can patterns of hemangiomas on the skin surface tell us?	141
143. Which hemangiomas are especially worrisome?	142
144. When is treatment indicated for infantile hemangiomas?	142
145. What are the most commonly used treatments for problematic infantile hemangiomas?	142
146. Why is an infant with a vascular tumor and new-onset thrombocytopenia so worrisome?	143
147. How do superficial hemangiomas differ from port wine stains?	143
148. What is ``simple´´ about a nevus simplex?	144
149. When are port wine stains associated with other anomalies?	144
150. How are port wine stain-type capillary malformations treated?	144
Vesicobullous disorders	144
151. What is the Nikolsky sign?	144
152. What are causes of skin blistering in childhood?	145
153. How is SSSS differentiated from toxic epidermal necrolysis (TEN)?	145
154. Why are neonates susceptible to SSSS?	145
155. Where can the S. aureus be found in patients with SSSS?	145
156. What is the likely diagnosis for a 4-year-old child who develops a 1-week history of widespread painful and pruritic...	145
157. What are the subtypes of epidermolysis bullosa (EB)?	146
158. Should epidermolysis bullosa blisters be ``popped´´?	146
159. Which disorder is associated with ``target lesions´´?	146
160. Is EM part of a continuum with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)?	146
161. What distinguishes SJS from TEN?	147
162. What medications are most commonly associated with SJS and TEN in children?	147
163. Is steroid therapya beneficial for the treatment of SJS or TEN?	147
164. What therapy should be considered for patients with rapidly progressive SJS or TEN?	147
165. What infection should be considered in a child with SJS and a cough?	147
166. In addition to hand-foot-mouth, which other body site is often affected in children with coxsackie virus infections?	147
167. Can acne be scrubbed away?	147.e1
168. Can lasers be used to treat acne?	147.e1
169. Are there any ``quick fixes´´ for acne?	147.e1
170. Can dilute bleach be used for purposes other than cleaning your clothes?	147.e1
171. Do soaps or clothes make any difference in atopic dermatitis?	147.e1
172. Frequency of bathing for children with atopic dermatitis: more or less?	147.e1
173. What is the most common side effect of topical pimecrolimus and tacrolimus?	147.e1
174. What is a cause of a localized triangular area of alopecia on the scalp of children?	147.e1
175. What is the ``flag sign´´?	147.e2
176. Is there an oral treatment available to treat scabies?	147.e2
177. What types of sunscreens are available?	147.e2
178. Describe causes of pseudoporphyria in children	147.e2
179. What are the clinical features of familial dysplastic nevus syndrome?	147.e2
180. Which hemangioma is an exception to the rule of gradual resolution?	147.e2
181. What is ``eczema coxsackium´´?	147.e2
Chapter 5: Emergency Medicine	148
Bioterrorism	148
1. Why are children more vulnerable to biologic agents than adults?	148
2. What are the three routes of transmission of anthrax?	148
3. How are the lesions of smallpox distinguished from varicella (chickenpox)?	148
4. How can the presenting symptoms of bubonic plague be differentiated from those of plague resulting from bioterrorism?	148
5. Why should families living near nuclear power plants keep potassium iodide (KI) in their medicine cabinets?	149
6. Why are children particularly vulnerable to terrorism in the form of explosive and blast attacks?	149
7. What categories of agents should be considered in the event of a chemical weapons attack?	149
8. What should be the practitioner's initial management when a chemical weapons event occurs?	149
Child abuse: physical and sexual	149
9. What are important historical indicators of possible child abuse?	149
10. What is the most common cause of severe closed head trauma in infants younger than 1 year?	150
11. Why is the diagnosis of abusive head trauma often overlooked?	150
12. What diagnostic tests may be contributory if abusive head trauma is suspected?	151
13. What physical examination findings are indicators of possible child abuse?	151
14. If retinal hemorrhages are noted in a child with seizures, how likely are the seizures to have caused the hemorrhages?	151
15. In a suspected victim of child abuse, is an ophthalmology exam looking for retinal hemorrhages routinely indicated as...	151
16. When should child abuse be considered in the event of an unexplained death of a child?	152
17. Which conditions with ecchymoses (bruising) can be mistaken for child abuse?	152
18. How are fractures dated radiographically in children?	153
19. What fractures are suggestive of child abuse?	153
20. How certain can a clinician be in attributing a femur fracture in a nonambulatory child to nonaccidental trauma?	154
21. What is the purpose of the skeletal survey?	154
22. What constitutes the skeletal survey?	154
23. Up to what age should a skeletal survey be ordered?	154
24. What is the value of a follow-up skeletal survey?	154
25. In addition to child abuse, what conditions must you consider as a cause of multiple unexplained long bone fractures ...	155
26. When are burn injuries suspicious for child abuse?	155
27. How do you recognize child abuse in a medical setting?	155
28. How often is sexual abuse committed by an individual known previously by the child or adolescent?	155
29. In the case of suspected prepubertal sexual abuse, how critical is it to perform the physical exam immediately on pre...	155
30. After the documentation of history and a careful physical examination, what evidence should be collected in cases of ...	156
31. After the documentation of history and a careful physical examination, what evidence should be collected in cases of ...	156
32. After the initial ED evaluation for sexual assault, what kind of follow-up care should the ED physician offer?	156
33. If a child who is not sexually active is diagnosed with an infection caused by an STI-associated organism, how likely...	156
34. Is the size of the hymenal opening an important finding in the diagnosis of sexual abuse?	157
35. What is the most common finding of the physical examination of a child who has been sexually abused?	157
36. What are the date-rape drugs?	157
37. How can you tell whether a patient has been given a date-rape drug?	157
Environmental injury	158
38. How do fresh- and salt-water drownings differ?	158
39. How is the duration of submersion predictive of outcomes in drownings?	158
40. What cardiovascular changes occur as body temperature falls?	159
41. What are the physiologic consequences of externally warming a severely hypothermic patient too rapidly?	159
42. What are acceptable rewarming methods for the hypothermic child?	159
43. What organ systems are affected in patients suffering from heat stroke?	159
44. How quickly can temperature rise inside an enclosed automobile?	159
45. What are characteristics of heat stroke?	160
46. Why are children more vulnerable to effects of external temperature changes?	160
47. What are the signs and symptoms of significant upper airway heat exposure in a patient who has been in a house fire?	160
48. What are the signs and symptoms of impending respiratory failure as a result of mucosal injury and edema from heat ex...	160
49. Which laboratory studies are needed for patients with suspected carbon monoxide (CO) poisoning?	160
50. What are the key aspects of treatment for carbon monoxide poisoning in children?	160
51. Why is carbon monoxide such a deadly toxin?	161
52. What is the pathophysiology of carbon monoxide poisoning?	161
53. What other serious exposure risk should one consider when managing a patient suffering from carbon monoxide poisoning?	161
54. What are the different degrees of burn injuries?	161
55. How does the ``rule of nines´´ apply in children?	161
56. Which burn injuries are indications for hospitalization?	162
57. Why are alkali burns worse than acid burns in the eye?	162
58. How do the injuries produced by lightning and high-voltage wires differ?	162
59. In electrical injury, is alternating or direct current more hazardous?	162
60. What agents are the most common causes of anaphylaxis seen in U.S. emergency rooms?	163
61. What are important considerations when treating frostbite in children?	163
Resuscitation	163
62. What are common problems identified in cardiopulmonary resuscitation (CPR) done by professionals?	163
63. What is the role for capnography during resuscitation?	163
64. Why is the airway of an infant or child more prone to obstruction than that of an adult?	164
65. How can the correct size of endotracheal tubes (ETTs) be estimated for a given patient?	164
66. When should cuffed versus uncuffed ETTs be used?	164
67. How should the appropriate depth of an ETT be calculated?	164
68. How should correct placement of an ETT be confirmed?	164
69. What emergency drugs can be given through an ETT?	164
70. What are the potential reasons for acute deterioration in an intubated patient?	165
71. When is atropine indicated during cardiopulmonary resuscitation?	165
72. When is the use of calcium indicated during cardiopulmonary resuscitation?	165
73. What are contraindications to the use of an intraosseous line?	165
74. Can laboratory tests be obtained from intraosseous lines?	165
75. What are the complications of intraosseous lines?	166
76. What features indicate that an intraosseous needle has been correctly placed?	166
77. How can a child's weight be estimated?	166
78. Name the potentially reversible causes of cardiac arrest.	166
79. What are the typical clinical findings associated with supraventricular tachycardia (SVT)?	166
80. If an infant develops SVT, how long before congestive heart failure (CHF) develops?	167
81. What factors may be predictive of outcomes after pediatric cardiac arrest?	167
82. Are fixed and dilated pupils a contraindication to resuscitation for a pediatric patient in cardiac arrest?	167
83. When should a failing resuscitation be stopped?	167
84. Why is resuscitation less successful in children than in adults?	167
85. Should family members be allowed to observe a resuscitation?	168
Shock	168
86. Are all children in shock hypotensive?	168
87. What are the signs and symptoms of early or compensated shock?	168
88. What are the signs and symptoms of late or uncompensated shock?	168
89. What external factors can affect the accuracy of measurement of capillary refill time in children and neonates?	168
90. How much blood volume can be lost before hypotension may be seen in children?	168
91. What defines hypotension in children (i.e., systolic blood pressure <5th percentile for age)?	169
92. What types of shock can occur in children?	169
93. What are the hallmarks of septic shock?	169
94. What are the key initial management items for septic shock?	169
95. Are corticosteroids recommended for the treatment of septic shock?	169
96. What is the most important pharmacologic therapy for anaphylactic shock?	170
97. What are the possible causes of shock in the newborn period?	170
98. A 4-day-old infant presents to the ED in shock with evidence of CHF and cyanosis. In addition to managing the airway ...	170
99. What are the four classes of medications that can be used to support cardiac output?	170
100. An 8-year-old presents to the ED after falling headfirst into an empty swimming pool. His heart rate is normal, yet ...	170
Toxicology	171
101. What are the most common poisonings in children younger than 6 years?	171
102. Which medications can kill a 10-kg toddler with 1 or 2 tablets, capsules, or teaspoonfuls?	171
103. What medication causes the most overdose deaths in children each year in the United States?	171
104. Name the toxicology ``time bombs.´´	172
105. What empirical drug therapies are indicated for the poisoned child who presents with altered mental status?	172
106. What is gastrointestinal decontamination?	172
107. How does single-dose activated charcoal work and when should it be considered?	172
108. For what substances is charcoal not recommended?	172
109. When is gastric lavage indicated?	172
110. What are the indications for whole bowel irrigation (WBI) in acute ingestions?	173
111. How is the manipulation of urinary pH used in treating poisonings?	173
112. What ingestions and exposures have available antidotes?	173
113. For which kinds of ingestions is naloxone considered an antidote?	174
114. Which ingestions are radiopaque on abdominal radiograph?	174
115. What causes the radiographic ``lead lines´´ of chronic lead poisoning?	174
116. What is a toxidrome?	174
117. What is the toxidrome for anticholinergics?	175
118. What breath odors may be associated with specific ingestions?	175
119. What are the limitations of the routine toxicology screen?	175
120. After use of marijuana, how long does a urine screen remain positive?	176
121. How do the types of alcohol ingestions vary?	176
122. Which alcohol is considered the most lethal?	176
123. What is the treatment for methanol and ethylene glycol ingestions?	176
124. What is ``MUDPILES´´?	176
125. How can pupillary findings assist in the diagnosis of toxic ingestions?	177
126. If a child has ingested an acetaminophen-containing product, when should the first acetaminophen level be obtained?	177
127. When should a ``NAC attack´´ begin?	177
128. How does NAC prevent hepatotoxicity in acetaminophen overdose?	177
129. What arterial blood gas pattern is classic for salicylate poisoning?	177
130. What are hidden salicylates?	177
131. What are the classic ECG findings associated with tricyclic antidepressants?	177
132. Which clinical and laboratory features correlate with an acutely elevated serum iron?	178
133. What are the four clinical stages of iron toxicity and the correlating pathophysiology?	178
134. Which is more toxic, drinking dishwashing detergent or toilet bowl cleaner?	178
135. Which hydrocarbons pose the greatest risk for chemical pneumonitis?	178
136. What is the differential diagnosis in a child who presents with confusion and lethargy?	178
137. A patient receiving an antiemetic drug (e.g., promethazine) who develops involuntary, prolonged, twisting, writhing ...	178
138. What do ``SLUDGE´´ and ``DUMBELS´´ have in common?	179
139. What metal intoxication can mimic Kawasaki disease?	179
140. Why is cyanide so toxic?	179
141. In what settings should cyanide poisoning be suspected?	179
142. What kinds of plants account for the greatest percentage of deaths due to plant poisonings?	179
143. Is mistletoe toxic?	179
144. Should swallowed disc batteries be removed?	180
145. What are the available methods used to remove a foreign body from the esophagus?	180
146. What recreational drug is most frequently associated with rave parties?	180
147. Why is ecstasy considered so dangerous?	180
Trauma	180
148. What are the major signs of a blowout fracture?	180
149. When evaluating a patient with an eye injury, when should you suspect a ruptured globe and how should you handle it?	181
150. When should an avulsed tooth be reimplanted?	181
151. What are the three most important considerations when evaluating nasal trauma?	181
152. How long can you wait before a broken nose in a child must be reduced?	182
153. After a motor vehicle collision, an 8-year-old presents with right-sided pain, a heart rate of 150 beats per minute,...	182
154. What are the signs and symptoms of a tension pneumothorax?	182
155. Which children with acute minor blunt head trauma require emergency CT scans?	182
156. What is the risk associated with CT scans in children?	182
157. When intracranial pressure is acutely elevated, how long is it before papilledema develops?	182
158. What are the components of the Glasgow Coma Scale (GCS)?	182
159. How does the location of cervical spine fractures vary between younger children and older children and adults?	183
160. What is SCIWORA?	183
161. Are single lateral cervical spine radiographs sufficient to ``clear´´ a patient after neck injury?	184
162. Why is left shoulder pain after abdominal trauma a worrisome sign?	184
163. A 5-year-old child has ecchymosis of the lower abdomen after a motor vehicle collision. What should you immediately ...	184
164. In a 7-year-old boy with a radiographically proven pelvic fracture, what diagnostic procedure should be done?	184
165. In this same patient as in question 164, blood at the tip of the penis is noted. Why is catheterization contraindicated?	184
166. What is the focus of the FAST examination?	184
167. In children with blunt abdominal trauma, are there clinical findings that predict low risk of clinically important i...	185
Wound repair	185
168. What advice should be given over the telephone regarding the transportation of an avulsed digit?	185
169. Which lacerations should be referred to a surgeon or an ED physician who is familiar with wound repair?	185
170. How many days should sutures remain in place?	185
171. When should a nerve injury be suspected in a finger laceration?	185
172. What should be done if nerve damage is suspected?	185
173. Which lacerations should not be sutured?	186
174. Which are at greater risk for infection, dog bites or cat bites?	186
175. Should antibiotic prophylaxis be given for dog, cat, and human bites?	186
176. Which animals most often carry the rabies virus?	186
177. Which are more likely to be rabid: cats or dogs?	187
178. If at a local petting zoo a playful 20-month-old child is bitten by a duck, scratched by a rabbit (breaking skin), s...	187
179. When is the use of lidocaine with epinephrine contraindicated as a local anesthetic?	187
180. What are methods for decreasing the pain of local lidocaine infiltration?	187
181. What are some of the ingredients in the alphabet soup of topical anesthetics?	187
182. When should tissue adhesives be considered or avoided?	187
183. In which situations might you choose absorbable over nonabsorbable sutures when repairing a pediatric laceration?	188
184. What is the proper fluid to use for wound irrigation?	188
185. How is conscious sedation best managed in children?	188
186. To reduce the exposure to radiation from a complete skeletal survey, can the survey be modified to include fewer x-r...	188.e1
187. If physical abuse is suspected, are physicians mandated to photograph physical findings?	188.e1
188. If a toddler suffers a full-thickness burn to the corner of the mouth after biting an electrical cord, what complica...	188.e1
189. What is a laryngeal mask airway (LMA)?	188.e1
190. What is the Sellick maneuver?	188.e1
191. Should activated charcoal be given to a sleepy 2-year-old girl who consumed half a bottle of a liquid antihistamine ...	188.e2
192. When can a toddler who may have swallowed some multivitamins be discharged home?	188.e2
193. If the abdominal CT scan is negative in a patient with blunt abdominal trauma, can you be certain that there is no i...	188.e2
194. In children with blunt thoracic trauma, are there clinical exam findings that predict low risk of significant injury?	188.e2
Chapter 6: Endocrinology	189
Adrenal disorders	189
1. What are the symptoms of adrenal insufficiency?	189
2. What distinguishes primary and secondary adrenal insufficiency?	189
3. What is the differential diagnosis of primary adrenal insufficiency?	189
4. What are the most common causes of secondary adrenal insufficiency?	189
5. What clinical clues suggest that adrenal insufficiency is a primary rather than a secondary problem?	189
6. What is the most common form of congenital adrenal hyperplasia (CAH)?	189
7. In newborns with CAH, why are girls likely to be diagnosed earlier than boys?	190
8. How do the major steroid preparations vary in potency?	190
9. How do physiologic, stress, and pharmacologic doses of hydrocortisone differ?	190
10. When does adrenal-pituitary axis suppression occur in prolonged glucocorticoid treatment?	190
Calcium metabolism and disorders	191
11. What are the causes of hypercalcemia?	191
12. An 8-year-old in a spica cast after hip surgery develops vomiting and a serum calcium concentration of 15.3 mg/dL. H ...	191
13. Is it the Chvostek or Trousseau sign that gets the tap?	192
14. What is hypoparathyroidism?	192
15. In what clinical circumstances should hypoparathyroidism be suspected?	192
16. What are the main causes of hypocalcemia in children?	192
17. What is the most likely diagnosis in a child with hypocalcemia who has abnormally shaped fingers?	193
Clinical syndromes	194
18. How does the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) develop?	194
19. What is cerebral salt wasting and how is it separated from SIADH?	194
20. What are the five criteria for the diagnosis of SIADH?	194
21. What clinical features suggest diabetes insipidus (DI)?	194
22. How is the diagnosis of DI made?	194
Diabetic ketoacidosis	195
23. What is diabetic ketoacidosis (DKA)?	195
24. What percentage of newly diagnosed diabetic patients present with symptoms of DKA?	195
25. What are the mainstays of therapy for DKA?	195
26. What should be the initial fluid management in DKA?	195
27. Why is a falling serum sodium concentration during the treatment of DKA of concern?	196
28. What is the typical potassium status in children with DKA?	196
29. Why do potassium levels fall during the management of DKA?	196
30. Should bicarbonate be used for the treatment of children with DKA?	196
31. Are there any indications for the use of bicarbonate?	197
32. When should glucose be added to the intravenous fluids in patients with DKA?	197
33. In the past, a bolus of insulin was given at the start of therapy for DKA. Is that still recommended?	198
34. Is continuous or bolus insulin better for the initial treatment of DKA?	198
35. Which corrects sooner during continuous insulin administration: hyperglycemia or acidosis?	198
36. What is the main cause of mortality in DKA?	198
37. What risk factors are associated with the development of cerebral edema?	198
38. What signs and symptoms suggest worsening cerebral edema during the treatment of DKA?	199
Diabetes mellitus	199
39. What are the risks of a child developing type 1 diabetes (T1D) if one sibling or parent is affected?	199
40. How long does the ``honeymoon´´ period last in patients with newly diagnosed T1D?	199
41. How do the types of insulin vary in their onset and duration of action?	199
42. When should the Somogyi phenomenon be suspected?	200
43. What causes the ``dawn phenomenon´´?	200
44. How rapidly can renal disease develop after the onset of diabetes mellitus?	200
45. How is hemoglobin A1C (HbA1C) helpful for monitoring glycemic control?	201
46. What are the goals for hemoglobin A1C?	201
47. What pathophysiologic process characterizes type 2 diabetes (T2D)?	201
48. Is the prevalence of T2D in children increasing?	201
49. What historical and clinical features suggest type 2 rather than type 1 diabetes?	201
50. What laboratory features are helpful to distinguish T1D from T2D?	202
51. What is acanthosis nigricans?	202
52. How is T2D diagnosed?	202
53. Which pediatric patients should be screened for T2D?	203
54. What hemoglobin A1C level is sufficient to diagnose diabetes?	203
55. When should oral hypoglycemic agents be considered as part of therapy?	203
Growth disturbances	203
56. How do the growth rates of boys and girls differ?	203
57. What is the best predictor of a child´s eventual adult height?	203
58. When have most children achieved the height percentile that is consistent with parental height?	204
59. When is a detailed evaluation for short stature warranted?	204
60. Name the major categories of causes of short stature	204
61. In a child with short stature, what rate of growth makes an endocrine abnormality unlikely?	204
62. When evaluating a short child, why should you ask when the parents reached puberty?	204
63. When does the pubertal growth spurt occur?	204
64. Are upper to lower body ratios helpful for the diagnosis of growth problems?	205
65. What laboratory studies should be obtained when evaluating short stature?	205
