[Acute intermittent porphyria--an autopsy case].
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Biomedical subjects
Publications and source records attributed to S Toyoda.
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The activities of several glycosidases (beta-galactosidase, beta-glucosidase, N-acetyl-beta-glucosaminidase) were demonstrated in human bile. The enzyme activities are increased about 100 times after exclusion of bile salts and other small molecular compounds by Sephadex G-50 gel filtration. The use of 4-methylumbelliferyl derivatives as substrates was useful as measurement of the bile enzyme activities are not altered in the presence of bile pigments. Enzyme characteristics of bile glycosidases were determined: pH optimum and isoelectric point. The bile glycosidase activities were also measured in various hepatobiliary disorders (cholelithiasis, cancer of gallbladder, acute hepatitis, liver cirrhosis and fatty liver). The glycosidase activities in bile from patients with liver diseases, as well as with cholelithiasis, were generally decreased. Isoelectric focusing patterns of biliary glycosidases were similar for specimens from patients with hepatobiliary disorders as compared to normal.
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Resection of metastatic liver cancer in a patient with an anomalous intrahepatic portal system is presented. A 67-year-old man was referred to our hospital with a liver tumor. Percutaneous transhepatic portography demonstrated that the right anterior branch of the portal vein rose from the left portal vein with agenesis of the umbilical portion. Cholangiography disclosed obstruction of the right posterior segmental bile duct by the tumor. Barium enema demonstrated a Borrmann type II cancer in the sigmoid colon. Right hepatic lobectomy and high anterior resection of the colon were performed, and postoperative histologic examination confirmed that the liver tumor was metastatic adenocarcinoma of the colon. The ligamentum teres arose from the right anterior portal vein and separated the left medial segment from the right anterior portal segment of the liver. These anomalies led to the performance of right lobectomy instead of trisegmentectomy and the patient died 10 months later of hepatic recurrence. This case emphasizes the importance of delineating the hepatic vascular anatomy before liver resection and illustrates one of the pitfalls related to portal venous anomalies.