BOOK
Primary Immunodeficiency Disorders, An Issue of Immunology and Allergy Clinics of North America 35-4, E-Book
(2016)
Additional Information
Book Details
Abstract
This issue of Immunology and Allergy Clinics of North America, Guest Edited by Anthony Montanaro, MD, is devoted to Primary Immunodeficiency Disorders. Dr. Montanaro has assembled a group of expert authors to review the following topics: Overview of Immunodeficiency Disorder; Severe Combined Immunodeficiency Disorder; Specific Antibody Deficiencies; Common Variable Immunodeficiency; Pulmonary Manifestations of Primary Immunodeficiency Disorders; Approach to the Child with Recurrent Infections; Immunoglobulin Treatment for Primary Immunodeficiency; Bone Marrow Transplantation for Primary Immunodeficiency; and Autoimmune Manifestations of Primary Immunodeficiency.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Primary Immunodeficiency\rDisorders | i | ||
| Copyright | ii | ||
| Contributors | iii | ||
| EDITOR | iii | ||
| AUTHORS | iii | ||
| Contents | v | ||
| Preface: Primary Immunodeficiency Disorders | v | ||
| Overview of Immunodeficiency Disorders | v | ||
| Approach to Children with Recurrent Infections | v | ||
| Common Variable Immunodeficiency: Diagnosis, Management, and Treatment | v | ||
| Specific Antibody Deficiencies | v | ||
| Severe Combined Immunodeficiency Disorders | vi | ||
| Hematopoietic Stem Cell Transplant for Immune Deficiency and Immune Dysregulation Disorders | vi | ||
| Immunoglobulin Replacement Therapy for Primary Immunodeficiency | vi | ||
| Autoimmune Disease in Primary Immunodeficiency: At the Crossroads of Anti-Infective Immunity and Self-Tolerance | vii | ||
| Pulmonary Manifestations of Primary Immunodeficiency Disorders | vii | ||
| Primary Immunodeficiency Masquerading as Allergic Disease | vii | ||
| IMMUNOLOGYAND ALLERGY\rCLINICS OF NORTH AMERICA | viii | ||
| FORTHCOMING ISSUES | viii | ||
| February 2016 | viii | ||
| May 2016 | viii | ||
| August 2016 | viii | ||
| RECENT ISSUES | viii | ||
| August 2015 | viii | ||
| May 2015 | viii | ||
| February 2015 | viii | ||
| Preface: Primary Immunodeficiency Disorders | ix | ||
| Overview of Immunodeficiency Disorders | 599 | ||
| Key points | 599 | ||
| INTRODUCTION | 599 | ||
| HISTORY | 600 | ||
| EPIDEMIOLOGY | 600 | ||
| CLASSIFICATION | 601 | ||
| Predominantly Antibody Deficiencies | 601 | ||
| Combined Immunodeficiencies | 601 | ||
| Phagocytic Defects | 601 | ||
| Complement Deficiencies | 601 | ||
| Complement defects | 601 | ||
| Disorders of Innate Immunity | 601 | ||
| Nuclear factor kappa B pathway defect/anhidrotic ectodermal dysplasia | 603 | ||
| Toll-like receptor signaling pathway deficiency | 603 | ||
| Pyogenic infections | 603 | ||
| Herpes simplex encephalitis | 603 | ||
| Natural killer cell deficiency | 603 | ||
| DISORDERS OF IMMUNE DYSREGULATION | 614 | ||
| Familial Hemophagocytic Lymphohistiocytosis | 614 | ||
| Immunodeficiency; Polyendocrinopathy, Enteropathy, X Linked; and Polyendocrinopathy, Enteropathy, X Linked–like Disorders | 614 | ||
| Lymphoproliferative Disorders Associated with Epstein-Barr Virus (EBV) | 614 | ||
| Autoimmunity Without Lymphoproliferation | 614 | ||
| Autoimmune Lymphoproliferative Syndrome | 614 | ||
| AUTOINFLAMMATORY DISORDERS | 615 | ||
| Periodic Fever Syndrome | 615 | ||
| PHENOCOPIES OF PRIMARY IMMUNODEFICIENCY DISORDERS | 615 | ||
| DIAGNOSIS | 615 | ||
| HISTORY AND PHYSICAL EXAMINATION | 615 | ||
| LABORATORY INVESTIGATIONS | 616 | ||
| Screening Tests | 616 | ||
| Second-tier Tests | 616 | ||
| TREATMENT | 616 | ||
| Definitive Treatment | 616 | ||
| Symptomatic Treatment | 620 | ||