66. In a very obese child, how does height measurement help determine whether an endocrinopathy might be the cause?	205
67. How does a growth chart help determine the diagnosis of failure to thrive?	205
68. How can one track growth in children who have spinal cord abnormalities or severe scoliosis?	207
69. What is bone age?	207
70. Why is a bone age determination helpful for evaluating short stature?	207
71. What features suggest constitutional delay as a cause of short stature?	207
72. How is constitutional delay managed?	207
73. Should growth hormone therapy be given to the normal short child?	207
74. What are the clinical manifestations of growth hormone excess?	208
Hypoglycemia	208
75. How is hypoglycemia defined?	208
76. What are the clinical findings associated with hypoglycemia?	208
77. What are the causes of childhood hypoglycemia?	208
78. An unconscious 3-year-old girl is brought to the emergency department with a serum glucose concentration of 26mg/dL. ...	209
79. In patients with acute hypoglycemia, what are the treatment options?	209
Hypothalamic and pituitary disorders	209
80. What clinical signs or symptoms suggest hypothalamic dysfunction?	209
81. List the intracranial processes that can interfere with hypothalamic-pituitary function	210
82. Why is it bad to have a ``Turkish saddle´´ that is too large?	210
83. Which tests are useful for studying suspected hypothalamic and pituitary malfunction?	210
Sexual differentiation and development	210
84. An infant is born with ``ambiguous genitalia.´´ What features of the history and physical examination are key in the ...	210
85. What are the causes of a DSD?	211
86. Which studies are essential for the evaluation of a DSD?	211
87. What major criteria are used to define a micropenis?	212
88. What are the main concerns to be addressed during the initial evaluation of a 1-month-old infant with micropenis?	212
89. Discuss the terms that denote aspects of precocious sexual development.	212
90. Boys or girls: Who is more likely to have an identifiable cause for precocious puberty?	212
91. A 7 -year-old girl develops breast buds and pubic hair. Is this normal or precocious?	213
92. Breast buds are noted on a 2-year-old girl. Is this worrisome?	213
93. Which aspects of the physical examination are particularly important when evaluating a patient with precocious puberty?	213
94. Which radiologic and laboratory tests are indicated for the evaluation of precocious puberty?	213
95. When does the male voice begin to crack?	214
Thyroid disorders	214
96. Which thyroid function tests are ``standard´´?	214
97. What signs and symptoms in an infant suggest congenital hypothyroidism?	214
98. What causes congenital hypothyroidism?	214
99. How effective are screening programs for congenital hypothyroidism?	215
100. Discuss the risks of delaying treatment for congenital hypothyroidism.	215
101. A suspected goiter (diffuse enlargement of the thyroid gland) is noted during a routine examination of an asymptomat...	215
102. What is the most common cause of acquired hypothyroidism in childhood?	216
103. What is the most common clinical presentation of Hashimoto thyroiditis?	216
104. What should a parent be told about the prognosis of a child who has euthyroid goiter caused by chronic lymphocytic t...	216
105. What other autoimmune diseases are associated with chronic lymphocytic thyroiditis?	216
106. What does a normal T4 and an elevated TSH suggest?	216
107. What is the most common cause of hyperthyroidism in children?	216
108. In addition to Graves disease, what conditions may cause hyperthyroidism?	217
109. Describe the typical features of hyperthyroidism that occur as a result of Graves disease.	217
110. What causes Graves disease?	217
111. Why does exophthalmos occur in Graves disease?	217
112. What treatment options are available for children with Graves disease?	217
113. Describe the principal modes of actions and the side effects of medications used to treat Graves disease.	218
114. Has radioactive iodide fallen into disfavor as a treatment option for Graves disease?	218
115. During a routine physical examination, a solitary thyroid nodule is palpated on an asymptomatic 10-year-old child. C...	219
116. How should this solitary thyroid nodule be investigated?	219
117. How is the euthyroid sick syndrome diagnosed?	219
118. How do antiepileptic medications affect thyroid function tests?	219.e1
119. Of what value is the T3 resin uptake (T3RU) test?	219.e1
120. How common is goiter (thyroid enlargement) in newborns with congenital hypothyroidism?	219.e1
Chapter 7: Gastroenterology	220
Clinical issues	220
1. What is the definition of failure to thrive?	220
2. What is the differential diagnosis of FTT?	220
3. How is FTT evaluated?	220
4. What features of history or on physical examination suggest a medical condition leading to FTT?	220
5. How is the diagnosis of ``pinworms´´ made?	220
6. What characterizes functional abdominal pain in children?	221
7. In children with abdominal pain, what historical features suggest a possible organic or serious cause?	221
8. What treatments are used for functional abdominal pain in children?	221
9. What is intractable singultus?	222
10. What is the most commonly ingested foreign body?	222
11. Which is potentially more dangerous after ingestion: a penny made in 1977 or one made in 1987?	222
12. What is the difference radiographically between a coin in the esophagus and a coin in the trachea?	222
13. Why is ingestion of a button battery more dangerous than ingestion of a coin?	222
14. Which is more dangerous, 2 magnets that are swallowed together or 2 magnets that are swallowed separately?	223
15. What are the indications for emergent foreign body removal?	223
16. What is the grim news about Rapunzel syndrome?	223
17. What is the most common clinical presentation of juvenile polyps in children?	223
18. What are the types of colonic polyps?	223
19. Why is it important to confirm a diagnosis of juvenile polyposis?	223
20. How is ascites diagnosed by physical examination?	224
Constipation	224
21. What constitutes constipation in childhood?	224
22. What features suggest an organic etiology for constipation?	224
23. What is the most important component of the physical examination when evaluating constipation?	225
24. What are some common triggers of constipation in healthy infants and children?	225
25. Which clinical features differentiate chronic retentive constipation from Hirschsprung disease?	225
26. How is Hirschsprung disease diagnosed?	225
27. What is the most common cause of encopresis?	226
28. How should children with chronic constipation and encopresis be managed?	226
Diarrhea and malabsorption	227
29. What time frame distinguishes acute and chronic diarrhea?	227
30. What is the most common cause worldwide of epidemic diarrhea?	227
31. What are other common causes of acute diarrhea?	227
32. Which historical questions are key when seeking the cause of diarrhea?	227
33. In what settings can diarrhea be a severe, life-threatening illness?	228
34. Why is true diarrhea during the first few days of life especially concerning?	228
35. What are the most useful stool tests for diagnosing fat malabsorption?	228
36. What stool test is most useful for helping diagnose GI protein loss?	228
37. How do patterns of secretory or enterotoxigenic and inflammatory diarrhea vary?	228
38. What is the primary pathophysiologic difference between secretory and osmotic diarrhea?	228
39. How can osmotic diarrhea be distinguished from secretory diarrhea?	228
40. How should children with secretory diarrhea be managed?	229
41. What rare tumors can cause true secretory diarrhea?	229
42. What features characterize ``toddler diarrhea´´?	229
43. What is primary lactose intolerance?	229
44. How does late-onset lactase deficiency vary by ethnicity?	229
45. What conditions produce secondary lactose intolerance?	230
46. How is lactose intolerance diagnosed?	230
47. What is the role of stool elastase measurement?	230
48. What three individual clinical features are the most accurate for predicting 5% dehydration?	230
49. How accurate are urine specific gravity and blood urea nitrogen (BUN) measurements as means of assessing dehydration ...	231
50. How do various oral rehydration solutions differ in composition from other liquids that are commonly used for rehydra...	231
51. How can the World Health Organization (WHO) oral electrolyte (rehydration) solution be duplicated?	231
52. What traditional approaches to feeding during diarrhea are no longer recommended and should be avoided?	231
53. What is the role of antiemetic agents in children with gastroenteritis?	231
54. What are non-antimicrobial drug therapies for diarrhea?	232
55. What is the role of probiotic organisms in the treatment of antibiotic-associated diarrhea?	232
56. Why is Salmonella enteritis so concerning in a child who is younger than 12 months?	232
57. What are the clinical manifestations of typhoid fever?	232
58. Who was Typhoid Mary?	233
59. What is the most common cause of travelers´ diarrhea?	233
60. How can travelers´ diarrhea be prevented?	233
61. Which bacterial gastroenteritides may benefit from antimicrobial therapy?	233
62. What strains of E. coli are associated with diarrhea?	234
63. What clinical entity has been attributed to EHEC, specifically strain O157:H7?	234
64. What is the most common cause of antibiotic-associated colitis?	234
65. How is the diagnosis of C. difficile made?	234
66. How common is asymptomatic C. difficile carriage?	234
67. Why are alcohol-based sanitizers insufficient when examining patients with C. difficile?	235
68. What are the three most common presenting symptoms of giardiasis?	235
69. How reliable are the various diagnostic methods for detecting Giardia?	235
70. What are the potential complications of amebiasis?	235
71. What is gluten?	236
72. What classic clinical features suggest celiac disease?	236
73. What are possible nongastrointestinal manifestations of celiac disease?	236
74. What is the appropriate screening test for celiac disease?	236
75. What is the definitive way to diagnose celiac disease?	237
76. What is the mainstay of treatment for celiac disease?	237
Esophageal disorders	237
77. What is the likely diagnosis for an infant with excessive secretions and choking episodes in whom a nasogastric tube ...	237
78. What underlying diagnoses should be considered in a patient who presents with a meat impaction in the esophagus?	237
79. What is the most common condition that might present as a food impaction in an adolescent?	237
80. How is EoE diagnosed?	237
81. What are common endoscopic findings in EoE?	238
82. What causes esophageal eosinophilia in EoE?	239
83. What are the symptoms of EoE?	239
84. What are the therapies for EoE?	239
Food allergies	240
85. What are the most common food allergies in children?	240
86. How are adverse food reactions characterized?	240
87. What can be the acute manifestations of milk protein allergy in childhood?	240
88. What is the most common chronic manifestation of milk protein allergy?	240
89. What likely condition does a birch-allergic child have who develops tongue swelling when eating an apple?	240
90. Can dietary manipulation in the first few months of life reduce the risk for atopic dermatitis and food allergies?	240
91. What are the symptoms of allergic proctocolitis or milk protein intolerance in an infant?	241
92. Does the diagnosis of allergic proctocolitis in infants usually require endoscopy?	241
Gastrointestinal bleeding	241
93. What features on physical examination can help identify an unknown cause of GI bleeding?	241
94. In patients with acute GI bleeding, how may vital signs indicate the extent of volume depletion?	242
95. What is the simplest way of differentiating upper GI from lower GI bleeding?	242
96. How does the type of bloody stool help pinpoint the location of a GI bleed?	242
97. What can cause false-negative and false-positive results when stool testing for blood?	243
98. How do the causes of lower GI bleeding vary by age group?	243
99. A previously asymptomatic 18-month-old child has large amounts of painless rectal bleeding (red but mixed with darker...	243
100. Worldwide, what is the most common cause of GI blood loss in children?	243
101. How do the causes of upper GI bleeding vary by age group?	243
102. What is the most likely cause of hematemesis in a healthy term infant?	244
103. What are the two most likely causes of visible blood in the stool of an otherwise healthy infant?	244
104. What are the six most common causes of massive GI bleeding in children?	244
Gastrointestinal dysmotility	244
105. How rapidly do infants outgrow GER?	244
106. When does GER become GERD (gastroesophageal reflux disease)?	244
107. What are the diagnostic methods for GER?	245
108. How effective are nonpharmacologic agents as treatments for suspected GER?	245
109. How effective are H2 blockers and proton pump inhibitors (PPIs) in the treatment of GER?	245
110. An infant with known GER who periodically arches his or her back may have what syndrome?	245
111. What is a Nissen fundoplication?	245
112. Which patients are candidates for fundoplication?	246
113. A teenage girl has symptoms of swallowing difficulties improved by positional head and neck changes, nocturnal regur...	246
114. What are the key tests to diagnose achalasia?	246
115. What are treatment options for achalasia?	246
116. What are the common symptoms of gastroparesis?	247
117. In what clinical settings should gastroparesis be suspected?	247
118. How is postinfectious gastroparesis diagnosed?	247
119. How is gastroparesis treated?	247
120. A 12-year-old who presents with weight loss and a history of effortlessly and involuntarily regurgitating many meals...	247
Hepatic, biliary and pancreatic disease	247
121. What laboratory tests are commonly used to evaluate liver disease?	247
122. What conditions are associated with elevations of aminotransferases?	248
123. What is the most frequent cause of chronically elevated aminotransferases among children and adolescents in the Unit...	248
124. What is the main reason for the apparent increase in pediatric NAFLD?	248
125. What is the best way to screen for NAFLD?	249
126. Why is it important to determine whether an elevated bilirubin is conjugated or unconjugated?	249
127. When are levels of conjugated bilirubin considered abnormal?	249
128. What are the common causes of neonatal hepatitis and neonatal cholestasis?	249
129. What is the likelihood of chronic hepatic disease developing after acute infections with hepatitis viruses A to G?	250
130. Other than viral hepatitis, what are other common causes of acute and chronic hepatitis in children?	250
131. How is α1-antitrypsin deficiency most likely to present in infants and children?	250
132. Why is measuring the level serum level of α1-antitrypsin not enough to diagnose α1-antitrypsin deficiency?	250
133. What is the metabolic defect in patients with Wilson disease?	250
134. What are the treatments of choice for Wilson disease?	251
135. A 3-year-old child who experiences mild fluctuating jaundice in times of illness ``just like his Uncle Kevin´´ is li...	251
136. What are the clinical findings of portal hypertension?	251
137. How does autoimmune hepatitis (AIH) typically present?	251
138. A patient with liver failure develops confusion. Why worry?	251
139. What is the most common indication for pediatric liver transplantation?	251
140. Calculous and acalculous cholecystitis: what are the differences?	251
141. Which patients are at risk for cholelithiasis?	252
142. What are the possible causes of pancreatitis in children?	252
143. What is the typical presentation of acute pancreatitis in children?	252
144. Which enzyme is a more sensitive marker of pancreatic injury in children: amylase or lipase?	253
145. What conditions may be associated with hyperamylasemia?	253
Inflammatory bowel disease	253
146. What is the epidemiology of pediatric inflammatory bowel disease (IBD)?	253
147. How do ulcerative colitis and Crohn disease vary in intestinal distribution?	253
148. What features differentiate ulcerative colitis from Crohn disease?	253
149. What is the role of serologic panels in the diagnosis of IBD?	254
150. What are the extraintestinal manifestations of pediatric IBD?	255
151. What pharmacologic therapies are used in the treatment of ulcerative colitis and Crohn disease?	255
152. How are therapies chosen for IBD?	255
153. Is there a potential role for thalidomide in the treatment of Crohn disease?	255
154. In a child who has been diagnosed with Crohn disease, what are potential long-term complications?	256
155. Are children with IBD at increased risk for malignancy?	256
156. When is surgery indicated for children with IBD?	256
Nutrition	256
157. What are various requirements for protein, fat, and carbohydrates?	256
158. If recommended caloric intakes are maintained, what is normal daily weight gain of young children?	257
159. What are the recommended bottle feedings by age?	257
160. Why should whole cow milk not be introduced until 1 year of age?	258
161. Why is honey not recommended for infants during the first year of life?	258
162. How is nutritional status objectively assessed in children?	258
163. What features on examination of the scalp, eyes, and mouth suggest problems of malnutrition?	258
164. How do marasmus and kwashiorkor differ clinically?	258
165. What vitamins and minerals are often deficient in strict vegans and some vegetarians?	259
166. What two factors make vitamin D deficiency such a common problem?	259
167. How much vitamin D should children receive on a daily basis?	259
168. What are the cutoffs for vitamin D deficiency and sufficiency?	260
Obesity and lipid disorders	260
169. What are the weight status categories for children in terms of body mass index (BMI) percentile?	260
170. What screening laboratory tests should be done for obese children?	260
171. What are the different types of cholesterol?	260
172. Why is the promotion of cardiovascular health and the identification of specific risk factors important in pediatric...	260
173. What are the screening guidelines for lipids?	261
174. What are the cutoffs for abnormal lipid levels?	261
175. What are the American Heart Association dietary strategies for all children older than 2 years?	261
176. How are the primary genetic hyperlipidemias classified?	262
177. What is the most common hyperlipidemia in childhood?	262
Surgical issues	262
178. What is the natural history of an umbilical hernia?	262
179. Which umbilical hernias warrant surgical repair?	262
180. When should an infant with inguinal hernia have it electively repaired?	263
181. Does surgical repair of one hernia warrant intraoperative exploration for another on the opposite side?	263
182. How are incarcerated inguinal hernias reduced?	263
183. Under what clinical settings should manual reduction of an inguinal hernia not be attempted?	263
184. What is the significance of green vomiting during the first 72 hours of life?	263
185. What are the clinical findings of malrotation of the intestine?	264
186. What causes the intestinal obstruction in malrotation?	264
187. In an asymptomatic child with an incidental finding of malrotation, is surgery indicated?	264
188. What is the most common cause of intestinal obstruction in young children?	265
189. In what settings should intussusception be suspected?	265
190. How commonly does intussusception appear with the classic findings?	265
191. What causes intussusception?	265
192. What is the most common type of intussusception?	265
193. How is intussusception diagnosed?	266
194. How frequently does intussusception recur?	266
195. Rotavirus vaccine and intussusception: how are they intertwined?	266
196. Duodenal or jejunoileal atresia: which is associated with other embryonic abnormalities?	266
197. What is the classic radiographic finding in duodenal atresia?	266
198. How does the infant with biliary atresia classically present?	266
199. What is the surgical procedure for biliary atresia?	267
200. Which is accompanied by more complications: high or low imperforate anus?	267
201. What is the classic presentation of pyloric stenosis?	267
202. How is pyloric stenosis diagnosed?	267
203. What is the mechanism of hyperbilirubinemia in babies with pyloric stenosis?	267
204. In a patient with suspected pyloric stenosis, why is an acidic urine very worrisome?	267
205. What is the connection between pyloric stenosis and macrolide antibiotics?	268
206. What is the short bowel syndrome?	268
207. Why are infants with short bowel syndrome prone to renal calculi?	268
208. In extensive small bowel resection, how much is ``too much´´?	268
209. Appendicitis in children: clinical, laboratory, or radiologic diagnosis?	269
210. How specific is the diagnosis of appendicitis if an appendicolith is noted on radiograph?	269
211. Should a digital rectal examination be performed on all children with possible appendicitis?	269
212. In children taken to surgery for suspected appendicitis, how often is perforation of the appendix present?	270
213. Should children with acute abdominal pain be given analgesia before a diagnosis?	270
214. In what clinical settings is rectal prolapse most commonly seen?	270.e1
215. List the indications for lower gastrointestinal colonoscopy or endoscopy in children.	270.e1
216. How can you tell a coin from a button battery on x-ray?	270.e1
217. What are the indications for nonemergent foreign body removal?	270.e1
218. What are risk factors for the formation of phytobezoars?	270.e1
219. What tests, in addition to those ruling out Hirschsprung disease, are commonly considered for refractory cases of co...	270.e1
220. Which children should be seen for the medical evaluation of acute diarrhea?	270.e2
221. What is the differential diagnosis of chronic diarrhea in different age groups?	270.e2
222. What is the physiologic basis for oral rehydration therapy?	270.e2
223. What are the basic principles guiding optimal treatment of children with diarrhea and mild dehydration?	270.e2
224. What is the most common treatment for travelers´ diarrhea in children?	270.e3
225. Which patients are particularly susceptible to giardiasis?	270.e3
226. What common conditions are associated with an increased risk for celiac disease?	270.e3
227. What is the management for massive upper GI bleeding in children?	270.e3
228. Why is the buffering of gastric acid important for controlling upper GI bleeding?	270.e3
229. What are the most common complications of fundoplication?	270.e3
230. How does the presentation of achalasia differ with age?	270.e4
231. Can hepatitis C virus (HCV) be acquired vertically at birth?	270.e4
232. Is a decreased ceruloplasmin pathognomonic to Wilson disease?	270.e4
233. An infant with cholestasis, triangular facies, and a pulmonic stenosis murmur is likely to have what syndrome?	270.e4
234. What are the common side effects of the immunomodulators?	270.e4
235. What is the difference between Infliximab and adalimumab?	270.e4
236. What is the mechanism of enteral nutrition therapy for IBD?	270.e4
237. What is the postoperative prognosis for Crohn disease and ulcerative colitis?	270.e4
238. When and how should solids be introduced into an infants diet?	270.e5
239. What are risk factors for low vitamin D levels?	270.e5
240. What comorbidities are associated with obesity?	270.e5
241. How do causes of intestinal obstruction vary by age?	270.e5
242. When should a Kasai procedure be performed?	270.e6
243. What conditions may mimic appendicitis?	270.e6
Chapter 8: Genetics	271
Clinical issues	271
1. Which disorders with ethnic and racial predilections most commonly warrant maternal screening for carrier status?	271
2. Why are mitochondrial disorders transmitted from generation to generation by the mother and not the father?	271
