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EDITOR:

Sawsan Okasha Prof. of Pediatrics Head of Pediatric Hepatology Unit Cairo University May 2023

Pages:  199                                                                                                                                 

: CONTENTS

  CHAPTER 1: INTERPRETATION OF LIVER FUNCTION TESTS 10 

CHAPTER 2: THE JAUNDICED BABY 14 

Unconjugated Hyperbilirubinemia 14 

o Breast Milk Jaundice 14 

o Crigler Najjar Syndrome 15 

o Other Hyperbilirubinemic Syndromes 18 

 Approach to the Infant with Cholestasis 20

 - Management of Neonatal Cholestasis 26 

- Biliary Atresia 32 - Choledochal Malformation 39

 - Alagille Syndrome 41 -

 Cytomegalovirus Infection 42 

- Progressive Familial Intrahepatic Cholestasis 44

 - Bile Acid Synthesis Defects 46

CHAPTER 3: MONOGENIC LIVER DISEASES 47 

- Glycogen Storage Diseases 49

 - Galactosemia 55

 - Niemann-Pick Disease 57

 - Hepatorenal Tyrosinemia 59 

- Cystic Fibrosis 62 - 

Alpha-1-Antitrypsin Deficiency 65 

- Wolman Disease and Cholesterol Ester Storage Disease 65 

- Citrin Deficiency 66

CHAPTER 4: ACUTE HEPATITIS 67 - 

Hepatitis A Viral Infection 67 

CHAPTER 5: PEDIATRIC ACUTE LIVER FAILURE 72 

- Acetaminophen Overdose 80 

 Guidelines for Pediatric Plasma Transfusion 85 

CHAPTER 6: CHRONIC LIVER DISEASE 86 -

 Hepatitis B Viral Infection 91 -

 Hepatitis C Viral Infection 95 -

 Post-Exposure Management of Needle Stick Injury 99 - 

Wilson Disease 101 -

 Autoimmune Hepatitis 112 -

 Non-Alcoholic Fatty Liver Disease 117 -

 Fibrocystic Liver Disease 123 -

 Causes of Biliary Tract Disorders 126

 CHAPTER 7: HEPATIC ENCEPHALOPATHY 127 -

 Nutritional Guidelines in Cirrhotic Patients with Hepatic Encephalopathy 133

 CHAPTER 8: ASCITES 135 

CHAPTER 9: SPONTANEOUS BACTERIAL PERITONITIS 142 

 CHAPTER 10: PORTAL HYPERTENSION 145 -

 Prehepatic (Extrahepatic) Portal Hypertension 147

 CHAPTER 11: PEDIATRIC GALLSTONES 152 

CHAPTER 12: LIVER ABSCESS 154  

CHAPTER 13: NUTRITIONAL ASSESSMENT AND MANAGEMENT OF PEDIATRIC LIVER DISEASES 157 

CHAPTER 14: LIVER INVOLVEMENT IN SYSTEMIC DISEASES 163 -

 Bacterial Sepsis 163 -

 Cardiovascular Disease and Circulatory Failure 164 -

 Collagen Vascular Diseases 165 -

 Endocrine Disorders 166 -

 Hematological Diseases, Coagulalopathies and Malignancies 167 -

 Gastrointestinal Diseases 168 -

 Nutritional Disorders 169 -

 Immunodeficiency Disorders 170 -

170 COVID-19 

 CHAPTER 15: LIVER TRANSPLANTATION 171 

CHAPTER 16: PEDIATRIC LIVER IMAGING 177

 CHAPTER 17: LIVER BIOPSY 180

LIST OF ABBREVIATIONS 186

BIBLIOGRAPHY 192

Preface

Nearly two decades have elapsed since the first edition of this book. Major advances have occurred in the diagnosis of pediatric liver diseases, particularly in the field of genetic disorders. With the advances in next generation sequencing techniques, many monogenic pediatric liver disorders are becoming precisely diagnosed, as several of them may have common clinical and biochemical presentations especially in the field of cholestatic disorders of infancy. These advances revealed a need for adding a chapter on “Monogenic Liver Diseases” and to stress in the chapter on “Acute Liver Failure” on the need to reach a molecular diagnosis lest the genetic cause may be a contraindication to liver transplantation. 

This guide has always been directed to the General Pediatrics residents, as a source of practical knowledge that enables them to manage their pediatric patients with liver disease. This guide does not elaborate on theoretical knowledge but is rather a practical guide for diagnosis and management of pediatric liver diseases, emphasizing the clinical presentation, diagnosis and management of these conditions. This inspired us to write detailed management of: e.g. post-Kasai portoenterostomy fever, acute liver failure (with special stress on acetaminophen overdose), hepatic encephalopathy, gastrointestinal bleeding, ascites, spontaneous bacterial peritonitis, plasma transfusion, immunization strategies in special situations and liver involvement in systemic disorders. We hope that this guide will be of help to adult hepatologists to whom our pediatric liver patients will be transitioned in the future; as they have to be acquainted with these disorders that present in early life, but thanks to proper management, these infants and children are now surviving to adult life.