| Prevention of Manifestations/Complications | 620 | ||
| Follow-up/Monitoring | 620 | ||
| PROGNOSIS | 620 | ||
| FUTURE OF THE STUDY OF PRIMARY IMMUNODEFICIENCY DISORDERS | 620 | ||
| REFERENCES | 621 | ||
| Approach to Children with Recurrent Infections | 625 | ||
| Key points | 625 | ||
| INTRODUCTION | 625 | ||
| SYMPTOMS/SIGNS | 626 | ||
| DIAGNOSTIC TEST/IMAGING STUDY | 628 | ||
| DIFFERENTIAL DIAGNOSIS | 630 | ||
| TREATMENT | 632 | ||
| MANAGEMENT | 633 | ||
| SUMMARY | 634 | ||
| REFERENCES | 634 | ||
| Common Variable Immunodeficiency | 637 | ||
| Key points | 637 | ||
| INTRODUCTION | 637 | ||
| DIAGNOSIS | 638 | ||
| Initial Evaluation | 638 | ||
| Differential Diagnosis | 638 | ||
| Pitfalls | 638 | ||
| Classification | 639 | ||
| Genetics | 639 | ||
| Radiography | 644 | ||
| EPIDEMIOLOGY | 644 | ||
| PATHOGENESIS | 644 | ||
| Cell Biology | 644 | ||
| Role of Chronic Immune Activation | 646 | ||
| MANAGEMENT | 646 | ||
| Prognosis | 646 | ||
| Clinical Monitoring | 647 | ||
| Treatment Approach | 647 | ||
| Specific Complications | 648 | ||
| Diseases of the gastrointestinal tract and liver | 648 | ||
| Pulmonary disease | 648 | ||
| Anemia and thrombocytopenia | 649 | ||
| Granuloma | 649 | ||
| Malignancy | 650 | ||
| Arthritis | 650 | ||
| Infection | 650 | ||
| CONCLUDING REMARKS | 650 | ||
| REFERENCES | 651 | ||
| Specific Antibody Deficiencies | 659 | ||
| Key points | 659 | ||
| INTRODUCTION | 659 | ||
| PATHOPHYSIOLOGY | 660 | ||
| EPIDEMIOLOGY | 661 | ||
| NATURAL HISTORY | 661 | ||
| SYMPTOMS | 661 | ||
| DIAGNOSTIC EVALUATION | 662 | ||
| Initial Approach to the Patient | 662 | ||
| Laboratory Considerations Regarding Antipneumococcal Antibody Titers | 663 | ||
| Interpretation of the Pneumococcal Polysaccharide Response | 664 | ||
| Diagnosis of Specific Antibody Deficiency Phenotypes | 665 | ||
| MANAGEMENT | 666 | ||
| Immunization | 666 | ||
| Antibiotics | 666 | ||
| Immunoglobulin Replacement Therapy | 667 | ||
| PROGNOSIS | 667 | ||
| FUTURE CONSIDERATIONS | 668 | ||
| SUMMARY | 668 | ||
| REFERENCES | 669 | ||
| Severe Combined Immunodeficiency Disorders | 671 | ||
| Key points | 671 | ||
| INTRODUCTION | 671 | ||
| Severe Combined Immunodeficiency Disease | 672 | ||
| Definition | 672 | ||
| Molecular defects | 672 | ||
| B-positive, natural killer–negative severe combined immunodeficiency disease | 673 | ||
| B-negative, natural killer–positive severe combined immunodeficiency disease | 673 | ||
| B-positive, natural killer–positive severe combined immunodeficiency disease | 675 | ||
| B-negative, natural killer–negative severe combined immunodeficiency disease | 675 | ||
| Special considerations: T-positive severe combined immunodeficiency disease | 676 | ||
| Clinical evaluation and management | 676 | ||
| Treatment | 679 | ||
| History | 679 | ||
| Hematopoietic bone marrow stem cell transplant | 679 | ||
| Gene therapy | 682 | ||
| Congenital Athymia | 682 | ||
| Diagnostic considerations | 682 | ||
| Treatment strategies | 683 | ||
| Thymus transplant | 683 | ||
| Bone marrow transplant or mature T-cell infusions | 684 | ||
| Newborn Screening for Severe Combined Immunodeficiency Disorders | 684 | ||
| Implementation and outcomes | 684 | ||
| Considerations | 684 | ||
| FUTURE CONSIDERATIONS/SUMMARY | 685 | ||
| REFERENCES | 685 | ||
| Hematopoietic Stem Cell Transplant for Immune Deficiency and Immune Dysregulation Disorders | 695 | ||
| Key points | 695 | ||
| INTRODUCTION | 695 | ||
| IMPORTANT POINTS TO CONSIDER WHEN CONTEMPLATING HEMATOPOIETIC STEM CELL TRANSPLANT FOR PRIMARY IMMUNODEFICIENCY DISORDER | 696 | ||
| STEM CELL DONOR SELECTION | 698 | ||