3. What is genetic imprinting?	271
4. What is uniparental disomy?	272
5. What is the etiology of arthrogryposis congenita?	272
6. How common are genetic causes of hearing loss in childhood?	273
7. What is the most common genetic mutation in infants with prelingual hearing loss?	273
8. What are the genetic causes of microcephaly?	273
9. Are older fathers at increased risk of having a child with a genetic disease?	273
10. What is the most common genetic lethal disease?	273
11. What are the syndromes associated with macrosomia (large baby syndromes)?	274
12. What is the ``H3O´´ of Prader-Willi syndrome?	274
13. A child with supravalvular aortic stenosis, small and abnormally shaped primary teeth, low muscle tone with joint lax...	274
14. What are the two most common forms of dwarfism that are recognizable at birth?	274
15. What chromosomal abnormality is found in cri-du-chat syndrome?	275
16. Is there a ``Catch-22´´ to the Catch-22 syndrome?	275
17. For what condition are patients with isolated limb hypertrophy at risk?	275
18. After Down syndrome, what are the next most common autosomal trisomies in live-born children?	276
19. What are the reasons that a condition might be genetically determined but the family history would be negative?	276
20. What online resources are available for a pediatrician who suspects a child has a genetic syndrome or would like addi...	277
Down syndrome	277
21. What are the common physical characteristics of children with Down syndrome?	277
22. Are Brushfield spots pathognomonic for Down syndrome?	278
23. What is the chance that a newborn with a simian crease has Down syndrome?	278
24. Why is an extensive cardiac evaluation recommended for newborns with Down syndrome?	279
25. What proportion of infants with Down syndrome has congenital hypothyroidism?	279
26. Infants with Down syndrome are at increased risk for a number of conditions during early infancy. What are they?	279
27. What is the most common malignancy in an infant with Down Syndrome?	279
28. What is the genetic basis for Down syndrome?	279
29. What chromosomal abnormalities are related to maternal age?	279
30. How does the risk for having an infant with Down syndrome change with advancing maternal age?	280
31. Who was Down of Down syndrome?	280
Dysmorphology	280
32. What is the clinical significance of a minor malformation?	280
33. Do infants with the LEOPARD syndrome have spots?	280
34. Which is correct: CHARGE syndrome or CHARGE association?	281
35. What is the proper way to test for low-set ears?	281
36. How is hypertelorism distinguished from telecanthus?	281
37. What is the inheritance pattern of cleft lip and palate?	282
38. Which syndromes are associated with colobomas of the iris?	282
Genetic principles	283
39. Identify the common symbols used in the construction of a pedigree chart	283
40. How can the same genotype lead to different phenotypes?	283
41. When a geneticist says they are going ``FISH´´ing, what does that mean?	283
42. What is currently the best method for detecting small chromosome deletions and duplications?	284
Inborn errors of metabolism	284
43. What types of inherited metabolic conditions are routinely screened by most states?	284
44. In what settings should inborn errors of metabolism be suspected?	284
45. What are the main categories of specialized laboratory testing to detect an IEM?	284
46. What are the main principles of treatment for IEM?	285
47. What are the main features of phenylketonuria (PKU)?	285
48. What are the main characteristics of a patient with glycogen storage disease type 1 (GSD 1)?	285
49. What are the main features of a patient with mucopolysaccharidosis?	285
50. An 8-month-old presents with vomiting, lethargy, hypoglycemia and no ketones on urinalysis. What condition is likely?	287
51. What features should raise suspicion of mitochondrial disease?	287
52. What is the most common presentation of childhood-onset mitochondrial disease?	287
53. When you are rounding in the well newborn nursery, one of the infants has an unusual odor. What are the typical body ...	287
54. Which inborn errors of metabolism can result in fetal hydrops?	289
55. Which metabolic disorders can present as sudden unexpected death syndrome (SUDS)?	289
56. One of the infants in your care dies from a suspected IEM. What postmortem investigations are key?	289
Sex-chromosome abnormalities	289
57. Does the Lyon hypothesis refer to the ``king of beasts´´?	289
58. What are the features of the four most common sex-chromosome abnormalities?	289
59. Of the four most common types of sex-chromosome abnormalities, which is identifiable at birth?	291
60. What are the differences between Noonan syndrome and Turner syndrome?	292
61. What is the second most common genetic form of mental retardation?	292
62. What are the characteristic facial features of fragile X syndrome?	292
63. What is the nature of the mutation in fragile X syndrome?	292
64. What are the associated medical problems of fragile X syndrome in males?	293
65. What is the outcome for girls with fragile X?	293
Teratology	293
66. Which drugs are known to be teratogenic?	293
67. Describe the characteristic features of the fetal hydantoin syndrome	294
68. A pregnant female sommelier asks you what amount of Chateauneuf Du Pape is safe to ingest during pregnancy.	294
69. What are the frequent facial features of the fetal alcohol syndrome?	294
70. What happens to children with fetal alcohol syndrome when they grow up?	295
71. What syndromes with a genetic basis should be considered in a patient with ambiguous genitalia?	295.e1
72. What is the genetic basis of Angelman syndrome?	295.e1
73. Why are patients with Marfan syndrome at risk for sudden cardiac death?	295.e1
74. List the syndromes and malformations associated with congenital limb hemihypertrophy	295.e1
75. What are the most common chromosome deletion syndromes?	295.e1
76. Which of the inborn errors of metabolism are associated with liver disease?	295.e1
Chapter 9: Hematology	296
Bone marrow failure	296
1. What are the types of bone marrow failure?	296
2. What are the causes of acquired aplastic anemia?	296
3. What is the definition of severe aplastic anemia?	296
4. What are the treatments and prognosis for children with aplastic anemia?	296
5. What is the probable diagnosis of a 6-year-old child with pancytopenia, short stature, abnormal thumbs, and areas of h...	297
6. How is the diagnosis of Fanconi anemia made?	297
7. A 1-year-old child presents with pallor and lethargy and is found to have a normocytic anemia (hemoglobin 3.5 g/dL). T ...	297
8. Why is distinguishing between the two conditions extremely important?	297
9. How are the two conditions diagnosed?	297
10. What is Kostmann syndrome?	298
11. You are asked to evaluate a 9-month-old male with eczema and recurrent respiratory infections who was found to be thr...	298
12. A 4-year-old with failure to thrive and chronic diarrhea has a normal sweat test but is noted to have neutropenia on ...	298
Clinical issues	298
13. What is the hemoglobin value below which children are considered to be anemic (lower limit of normal)?	298
14. When does the physiologic anemia of infancy occur?	298
15. Why does the physiologic anemia of infancy occur?	298
16. In what settings of shortened RBC survival can the reticulocyte count be normal or decreased?	299
17. How does the pathophysiology of anemia differ in chronic and acute infection?	299
18. Describe the differential diagnosis for children with splenomegaly and anemia.	299
19. What is the significance of a leukemoid reaction?	299
20. Name the three most common causes of eosinophilia in children in the United States.	299
21. What conditions are associated with extreme elevations of eosinophils in children?	299
22. A 14-month-old child presents symptoms including marked cyanosis, lethargy, and normal oxygen saturation by pulse oxi...	300
23. What is the treatment for methemoglobinemia?	300
24. Why are infants at greater risk for the development of methemoglobinemia?	300
25. What are the critical steps in planning for a teenager with a chronic hematologic condition to transition to adult-or...	300
Coagulation disorders	300
26. What features on history or physical examination help pinpoint the cause of a bleeding problem?	300
27. What do the activated partial thromboplastin time (aPTT) and the prothrombin time (PT) measure in the basic clotting ...	301
28. What are the possible causes of a prolonged aPTT and PT?	301
29. What is the INR?	301
30. What are the frequency and the inheritance patterns of common bleeding disorders?	302
31. Why is the lack of a family history of bleeding problems only moderate evidence against the likelihood of hemophilia ...	302
32. What are the clinical classifications for hemophilia A and B?	302
33. What are the primary measures for achieving hemostasis in individuals with bleeding disorders?	303
34. To what degree should factor levels be raised for patients with hemophilia with or without life-threatening hemorrhage?	303
35. How are doses of replacement factors calculated?	303
36. In patients with severe hemophilia, can prophylaxis with factor replacement prevent severe hemorrhage?	303
37. What are the half-lives of exogenously administered factors VIII and IX?	304
38. Are longer-acting factors VIII and IX available?	304
39. What can cause an elevation of the PT when other coagulation testing is normal?	304
40. Who gets hemophilia C?	304
41. Why is factor IX deficiency also called ``Christmas disease´´?	304
42. What is the von Willebrand factor (vWF)?	305
43. What are the coagulation abnormalities in von Willebrand disease?	305
44. What are initial diagnostic tests for suspected von Willebrand disease?	305
45. What does the ristocetin cofactor assay measure?	305
46. How is von Willebrand disease treated?	305
47. In an adolescent with menorrhagia, how likely is a bleeding disorder?	305
48. How does DDAVP work in the treatment of von Willebrand disease?	305
49. Should children awaiting surgery undergo routine preoperative screening for potential abnormal bleeding?	306
50. What is the role of vitamin K in coagulation?	306
51. In what settings outside the newborn period can vitamin K abnormalities contribute to a bleeding diathesis?	306
52. What is the best test for distinguishing coagulation disturbances that result from hepatic disease, DIC, and vitamin ...	306
53. What is DIC?	307
54. What tests are valuable for the diagnosis of suspected DIC?	307
55. What is the treatment of choice for DIC?	307
56. What are the common hereditary disorders that predispose a child to thrombosis?	307
57. What are the inheritance patterns of the hypercoagulable states?	307
58. In an adolescent with an unprovoked deep vein thrombosis (DVT), what risk factors need to be assessed?	308
59. What anatomic variants will predispose individuals to venous thromboses?	308
60. What are the mechanisms for low molecular weight heparin and pentasaccharide as antithrombotic agents?	308
61. What are the direct thrombin inhibitors?	308
Developmental physiology	308
62. How do immunoglobulin (Ig) levels change during the first years of life?	308
63. Why are antibodies not produced by the fetus in appreciable quantities?	308
64. What is the role of the thymus?	309
65. At what age does thymic function cease?	309
66. How does neutrophil function in the neonate compare with that of adults?	309
Hematology laboratory	309
67. Of the seven red-cell parameters given by a Coulter counter, which are measured and which are calculated?	309
68. How does the mean corpuscular volume help provide a quick screen of the possible causes of anemia?	309
69. What is a quick rule of thumb for approximating MCV?	309
70. In addition to an elevated reticulocyte count, what laboratory studies suggest increased destruction (rather than dec...	309
71. Why must the reticulocyte count sometimes be corrected?	310
72. What is the significance of targeting on an RBC smear?	310
73. In what conditions are Howell-Jolly bodies found?	310
74. What is the cause of Heinz bodies?	310
75. What makes an ``atypical lymphocyte´´ atypical?	311
76. A patient with oculocutaneous albinism has repeated Staphylococcus aureus infections and the peripheral smear shown i...	311
Hemolytic anemia	312
77. What clinical features are suspicious for hemolytic anemia?	312
78. What two types of RBC forms are commonly seen on the peripheral smear in patients with hemolytic anemia?	312
79. Name the two most common inherited disorders of red-cell membranes	312
80. Which disorder is most commonly associated with an elevated MCHC?	312
81. What is the osmotic fragility test?	312
82. What is the difference between alloimmune and autoimmune hemolytic anemia?	313
83. In which settings do alloimmune and AIHA most commonly appear?	313
84. How does the cause of AIHA vary by age?	313
85. What is the most important test to establish the diagnosis of AIHA?	313
86. What are the differences between autoimmune hemolytic anemias caused by ``warm´´ and ``cold´´ erythrocyte autoantibodies?	313
87. A 6-year-old presents with acute anemia, fatigue, jaundice, and dark urine after an early spring swim in a local quar...	313
88. An 8-year-old black male developed jaundice and very dark urine 24 to 48 hours after beginning nitrofurantoin for a u...	313
89. Why are ``bite cells´´ seen in patients with G6PD deficiency´´?	314
90. In a patient with G6PD deficiency, why is the initial diagnosis often difficult in the acute setting?	314
91. What is favism?	314
Immunodeficiency	314
92. How is neutropenia defined?	314
93. How do children with neutrophil disorders present?	315
94. What is the most common cause of transient neutropenia in children?	315
95. Excluding intrinsic defects in myeloid stem cells, what conditions are associated with neutropenia in children?	315
96. Which is the most common form of chronic childhood neutropenia?	315
97. How common are primary immunodeficiencies?	315
98. What are the typical clinical findings of the various primary immunodeficiencies?	316
99. What is the single most important laboratory test if SCID is suspected?	316
100. Why are male children more likely to suffer from a primary immunodeficiency?	317
101. Which is the most common type of primary immunodeficiency?	317
102. What are the diagnostic criteria for IgA deficiency?	317
103. What is the association of autoimmune disorders and IgA deficiency?	317
104. Why is immunoglobulin therapy not used as a treatment for selective IgA deficiency?	317
105. In an infant with panhypogammaglobulinemia, how can the quantitation of B and T lymphocytes in peripheral blood help...	317
106. What is the underlying disorder in an 8-year-old girl with atypical eczema, pneumatoceles, and bouts of severe furun...	318
107. What are the proven indications for intravenous immunoglobulin (IVIG) therapy?	318
108. What are the pharmacologic characteristics of IVIG?	318
109. What are the adverse reactions to IVIG?	318
110. Which viral infections can result in hypogammaglobulinemia in the immunocompetent individual?	319
111. What is the classic triad of Wiskott-Aldrich syndrome?	319
112. What is the likely diagnosis of a patient presenting with a progressive ataxia, conjunctival abnormalities, and recu...	319
113. What disease did the ``bubble boy´´ have?	320
114. Describe the molecular defect of chronic granulomatous disease (CGD)	320
115. Which laboratory tests are used for the diagnosis of CGD?	320
116. What types of infections are commonly seen in children with CGD?	320
117. Which disorder has to be considered in a newborn patient with delayed separation of the umbilical cord?	320
118. Which potential life-threatening disorder of the complement system is associated with nonpruritic swelling and occas...	320
Immunology laboratory	321
119. Which are the initial screening tests for a suspected immunodeficiency?	321
120. Which laboratory tests allow for a broad evaluation of the humoral immune system?	321
121. Which diagnostic tests allow for the specific evaluation of T-cell functions?	321
122. What is the value of skin testing for the diagnosis of T-cell deficiencies?	321
123. What is the importance of the CD4/CD8 ratio?	321
124. Which laboratory tests appropriately evaluate the phagocytic system?	322
125. How is the classic complement cascade evaluated?	322
Iron-deficiency anemia	322
126. What is the worlds most common single-nutrient deficiency?	322
127. At what age do exclusively breast-fed infants become at risk for iron deficiency?	322
128. Why are infants who begin consuming cow milk at an early age susceptible to iron-deficiency anemia?	323
129. As iron becomes depleted from the body, what is the progression at which laboratory tests change?	323
130. How might the reticulocyte hemoglobin content be helpful for the diagnosis of iron deficiency?	323
131. Why are tests for iron stores more difficult to interpret during acute inflammatory states?	324
132. What is the role of hepcidin in iron metabolism?	324
133. What are the common causes of microcytic anemia in children?	324
134. How is the RDW useful for distinguishing causes of microcytic anemia?	324
135. What is the Mentzer index?	324
136. In a child with suspected iron-deficiency anemia, is a therapeutic trial with iron an acceptable diagnostic approach?	324
137. After iron therapy is initiated, how early can a response be detected?	324
138. What foods affect the bioavailability of nonheme iron?	324
139. What are the options for use of parenteral iron therapy?	325
140. What are the differences between pica, geophagia, and pagophagia?	325
141. What is the derivation of the term pica?	325
142. Discuss the relationship between iron deficiency and development in infants and toddlers.	325
143. What are the risk factors for iron deficiency or iron-deficiency anemia in a 1-year-old?	325
144. Why are iron-deficient children at increased risk for lead poisoning?	325
145. How and when should younger children be screened for iron deficiency?	326
Megaloblastic anemia	326
146. What is megaloblastic anemia?	326
147. Is megaloblastic anemia the most common cause of macrocytic anemia?	326
148. What findings on a CBC are suggestive of megaloblastic anemia?	326
149. What are the causes of vitamin B12 (cobalamin) deficiency in children?	326
150. What are the best dietary sources of folate and B12?	327
151. What is pernicious anemia?	327
152. A 10-month-old child who was exclusively fed goat milk is likely to develop what type of anemia?	327
Platelet disorders	327
153. How can a platelet count be estimated from a peripheral smear?	327
154. What are the main pathophysiologic processes that can result in thrombocytopenia?	327
155. A previously healthy 3-year-old child develops mucosal petechiae, multiple ecchymoses, and a platelet count of 20,00...	327
156. What microscopic features would suggest a diagnosis other than ITP in a patient with a platelet count of 20,000/mm3?	327
157. What is the natural history of acute childhood ITP?	327
158. In a toddler with suspected ITP, what is the significance of a palpable spleen on examination?	328
159. In patients with suspected ITP, should a bone marrow evaluation be done?	328
160. When should medical treatment be given for acute ITP without active bleeding?	328
161. What are thrombocytopenic precautions in children?	328
162. How do treatments for ITP compare?	328
163. Which children with ITP are candidates for splenectomy?	329
164. What evaluations should be considered in a patient with persistent refractory thrombocytopenia?	329
165. How is neonatal alloimmune thrombocytopenia diagnosed and treated?	329
166. In what conditions of children is thrombocytosis most commonly seen?	329
167. What level of thrombocytosis requires treatment?	329
Sickle cell disease	330
168. What is the mutation that results in sickle cell disease?	330
169. Why is sickle cell disease often asymptomatic during the first months of life?	330
170. What are the various genotypes that can cause the clinical syndrome of sickle cell disease?	330
171. What are the two major pathophysiologic mechanisms in sickle cell anemia that cause the morbidities associated with ...	330
172. A 6-month-old black male has painful swelling of both hands. What is the most likely diagnosis?	330
173. When does functional asplenia occur in children with sickle cell disease?	331
174. What is the most common cause of death in children with sickle cell disease?	331
175. What are the four main categories of acute events requiring intervention in patients with sickle cell disease?	331
176. How should a child with a vasoocclusive (painful) event be managed?	331
177. A 15-month-old with sickle cell disease presents with pallor and fatigue, but no jaundice. On exam, his spleen is pa...	331
178. What is ``acute chest syndrome´´ in sickle cell patients?	332
179. How should the acute chest syndrome in sickle cell patients be treated?	332
180. How often is priapism a problem in children with sickle cell disease?	332
181. What are some long-term morbidities associated with sickle cell disease?	332
182. How can stroke be prevented in children with sickle cell disease?	332
183. If initiated for either primary or secondary stroke prevention, when should blood transfusions be discontinued?	333
184. What is the primary mechanism by which hydroxyurea is beneficial for sickle cell disease?	333
185. Hydroxyurea treatment in young children: how early and how beneficial?	333
186. How common is the sickle cell trait in the United States?	334
187. Does sickle cell trait have any significant morbidity?	334
188. What is the second most common worldwide hemoglobin variant?	334
Thalassemia	334
189. What are the thalassemias?	334
190. Where was β-thalassemia first described?	334
191. What accounts for the variability in the clinical expression of the thalassemias?	334
192. How is the diagnosis of thalassemia made in most clinical laboratories?	335
193. Describe the clinical features of the alpha-thalassemia syndromes	335
194. What is hemoglobin Barts?	335
195. What are the clinical features of the beta-thalassemia syndromes?	335
196. How can coexistent iron deficiency increase the difficulty of diagnosing beta-thalassemia?	335
197. What are the adverse effects of chronic transfusional iron overload in children with thalassemia?	336
198. What are the two most common diseases that are associated with transfusion-related iron overload?	336
199. How do you reduce iron accumulation in children who require repeated transfusions?	336
200. In addition to iron loading, what are additional risks of chronic transfusion therapy?	336
Transfusion issues	336
201. What is the difference between the direct and indirect Coombs tests?	336
202. What is the difference between forward and reverse blood typing?	337
203. What can cause a patient to be ABO indeterminate?	337
204. What is a naturally forming RBC antibody?	337
205. What is the difference between a type and screen and a type and cross?	337
206. What are the indications for the use of leukoreduced RBCs?	337
207. What is the estimated total blood volume of children?	337
208. What is the RBC transfusion threshold for infants <4 months of age?	338
209. What is the packed red blood cell (PRBC) transfusion threshold for children >4 months of age?	338
210. In patients with severe chronic anemia, how rapidly can transfusions be given?	338
211. At typical doses, what are the expected increases and likely average survival of packed RBCs, platelets, and fresh f...	338
212. What are the components of cryoprecipitate?	339
213. What are the most common types of transfusion reactions?	339
214. What is the most common cause of transfusion-related death in the United States?	339
215. What is the difference between an acute hemolytic transfusion reaction and a delayed hemolytic transfusion reaction?	339
216. Why do some blood products require irradiation?	339
