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…
The term neonatal hepatitis originated in the 1950s when few causes of neonatal liver disease were identified, and pathologists recognized a characteristic histological appearance of the neonatal liver in response to injury. The term has since been used to refer…
Introduction This chapter summarizes how the hepatobiliary system develops, from a sheet of undifferentiated cells at 3 weeks’ gestation to an organ capable of performing a myriad of metabolic, synthetic, and detoxifying functions weeks later. The chapter divides hepatobiliary development…
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 and the portal tracts (with portal veins and hepatic arteries)…
Optimal interpretation of endoscopic gastrointestinal biopsy specimens requires adequate clinical information including medication history and illnesses that may have associated gastrointestinal findings. The clinical history can guide additional studies in addition to the standard hematoxylin and eosin staining. Precise identification…
The gastrointestinal (GI) tract has evolved specific mechanisms to allow ingestion of nutrients, their transport for digestion and absorption, and finally the expulsion of unused portions. This aboral propulsion of gastrointestinal contents is orchestrated by the complex interaction between the…