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EDITOR:
Sawsan Okasha Prof. of Pediatrics Head of Pediatric Hepatology Unit Cairo University May 2023
Pages: 199
- Language: English
- Format: PDF
- Size : 2.39 MB
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.