217. In what clinical settings is apheresis utilized?	340
218. How are transfusion-transmitted diseases prevented?	340
219. What role does molecular testing play in providing blood products to patients?	340
220. Do socioeconomic factors affect the ability of pediatric patients to successfully transition health care?	340.e1
221. Which are the characteristics of immunoglobulin transport across the placenta?	340.e1
222. What are the allergies associated with IgA deficiency?	340.e1
223. Is there a role for stimulating platelet production in ITP therapy?	340.e1
224. In addition to stroke, what other sickle cell-related conditions benefit from chronic transfusion regimens?	340.e1
225. What are the indications for platelet transfusion in neonates and children?	340.e1
Chapter 10: Infectious Diseases	341
Anti-infective therapy	341
1. What are the main features of penicillins?	341
2. What are the different classes and spectra of activity of penicillins?	341
3. In patients for whom the history lists ``penicillin allergy,´´ how commonly is a true allergy present on testing?	342
4. If a 16-year-old male develops a pruritic, maculopapular rash 1 week after starting treatment with amoxicillin for an ...	342
5. What are the similarities between penicillins and cephalosporins?	342
6. How do the ``generations´´ of cephalosporins differ from one another?	342
7. What are the differences among first-, second-, third-, and fourth-generation cephalosporins?	342
8. Can cephalosporins be safely given to patients who are allergic to penicillin?	343
9. What are the two primary mechanisms of resistance to β-lactam antibiotics?	343
10. What are the main features of carbapenems?	343
11. What is the role of ``double antimicrobial coverage´´?	343
12. What are the ``ESKAPE´´ organisms?	344
13. How can the emergence of antibiotic-resistant pathogens be minimized?	344
14. What is the distinction between community-associated methicillin-resistant S. aureus (CA-MRSA) and hospital acquired ...	344
15. Why is the D-test done?	344
16. Is mupirocin useful in the eradication of S. aureus in colonized children?	345
17. What is the ``red man syndrome´´ and which antibiotic is it associated with?	345
18. How should infections with vancomycin-resistant enterococci be managed?	345
19. Is vancomycin still effective against all staphylococci?	346
20. In what situations may treatment with vancomycin be considered appropriate?	346
21. Are fluoroquinolones safe to use in children?	346
22. What are the uses of ribavirin?	347
23. Why is chicken soup so helpful for upper respiratory infections (URIs)?	347
24. Is there any physiologic basis to the adage ``starve a fever, feed a cold´´?	347
Clinical issues	347
25. Name the three stages of pertussis infection (whooping cough)	347
26. What is the most common cause of death in children with whooping cough?	348
27. Is antibiotic therapy of value in pertussis infection?	348
28. What are ways on physical exam to help distinguish swelling as a result of mumps from swelling caused by lymphadenitis?	348
29. What is the empiric treatment of a skin and soft tissue infection (SSTI) in the setting of the increasing prevalence ...	348
30. What are the distinguishing features of staphylococcal scalded skin syndrome, staphylococcal toxic shock syndrome, an...	349
31. Can antiviral medications be used to prevent or treat oral herpes simplex virus (HSV) infections?	350
32. What is the proper medical term for oral thrush?	350
33. What is the most common specific etiology diagnosed in patients with systemic febrile illness after international travel?	350
34. What is the classic triad of malaria?	350
35. How is malaria diagnosed?	351
36. Which illness is associated with the term ``breakbone fever´´?	351
37. What causes leptospirosis?	351
38. What are the phases of leptospirosis?	351
39. Which organisms are particularly dangerous to clinical microbiology laboratory workers?	352
Congenital infections	352
40. Which congenital infections cause cerebral calcifications?	352
41. What are the late sequelae of congenital infections?	352
42. What is the most common congenital infection?	353
43. How common is hearing loss from congenital CMV?	353
44. How is CMV transmitted from mother to infant?	353
45. How should congenital CMV be treated?	353
46. What is the risk to the fetus if the mother is infected with parvovirus B19 during pregnancy?	353
47. What are the consequences of primary varicella infection during the first trimester?	353
48. What are the indications for postexposure prophylaxis for varicella in the newborn?	353
49. Do urogenital mycoplasmas have a role in neonatal disease?	354
50. What are the features of congenital rubella syndrome (CRS)?	354
51. Should all pregnant women be screened for HSV infection during pregnancy?	354
52. What are risk factors for the development of neonatal HSV disease?	354
53. What are the three forms of neonatal HSV disease?	355
54. When should HSV disease be suspected in newborn or infants?	355
55. How should the neonate with suspected HSV disease be treated?	355
56. In which groups of women is prenatal hepatitis B surface antigen (HBsAg) screening recommended?	355
57. What is the risk to the fetus if the mother is infected with hepatitis B virus?	356
58. How should infants born to mothers with hepatitis B infection be managed?	356
59. How should infants born to mothers with hepatitis A infection be managed?	356
60. How should infants born to mothers with hepatitis C infection be managed?	356
61. How do the clinical features of early and late congenital syphilis differ?	356
62. How is the diagnosis of congenital syphilis made?	356
63. What are the pitfalls of RPR and VDRL testing?	357
64. If a pregnant woman is found to have Chlamydia trachomatis in her birth canal, what is the most appropriate course of...	358
65. Should newborns of mothers with untreated chlamydial infection receive prophylactic antibiotic therapy?	358
66. What is the risk to a fetus after primary maternal Toxoplasma infection?	358
67. What is the typical presentation of congenital toxoplasmosis?	359
68. How can a woman minimize the chance of acquiring a Toxoplasma infection during pregnancy?	359
Emerging infectious diseases	359
69. Which mycobacterium may infect someone who has a home aquarium?	359
70. What are possible sources for an anthrax infection in a 17-year-old who lives on a cattle farm, makes and plays drums...	359
71. What are the novel coronaviruses?	359
72. What viral etiology should be considered in a patient with acute, unexplained respiratory illness who is not febrile?	360
73. What two diseases in particular should be in the differential diagnosis for a traveler returning from the Caribbean w...	360
74. What is the most common viral intestinal infection you can get from eating at a salad bar?	360
75. When was the Ebola virus first discovered?	360
76. What are the clinical features of an Ebola infection?	360
77. What is the natural reservoir host of the Ebola virus?	361
The febrile child	361
78. At what temperature does a child have fever?	361
79. Where did the popular notion that a normal temperature is 98.6 °F originate?	361
80. How does temperature vary among different body sites?	361
81. How accurate is parental palpation for fever in infants?	361
82. How should the temperature of young infants be taken?	362
83. How do environmental factors affect an infant's temperature?	362
84. What is occult bacteremia?	362
85. How has the pneumococcal vaccine (PCV) affected the incidence of occult bacteremia?	362
86. What is meant by ``serotype replacement´´?	362
87. What is the proper way to evaluate and manage febrile illness in neonates 28 days?	363
88. What is the evaluation and management of febrile illness in infants >28 days to 90 days?	363
89. How should older infants and toddlers (3 to 36 months old) with fever and no apparent source be managed?	363
90. When is a chest radiograph indicated for a febrile young infant?	364
91. What is the approach for a 2-week-old, otherwise healthy, afebrile, full-term female who presents to the ED with mast...	364
92. What is a CLABSI?	364
93. How long should one wait before a blood culture is designated negative?	364
94. What is the utility of so-called ``rapid´´ pathogen testing?	365
95. When is a fever considered a fever of unknown origin (FUO)?	365
96. What is the eventual etiology of fever in children with FUO?	365
97. How should a child with FUO be evaluated?	365
98. What is PFAPA?	366
99. In addition to PFAPA, which syndromes are associated with periodic fevers?	366
Human immunodeficiency virus infection	366
100. How common is the maternal-to-infant transmission of HIV?	366
101. What drugs are recommended for reducing the maternal-to-infant transmission of HIV?	367
102. What are the risk factors for perinatal transmission of HIV?	367
103. Should HIV-infected women breast-feed?	367
104. How is an infection with HIV confirmed in the newborn infant?	368
105. What are the earliest and most common manifestations of congenital HIV infection?	368
106. When should Pneumocystis prophylaxis begin and end for an HIV-exposed infant?	368
107. Among patients <13 years of age with HIV infection, how is the severity of HIV illness classified?	369
108. What is the significance of the ``viral load´´?	369
109. What are the classes of antiretroviral agents (ARTs) used to treat HIV?	369
110. What are the common toxicities associated with antiretroviral therapy?	369
111. How should nonadherence to HIV medication be addressed?	371
112. Should a classroom teacher be told that a child is HIV positive?	371
113. What are the risk factors for HIV transmission after a needlestick injury?	371
Immunizations	372
114. What is the derivation of the word ``vaccination´´?	372
115. Why are the buttocks a poor location for intramuscular (IM) injections in infants?	372
116. When administering an IM vaccination, is aspiration necessary before injection?	372
117. Is there any risk associated with administering multiple vaccines simultaneously?	372
118. Should premature babies receive immunization on the basis of postconception age or chronologic age?	373
119. Which vaccines are egg-embryo-based vaccines?	373
120. What is the difference between whole-cell and acellular pertussis vaccines?	373
121. What are the absolute contraindications to pertussis immunization?	373
122. How long does protection against pertussis last after immunization?	374
123. What is cocooning?	374
124. Which vaccines offer protection against cervical cancer?	374
125. How effective is the pneumococcal conjugate vaccine?	374
126. What is the ``grandparent effect´´ of vaccination?	375
127. What serogroup capable of causing meningococcal infections is lacking in licensed polyvalent vaccines in the United ...	375
128. How effective is the varicella vaccine if given after exposure to the illness?	375
129. Is the MMR vaccine effective in preventing measles if given after exposure to the illness?	376
130. Of the vaccines included in the routine schedule, which ones contain live viruses?	376
131. What are the indications for palivizumab?	376
132. What are the recommendations regarding the administration of live-virus vaccines to patients receiving corticosteroi...	376
133. What is thimerosal?	377
134. Does thimerosal or any vaccine or vaccine combination cause autism?	377
135. How should parents who refuse vaccinations be handled?	377
Infections with rash	377
136. What is the traditional numbering of the ``original´´ six exanthemas of childhood, and when were they first described?	377
137. What conditions are associated with fever and petechiae?	378
138. What are the three Cs of measles?	378
139. What do Koplik spots look like?	378
140. What is ``atypical´´ about atypical measles?	379
141. How is measles diagnosed?	379
142. Why is postmeasles blindness so common in underdeveloped countries?	379
143. How is measles treated?	379
144. What are the most feared neurologic complications of measles?	380
145. Which viruses comprise the human herpesviruses (HHV)?	380
146. What is the derivation of the word herpes?	380
147. What are the typical features of roseola (exanthema subitum)?	380
148. What causes roseola?	380
149. How common is human herpesvirus type-6 (HHV-6) infection in children?	381
150. What is the spectrum of disease caused by parvovirus B19?	381
151. Describe the characteristic rash of Rocky Mountain spotted fever (RMSF) from Rickettsia rickettsii.	381
152. Why is doxycycline recommended for all ages in patients with suspected RMSF?	381
153. How long after exposure to chickenpox (varicella) do symptoms develop?	382
154. What is the risk for varicella-associated complications in normal children 1 to 14 years old?	382
155. How common are second episodes of varicella after natural infection?	382
156. What is herpes zoster?	382
157. In children with herpes zoster, what is the distribution of the rash?	382
158. Is it possible to get herpes zoster after the varicella vaccine?	383
159. Should chickenpox be treated with an antiviral medication?	383
160. Should healthy children with zoster be treated with antiviral medications?	384
161. Who gets herpes gladiatorum?	384
162. What is eczema herpeticum?	384
163. What is hand-foot-and-mouth disease?	384
164. What is the spectrum of disease caused by enterovirus?	384
165. Why do real-time PCR positive test results indicate both ``rhinovirus/enterovirus´´?	385
Influenza	385
166. What is the difference between an epidemic, an outbreak, and a pandemic?	385
167. What are the types of influenza viruses?	385
168. What are the functions of hemagglutinin and neuraminidase?	385
169. What clinical features typically distinguish an infection with an influenza virus from the common cold?	385
170. What is the difference between ``antigenic shift´´ and ``antigenic drift´´?	385
171. What made the influenza A H1N1 pandemic strain of 2009 so novel?	385
172. Which patients should not receive the live-attenuated influenza vaccine?	386
173. What are the main antiviral medications used as treatment for influenza?	387
174. Does influenza display resistance to antiviral medications?	387
175. What are the indications for antiviral medications for influenza in children?	387
176. What complications can be associated with influenza infections?	387
177. What bacterial coinfection is most commonly identified in influenza-associated pediatric deaths?	388
Lymphadenitis and lymphadenopathy	388
178. What are the most common causes of acute and chronic lymphadenitis in normal, otherwise healthy children?	388
179. What infectious etiology should be considered in a toddler with an intensely erythematous but minimally tender subma...	388
180. How is the diagnosis of NTM disease made?	388
181. How is NTM lymphadenitis treated?	388
182. What infectious etiology should be considered in a child with swollen, tender axillary nodes?	388
183. What is the typical course of the lymphadenitis in cat-scratch disease?	388
184. What are other manifestations of cat-scratch disease in addition to lymphadenopathy?	389
185. What are the presentations of Epstein-Barr virus (EBV) infection?	389
186. How was the monospot test developed?	389
187. What is the natural course of serologic responses to EBV infection?	389
188. When are steroids indicated for children with EBV infection?	389
189. What are the clinical presentations of acquired CMV infection?	390
190. What is the most common form of tularemia?	390
191. What vectors are commonly associated with tularemia?	390
192. Which other organisms can cause a mononucleosis-like clinical picture?	390
193. Why is it called ``mononucleosis´´?	390
Meningitis	391
194. What are the most common signs and symptoms of meningitis in infants <2 months?	391
195. What percentage of neonates <30 days of age with bacterial sepsis and positive blood cultures have meningitis?	391
196. What is the most common cause of viral meningitis?	391
197. What is the diagnostic test of choice for enteroviral meningitis?	391
198. What are common arthropod-borne viral causes of meningoencephalitis in the United States?	391
199. Should computed tomography (CT) scans be performed before an LP during the evaluation of possible meningitis?	391
200. What is the range of normal parameters for CSF in infants and children who do not have meningitis?	392
201. If bloody CSF is collected during LP, how is CNS hemorrhage distinguished from a traumatic artifact?	392
202. How is a traumatic LP interpreted?	392
203. What is the best way to position the patient for an LP?	392
204. How do the CSF findings vary in bacterial, viral, fungal, and tuberculous meningitis in children beyond the neonatal...	393
205. What CSF indices help in the diagnosis of bacterial versus viral meningitis?	393
206. When is the best time to obtain a serum glucose level in an infant with suspected meningitis?	393
207. Does antibiotic therapy before LP affect CSF indices?	393
208. How quickly is the CSF sterilized in children with meningitis?	393
209. What are the most common organisms responsible for bacterial meningitis in the United States?	394
210. What are the drugs of choice for the empiric treatment of bacterial meningitis in children >1 month?	394
211. What is the role of corticosteroids in the treatment of bacterial meningitis?	395
212. How long after treatment has been initiated must individuals with meningitis remain on droplet precautions?	395
213. Should children receiving therapy for bacterial meningitis undergo repeat LP?	395
214. What is the accepted duration of treatment for bacterial meningitis?	395
215. In a patient with meningitis, what are the findings that suggest intracranial complications and provide indications ...	396
216. What are the most common causes of prolonged fever in patients with meningitis?	396
217. What should the parents of a child with bacterial meningitis be told about long-term outcomes?	396
218. How should contacts of children with N. meningitidis disease be managed?	397
219. What is the most common parasitic infection of the CNS?	397
Ocular infections	397
220. Among neonates with conjunctivitis, what is the timing for the various etiologies?	397
221. What is the best method of prophylaxis for ophthalmia neonatorum?	397
222. Can newborns with chlamydial conjunctivitis be treated with topical therapy alone?	397
223. In children with conjunctivitis and otitis media, what are the most likely etiologic agents?	398
224. Can bacterial conjunctivitis be distinguished from viral conjunctivitis on clinical grounds alone?	398
225. What is keratoconjunctivitis?	398
226. What are the most common causative organisms of acute bacterial conjunctivitis?	398
227. How does the treatment vary by age for suspected acute bacterial conjunctivitis?	398
228. What should be the specific treatment for a 5-year-old diagnosed with infectious conjunctivitis in an outpatient set...	398
229. What is Parinaud oculoglandular syndrome?	399
230. How is orbital cellulitis distinguished from periorbital (or preseptal) cellulitis?	399
231. What is the pathogenesis of periorbital and orbital cellulitis?	399
232. What are the most common organisms causing orbital cellulitis?	400
233. What are treatment options for orbital cellulitis?	400
234. What is the difference between a hordeolum, a stye, and a chalazion?	400
235. Why is the ``ciliary flush´´ particularly worrisome when evaluating a patient with a pink or red eye?	400
236. What organism should not be overlooked when treating ocular infections following penetrating trauma?	400
Otitis media	400
237. Is ear pulling a reliable sign of infection?	400
238. What are the landmarks of the tympanic membrane?	401
239. What are the most reliable ways, on physical examination, to accurately diagnosis AOM?	401
240. What are the most common viral and bacterial agents that cause AOM?	401
241. What is the ``watchful waiting´´ approach for otitis media?	402
242. Should all children with AOM be treated with antibiotics?	402
243. What is the recommended therapy for children for whom treatment for AOM is indicated?	402
244. After an acute episode of otitis media (OM), how long does the middle ear effusion persist?	402
245. What are the indications for tympanostomy tubes?	402
246. Should a child with tympanostomy tubes be allowed to swim?	403
247. A child with the acute onset of ear pain and double vision likely has what condition?	403
248. What are differences between acute and chronic mastoiditis?	403
249. What are the potential complications of mastoiditis?	403
250. What famous playwright died of mastoiditis?	403
Pharyngeal and laryngeal infections	403
251. Can group A β-hemolytic streptococcal (GAS) pharyngitis reliably be distinguished from viral causes?	403
252. What is the typical rash of scarlet fever?	404
253. Why is a throat culture for GAS advised if a rapid antigen detection test is negative?	404
254. What is the rationale for the treatment of GAS pharyngitis?	404
255. What is the recommended treatment for GAS pharyngitis?	404
256. Why do some clinicians use treatments other than penicillins for GAS pharyngitis?	404
257. How does one differentiate a patient with a sore throat who is a streptococcal carrier with an intercurrent viral ph...	405
258. When can children treated for positive streptococcal throat cultures return to school or day care?	405
259. How commonly do children <3 years of age develop GAS pharyngitis?	405
260. How long after the development of streptococcal pharyngitis can treatment be initiated and still effectively prevent...	405
261. What diagnosis should be suspected in a teenager with pharyngitis followed by multifocal pneumonia and sepsis?	406
262. What is the difference between herpangina and Ludwig angina?	406
263. What is quinsy?	406
264. How is a peritonsillar abscess distinguished from peritonsillar cellulitis?	406
265. What radiographic features suggest the diagnosis of a retropharyngeal abscess?	406
266. Which age group is most susceptible to retropharyngeal abscess?	407
267. What are the indications for tonsillectomy in children 1 to 18 years old?	407
268. How should children with epiglottitis be managed?	407
269. What are the bacterial causes of epiglottitis?	408
270. How is epiglottitis distinguished clinically from croup?	408
271. What are the criteria for the admission of a child with viral croup?	408
272. Are steroids efficacious for the treatment of croup?	408
273. If a child has received racemic epinephrine as a treatment for croup, is hospitalization required?	409
274. Is a cool-mist vaporizer truly of benefit for patients with croup?	409
275. What are membranous croup and pseudomembranous croup?	409
Sinusitis	409
276. When do the sinuses develop during childhood?	409
277. Does a thick, green nasal discharge on day 2 of a respiratory illness indicate a bacterial sinus infection?	410
278. What is the typical presentation of sinusitis in children?	410
279. What is the role of sinus imaging in the diagnosis of sinusitis?	410
280. What should be suspected in an adolescent male with a very severe frontal headache in the setting of sinusitis?	410
281. When should imaging be considered in cases of sinusitis?	410
282. Which organisms are responsible for acute and chronic sinusitis in the pediatric age group?	411
283. What is the management of acute sinusitis?	411
284. List the predisposing factors for the development of chronic sinusitis	411
Tuberculosis	411
285. How effective is the Bacillus Calmette-Guerin (BCG) vaccination?	411
286. When are the steps in screening for M. tuberculosis?	411
287. How is the Mantoux test interpreted in the context of clinical signs and symptoms and epidemiologic risk factors, su...	412
288. What are the reasons for a false-negative tuberculin skin test (TST)?	412
289. How does BCG immunization influence TB skin testing?	412
290. What is the role of interferon-γ release assays (IGRAs) in the diagnosis of TB in children?	412
