Orthotopic liver transplantation (LTX) has become an accepted means for the treatment of end-stage liver disease in both adults and children. The history of pediatric LTX starts with the first human attempt at transplantation by Dr. Starzl in 1963. The…
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 outcome for infants and children. Liver failure is a loss…
Portal hypertension (PHT) is a rare problem in pediatrics that can affect multiple organ systems. Prolonged increases in portal pressure can result in life-threatening complications and lead to an array of chronic morbidities. The most common causes of PHT in…
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 understanding of many of these infectious and inflammatory diseases. New…
Introduction Nonalcoholic fatty liver disease (NAFLD) has emerged as the most common form of chronic liver disease in children and has evolved into a serious public health issue. Similar to its presentation in adults, pediatric NAFLD is tightly associated with…
The liver is the central organ responsible for carbohydrate metabolism. The liver stores carbohydrates in the form of glycogen and synthesizes glucose through glycogen breakdown and gluconeogenesis. Glucose is an essential nutrient for the function of both the central nervous…
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 in 1963 by Laurell and Eriksson. The association between α1-antitrypsin…
Defects In Fatty Acid Oxidation Fatty acid oxidation (FAO) provides an important source of energy during fasting and physiologic stress, especially in childhood when glycogen stores are limited. Hepatic FAO produces ketone bodies, which are an important secondary energy source…
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 retention of substances normally excreted into the bile, such as…
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 the so-called neonatal cholestasis syndrome. Disorders of the bile ducts…