Liver Failure
Pediatric liver disease is a significant cause of morbidity and mortality worldwide. Advances in diagnosis and treatment, including the successful development of transplantation, have dramatically improved the natural history and…
Liver Pathology
Functional Anatomy and Histology of the Liver The liver is composed of lobules of hepatocytes that are bracketed by vascular structures (with the central or hepatic veins) on one side…
Pediatric Cholestatic Liver Disease
Cholestasis is defined as a pathologic state of reduced bile formation or flow. Most cholestatic conditions can be classified as either obstructive or hepatocellular in origin and result in the…
Acute and Chronic Hepatitis
Hepatitis is defined as inflammatory liver injury regardless of cause. Discoveries in the field of molecular biology, microbiology, metabolism, and immunology have greatly expanded the viral “hepatitis alphabet” and our…
Biliary Atresia and Neonatal Disorders of the Bile Ducts
Children with primary disorders of the bile ducts present early in life with classic signs of prolonged conjugated jaundice, pale stools, and dark urine. They represent an important group within…
Stomas of the Small and Large Intestine
Stomas of the small and large intestine, whether temporary or permanent, are used in the management of a wide variety of benign and malignant conditions in pediatric patients. Creation of…
Abnormalities of Hepatic Protein Metabolism
Three disorders are discussed in this chapter, including α1-antitrypsin deficiency, tyrosinemia, and urea-cycle enzyme defects. α1-Antitrypsin Deficiency The description of α1-antitrypsin deficiency and its association with lung disease was reported…
Nonalcoholic Fatty Liver Disease
Nonalcoholic fatty liver disease (NAFLD) has evolved as the most common form of chronic liver disease in children and has become a serious public health issue. As in adults, NAFLD…
Neonatal Hepatitis
The term neonatal hepatitis originated in the 1950s when few causes of neonatal liver disease were identified, and pathologists recognized a characteristic histologic appearance of the neonatal liver in response…
Mitochondrial Hepatopathies: Disorders of Fatty Acid Oxidation and the Respiratory Chain
Inherited mitochondrial disorders of fatty acid oxidation (FAO) and the mitochondrial respiratory chain both affect mitochondrial oxidative phosphorylation. The respiratory chain is made up of five separate complexes in the…