291. How are IGRA results interpreted?	413
292. How should a patient with a positive TST or IGRA be evaluated?	413
293. How common is HIV and TB coinfection?	413
294. What is latent tuberculosis infection (LTBI) and why is it treated?	413
295. In a younger child suspected of having TB disease, what is the utility of gastric aspirates?	413
296. What is the role of nucleic acid amplification testing (NAAT) in the diagnosis of TB?	413
297. How do the manifestations of active pulmonary TB on chest radiograph differ between adults and children?	414
298. How are children with active pulmonary TB treated?	414
299. Why are multiple antibiotics used for the treatment of TB disease?	414
300. What are the signs of TB meningitis?	414
301. What is the importance of DOT in the treatment of TB?	414
302. Why is pyridoxine supplementation given to patients who are receiving isoniazid?	415
303. Why do children with TB rarely infect other children?	415
304. In addition to TB, what other airborne microbes can cause respiratory disease?	415
305. Which famous U.S. First Lady died of TB?	415
Chapter 11: Neonatology	416
Clinical issues	416
1. When does the ductus arteriosus (DA) close in well newborn infants?	416
2. Why is it not always possible to auscultate a murmur in an infant with a ventricular septal defect (VSD) on the first ...	416
3. What are the three main types of congenital heart disease, and how do they present in the newborn period?	416
4. What is the chance that an extremely preterm infant will survive without significant impairment?	416
5. What interventions are indicated in future pregnancies after a mother delivers a preterm infant?	417
6. Which infants require ophthalmologic evaluation for retinopathy of prematurity (ROP)?	417
7. What are the stages of ROP?	418
8. What are the indications for treatment for ROP?	418
9. When should hearing evaluations be repeated after neonatal intensive care unit (NICU) discharge?	418
10. What are the manifestations of drug withdrawal in the neonate?	418
11. What is the recommended pharmacologic treatment for neonatal abstinence syndrome (NAS) from opioid withdrawal?	419
12. Should breastfeeding be initiated in infants with NAS?	419
13. If maternal drug abuse is suspected, which specimen from the infant is most accurate for detecting exposure?	419
14. Does in utero exposure to selective serotonin reuptake inhibitors (SSRIs) result in neonatal withdrawal?	419
15. What is the difference between ``sudden unexpected infant death (SUID)´´ and ``sudden infant death syndrome (SIDS)´´?	419
16. What is the ``Back to Sleep´´ program?	420
17. What additional recommendations have been made regarding the most ideal sleep environment for infants?	420
18. Do home apnea monitors help prevent sudden infant death syndrome (SIDS)?	420
Delivery room issues	420
19. When should the umbilical cord be clamped after birth?	420
20. How long has meconium been present in the amniotic fluid if an infant has evidence of meconium staining?	420
21. Is meconium staining a good marker for neonatal asphyxia?	421
22. If meconium is noted before or during the time of delivery, what is the recommended course of action?	421
23. During asphyxia, how is primary apnea distinguished from secondary apnea?	421
24. How should apnea be managed in the delivery room?	421
25. What are the clinical signs of adequate ventilation?	421
26. What are the pros and cons of the T-piece resuscitator?	422
27. How does one estimate the size of the endotracheal tube required for resuscitation?	422
28. What is the ``7-8-9´´ rule?	422
29. When should epinephrine be given during resuscitation in the delivery room?	422
30. Should sodium bicarbonate be given during resuscitation in the delivery room?	422
31. When should a laryngeal mask airway (LMA) be used in neonatal resuscitation?	423
32. What is the role for CO2 detectors in neonatal resuscitation?	423
33. What techniques are available to keep preterm infants warm in the delivery room?	423
34. What amount of oxygen is recommended for neonatal resuscitation?	424
35. After a ``traumatic´´ delivery, what are the possible injured systems?	424
36. What bone is the most frequently fractured in the newborn?	424
37. Who was Virginia Apgar, and how does one remember her score?	425
38. Is a low Apgar score alone sufficient to diagnose a neonate as asphyxiated?	425
39. When should neonatal resuscitation be stopped?	425
Fetal issues	425
40. What is the most accurate method of pregnancy dating?	425
41. What features constitute the biophysical profile?	426
42. What factors influence biophysical profile performance?	426
43. What are the increased risks of twin pregnancies?	426
44. Why are monozygotic twins considered higher risk than dizygotic twins?	426
45. What are the known benefits of antenatal corticosteroids and when are they indicated?	426
46. What are the potential fetal effects associated with diabetes during pregnancy?	426
47. How is hypertension in pregnancy classified?	427
48. What are the clinical consequences for the fetus from maternal preeclampsia?	427
49. How does postmaturity differ from dysmaturity?	427
50. What is the first bone in the human fetus to ossify?	427
51. What external characteristics are useful for estimating gestational age?	427
52. At what gestational age does pupillary reaction to light develop?	428
53. At what gestational age does a sense of smell develop?	428
54. When does the fetal heart begin to contract in utero?	428
55. How does fetal circulation differ from neonatal circulation?	428
56. What is the normal rate of head growth in the preterm infant?	428
57. What morbidities (short-term and long-term) are known to occur more frequently in growth-restricted babies?	429
58. When do premature infants ``catch up´´ on growth charts?	429
Gastrointestinal issues	429
59. When does the newborn infant´s stomach begin to secrete acid?	429
60. When is meconium usually passed after birth?	429
61. How is gastroschisis differentiated from omphalocele in the newborn infant?	429
62. Which conditions are associated with intra-abdominal calcifications?	430
63. What is necrotizing enterocolitis (NEC)?	430
64. Is pneumatosis intestinalis pathognomonic for NEC?	430
65. What are the most important risk factors for NEC in preterm infants?	431
66. Is it safe to feed infants with umbilical catheters?	431
67. How long should infants with NEC receive nothing by mouth?	431
68. Does the feeding of immunoglobulin to infants as prophylaxis prevent NEC?	431
69. Is there a role for probiotics in the prevention of NEC?	431
70. Are trophic feedings beneficial to preterm infants?	431
Hematologic issues	432
71. When does the switch from fetal to adult hemoglobin synthesis occur in the neonate?	432
72. Does the definition of anemia vary by gestational age?	432
73. How does the hemoglobin concentration change during the first few days of life?	432
74. When and at what dose should iron supplementation be initiated and for how long should it be maintained?	432
75. Should erythropoietin be used in preterm infants?	432
76. How can Rh disease be prevented?	432
77. Why is the direct Coombs test frequently negative or weakly positive in infants with ABO incompatibility?	432
78. If fetomaternal hemorrhage is suspected as a cause of neonatal anemia, how is this diagnosed?	433
79. If a gastric aspirate contains blood shortly after birth, what test can determine whether the blood is swallowed mate...	433
80. How is polycythemia defined?	433
81. What are the clinical manifestations of polycythemia?	433
82. Which infants with polycythemia should be treated?	433
83. What is the definition of thrombocytopenia in the neonate?	433
84. At what platelet count should platelet transfusion be considered?	433
85. What features on physical examination suggest a specific cause of thrombocytopenia?	434
86. What is the most common cause of severe thrombocytopenia in the first day of life?	434
87. What treatment is available during subsequent pregnancies for mothers who have had a child affected with NAIT?	434
88. When do the prothrombin time and partial thromboplastin time ``normalize´´ to adult values?	434
89. How is disseminated intravascular coagulation (DIC) diagnosed in the neonate?	435
90. How should newborn infants with DIC be managed?	435
91. What causes hemorrhagic disease of the newborn?	435
92. What are the risks and benefits of restrictive versus liberal transfusion criteria in preterm infants?	435
Hyperbilirubinemia	435
93. What are the normal changes in bilirubin levels in full-term healthy newborns?	435
94. How should infants be assessed for jaundice before discharge?	435
95. How soon should infants be evaluated after discharge?	436
96. What is believed to be the fraction of bilirubin that is toxic to the CNS?	436
97. Which infants are ``set-ups´´ for ABO incompatibility?	436
98. What screening tests should pregnant women have to identify infants at risk for hyperbilirubinemia?	436
99. What are the clinical features of bilirubin toxicity?	437
100. When should phototherapy be instituted in infants who are at least 35 weeks of gestational age?	437
101. What distinguishes breastfeeding jaundice from breast milk jaundice?	437
102. Why should infants at risk for breastfeeding jaundice be fed more frequently?	438
103. Where does bilirubin go when you turn on the lights?	438
104. What are the factors that affect the efficacy of phototherapy?	438
105. What are the contraindications to phototherapy?	438
106. What are the common adverse effects of phototherapy?	438
107. A newborn develops dark skin discoloration and dark urine after beginning phototherapy. What is the diagnosis?	438
108. What are the complications of exchange transfusions in the newborn?	439
109. What is the relationship between neonatal hyperbilirubinemia and urinary tract infection (UTI)?	439
110. Can transcutaneous bilirubin measurements be used in place of serum levels?	439
111. What is the role of metalloporphyrins in the treatment of hyperbilirubinemia?	439
112. What is the role of IVIG use in the treatment of hyperbilirubinemia?	439
Infectious disease issues	440
113. What is the best method of umbilical cord care during the immediate neonatal period?	440
114. Can sepsis be distinguished from other causes of respiratory distress in the neonate?	440
115. What is the single most important factor in determining whether an infant should be evaluated and treated for possib...	440
116. Should a lumbar puncture (LP) be performed on all newborns as part of the sepsis evaluation?	440
117. List the contraindications to the performance of an LP.	440
118. What are normal CSF values for healthy neonates?	441
119. What is the preferred strategy for identifying women for intrapartum GBS prophylaxis?	441
120. Which women should receive intrapartum antibiotic prophylaxis?	441
121. What is the typical clinical presentation of early-onset GBS disease?	441
122. Do intrapartum antibiotics change the clinical presentation of early-onset GBS sepsis?	442
123. What are the most common pathogens that are responsible for late-onset sepsis in the newborn infant?	442
124. What are the major risk factors for nosocomial sepsis?	442
125. Is methicillin-resistant S. aureus (MRSA) a significant pathogen in the NICU?	442
126. How is systemic candidiasis diagnosed in the neonate?	442
127. Should preterm infants receive prophylaxis for the prevention of Candida infections?	442
128. What is the risk for prenatal viral transmission in infants born to mothers with hepatitis B?	443
129. Should preterm infants receive hepatitis B vaccine during their newborn stay in the hospital?	443
130. What prophylactic medications should be given to infants born to HIV-positive mothers?	443
131. When is treatment for congenital cytomegalovirus (CMV) infection indicated?	443
Metabolic issues	443
132. How frequently are the various metabolic disorders detected by newborn screening?	443
133. In what settings should inborn errors of metabolism be suspected?	444
134. What key urine odors are associated with inborn errors of metabolism?	444
135. One of the key presenting features of inborn errors of metabolism is the presence or absence of metabolic acidosis. ...	444
136. What is the definition and management of neonatal hypoglycemia?	445
137. When is hypoglycemia most likely to occur in a neonate?	445
138. What is the prognosis of infants with hypoglycemia?	445
139. What features on physical examination suggest the etiology of hypoglycemia?	446
140. What is the differential diagnosis for an infant with hypoglycemia?	446
141. Should insulin be used to treat preterm infants with hyperglycemia?	446
142. What are the manifestations of hypocalcemia in the neonate?	446
143. Which neonates are at highest risk to develop hypocalcemia?	446
144. When should hypocalcemia be treated in the neonate?	446
145. How should symptomatic hypocalcemia be treated?	447
146. What are the causes of neonatal hypomagnesemia?	447
147. In which neonates should the serum magnesium concentration be measured?	447
148. How is hypomagnesemia treated?	447
Neurologic issues	447
149. After a difficult delivery, what three major forms of extracranial hemorrhage can occur?	447
150. If a cephalhematoma is suspected, should a skull radiograph be performed to evaluate for fracture?	448
151. When screening for IVH, when is the best time to perform an ultrasound?	448
152. What is the standard grading system for IVH?	448
153. What is the cause of hydrocephalus after an intracranial hemorrhage?	448
154. What factors predispose premature infants to the development of periventricular leukomalacia?	448
155. What modalities should be used to detect periventricular leukomalacia?	449
156. What is the most common brachial plexus palsy?	449
157. What is Klumpke paralysis?	449
158. In newborns with facial paralysis, how is peripheral nerve involvement distinguished from central nerve involvement?	450
159. Is ankle clonus normal in the newborn infant?	450
160. What is therapeutic hypothermia?	450
161. Which infants should be treated with therapeutic hypothermia?	450
162. How is neonatal encephalopathy evaluated and classified?	450
163. What is the most common cause of a neonatal seizure?	450
Nutrition	451
164. How many calories are required daily for growth in a healthy, growing preterm infant?	451
165. How should enteral feedings be started in the preterm infant?	451
166. What are the medical benefits of breastfeeding?	451
167. How does the composition of maternal breast milk differ for a full-term versus a premature baby?	452
168. How does colostrum differ from mature human breast milk?	452
169. How do fore milk and hind milk differ?	452
170. What are contraindications to breastfeeding?	452
171. What advice should be given to a mother who plans to express and save breast milk for later feedings?	452
172. What are the advantages of a 60/40 whey-to-casein ratio in infant formulas?	453
173. ``Low-iron´´ or ``regular iron-fortified´´ formulas: which are preferred for infants?	453
174. Is vitamin supplementation necessary for exclusively breast-fed term infants?	453
175. What is nonnutritive sucking?	453
176. Which fatty acids are essential for the neonate?	453
177. What are the proven advantages of supplementing formulas with long-chain polyunsaturated fatty acids?	453
178. What are the manifestations of essential fatty acid deficiency?	454
179. What are the manifestations of vitamin E deficiency in the neonate?	454
Respiratory issues	454
180. What causes infants to grunt?	454
181. What do hyperpnea and tachypnea signify in the neonate?	454
182. Until what age are infants obligate nose breathers?	454
183. What conventional mechanical ventilator settings are likely to affect Po2 and Pco2?	454
184. What factors determine the mean airway pressure on a conventional ventilator?	454
185. What are the physiologic effects of PEEP?	454
186. What are the effects of severe hypercarbia (Pco2100mm Hg) if there is no associated hypoxia?	454
187. What are the most common causes of respiratory distress in term or late preterm infants?	455
188. What are the risks and benefits of oxygen therapy in preterm infants?	455
189. What is respiratory distress syndrome (RDS)?	455
190. What is the composition and function of surfactant?	456
191. When is treatment with exogenous surfactant indicated in newborn infants?	456
192. What is persistent pulmonary hypertension of the newborn (PPHN)?	456
193. Is there a role for sildenafil (Viagra) in the treatment of PPHN?	456
194. What is the pathophysiology and typical course of transient tachypnea of the newborn (TTN)?	456
195. Why are fetuses with anhydramnios or severe oligohydramnios at risk for respiratory problems after birth?	457
196. Has nasal prong CPAP been proven to decrease the risk for BPD?	457
197. What is the mechanism of action of inhaled nitric oxide (iNO) in the management of pulmonary hypertension?	457
198. Which infants benefit most from extracorporeal membrane oxygenation (ECMO)?	457
199. What is the most common type of congenital diaphragmatic hernia (CDH)?	457
200. What is bronchopulmonary dysplasia (BPD)?	458
201. In the current era of neonatal medicine, how is BPD or CLD (chronic lung disease) diagnosed?	458
202. What are the respiratory benefits of caffeine therapy in preterm infants?	459
Chapter 12: Nephrology	460
Acid-base, fluids, and electrolytes	460
1. How is the cause of hyponatremia established?	460
2. What is the emergency treatment of symptomatic hyponatremia?	460
3. How is the cause of hypernatremia established?	461
4. Why can correcting hypernatremia too rapidly cause seizures?	461
5. What is the differential diagnosis of nephrogenic diabetes insipidus (NDI)?	461
6. An infant boy presents with severe dehydration, polyuria, and hypernatremia. What is the first renal diagnosis that sh...	462
7. What are the clinical and physiologic consequences of progressive hypokalemia (low potassium)?	462
8. What are the causes of hypokalemia?	462
9. Which foods are high in potassium?	462
10. List the causes of hyperkalemia in children.	462
11. When are calcium infusions indicated in a patient with elevated serum potassium?	463
12. What are key aspects in the treatment of hyperkalemia?	463
13. What are the causes of periodic paralysis syndromes involving both high and low potassium levels?	463
14. What is the undetermined serum anion gap?	463
15. How is the serum anion gap helpful in the evaluation of a metabolic acidosis?	464
16. What are the causes of an elevated serum anion gap acidosis?	464
17. How limited is the respiratory response to metabolic alkalosis?	464
18. What is the differential diagnosis in a child presenting with symptoms of primary metabolic alkalosis?	464
19. Why is the urine pH often acidic (pH5.0 to 5.5) in a child with metabolic alkalosis from severe vomiting?	464
Acute kidney injury	465
20. Why has the term acute kidney injury replaced acute renal failure?	465
21. What clinical and laboratory observations are useful for distinguishing prerenal oliguria (decreased effective circul...	465
22. What is the most common cause of AKI in young children in the United States?	465
23. What is the triad of clinical findings of HUS?	466
24. What are the causes of HUS?	466
25. Does the use of antibiotic therapy in children with diarrhea caused by E. coli OH157:H7 prevent HUS?	467
26. Can anything be done to lessen the severity of the renal disease in children with HUS caused by E. coli OH157:H7?	467
27. Why is Shiga toxin-associated HUS so terrifying for patients, family, and physicians?	467
28. What is meant by atypical hemolytic uremic syndrome (aHUS)?	467
29. What are indications for dialysis in AKI?	468
Chronic kidney disease	468
30. Based on GFR estimations and serum creatinine levels, how are AKI and chronic kidney disease (CKD) defined?	468
31. What are the stages of CKD?	468
32. What are the main causes of CKD in children that result in renal transplantation?	468
33. Your nephrology attending likes to use the Socratic method of teaching. ``What are four major endocrine and cardiac m...	469
34. What is the new term for renal osteodystrophy?	469
35. What hormonal elevation is key in the pathogenesis of CKD-MBD?	469
36. Why is it important to recognize CKD-MBD at an early stage?	469
37. Why is FGF23 an important evolving concept in CKD-MBD?	469
38. What are the ciliopathies?	469
39. Nephronophthisis is difficult to pronounce and spell, but what is it?	470
40. Which is more common in children: autosomal dominant polycystic kidney disease (ADPKD) or autosomal recessive polycys...	470
41. What anatomic features characterize ADPKD?	470
42. How is ADPKD diagnosed and managed?	470
43. Why was the term infantile polycystic kidney disease replaced with ARPKD?	470
Enuresis/dysfunctional voiding	471
44. How common is nocturnal enuresis in older children?	471
45. Why does nighttime bed-wetting persist in some children?	471
46. What treatments are available for nocturnal enuresis?	471
47. A 7-year-old presents with problems of intermittent daytime urinary incontinence with a normal urinalysis and a negat...	471
48. What is the term for extraordinary daytime urinary frequency?	472
49. Why is giggle incontinence not a laughing matter?	472
50. What is the normal bladder capacity in children?	472
Glomerular diseases	472
51. What constellation of findings defines nephrotic syndrome?	472
52. What differentiates nephrotic syndrome from nephritis?	472
53. A 12-year-old girl complains of a sore throat and passes painless coke-colored urine for 2 days. When you see her a f...	473
54. What is the long-term prognosis for patients with IgAN?	473
55. During the evaluation of a patient with hematuria, what features suggest acute glomerulonephritis, chronic glomerulon...	473
56. If glomerulonephritis is suspected, what laboratory tests should be considered?	474
57. Which glomerulonephritides have a genetic basis?	474
58. Which types of glomerulonephritis are associated with hypocomplementemia?	474
59. Does the treatment of streptococcal skin or pharyngeal infections prevent ASPGN?	474
60. What is the usual time course for ASPGN?	474
61. What percentage of children with ASPGN have elevated levels of serum anti-streptolysin O titers?	475
62. If pharyngitis and the brown urine occur on the same day or within 1 or 2 days, does this make ASPGN less likely?	475
63. A 7-year-old girl has a typical presentation of poststreptococcal glomerulonephritis with positive ASO titers and low...	475
64. You are on rounds and a know-it-all nephrology fellow asks you ``what are the renal complications of HIV infection?´´	475
Hematuria	476
65. How common is hematuria in children?	476
66. What is the most identifiable cause of microscopic hematuria?	476
67. What distinguishes lower from upper tract bleeding?	476
68. Why does recurrent macroscopic hematuria ``gross´´ us out?	477
69. If a healthy 10-year-old boy has bright red blood at the end of a previously clear urine stream, what is the likely d...	477
70. A concerned mother of infant twins brings in a diaper from each with one having pink urine stains and the other blue ...	477
71. What evaluations should be considered during the evaluation of isolated hematuria?	477
72. What condition should be suspected in a 9-year-old with a history of hearing loss and visual deficits who presents wi...	478
Hypertension	478
73. How is hypertension defined in children?	478
74. Why are repeat visits necessary to diagnose hypertension?	479
75. How do you determine the optimum cuff size for obtaining a blood pressure?	479
76. Which Korotkoff sound best represents diastolic blood pressure?	479
77. What is ambulatory blood pressure monitoring (ABPM)?	479
78. What are the advantages and limitations of ABPM?	479
79. In what settings is ABPM particularly useful?	479
80. When should hypertension be treated in the neonate?	480
81. What are the indications for the pharmacologic treatment of hypertension in children and in adolescents?	480
