Reversibility of extensive liver damage in galactosemia.
An infant with galactosemia is reported in whom extensive liver damage developed by 1 month of age. Liver biopsy obtained prior to treatment indicated extensive periportal and intralobular fibrosis, ductular cysplasia. "pseudoglandular" transformation, and distortion of periportal vasculature. Three months after institution of a galactose-free diet, clinical and biological evidence of liver disease disappeared, and follow-up biopsy at 5 months of age showed normal hepatic histology. These findings demonstrate that functional and histological abnormalities consistent with cirrhosis can be completely reversed by dietary management in galactosemia.
D001707:Biopsy, Needle; D005260:Female; D005693:Galactosemias / Q000150:complications / Q000178:diet therapy* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000178:diet therapy*