| Donor Types | 698 | ||
| Matched related donor | 698 | ||
| Matched unrelated donor | 699 | ||
| Cord blood donor | 700 | ||
| Mismatched related donor | 700 | ||
| Bone Marrow Versus Mobilized Peripheral Blood Stem Cells | 700 | ||
| Stem Cell Graft Manipulation | 701 | ||
| CONDITIONING REGIMEN | 701 | ||
| Myeloablative Conditioning Agents Versus Immunosuppressive Conditioning Agents | 701 | ||
| No Conditioning | 702 | ||
| Minimal Intensity Conditioning | 703 | ||
| Reduced Intensity Conditioning | 703 | ||
| Myeloablative Conditioning | 703 | ||
| GRAFT-VERSUS-HOST DISEASE AND PROPHYLAXIS | 703 | ||
| GRAFT FAILURE | 704 | ||
| DONOR CHIMERISM | 704 | ||
| Assessment of Donor Chimerism | 705 | ||
| SUMMARY | 707 | ||
| REFERENCES | 707 | ||
| Immunoglobulin Replacement Therapy for Primary Immunodeficiency | 713 | ||
| Key points | 713 | ||
| INTRODUCTION | 713 | ||
| CLINICAL APPLICATIONS | 714 | ||
| DOSAGE | 716 | ||
| Starting Dose | 716 | ||
| Dose Adjustment | 716 | ||
| ROUTES OF ADMINISTRATION | 716 | ||
| Intravenous Immunoglobulin | 717 | ||
| Subcutaneous Immunoglobulin | 717 | ||
| PATIENT MONITORING | 719 | ||
| ADVERSE EFFECTS | 719 | ||
| Acute Systemic Adverse Events During or Immediately After Immunoglobulin G Infusions | 720 | ||
| Anaphylaxis Versus Anaphylactoid Reactions | 722 | ||
| Reactions Due to Anti-immunoglobulin A Antibodies | 722 | ||
| Aseptic Meningitis | 722 | ||
| Renal Complications | 723 | ||
| Acute Hemolysis and Hemolytic Anemia | 723 | ||
| Thromboembolic Events | 724 | ||
| Infectious Complications | 724 | ||
| Reactions to Subcutaneous Immunoglobulin | 725 | ||
| SUMMARY | 725 | ||
| ACKNOWLEDGMENTS | 725 | ||
| REFERENCES | 726 | ||
| Autoimmune Disease in Primary Immunodeficiency | 731 | ||
| Key points | 731 | ||
| INTRODUCTION | 731 | ||
| DEFECTIVE TOLERANCE IN PRIMARY IMMUNODEFICIENCIES | 732 | ||
| Defects in Thymic Central Tolerance | 732 | ||
| Apoptosis in Tolerance | 737 | ||
| Regulatory T Cells and Peripheral Tolerance | 737 | ||
| Central and Peripheral B-Cell Tolerance | 738 | ||
| Contraction of the Immune Response | 740 | ||
| AUTOIMMUNE AND INFLAMMATORY COMPLICATIONS OF COMMON VARIABLE IMMUNODEFICIENCY | 740 | ||
| Autoimmune Cytopenias and Rheumatologic Complications | 741 | ||
| Enteropathy | 741 | ||
| Granulomatous Lymphocytic Infiltrative Lung Disease | 742 | ||
| Nodular Regenerative Hyperplasia and Autoimmune Hepatitis | 743 | ||
| SUMMARY | 744 | ||
| REFERENCES | 744 | ||
| Pulmonary Manifestations of Primary Immunodeficiency Disorders | 753 | ||
| Key points | 753 | ||
| INTRODUCTION | 753 | ||
| PULMONARY INFECTIOUS COMPLICATIONS OF PRIMARY IMMUNODEFICIENCY DISORDER | 754 | ||
| NONINFECTIOUS COMPLICATIONS OF PRIMARY IMMUNODEFICIENCY DISORDER | 754 | ||
| OBSTRUCTIVE AIRWAY DISEASE AND BRONCHIECTASIS | 756 | ||
| Bronchiectasis | 756 | ||
| Diagnosis | 757 | ||
| Treatment | 757 | ||
| PARENCHYMAL/INTERSTITIAL LUNG DISEASE IN PRIMARY IMMUNODEFICIENCY DISORDER | 761 | ||
| MALIGNANCY | 763 | ||
| AUTOIMMUNITY IN PRIMARY IMMUNODEFICIENCY DISORDER | 763 | ||
| SCREENING AND TESTING RECOMMENDATIONS | 764 | ||
| OTHER POINTS | 764 | ||
| Smoking Cessation | 764 | ||
| Vaccine-Preventable Lung Disease | 765 | ||
| SUMMARY | 765 | ||
| REFERENCES | 765 | ||
| Primary Immunodeficiency Masquerading as Allergic Disease | 767 | ||
| Key points | 767 | ||
| INTRODUCTION | 767 | ||
| ALL THAT ITCHES IS NOT ALWAYS ECZEMA | 768 | ||
| Pathophysiology | 768 | ||
| PRIMARY IMMUNE DEFICIENCY PRESENTING WITH NEWBORN AND INFANT ECZEMA | 768 | ||
| Human Immunodeficiency Virus | 769 | ||
| Omenn Syndrome | 769 | ||
| Pathogenesis | 769 | ||
| Diagnosis | 769 |