82. During the evaluation of a child with elevated blood pressure, what risk factors should be considered for identificat...	480
83. What historical information suggests a secondary cause of hypertension?	481
84. What is the most common cause of renal artery stenosis in children in the United States?	481
85. List the features on physical examination that suggest a secondary cause of hypertension	481
86. What are the categories of antihypertensive medications used for outpatient management of hypertensive children?	482
87. A 12-year-old girl is referred for evaluation of severe hypertension. She has hypernatremia, hypokalemia, and metabol...	482
88. Why should patients with hypertension and/or those using diuretics avoid true licorice?	483
Proteinuria/nephrotic syndrome	483
89. How does the dipstick test for urine protein compare with the sulfosalicylic method?	483
90. What is microalbuminuria?	483
91. In what clinical setting is microalbuminuria a particularly important finding?	483
92. What is the best spot method for determining the amount of proteinuria?	483
93. An asymptomatic 11-year-old boy is found to have 2+ protein on dipstick testing during a routine checkup. How should ...	484
94. How is the diagnosis of orthostatic proteinuria established?	484
95. What additional evaluation should be done for a patient with persistent proteinuria?	484
96. What is the natural history of orthostatic proteinuria?	484
97. What level constitutes significant proteinuria?	484
98. In a child with hematuria, can proteinuria be attributed to the protein that is contained in whole blood?	484
99. Can proteinuria be caused by leukocytes or mucus in the urine?	484
100. At what serum albumin concentration does edema develop?	484
101. What is the most common form of nephrotic syndrome in childhood?	485
102. What is the most important historical factor to consider when assessing a patient for possible MCNS?	485
103. What are the typical clinical features and therapeutic responses seen in patients with MCNS?	485
104. What are the indications for furosemide and albumin infusions in patients with nephrotic syndrome?	485
105. Name two important complications of MCNS	485
106. What are the prognostic factors in MCNS?	485
107. A 5-year-old child presents with puffy eyes and the laboratory features of the nephrotic syndrome, but fails to resp...	485
Renal function assessment and urinalysis	486
108. What is the simplest way to estimate the glomerular filtration rate (eGFR) in the absence of a timed urine collection?	486
109. How can you be confident that a 24-hour urine collection (for any determination) is complete?	486
110. When should routine urinalyses (UA) be performed in the pediatric age group?	486
111. What are the maximal and minimal renal dilutional and concentrating capabilities?	487
112. What is the difference between urine specific gravity (SG) and urine osmolality?	487
113. Which urinary crystals are always pathologic?	487
Surgical issues	488
114. What is the most common cause of urinary tract obstruction in the newborn?	488
115. What is the most common renal abnormality detected on antenatal ultrasound?	488
116. What are the possible causes of prenatal hydronephrosis?	488
117. What is the most common cause of kidney disease of children worldwide?	488
118. Is unilateral renal agenesis (URA) a benign condition?	488
119. Should a child with a single kidney be allowed to play football?	488
Tubular disorders	489
120. In what settings should renal tubular acidosis (RTA) be considered?	489
121. What are defects in each type of primary RTA?	489
122. What are clinical and laboratory features of the primary RTAs?	489
123. How is determining the urine anion gap helpful in the evaluation of metabolic acidosis?	489
124. How is RTA diagnosed with utilizing a urine anion gap in a patient with a hyperchloremic metabolic acidosis and a no...	490
125. What is the recommended alkali therapy for the treatment of various forms of RTA?	490
126. What is most common cause of the renal Fanconi syndrome?	490
127. A 2-year-old girl, generally healthy with height just below the 5th percentile, was noted to have blinking problems ...	490
128. Glucosuria is detected on repeated urine dipstick testing in a 5-year-old boy, but the blood glucose is always norma...	491
129. What is the clinical presentation of acute interstitial nephritis (AIN)?	491
130. What medications are causes of AIN?	491
131. What laboratory abnormalities are seen in patients with AIN?	491
Urinary Tract infections	491
132. What two features on the dipstick test are used to evaluate possible UTIs?	491
133. How helpful are dipstick testing and microscopic analysis of urine as screening tests for UTIs?	491
134. Can the diagnosis of UTI be made on the basis of urinalysis alone?	492
135. What bacterial counts constitute a positive urine culture?	492
136. A child has painful, frequent urination and a culture revealed a UTI, but the original urinalysis had a negative nit...	492
137. What factors can cause a low colony count despite a definite urinary infection?	492
138. Why should urine specimens be refrigerated if they cannot be immediately processed?	493
139. What are the common presenting signs and symptoms of a UTI in an infant?	493
140. How common are UTIs in young febrile infants?	493
141. What pathogens are associated with UTIs in children?	493
142. How is cystitis distinguished clinically from pyelonephritis?	493
143. What is the diagnostic approach for a possible UTI for a female infant 3 to 24 months of age with no known urinary t...	493
144. Which patients with UTIs require hospitalization and parenteral antibiotics?	494
145. Should all pediatric patients with clinical pyelonephritis be hospitalized?	495
146. What is the expected resolution of fever after a child is started on an antibiotic for a UTI?	495
147. What is the duration of antibiotic therapy for a UTI?	495
148. Are repeat cultures required at the end of therapy for a patient without symptoms?	495
149. In what patients are prophylactic antibiotics indicated for UTIs?	495
150. Is cranberry juice helpful in the management of recurrent UTIs in children?	496
151. Do patients with an initial UTI require imaging studies?	496
152. What imaging studies can be used for patients with UTIs who warrant evaluation?	496
Urogenital issues	497
153. What are risks associated with circumcision?	497
154. Is circumcision now medically indicated?	497
155. What distinguishes phimosis and paraphimosis?	497
156. What is hypospadias?	497
157. How common are undescended testicles at birth?	497
158. When should undescended testicles be repaired?	497
159. How do you treat labial adhesions?	498
Urolithiasis	498
160. Why are kidney stones increasing in frequency in children in the United States?	498
161. What are the clinical findings in pediatric urolithiasis?	498
162. What is the composition of kidney stones in children?	498
163. What is the most common cause of pediatric urinary calculi?	498
164. How is hypercalciuria defined in pediatrics?	499
165. What are the appropriate laboratory studies for the initial evaluation of children with renal stones?	499
166. When is lithotripsy or surgery indicated for children with kidney stones?	499
Vesicoureteral reflux	499
167. How is vesicoureteral reflux (VUR) graded?	499
168. What is the natural history of VUR?	500
169. How is VUR managed: medically or surgically?	500
170. Are antibiotics effective to prevent recurrent UTIs in children with reflux?	500
171. Should asymptomatic siblings of a patient with VUR have urologic imaging done as a screen for reflux?	500
172. Why are some children with congestive heart failure (CHF) hyponatremic while others are not?	500.e1
173. What treatment is available for NDI?	500.e1
174. Which children with acute kidney injury are at the greatest risk for hyperkalemia?	500.e1
175. In a child with HUS, does the presence of a low C3 level have any special significance?	500.e1
176. What is urotherapy?	500.e1
177. Why is constipation associated with daytime urinary symptoms?	500.e1
178. What is vaginal reflux?	500.e2
179. The mother consults Dr. Google when the hematuria recurs and requests an ear, nose, and throat (ENT) consult because...	500.e2
180. A 15-year-old black girl with sickle cell trait has gross hematuria for the second time 1 day after playing in a lac...	500.e2
181. What are the most possible causes of hypertension by age group?	500.e2
182. Why doesnt eating more protein restore the serum albumin concentration to normal in individuals with the nephrotic s...	500.e3
183. For which patients with MCNS should additional therapies be considered?	500.e3
184. What are the alternative therapies for children with MCNS?	500.e3
185. What must patients, family members, and prescribers be aware of when offering cyclophosphamide or tacrolimus?	500.e3
186. What are the risks associated with the infusion of albumin with furosemide?	500.e3
187. What physical findings should prompt a search for an underlying renal abnormality?	500.e3
188. A young girl diagnosed with AIN has developed a painful red eye. Could there be a connection?	500.e3
189. Which patients with UTIs are at higher risk for having an abnormality?	500.e4
190. How does altering the urine pH affect renal calculi?	500.e4
191. In addition to reflux, what other pathologic bladder findings may be noted on a VCUG?	500.e4
192. What are the pros and cons of a VCUG versus a radionuclide cystogram (RNC) for the evaluation of reflux?	500.e4
Chapter 13: Neurology	501
Antiepileptic drugs	501
1. Should treatment with antiepileptic drugs (AEDs) be started after the first afebrile seizure in a child?	501
2. What is the advantage of monotherapy chosen according to the epilepsy syndrome?	501
3. Which AEDs are recommended for primary generalized tonic-clonic seizures in children over 1 month of age?	501
4. What is the drug of choice for absence epilepsy?	502
5. Can AEDs paradoxically cause a worsening of seizures?	502
6. When should blood levels be obtained if seizures are poorly controlled, compliance is questionable, or drug toxicity s...	502
7. What are the suggested therapeutic ranges of AEDs?	502
8. What are the typical dose-related side effects of AEDs?	503
9. What idiosyncratic drug reactions are associated with antiepileptic medications?	503
10. Which children are most susceptible to valproic acid-induced acute hepatic failure?	504
11. What are the warning signs and symptoms of hypersensitivity syndromes to AEDs?	504
12. What is Diastat?	504
13. Are there other options for outpatient treatment of severe recurrent and prolonged seizures in children?	504
14. After what period can AEDs be safely discontinued?	505
15. When the decision is made to discontinue AEDs, should the tapering period be long or short?	505
Cerebral palsy	505
16. What is cerebral palsy?	505
17. What are the most common brain lesions seen on magnetic resonance imaging (MRI) in children with cerebral palsy?	505
18. What are the Levine (POSTER) criteria for the diagnosis of CP?	505
19. What are the types of cerebral palsy?	506
20. What proportion of CP is related to birth asphyxia?	506
21. How well do Apgar scores correlate with the development of CP?	506
22. Why is CP difficult to diagnose clinically during the first year of life?	507
23. What behavioral symptoms during the first year should arouse suspicion about the possibility of CP?	507
24. What gross motor delays are diagnostically important in the infant with possible CP?	507
25. What problems are commonly associated with CP?	508
26. What features in an infant suggest a progressive central nervous system (CNS) disorder rather than CP as the cause of...	508
27. What therapies are used to treat the spasticity and dystonia of cerebral palsy?	508
Cerebrospinal fluid	508
28. What is normal cerebrospinal fluid (CSF) pressure?	508
29. What are the normal CSF volumes in an infant, child, and adolescent?	509
30. What are the common causes of an elevated CSF protein?	509
31. What CSF findings suggest metabolic disease as a cause of neurologic signs and symptoms?	509
32. Why is a stylet used during a lumbar puncture?	510
33. As tests of meningeal irritation, what constitutes a positive Kernig or Brudzinski sign?	510
34. How do the manifestations of increased intracranial pressure (ICP) differ in an infant compared with an older child?	510
35. What comprises the Cushing triad?	510
36. How is hydrocephalus classified?	511
37. What is the normal growth rate of head circumference during the first year of life?	511
38. What are the complications of ventricular shunts?	511
39. What are the characteristic features of idiopathic intracranial hypertension?	511
40. What causes idiopathic intracranial hypertension?	511
41. What treatment is recommended for severe cases of idiopathic intracranial hypertension?	512
Clinical issues	512
42. What are the key questions in a neurologic evaluation?	512
43. What are general rules that govern localization of a potential neurologic problem?	512
44. What distinguishes the pediatric neurologic examination?	512
45. What are the advantages and disadvantages of various imaging procedures used in pediatric neurologic evaluation?	512
46. A child presents with progressive left leg weakness and diplopia, especially when looking toward the left. Where is t...	513
47. A dilated and unreactive pupil indicates the compression of what structure?	513
48. Pinpoint pupils and respiratory changes indicate the compression of what structure?	514
49. How does the presentation of stroke differ between infants and older children?	514
50. What is the differential diagnosis of stroke in children?	514
51. What is the derivation of ``moyamoya´´ in moyamoya disease?	514
52. A child who develops weakness, incontinence, and ataxia 10 days after a bout of influenza likely has what diagnosis?	515
53. In patients with acute injury to the brain, what two types of edema may occur?	515
54. What are the treatments for increased ICP?	515
55. How is brain death defined?	515
56. How is the diagnosis of brain death in children made?	516
57. Compare the persistent vegetative state with the minimally conscious state.	516
58. What is the differential diagnosis of an intracranial bruit?	516
59. In a previously normal child who develops acute ataxia, what are the two most common diagnoses?	516
60. What are the causes of toe walking?	517
61. What is the significance of a positive Babinski response?	517
62. A 7-year-old child with progressive ataxia, kyphoscoliosis, nystagmus, pes cavus (high arch), and an abnormal electro...	517
63. What clinical features help distinguish peripheral from central vertigo?	517
64. In what settings is hyperacusis noted?	517
65. What is the most common cause of neonatal asymmetric crying facies?	518
66. What are the common causes of peripheral seventh-nerve palsy?	518
67. How is peripheral seventh-nerve palsy distinguished from central seventh-nerve palsy?	519
68. During recovery from Bell palsy, why do the eyes water at mealtime?	519
69. When are ``dolls eyes´´ movements considered normal or abnormal?	519
70. How are cold calorics done?	519
71. What causes pinpoint pupils?	519
72. What is the differential diagnosis of ptosis?	519
73. What does the Marcus Gunn pupil detect?	520
74. How is the swinging flashlight test done to detect a Marcus Gunn pupil?	520
Epilepsy	520
75. What is epilepsy?	520
76. What is the long-term outcome for children with epilepsy?	520
77. How often are EEGs abnormal in healthy children?	520
78. Should an EEG be done on all children who have a first afebrile seizure?	521
79. In a patient with a suspected seizure disorder, but a normal EEG, how can the sensitivity of the EEG be increased?	521
80. Which types of epilepsy are characterized by specific EEG findings?	521
81. Should all children with a new-onset afebrile, unprovoked generalized seizure have a CT or MRI evaluation?	521
82. Which disorders commonly mimic epilepsy?	521
83. What are ways to distinguish psychogenic nonepileptic seizures (PNES) from epileptic seizures?	522
84. What are the categories of seizures in children?	522
85. What are structural and metabolic causes of seizures?	523
86. If a previously normal child has an afebrile, generalized tonic-clonic seizure, what should parents be told about the...	523
87. What are the most common inherited seizure or epilepsy syndromes?	524
88. What are the clinical features of rolandic epilepsy?	524
89. What distinguishes typical and atypical absence seizures?	524
90. In a child who is suspected of having absence seizures, how can a seizure be elicited during an examination?	524
91. What percentage of patients with absence seizures also have occasional grand mal seizures?	524
92. What is the prognosis for children with absence epilepsy?	524
93. A teenager, like his father, develops brief, bilateral, intermittent jerking of his arms. What seizure disorder is he...	525
94. What are myoclonic seizures?	525
95. What distinguishes atonic and akinetic seizures?	525
96. Which seizure types constitute the ``epileptic encephalopathies´´?	525
97. What is the classic triad of epileptic (infantile) spasms?	525
98. What characterizes hypsarrhythmia?	525
99. How commonly is a cause identified in epileptic spasms?	526
100. What is the prognosis for infants with epileptic spasms?	526
101. What is the treatment of choice for epileptic spasms?	526
102. What is the most likely diagnosis in a child of Ashkenazi descent with stimulus-sensitive seizures, cognitive deteri...	526
103. A patient with seizures, microcephaly, and a low CSF glucose but a normal serum glucose has what likely condition?	527
104. What is the clinical triad of the Lennox-Gastaut syndrome?	527
105. A 5-year-old with a history of normal language development who develops seizures and inattention to speech with seve...	527
106. How is status epilepticus defined?	528
107. Why is status epilepticus so dangerous?	528
108. What should be done in the first 10 minutes for a child who presents with an ongoing seizure?	528
109. What is the most common cause of refractory seizures?	528
110. What is the role of the ketogenic diet for the treatment of seizures?	528
111. What is the role of the vagus nerve stimulator in seizure control?	529
112. What should a teenager with epilepsy be told about the potential of obtaining a drivers license?	529
113. What are some common seizure triggers about which families should be counseled?	529
114. When should a child be referred for possible epilepsy surgery?	529
Febrile seizures	530
115. How are febrile seizures defined?	530
116. What is the likelihood of recurrence of a febrile seizure?	530
117. What features make a febrile seizure complex rather than simple?	530
118. Why are complex febrile seizures more worrisome than simple febrile seizures?	530
119. When should a lumbar puncture (LP) be performed as part of the evaluation of a child <12months of age with a simple ...	530
120. Are EEG or neuroimaging studies indicated for a child with a simple febrile seizure?	530
121. Do prolonged febrile seizures result in an increased peripheral white blood cell count?	531
122. What ancillary testing should be considered in a patient with a complex febrile seizure?	531
123. What is the risk for epilepsy after a simple febrile seizure?	531
124. What is the long-term outcome for children with febrile seizures?	531
125. After a febrile seizure, should a child be treated with prophylactic AEDs?	532
126. Is the aggressive use of antipyretic therapy at the start of a febrile illness effective in reducing the likelihood ...	532
Headache	532
127. What are the emergency priorities when evaluating a child with a severe headache?	532
128. When should neuroimaging be considered in a child with headache?	532
129. What are the three primary headache disorders in children?	533
130. What are the clinical features of migraine headaches in children?	533
131. Which physical findings are important during the initial evaluation of possible migraine headache?	533
132. When do children begin to have migraine headaches?	534
133. Which foods have been associated with the development of migraine headaches?	534
134. What is familial hemiplegic migraine?	534
135. What is the likely diagnosis for a 10-year-old girl with a history of headaches and a family history of migraines wh...	534
136. How do the triptans work to treat an acute migraine headache?	534
137. What nonpharmacologic therapies are available for the prevention of migraine?	534
138. What categories of medication are available for the prevention of migraine in children?	535
139. Who should be started on prophylactic medication for migraine headaches?	535
140. How long are the prophylactic medications continued?	535
141. What distinguishes tension-type headaches from migraines?	535
Movement disorders	535
142. What are the various types of pathologic hyperkinetic movements?	535
143. What techniques can be used to elicit abnormal movements (particularly chorea)?	536
144. What disorders are commonly associated with the various hyperkinetic movements?	536
145. What constitutes a tic?	536
146. What is the range of clinical tics?	536
147. What are the causes of a tic?	536
148. How should simple tics be treated?	536
149. What comorbidities occur in children with tics?	537
150. When do tics warrant pharmacologic intervention?	537
151. What are the diagnostic criteria for Tourette syndrome?	537
152. What is coprolalia?	537
153. What behavioral problems are associated with Tourette syndrome?	537
154. Why is the diagnosis of Tourette syndrome commonly delayed?	537
155. What is the cause of tardive dyskinesia?	538
156. For a patient taking neuroleptic medication, how long must therapy last before symptoms of tardive dyskinesia can de...	538
157. What is neuroleptic malignant syndrome?	538
158. Which movement disorder in children presents with ``dancing eyes and dancing feet´´?	538
Neonatal seizures	538
159. How are neonatal seizures classified?	538
160. Why are generalized seizures uncommon in newborns?	538
161. What is the most common type of clinical seizure during the neonatal period?	538
162. What are the causes of neonatal seizures?	538
163. In premature and full-term infants, how do the causes of seizures vary with regard to relative frequency and time of...	539
164. What is an acceptable workup in a newborn with seizures?	539
165. In what settings should an inborn error of metabolism be suspected as a cause of neonatal seizures?	539
166. How are seizures differentiated from tremors in the neonate?	540
167. What are the treatment options for neonatal seizures?	540
168. What is the treatment for refractory seizures in the neonate?	540
169. Of what prognostic value is the interictal EEG in a neonate with seizures?	540
170. After an infant has recovered from a seizure, how long should medication be continued?	541
171. In patients with neonatal seizures, how does the cause affect the prognosis?	541
Neurocutaneous syndromes	541
172. What are the three most common neurocutaneous syndromes?	541
173. What are the inheritance patterns of the various neurocutaneous syndromes?	541
174. What are the diagnostic criteria for neurofibromatosis-1 (NF1)?	542
175. How does NF1 differ from NF2?	542
176. How common are café-au-lait spots at birth?	542
177. If a 2-year-old child has seven café-au-lait spots that are larger than 5mm in diameter, what is the likelihood that...	542
178. What are Lisch nodules?	542
179. How common is a positive family history in cases of NF1?	543
180. What are the primary diagnostic criteria for tuberous sclerosis complex (TSC)?	543
181. What is the classic triad of TSC?	543
182. What is the most common presenting symptom of TSC?	544
183. What are skin findings in patients with tuberous sclerosis?	544
184. Why is the term adenoma sebaceum a misnomer when used to describe patients with tuberous sclerosis?	544
