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Cholestatic Liver Diseases, An Issue of Clinics in Liver Disease, E-Book

Cholestatic Liver Diseases, An Issue of Clinics in Liver Disease, E-Book

Cynthia Levy

(2013)

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Book Details

Abstract

Dr. Levy is a key leader in the field of hepatology , and she has enlisted authors who are top experts in their fields to submit state-of-the-art clinical reviews on cholestatic liver diseases. Articles are devoted to genetic determinants of cholestasis, nuclear receptors as drug targets, drug-induced cholestasis, primary sclerosing cholangitis, therapeutic advances in primary biliary cirrhosis, overlap syndromes, autoimmune sclerosing cholangitis, secondary sclerosing cholangitis, Focus is also on familial cholestasis, allagile syndrome and other hereditary causes of cholestasis, Systemic causes of cholestasis, advances in pathogenesis and treatment of pruritus, care of the cholestatic patient, and transplantation for cholestatic diseases . Readers will come away with a full overview of the diagnosis and treatment of cholestatic liver diseases.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Clinics in Liver Disease: Cholestatic Liver Diseases i
Copyright Page ii
Table of Contents vii
CLINICS IN LIVER DISEASE xi
Preface: Cholestatic Liver Diseases xiii
Contributors iii
Chapter 1. Genetic Determinants of Cholestasis 147
INTRODUCTION 147
INHERITED CHOLESTATIC SYNDROMES 149
ACQUIRED CHOLESTATIC SYNDROMES 153
SUMMARY 156
REFERENCES 156
Chapter 2. Nuclear Receptors as Drug Targets in Cholestatic Liver Diseases 161
INTRODUCTION 161
NUCLEAR BA RECEPTOR FXR AND ITS BIOLOGY 162
FXR IN CHOLESTATIC LIVER DISEASES 165
THERAPEUTIC POTENTIAL OF FXR IN CHOLESTASIS 166
NUCLEAR XENOBIOTIC RECEPTORS PXR AND CAR AND THEIR BIOLOGY 168
PXR AND CAR IN CHOLESTATIC LIVER DISEASES 169
PXR AND CAR AS THERAPEUTIC TARGETS 169
VDR AND ITS BIOLOGY 171
VDR AND CHOLESTATIC LIVER DISEASES 171
VDR AS THERAPEUTIC TARGET 172
PPARS AND THEIR BIOLOGY 172
PPARS AND LIVER DISEASES 173
PPARS AS THERAPEUTIC TARGETS 173
GR AND ITS BIOLOGY 174
GR AS THERAPEUTIC TARGET 175
URSODEOXYCHOLIC ACID — CURRENT ANTI-CHOLESTATIC DRUG STANDARD AND ITS EFFECTS ON NRS 175
SUMMARY AND FUTURE PERSPECTIVES 176
REFERENCES 176
Chapter 3. Drug-Induced Cholestasis 191
INTRODUCTION 191
APPROACH TO THE PATIENT WITH SUSPECTED DRUG-INDUCED CHOLESTATIC LIVER INJURY 192
INCIDENCE OF CHOLESTATIC LIVER INJURY CAUSED BY DRUGS 193
RISK FACTORS 193
VANISHING BILE DUCT SYNDROME 195
THE PATHOLOGY OF DRUG-INDUCED CHOLESTASIS 195
THE PATHOPHYSIOLOGY OF DRUG-INDUCED CHOLESTASIS 198
MOST IMPORTANT DRUGS LEADING TO CHOLESTATIC LIVER INJURY 199
THE PROGNOSIS OF PATIENTS WITH CHOLESTASIS CAUSED BY DRUGS 202
REFERENCES 203
Chapter 4. Primary Sclerosing Cholangitis 211
