Abstract
37 year-old man in early stage of primary biliary cirrhosis suffering from prolonged jaundice and generalized itching was reported in this paper.
After the treatment of D-Penicillamine, jaundice and liver function tests were markedly improved, and the copper contents in hepatocytes of liver tissue were significantly decreased.
In three months before entry he experienced icterus and itching on the skin. On the day of admission, the liver was palpable 3cm below the right costal margin, total bilirubin was 8.4mg/dl, serum GOT was 45, GPT was 26 and alkaline phosphatase was 15.3 U (K.A.). After entry jaundice and serum alkaline phosphatase and ceruloplasmin levels were increased, and anti-mitochondrial antibody was positive.
Laparotomic investigation disclosed the precirrhotic stage of the liver without the extrahepatic obstruction of bile ducts and liver biopsy specimens showed the early stage of primary biliary cirrhosis with massive deposits of copper granules in most hepatocytes. Electromicroscopical studies revealed proliferation and enlargement of endoplasmic reticulum, dilatation of intracristal space in mitochondria, and dilatation of bile canalicula with amorphous substances and shortening & reduction of microvilli.