185. What is the ``tuber´´ of tuberous sclerosis?	545
186. What is the tissue type of a shagreen patch?	545
187. Which types of facial port-wine stains are most strongly associated with ophthalmic or CNS complications?	545
188. What are the three stages of incontinentia pigmenti?	545
189. What is the likely diagnosis for a 7-year-old who is noted to have recurrent nosebleeds, cutaneous telangiectasias o...	545
Neuromuscular disorders	546
190. How can the anatomic site responsible for muscle weakness be determined clinically?	546
191. What are the causes of acute generalized weakness?	546
192. If a child presents with weakness, what aspects of the history and physical examination suggest a myopathic process?	547
193. What is the significance of a Gowers sign?	547
194. How does electromyography help differentiate between myopathic and neurogenic disorders?	547
195. How is pseudoparalysis distinguished from true neuromuscular disease?	548
196. Why is it important to localize the cause of hypotonia?	548
197. How can you detect myotonia clinically?	548
198. How do the presentations of the two forms of myotonic dystrophy differ?	548
199. In a newborn with weakness and hypotonia, what obstetric and delivery features suggest a diagnosis of congenital myo...	548
200. How is myotonic dystrophy an example of the phenomenon of ``anticipation´´?	548
201. How does the pathophysiology of infant botulism differ from that of food-borne and wound botulism?	549
202. What is the earliest indication for intubation in an infant with botulism?	549
203. In an infant with severe weakness and suspected botulism, why is the use of aminoglycosides relatively contraindicated?	549
204. What are the two most common symptoms in children with juvenile myasthenia gravis?	549
205. What are the risks to a neonate who is born to a mother with myasthenia gravis?	549
206. How does the pathophysiology of juvenile versus congenital myasthenia gravis differ?	549
207. How is the edrophonium (Tensilon) test done?	550
208. Does a negative antibody test exclude the diagnosis of juvenile myasthenia gravis?	550
209. What are the four characteristic features of damage to the anterior horn cells?	550
210. What processes can damage the anterior horn cells?	550
211. What is the primary genetic abnormality in infants and children with spinal muscular atrophy (SMA)?	550
212. How are the inherited progressive spinal muscular atrophies distinguished?	550
213. What are muscular dystrophies?	550
214. What is the clinical importance of dystrophin?	550
215. How are Duchenne and Becker muscular dystrophies distinguished?	550
216. What causes the calf hypertrophy seen in Duchenne muscular dystrophy?	552
217. Is corticosteroid therapy effective for the treatment of Duchenne muscular dystrophy?	552
218. What is the most likely diagnosis in a child with progressive walking difficulties evolving over several days?	552
219. What are subtypes of GBS?	553
220. What CSF findings are characteristic of GBS?	553
221. Outline the management of acute GBS.	553
222. What is the prognosis for children with GBS?	553
223. Does multiple sclerosis (MS) present during childhood?	554
Spinal cord disorders	554
224. Which sacral dimples and coccygeal pits in a newborn are concerning for an occult spinal dysraphism (OSD)?	554
225. What are the two main features of the Chiari malformations?	554
226. What are the types of Chiari malformations?	554
227. What are types of spina bifida?	555
228. How common are asymptomatic spinal anomalies in normal children?	556
229. What is the full anatomic expression of myelomeningocele?	556
230. What is the likelihood that a patient with myelomeningocele will have hydrocephalus?	556
231. What is the usual cause of stridor in a child with myelomeningocele?	556
232. What are the principal options for managing urinary incontinence in patients with myelomeningocele?	557
233. How frequently is myelomeningocele associated with intellectual disability?	557
234. In an infant born with myelomeningocele, how does the initial evaluation predict long-term ambulation potential?	557
Chapter 14: Oncology	558
Chemotherapy and radiation therapy	558
1. What was the first cytotoxic chemotherapeutic agent used for the treatment of children with leukemia?	558
2. Name the common cytotoxic chemotherapeutic drug classes.	558
3. We can thank the guinea pig for a major (albeit serendipitous) breakthrough in the treatment of childhood acute lympho...	558
4. Which chemotherapeutic agents are cell cycle dependent? In which phase are they most active?	558
5. What is the difference between adjuvant and neoadjuvant chemotherapy?	559
6. Why are most chemotherapeutic drug dosages based on body surface area (BSA)?	559
7. What is the difference between pharmacokinetics and pharmacodynamics?	559
8. What are the phases of clinical trials?	559
9. What is the major dose-limiting toxicity for the alkylating agents?	559
10. If one had to choose a single laboratory test to obtain before administering high-dose methotrexate, which one should...	559
11. What factors are associated with an increased risk for developing anthracycline-induced cardiotoxicity?	560
12. How did a periwinkle plant contribute to some longstanding chemotherapeutic agents?	560
13. What is the mechanism of action of vincristine?	560
14. What agent can limit the complication of hemorrhagic cystitis that occurs in some chemotherapeutic regimens?	560
15. What is a vesicant?	560
16. Which classes of chemotherapeutic agents have most commonly been implicated in causing secondary leukemias?	561
17. Why is intrathecal chemotherapy dose based on patient age, whereas systemic (oral, intravenous) dosing is based on we...	561
18. What antiemetic agents are most effective in the management of chemotherapy-induced nausea and vomiting?	561
19. Who develops the ``somnolence syndrome´´?	561
20. What is radiation recall?	561
21. What is a ``fraction´´ of radiation?	561
22. A 10-year-old girl is being treated for AML with a combination of high-dose cytarabine and daunorubicin. Five days af...	561
23. What are the long-term sequelae of chemotherapy?	562
24. Radiation therapy: what are its long-term effects?	562
Clinical issues	562
25. A patient has a central venous catheter and develops a fever. What should be done?	562
26. Describe three different types of infection that are associated with central venous catheters, and how the treatment ...	562
27. A patient undergoing chemotherapy is neutropenic and has a fever. What should be done?	562
28. A patient remains febrile and neutropenic despite appropriate antibiotics for several days. Is there cause for concern?	563
29. How should a patient who has oral candidiasis or esophageal candidiasis be treated?	563
30. After receiving broad-spectrum antibiotic therapy for 4 days for fever and neutropenia, a patient develops a new feve...	563
31. A 10-year-old in her second year of treatment for ALL has had all medications voluntarily stopped by her parents for ...	563
32. What paraneoplastic syndromes can occur in childhood?	563
33. What are the metabolic abnormalities in the tumor lysis syndrome?	564
34. In what settings is tumor lysis syndrome more likely to occur?	564
35. What are the current recommendations regarding management of tumor lysis syndrome?	564
36. What two pharmacologic agents can be used to prevent or treat hyperuricemia caused by tumor lysis syndrome?	564
37. A child with newly diagnosed leukemia experiences a rapid decline in hemoglobin soon after administration of rasburic...	564
38. A child undergoing induction chemotherapy for leukemia develops right lower quadrant pain and tenderness. What diagno...	564
39. What is the difference between a Broviac and a Port-A-Cath?	564
40. What is the differential diagnosis of an anterior mediastinal mass?	565
41. What is superior mediastinal syndrome? How is it managed?	565
42. Which tumor is the most common cause of superior mediastinal syndrome in children?	565
43. Why is a generous mediastinal shadow on a radiograph much more worrisome in a teenager than in an infant?	565
44. Which neoplasms are associated with hemihyperplasia?	565
45. Which cancers are often associated with splenomegaly?	566
46. What are the predictors of malignancy in the pediatric patient with peripheral lymphadenopathy?	566
47. What is the function of the Langerhans cells?	566
48. What are the features of Langerhans cell histiocytosis (LCH)?	566
49. What is an eosinophilic granuloma?	566
50. What are the common indications for transfusion support for children with cancer?	566
51. What are the most common symptoms experienced by oncology patients receiving end-of-life care?	566
Epidemiology	567
52. Although in psychic lore a ``seer´´ can look into the future, SEER has a different connotation for cancer researchers...	567
53. How do the types of cancers differ between adults and children?	567
54. What is the most frequently occurring childhood cancer?	567
55. How do the types and frequency of childhood cancers vary by age?	567
56. Where does cancer rank as a cause of death in younger children?	567
57. Is cancer the leading cause of death in teenagers and young adults?	567
58. What are the relative risks for children to develop leukemia?	568
59. Does cell phone usage increase the risk of cancers, specifically brain tumors?	568
60. Which cancers have a significant racial predilection?	568
61. What cancers are most commonly associated with a second neoplasm?	568
62. Are there any known transplacental carcinogens?	569
63. Is prenatal ultrasound associated with a risk for leukemia later in childhood?	569
64. Do children living near electrical power lines have an increased risk for developing cancer?	569
Leukemia	569
65. What are the most common clinical findings in the initial presentation of ALL?	569
66. What are the typical hematologic findings noted during the presentation of ALL?	570
67. What studies of tumor cells are useful for determining a patients prognosis?	570
68. Which patients with ALL have a poorer prognosis: younger or older children?	570
69. What is the significance of translocations of the MLL gene?	570
70. Although many prognostic factors have come and gone for childhood ALL, which two have remained significant for the pa...	571
71. Why do boys with ALL fare more poorly than girls?	571
72. Are race and ethnicity related to treatment outcome in patients with acute leukemia?	571
73. In the United States, what are the four most common types of pediatric leukemia, and about how many children are diag...	571
74. What is MRD and how is it used?	571
75. Is there a relationship between MRD and prognosis in children with ALL?	571
76. What is the acute risk for a very elevated blast count noted at the time of the initial diagnosis of leukemia?	572
77. What are the most common sites of extramedullary relapse of ALL?	572
78. What are known risk factors for acute myeloid leukemia?	572
79. What is a chloroma?	572
80. What are the two major classes of lymphomas?	572
81. What is the malignant cell of Hodgkin disease?	572
82. How is Hodgkin disease staged?	572
83. What is the difference between clinical and pathologic staging as it relates to Hodgkin disease?	573
84. What is the histologic classification of Hodgkin disease?	573
85. What is the prognosis for the various stages of Hodgkin disease?	573
86. From what cells do non-Hodgkin lymphoma (NHL) derive?	573
87. How is childhood NHL classified?	573
88. Are there specific chromosomal abnormalities in Burkitt lymphoma?	574
89. What is the role of geography in the classification of Burkitt lymphoma?	574
90. Who was Burkitt?	574
91. What differentiates B- and T-cell precursor leukemia from lymphoma?	574
Nervous system tumors	574
92. How are CNS tumors classified?	574
93. Where is the most common area for each tumor to occur?	574
94. What are the most common supratentorial brain tumors? What are their symptoms?	575
95. What are the most common infratentorial tumors? What are their symptoms?	575
96. Which common parameters should be closely monitored in a child after resection of a brain tumor?	575
97. Which cranial nerve abnormality is most common in children showing signs of increased intracranial pressure as the re...	575
98. What are the three ``Es´´ of the diencephalic syndrome?	576
99. What is Parinaud syndrome?	576
100. In addition to imaging studies, what should be included in the evaluation of a possible CNS germ cell tumor?	576
101. What are the key evaluations for a child with a newly-diagnosed medulloblastoma?	576
102. What is a ``dropped met´´?	576
103. What are the differences among a glioma, an astrocytoma, and glioblastoma multiforme?	576
104. What is the most common benign brain tumor found in children?	576
105. Is resection curative for patients with juvenile pilocytic astrocytoma?	576
106. Why is the prognosis for children with brainstem gliomas so poor?	577
107. What is leukocoria?	577
108. What is the heredity of retinoblastoma?	577
109. What is the ``two-hit´´ hypothesis of cancer, particularly retinoblastoma?	577
110. In what age group does retinoblastoma usually occur?	577
111. Patients with retinoblastoma are at increased risk for other tumors. How significant a risk is this?	577
112. What is the cell of origin of neuroblastoma?	577
113. What is the most common cancer in younger children?	577
114. What are the most common presentations of neuroblastoma?	578
115. What is Horner syndrome?	578
116. Where does neuroblastoma tend to metastasize?	579
117. What is meant by ``dancing eyes, dancing feet´´?	579
118. What urinary test aids in the diagnosis of neuroblastoma?	579
119. Which molecular abnormality is associated with a more aggressive form of neuroblastoma?	579
120. What does the S stand for in stage 4S neuroblastoma?	579
121. How can the site of spinal cord compression be clinically localized?	579
Solid non-nervous system tumors	579
122. What are the peak ages of incidence of the most common solid tumors of childhood?	579
123. What are ``blastemal´´ tumors?	579
124. What is the average age of diagnosis for Wilms tumor?	579
125. What are the three histologic components of a Wilms tumor?	580
126. How is Wilms tumor distinguished radiographically from neuroblastoma?	580
127. Where does Wilms tumor tend to metastasize?	580
128. What is a stage V Wilms tumor?	580
129. What factors influence the prognosis of a patient with Wilms tumor?	580
130. ``Small, round, blue cell tumor´´ is often used in the description of which childhood tumors?	580
131. Where are the most common locations of Ewing sarcoma?	580
132. What molecular abnormality is commonly seen in Ewing sarcoma?	580
133. What are the two most common sites of metastases for patients with Ewing sarcoma?	580
134. What type of tumor is an osteosarcoma?	580
135. Osteosarcoma generally arises in which part of the bone?	580
136. Do all patients with osteosarcoma require surgical resection of the primary tumor?	581
137. For patients with localized osteosarcoma, what factor is most predictive of a favorable outcome?	581
138. What do Ewing sarcoma and osteosarcoma have in common?	581
139. In what solid tumor has the surgical resection of pulmonary metastases been shown to result in long-term cure?	581
140. What is a limb salvage procedure?	582
141. What type of tumor is a rhabdomyosarcoma?	582
142. Where do rhabdomyosarcomas usually arise?	582
143. What sites of disease are associated with the best outcomes for children with rhabdomyosarcoma?	582
144. What are the two major histologic subtypes of rhabdomyosarcoma?	582
145. Which germ cell tumor is usually seen in young children?	582
146. Why are tumor markers assessed before surgery for teratomas and other germ cell tumors?	582
147. Virilization may be associated with which childhood cancer?	582
148. How great is the risk for malignant transformation in undescended testes?	582
149. What are the most common primary liver tumors of childhood?	582
150. Which tumor marker is most likely to be elevated in children with hepatic tumors?	583
151. Lance Armstrongs treatment for metastatic testicular germ cell tumor had an important modification from standard the...	583
Stem cell transplantation	583
152. What are the two main types of hematopoietic stem cell (HSC) transplant?	583
153. What is the importance of HLA matching in HSC transplant recipients?	583
154. What is the chance of siblings having the same human leukocyte antigen (HLA) type?	583
155. What is the chance of finding an HLA-matched unrelated donor?	583
156. What are the different sources of stem cells for transplantation?	583
157. What are the advantages and disadvantages of umbilical cord blood as the source for a stem cell transplantation?	584
158. How are stem cells collected?	584
159. What is the rationale behind an autologous transplant?	584
160. Are there nonmalignant indications for an HSC transplant?	584
161. In transplant medicine, to what does the term ``conditioning´´ refer?	584
162. What are the three most common categories of conditioning regimens?	584
163. What are the major side effects from total-body irradiation used in conditioning?	584
164. Which prophylactic measures should be taken after stem cell transplantation?	585
165. What is the most common early complication seen in patients after HSC transplant?	585
166. What are the major features of graft-versus-host disease (GVHD)?	585
167. How is GVHD managed?	585
168. What are the risk factors for GVHD?	585
169. What is the most likely diagnosis for a patient who experiences weight gain, right upper quadrant pain, and hepatome...	585
170. Which chemotherapeutic agents can be administered intrathecally to either treat or prevent meningeal malignancy?	585.e1
171. What are the common side effects of methotrexate?	585.e1
172. In what way do the side-effect profiles of cisplatin and carboplatin differ?	585.e1
173. What are the common complications of vincristine?	585.e1
174. What agent can limit the complication of hemorrhagic cystitis that occurs in some chemotherapeutic regimens?	585.e1
175. What are the differences between conventional external radiation, intensity-modulated radiation therapy (IMRT), and ...	585.e1
176. What treatment options exist for individuals who are allergic to sulfonamides and are unable to tolerate trimethopri...	585.e1
177. What PJP prophylactic agent has been associated with methemoglobinemia?	585.e2
178. Is it true that the PEG-asparaginase was affectionately named after its inventor?	585.e2
179. Why are blood products irradiated and leukocyte depleted?	585.e2
180. What is the most well-documented risk factor for hepatoblastoma?	585.e2
181. Which distinct form of AML uses an analog of a common vitamin as a core component of its treatment?	585.e2
182. What is the consensus regarding appropriate treatment for CML?	585.e2
183. If you saw a car with the license plate, ``FLT3 ITD,´´ what would the most likely interest of its driver be?	585.e2
184. What nuclear medicine agent has been useful in both the diagnosis and treatment of neuroblastoma?	585.e3
185. Patients with high-risk neuroblastoma require treatment with virtually all known therapeutic modalities to maximize ...	585.e3
186. Do all transplant patients require complete ablation of their recipient bone marrow?	585.e3
187. What is graft-versus-leukemia (GVL)?	585.e3
Chapter 15: Orthopedics	586
Clinical issues	586
1. What is torticollis?	586
2. What is the differential diagnosis for torticollis?	586
3. An x-ray of a 10-year-old boy taken to rule out an ankle fracture reveals a 4-mm, well-circumscribed lytic lesion in t...	586
4. What are two other common types of benign bone tumors in children?	586
5. How and why is it important to differentiate between an ABC and a UBC?	586
6. What is rickets?	586
7. What are the physical signs that are suggestive of rickets?	586
8. Which bones are known to develop aseptic (also called avascular) necrosis?	587
9. What are the inheritance patterns and clinical features of osteogenesis imperfecta?	587
10. McCune-Albright syndrome is associated with what skeletal abnormalities?	587
11. What are the causes of in-toeing gait (pigeon-toeing)?	587
12. Is in-toeing a problem?	588
13. When, if ever, does in-toeing need to be treated?	588
14. A 15-year-old with tibial pain (worse at night and relieved by nonsteroidal anti-inflammatory drugs) has a small lyti...	588
15. What is the clinical significance of limb-length discrepancy?	588
16. What are the possible causes of a limb-length discrepancy?	588
17. What are the general management principles for a limb-length discrepancy?	588
18. What is a nursemaid elbow?	589
19. How is a nursemaid elbow reduced?	589
20. What signs and symptoms suggest a serious cause of back pain in a child that warrants further evaluation?	590
21. What is the differential diagnosis of back pain in children?	590
22. Do school backpacks contribute to back pain?	590
23. What constitutes an orthopedic emergency?	590
Foot disorders	590
24. Do infants and children need shoes?	590
25. What is the most common congenital foot abnormality?	591
26. How is metatarsus adductus treated?	591
27. How is clubfoot distinguished from severe metatarsus adductus?	591
28. How are clubfeet treated?	591
29. What is a calcaneovalgus foot?	592
30. What should be suspected when pes cavus is noted on examination?	592
31. Should children with flexible flat feet be given corrective shoes?	592
32. When should I worry about a child with flat feet?	592
33. How does the cause of foot pain vary by age?	593
34. A 10-year-old boy with recurrent ankle sprains and painful flat feet should be evaluated for what possible diagnosis?	593
Fractures	593
35. What are the fractures patterns unique to children?	593
36. What is a buckle fracture?	593
37. What is a greenstick fracture?	593
38. What does plastic deformation mean?	593
39. Where are the most frequent sites of fractures among children?	593
40. What is an open fracture?	593
41. What is a toddler fracture?	594
42. How are growth-plate fractures classified?	594
43. In a patient with suspected fracture, what are the key points on physical examination?	594
44. What are the signs of compartment syndrome?	594
45. What is the treatment of compartment syndrome?	595
46. How do you treat clavicle fractures?	595
47. Is surgery ever indicated in clavicle fractures?	595
48. A teenager who punches a wall in anger typically incurs what fracture?	595
49. Children who fall on outstretched arms often suffer what type of fractures?	596
50. What does the presence of the posterior fat pad on an elbow x-ray suggest?	596
51. For a teenager with wrist trauma, why is palpation of the anatomic ``snuff box´´ a critical part of the physical exam?	596
52. Name the eight carpal bones of the wrist.	596
53. What is the difference between open and closed reductions?	596
54. In pediatric fractures, what amount of angulation is acceptable before reduction is recommended?	596
55. In which fractures will remodeling of bone not occur?	597
56. How long should fractures be immobilized?	597
57. How long do fractured clavicles and femurs take to heal?	597
Hip disorders	597
58. Why has DDH replaced CHD?	597
59. What are the Ortolani and Barlow maneuvers?	597
60. What is a positive Galeazzi sign?	598
61. What is the significance of a ``hip click´´ in a newborn?	598
62. What is the most reliable physical finding for a dislocated hip in the older child?	598
63. What other diagnostic signs are suggestive of a dislocated hip?	598
64. What radiographic studies are most valuable for diagnosing DDH during the newborn period?	599
65. Should all infants be routinely screened by ultrasound for DDH?	599
66. Who is at a higher risk for DDH?	599
67. What is the recommended timing for ultrasound evaluation when screening is indicated?	599
68. How is DDH treated?	599
69. What is the natural history of untreated DDH?	600
