Metabolic cirrhoses of infancy and early childhood.

Cirrhosis of the liver in infancy and early childhood is not a 
common phenomenon. However, because of the attendant morbidity and frequent mortality, 
it represents a significant diagnostic and therapeutic problem in this age 
group. This review will present briefly the metabolic cirrhoses of infancy, 
discuss currently proposed pathogenetic mechanisms and outline diagnostic techniques that may 
aid the pathologist in his approach to the problem of metabolic 
cirrhosis of the very young. Hereditary tyrosinemia, galactosemia, hereditary fructose intolerance, 
alpha1-antitrypsin deficiency, glycogenesis type IV (amylopectinosis), fibrocystic disease of the pancreas, 
Niemann-Pick disease, Gaucher's disease, mucopolysaccharidosis (Hurler-Hunter syndrome), Wolman's disease, hemochromatosis, Wilson's 
disease, cystinosis, the cerebrohepatorenal syndrome of Zellweger, Indian childhood cirrhosis, infantile 
polycystic disease of the liver and kidneys, congenital hepatic fibrosis, iatrogenic 
metabolic cirrhoses, and idiopathic congenital cirrhosis are discussed. 