Reversal of hepatic alpha-1-antitrypsin deposition after portacaval shunt.
End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 3 1/2 to almost 7 years. Postoperative liver biopsy material from two of the patients showed the typical histopathological changes caused by portal diversion, as well as an apparent reduction in the quantity of alpha-1-antitrypsin particles in the hepatocytes. The metabolic changes caused by portal diversion have apparently created a more favourable equilibrium between the synthesis and excretion of the abnormal alpha-1-antitrypsin.
D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000601:surgery; D007223:Infant; D008099:Liver / Q000201:enzymology* / Q000473:pathology; D008107:Liver Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery; D008111:Liver Function Tests; D008297:Male; D010641:Phenotype; D011167:Portacaval Shunt, Surgical; D019896:alpha 1-Antitrypsin Deficiency