ETIOPATHOGENESIS OF PRIMARY SCLEROSING CHOLANGITIS 211
EPIDEMIOLOGY AND NATURAL HISTORY 212
CLINICAL PRESENTATION 213
DIAGNOSIS 213
MANAGEMENT OF PATIENTS WITH PSC 216
FEATURES OF IBD IN PATIENTS WITH PSC 222
SUMMARY 222
REFERENCES 222
Chapter 5. Primary Biliary Cirrhosis: Therapeutic Advances 229
INTRODUCTION 229
MANAGEMENT 231
SUMMARY 238
REFERENCES 238
Chapter 6. Cholestatic Liver Disease Overlap Syndromes 243
CLINICAL SPECTRUM OF PBC 244
CLINICAL SPECTRUM OF AIH 246
CLINICAL SPECTRUM OF PSC 246
DEFINITION AND PREVALENCE OF OVERLAP SYNDROMES 247
DIFFERENTIAL DIAGNOSIS: IGG4 DISEASE 248
DIFFERENTIAL DIAGNOSIS: DRUG-INDUCED LIVER DISEASE 249
TREATMENT OF OVERLAP SYNDROMES 249
SUMMARY 250
REFERENCES 251
Chapter 7. IgG4-Associated Cholangitis 255
INTRODUCTION 255
IGG4-ASSOCIATED CHOLANGITIS 256
PATHOPHYSIOLOGY 256
CLINICAL FEATURES 256
DIAGNOSIS 260
NATURAL HISTORY 262
TREATMENT 263
SUMMARY 265
REFERENCES 265
Chapter 8. Secondary Sclerosing Cholangitis: Pathogenesis, Diagnosis, and Management 269
INTRODUCTION 269
COMMON CAUSES 270
CLINICAL SYMPTOMS AND DIAGNOSIS 273
RADIOLOGIC DIFFERENTIATION 273
MANAGEMENT, COMPLICATIONS, AND OUTCOMES 274
SUMMARY 274
REFERENCES 274
Chapter 9. Alagille Syndrome and Other Hereditary Causes of Cholestasis 279
INTRODUCTION 279
ALAGILLE SYNDROME 279
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS 283
BILE ACID SYNTHESIS DEFECTS 285
NIEMANN-PICK TYPE C DISEASE 286
CITRIN DEFICIENCY (CITRULLINEMIA TYPE II) 288
ARTHROGRYPOSIS-RENAL DYSFUNCTION-CHOLESTASIS SYNDROME 289
AAGENAES SYNDROME 290
NEONATAL ICHTHYOSIS-SCLEROSING CHOLANGITIS SYNDROME 290
ALPHA1 ANTITRYPSIN DEFICIENCY 291
NORTH AMERICAN INDIAN CHILDHOOD CIRRHOSIS 292
ZELLWEGER SYNDROME 293
CILIOPATHIES 293
SMITH-LEMLI-OPITZ SYNDROME 294
SUMMARY 295
REFERENCES 295
Chapter 10. Systemic Causes of Cholestasis 301
BACTERIAL INFECTIONS 301
FUNGAL HEPATITIS 304
MYCOBACTERIAL INFECTIONS 305
SYSTEMIC VIRAL INFECTIONS 305
HEPATIC SARCOIDOSIS 305
HEPATIC AMYLOIDOSIS 306
HEMOPHAGOCYTIC SYNDROME 307
SICKLE CELL DISEASE 308
LYMPHOMAS 308
SOLID ORGAN MALIGNANCIES 309
HEPATIC GRAFT-VERSUS-HOST DISEASE 309
ENDOCRINE DYSFUNCTION 310
CARDIAC DISEASE 310
SYSTEMIC AUTOIMMUNE DISEASES 311
TOTAL PARENTERAL NUTRITION 311
SUMMARY 313
REFERENCES 313
Chapter 11. Advances in Pathogenesis and Treatment of Pruritus 319
INTRODUCTION 319
STUDYING ITCH IN CHOLESTASIS 320
PATHOGENESIS OF PRURITUS 321
SUMMARY 325
REFERENCES 325
Chapter 12. Care of the Cholestatic Patient 331
INTRODUCTION 331
DIAGNOSIS 331
DIFFERENTIAL DIAGNOSIS 334
MANAGEMENT 336
SUMMARY 340
REFERENCES 340
Chapter 13. Liver Transplant for Cholestatic Liver Diseases 345
PRIMARY SCLEROSING CHOLANGITIS 345
PRIMARY BILIARY CIRRHOSIS 348
CHOLESTASIS ASSOCIATED WITH TOTAL PARENTERAL NUTRITION 349
BILIARY ATRESIA 350
ALAGILLE SYNDROME 351
MISCELLANEOUS CHOLESTATIC LIVER DISEASES 352
SUMMARY 354
REFERENCES 354
Index 361