70. What is the significance of a Trendelenburg gait?	600
71. What is the most common cause of a painful hip in a child <10years old?	600
72. How can transient synovitis be differentiated from septic arthritis?	600
73. What is LCP disease?	601
74. What are the pathologic stages of LCP disease?	601
75. What is the prognosis for children with LCP disease?	602
76. What condition does the child in Figure15-9 have?	602
77. How is the extent of femoral anteversion measured?	602
78. Is sitting in the ``W´´ position harmful?	602
79. Is there ever an indication to treat femoral anteversion?	603
80. What symptoms do children with slipped capital femoral epiphysis (SCFE) have?	603
81. What systemic conditions are associated with SCFE?	603
82. What does FAI stand for?	603
83. What new treatments are available for the treatment of hip pathology including DDH, FAI, and SCFE?	603
Infectious diseases	603
84. What percentage of septic arthritis is ``culture negative´´?	604
85. Where does acute hematogenous osteomyelitis most commonly localize in children?	604
86. What is the most common cause of acute hematogenous osteomyelitis?	604
87. How often are blood cultures positive in patients with osteomyelitis?	604
88. As osteomyelitis progresses, how soon do x-ray changes occur?	604
89. What is the best way to confirm the diagnosis of osteomyelitis?	604
90. How long should antibiotics be continued in patients with osteomyelitis and septic arthritis?	605
91. Which marker, C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), is more sensitive to assess inflammat...	605
92. When is open surgical drainage indicated in cases of osteomyelitis?	605
93. Why are treatment failures more common in osteomyelitis than in septic arthritis?	605
94. How is the diagnosis of discitis established?	606
95. What is the most important variable that influences mortality in necrotizing fasciitis?	606
96. What is the role of bone scintigraphy in children with obscure skeletal pain?	606
97. What are the phases of a bone scan?	606
Knee, tibia, and ankle disorders	606
98. What is the difference between valgus and varus deformities?	606
99. Are children normally knock-kneed or bowlegged?	607
100. Which bowlegged infants or toddlers require evaluation?	607
101. What are the causes of pathologic genu varum (bowleggedness) or genu valgus (knock knees)?	607
102. Which children are more likely to develop Blount disease?	607
103. How does tibial torsion change with age?	608
104. How effective is the Denis Browne splint for the treatment of tibial torsion?	608
105. Why are ligamentous injuries less common in children?	608
106. How are ankle sprains graded?	608
107. Which ankle sprains should be evaluated with an x-ray?	608
108. Should ankle sprains be casted?	608
109. What is the most significant mistake made during the evaluation of knee pain?	608
110. In acute injury, what injuries typically cause bleeding into the knee joint?	609
111. A 5-year-old boy with a painless swelling in the back of his knee has what likely condition?	609
112. How does patellofemoral stress syndrome occur?	609
113. What is the Q angle?	609
Spinal disorders	609
114. What are the different forms scoliosis?	609
115. Is scoliosis more common in boys or girls?	610
116. How likely is the progression of scoliosis?	610
117. What are the risk factors for progression of idiopathic scoliosis?	610
118. How is screening for spinal deformity performed?	610
119. What constitutes an abnormal scoliometer measurement?	610
120. How is scoliosis measured by the Cobb method?	611
121. How valuable are school-based screening programs for scoliosis?	612
122. What is the natural history of untreated severe idiopathic scoliosis?	612
123. When should surgery be considered for idiopathic scoliosis?	612
124. What type of surgery is typically performed for scoliosis correction?	612
125. Are the spines of young children fused as well?	612
126. So how are large curves in young children treated?	612
127. Are casts still used to treat scoliosis?	613
128. Is bracing an effective treatment for scoliosis?	613
129. What diagnosis should you consider in a teenage male with very poor posture that is not flexible?	613
130. What is the difference between spondylolysis and spondylolisthesis?	613
Sports medicine	614
131. A 12-year-old baseball player presents complaining of elbow pain. What diagnosis do you need to consider?	614
132. Which is worse for a baseball pitchers elbow: throwing curveballs or fastballs?	614
133. How can elbow and shoulder injuries be prevented in pitchers?	614
134. Do meniscal tears occur in younger children?	614
135. If a ninth-grade soccer player with knee swelling ``felt a pop´´ while scoring a goal, what are three possible diagn...	614
136. How is meniscal integrity assessed on examination?	614
137. What is the typical mechanism for an anterior cruciate ligament (ACL) tear?	615
138. How is ACL stability tested on examination?	615
139. Why are girls and young women more susceptible to ACL tears than their male counterparts?	615
140. Are ACL tears treated differently in children, adolescents, and adults?	616
141. Are there ways to prevent ACL tears?	616
142. A teenager has chronic knee pain, swelling, and occasional ``locking´´ of the knee joint, and his x-ray reveals incr...	616
143. What is the most likely diagnosis if a 12-year-old basketball player has painful swelling below both knees?	617
144. What is the likely diagnosis in a fifth-grade football player with heel pain and a positive ``squeeze test´´?	617
145. Which sports injuries are the most common in school-age children and adolescents?	617
146. What are the definitions of concussion and postconcussion syndrome?	617
147. What is the value of ``brain rest´´ in the treatment of concussion?	618
148. When should an athlete who has suffered a concussion be allowed to return to play?	618
149. Are stretching exercises helpful for congenital muscular torticollis?	618.e1
150. What advice should be given to a parent about buying shoes for a toddler?	618.e1
151. Which conditions are associated with coxa vara?	618.e1
152. How do the features of osteomyelitis in the neonate differ from those seen in the older child and adult?	618.e1
153. What predisposes a child or teenager to recurrent dislocation of the patella?	618.e1
154. Is there a genetic test for scoliosis?	618.e1
Chapter 16: Pulmonology	619
Allergic rhinitis	619
1. How common is allergic rhinitis?	619
2. In addition to chronic or recurrent nasal congestion, what features on history and physical examination suggest allerg...	619
3. What are three early-life risk factors for allergic rhinitis?	619
4. How does the time of year help identify the potential cause of allergic rhinitis?	620
5. When are allergy blood tests used?	620
6. What is an antigen-specific IgE ImmunoCAP?	620
7. Summarize the pros and cons of skin testing versus in vitro testing (e.g., IgE ImmunoCAP) for allergies	620
8. What are the recommended treatments for children with allergic rhinitis?	620
9. What are the major indoor (year-round) allergens?	621
10. How can you decrease cat allergen in the home?	621
11. Is there truly a dog breed that is ``hypoallergenic´´?	621
12. Which children should be considered for immunotherapy?	621
13. How common is exercise-induced bronchospasm in children with allergic rhinitis?	621
Asthma	622
14. If both parents are asthmatic, what is the risk that their child will have asthma?	622
15. When does asthma usually have its onset of symptoms?	622
16. Which children with wheezing at an early age are likely to develop chronic asthma?	622
17. What historical points are suggestive of an allergic basis for asthma?	622
18. What are other potential triggers for asthma?	622
19. What distinguishes EIA from EIB?	622
20. What is the time course of EIB?	623
21. How is EIB diagnosed?	623
22. A 15-year-old with repeated shortness of breath after track practice has suspected exercise-induced bronchospasm, but...	623
23. What mechanisms lead to airway obstruction during an acute asthma attack?	623
24. All that wheezes is not asthma. What are other noninfectious causes?	623
25. How is the severity of an acute asthma attack estimated?	624
26. Is a chest radiograph necessary for all children who wheeze for the first time?	624
27. What are the usual findings on arterial blood gas sampling during acute asthma attacks?	625
28. What are the indications for hospital admission in children with asthma?	625
29. List the possible acute side effects of albuterol and other β-agonists	625
30. What is the role of magnesium sulfate in acute asthma attacks?	625
31. How is chronic asthma severity classified among children 5 to 11 years of age?	625
32. What is the treatment of choice for patients with persistent asthma?	625
33. Do inhaled steroids affect growth in children?	627
34. What is anti-IgE treatment for asthma?	627
35. Is there a role for complementary and alternative medicines in the treatment of asthma?	627
36. How useful are pulmonary function tests when evaluating and following children with asthma?	627
37. What proportion of asthmatic children ``outgrow´´ their symptoms?	628
38. What diagnosis should be considered in a patient with poorly controlled asthma with recurrent infiltrates who has cen...	628
Bronchiolitis	628
39. What is the most important cause of lower respiratory tract disease among infants and young children?	628
40. What other agents cause bronchiolitis?	629
41. What are the best predictors of the severity of bronchiolitis?	629
42. What are the typical findings on a chest radiograph in a child with bronchiolitis?	629
43. Which patients with bronchiolitis are at risk for apnea?	629
44. Is the use of steroids justified for bronchiolitis?	629
45. Is inhalation therapy effective for bronchiolitis?	630
46. Is there a vaccine to prevent RSV infection?	630
47. Does infection with RSV confer lifelong protection?	630
48. If a 5-month-old child is hospitalized as a result of RSV bronchiolitis, what should the parents be told about the li...	630
Clinical issues	630
49. How is hemoptysis differentiated from hematemesis?	630
50. What are the indications for surgical repair of pectus excavatum?	631
51. What are the most common causes of chronic cough?	631
52. When should the diagnosis of psychogenic cough be considered?	631
53. What medications are most effective for cold symptoms in children?	632
54. Which is the more effective for cough in children: antihistamines, antitussives, mucolytics, decongestants, or honey?	632
55. What constitutes passive cigarette smoke?	632
56. What are the possible risks of passive cigarette smoke exposure?	632
57. How is clubbing diagnosed?	632
58. What are the causes of digital clubbing?	633
59. What is the pathophysiology of clubbing?	633
60. Nasal polyps are associated with which conditions?	633
61. A patient with chronic sinusitis and recurrent pulmonary infections has a chest radiograph that demonstrates a right-...	634
62. What percentage of children snore?	634
63. In which children who snore should obstructive sleep apnea (OSA) be suspected?	634
64. What evaluations should be performed on a child with suspected OSA?	634
65. What are the potential long-term consequences of OSA?	635
66. What is the most common cause of infantile stridor?	635
67. How can you clinically distinguish bilateral from unilateral vocal cord paralysis in an infant?	635
68. What is the most common cause of chronic hoarseness in children?	636
69. What are the most common symptoms and signs in children with suspected foreign body aspiration?	636
70. Which other clinical features are suggestive of foreign-body aspiration?	636
71. Are chest radiographs useful for evaluating a foreign-body aspiration?	636
72. On which side of the chest are foreign-body aspirations and aspiration pneumonias more common?	637
73. What are the possible mechanisms for the development of lung abscesses in children?	637
74. What are the typical clinical findings in patients with bronchiectasis?	637
75. A novice teenage mountain-climber develops headache, marked cough, and orthopnea at the end of a rapid 2-day climb. W...	637
76. What is the likely diagnosis of a child with diffuse lung disease, microcytic anemia, and sputum that contains hemosi...	637
77. How should a child with a spontaneous pneumothorax be managed?	638
78. Describe the clinical and radiographic features of a tension pneumothorax	638
79. What physical examination features suggest a pleural effusion?	638
80. In children with pleural effusions, how are exudates distinguished from transudates?	638
81. What pediatric diseases are associated with exudative and transudative pleural effusions?	639
82. What are possible treatments for infected parapneumonic effusions?	639
83. What is the value of chest physiotherapy (CPT) in patients with pediatric pulmonary disease?	640
84. Who was Ondine, and what was her curse?	640
Cystic fibrosis	640
85. What is the basic defect in patients with cystic fibrosis (CF)?	640
86. What is the incidence of CF in various ethnic groups?	640
87. What are the presenting signs and symptoms of CF?	641
88. How is the diagnosis of CF made?	641
89. What constitutes an abnormal sweat test?	641
90. How are newborns screened for CF?	641
91. When and why should children with cystic fibrosis be screened for possible CF-related diabetes mellitus?	641
92. What are the mainstays of pulmonary therapy for children with CF?	641
93. What is the role of Ivacaftor in the treatment of CF patients?	642
94. Which features of CF have prognostic significance?	642
Pneumonia	642
95. What agents cause pneumonia in children?	642
96. What are important trends in the etiology of pneumonia in the United States?	643
97. Are throat or nasopharyngeal cultures helpful for the diagnosis of pneumonia?	643
98. How often are blood cultures positive in children with suspected bacterial pneumonia?	643
99. How often are pleural fluid cultures positive in children with suspected bacterial pneumonia?	644
100. Can a chest radiograph reliably distinguish between viral and bacterial pneumonia?	644
101. What are indications for hospital admission in children with pneumonia?	644
102. What clinical clues suggest atypical pneumonia?	645
103. What are the causes of ``afebrile infant pneumonia´´ syndrome?	645
104. What are the clinical characteristics of chlamydial pneumonia in infants?	645
105. How helpful are cold agglutinins in the diagnosis of M. pneumoniae infections?	645
106. When do the radiologic findings of pneumonia resolve?	645
107. Do children with pneumonia need follow-up radiographs to verify resolution?	645
108. What are the causes of recurrent pneumonia?	646
109. How does the pH of a substance affect the severity of disease in aspiration pneumonia?	646
110. How should children with aspiration pneumonia be managed?	646
Pulmonary principles	646
111. In addition to underlying immunologic immaturity, why are infants more susceptible to an increased severity of respi...	646
112. At what age do alveoli stop increasing in number?	646
113. What is the normal respiratory rate in otherwise healthy children?	647
114. What is the significance of grunting respirations?	647
115. What is normal oxygen saturation in healthy infants who are <6months?	647
116. What is the difference among Kussmaul, Cheyne-Stokes, and Biot types of breathing patterns?	647
117. Why a sigh?	647
118. Is there a respiratory basis for yawning?	647
119. At what concentration is inspired oxygen toxic?	647
120. Why is a child who is receiving 100% oxygen more likely to develop atelectasis than one who is breathing room air?	648
121. At what Pao2 does cyanosis develop?	648
122. What are the causes of a reduced Pao2 associated with an increased A-aDo2 (alveolar-arterial oxygen tension differen...	648
123. How does the pulse oximeter work?	648
124. What are the disadvantages or limitations of pulse oximetry?	648
125. In infants with unilateral lung disease, should the good lung be up or down?	648
126. How can house dust mite (HDM) concentrations be minimized?	649.e1
127. Is a nebulizer more effective than a metered-dose inhaler (MDI) with a spacer for the treatment of asthma?	649.e1
Chapter 17: Rheumatology	650
Clinical issues	650
1. What is an ANA?	650
2. What is an ANA profile?	650
3. Should I order a profile instead of an ANA because it has more specificity?	650
4. What is the significance of the various antibodies included in the ANA profile?	650
5. A 6-year-old girl with a 2-month history of joint pain (onset after a viral illness) has a normal physical examination...	650
6. Is Raynaud phenomenon a disease?	650
7. When is a child considered to have hypermobile joints?	651
8. Which children can demonstrate a Gorlin sign?	651
9. In what settings can reactive arthritis occur?	651
10. What conditions are associated with gastrointestinal symptoms and arthritis?	651
11. One week after mild trauma, an 8-year-old girl has pain and tenderness in the right foot and leg, both of which are c...	652
12. How is complex regional pain syndrome managed?	652
13. Do children develop fibromyalgia?	652
Dermatomyositis and polymyositis	653
14. What are the criteria used for the diagnosis of juvenile dermatomyositis and polymyositis?	653
15. What skin changes are pathognomonic for dermatomyositis?	653
16. What are the other classic cutaneous findings of dermatomyositis among children?	654
17. Which infectious agents are known to cause myositis?	654
Juvenile idiopathic arthritis	654
18. Why is JRA becoming juvenile idiopathic arthritis (JIA)?	654
19. What is synovitis, and at what point is it considered chronic?	654
20. What is the most common chronic arthritis seen in children?	654
21. What are the diagnostic criteria for the classification of JIA?	654
22. What are the characteristics of the seven main subsets of JIA?	655
23. What percent of pediatric JIA presents as systemic JIA?	655
24. What is the pattern of fever and characteristic rash of the systemic-onset subset of JIA?	655
25. In addition to different clinical features, how is systemic JIA distinguished from other subgroups of JIA?	656
26. Why is it sometimes difficult to distinguish systemic JIA (sJIA) from leukemia?	656
27. In a patient with suspected rheumatic disease, what clinical features are more suggestive of malignancy?	656
28. What is the value of measuring ANA and rheumatoid factor (RF) in patients with JIA?	656
29. Are radiographs helpful for diagnosing JIA?	656
30. A patient with JIA who becomes ill with thrombocytopenia, profound anemia, and markedly elevated transaminases probab...	657
31. What are the main features of the macrophage activation syndrome?	657
32. What is the traditional first-line approach to JIA medical management?	657
33. What second-line agents have been used in the treatment of JIA?	657
34. When are corticosteroids indicated for children with JIA?	658
35. What are the most common side effects of prolonged corticosteroid therapy?	658
36. What are biologic agents?	658
37. Which children with JIA require the most frequent monitoring for uveitis?	658
38. What is the earliest sign of uveitis among patients with JIA?	659
39. What are the juvenile spondyloarthropathies under the revised classification system?	659
40. What are the characteristic clinical features of the juvenile spondyloarthropathies?	660
41. How is enthesitis diagnosed clinically?	660
42. Why is the diagnosis of ankylosing spondylitis difficult to make in children?	660
43. Where are the dimples of Venus?	661
Lyme disease	661
44. What criteria are used to diagnose Lyme disease?	661
45. What is the typical rash seen in Lyme disease?	661
46. How long after a tick bite does the rash of Lyme disease appear?	662
47. How is Lyme disease confirmed in the laboratory?	662
48. If infection ensues after a tick bite, how does Lyme disease progress?	662
49. How is the diagnosis of Lyme meningitis established?	662
50. How are Lyme disease and viral meningitis clinically differentiated?	662
51. Should lumbar punctures be done for patients with facial palsy and suspected Lyme disease?	663
52. How is Lyme arthritis differentiated from septic arthritis?	663
53. What is the prognosis for children diagnosed with Lyme arthritis?	663
54. What should be suspected if a patient with Lyme disease develops fever and chills after starting antibiotic treatment?	663
55. Should we follow Lyme disease course and response to therapy with titers?	663
56. Is antibiotic prophylaxis indicated for all tick bites?	663
57. What are other means of preventing Lyme disease?	664
Rheumatic fever	664
58. What is acute rheumatic fever?	664
59. What are the major Jones criteria for rheumatic fever?	664
60. What is acceptable proof of antecedent streptococcal pharyngitis when diagnosing acute rheumatic fever?	664
61. Which antistreptococcal antibodies are most commonly measured?	664
62. What are the common manifestations of carditis in patients with ARF?	665
63. How quickly can valvular lesions occur in children with ARF?	665
64. What are the typical characteristics of arthritis in patients with ARF?	665
65. What is the effect of aspirin therapy on the arthritis of rheumatic fever?	665
66. What is the rash of rheumatic fever?	665
67. What is Sydenham chorea?	666
68. Who was Saint Vitus?	666
69. Are corticosteroids of benefit for the treatment of ARF?	666
70. Can antibiotic prophylaxis for rheumatic fever ever be discontinued?	666
71. Where do PANDAS live in the world of pediatric rheumatology?	666
Systemic lupus erythematosus	667
72. What is systemic lupus erythematosus (SLE)?	667
73. What laboratory tests should be ordered in a child who is suspected of having SLE?	667
74. What are the most common manifestations of SLE in children?	667
75. What are the neurologic manifestations of SLE?	668
76. Which diseases should be considered in the differential diagnosis of children with a butterfly rash?	668
77. Should children with SLE undergo a renal biopsy?	668
78. How can the result of renal biopsy affect treatment of SLE?	669
79. When should high-dose corticosteroid therapy be considered for SLE management?	669
80. What is the association of antiphospholipid antibodies and lupus?	669
81. Which laboratory tests are useful for monitoring the effectiveness of therapy in patients with SLE?	669
82. What are the most common manifestations of neonatal lupus erythematosus?	670
83. What is the pathophysiology of the CCHB of NLE?	670
84. What are the common features of drug-induced lupus?	670
85. What are the most common causes of drug-induced lupus in children?	670
Vasculitis	671
86. What clinical features suggest a vasculitic syndrome?	671
87. How are the primary systemic vasculitides classified?	671
88. What are the two most common pediatric vasculitides?	671
89. Which infectious agents are associated with vasculitis?	671
90. What are the conditions that are grouped under the term pulmonary-renal syndromes?	671
91. What is the clinical triad of Behçet disease?	672
92. Should it be ``Henoch-Schönlein purpura´´ or ``Schönlein-Henoch purpura´´?	672
93. What are the characteristic laboratory findings of patients with Henoch-Schönlein purpura (HSP)?	672
94. What kinds of skin lesions are noted in patients with HSP?	672
95. In addition to the skin, what other organ systems are typically involved in HSP?	673
96. How often does chronic renal disease develop in children with HSP?	673
97. Why is the diagnosis of intussusception often difficult in patients with HSP?	673
98. When are corticosteroids indicated for the treatment of HSP?	673
99. What is acute hemorrhagic edema of infancy (AHEI)?	674
100. In which rheumatic diseases can ``cauliflower ears´´ be seen?	674
101. What is the difference between autoinflammatory and autoimmune diseases?	674
102. Are all autoinflammatory diseases characterized by periodic fever and genetic mutations?	674
Index	676

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