Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to investigate the disorders of carbohydrate metabolism and insulin secretion as well as their correlation in patients with liver cirrhosis, we performed an oral glucose tolerance test on 30 patients with liver cirrhosis proven by history, clinical findings, liver function test, radioisotope liver scanning, ultrasonic examination, gastroscopy, barium esophagogram and liver biopsy, compared with 20 healthy controls. Blood glucose and immunoreactive insulin were determined in both groups at 60 min intervals for 180 min. Results showed marked glucose intolerance with peak value 60 min after glucose load in cirrhotic patients with normal fasting blood glucose. Plasma IRI levels were significantly higher in cirrhotic patients than in normal subjects after glucose load (P less than 0.05), especially 180 min after (P less than 0.01). Twelve of 30 cases (40%) showed an abnormal OGTT curve. Of the 12 cases seven (23.3%) showed a diabetic OGTT curve, five (16.7%) an impaired OGTT curve. While eighteen of 30 cases (60%) showed an abnormal OGIRT curve. Among the 18 cases one (3.3%) presented hypersecretic OGIRT curve, twelve (40.0%) with delayed and prolonged peak, and five (16.7%) with hypersecretic OGIRT curve and delayed, prolonged peak.
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PMID:[Observation of oral glucose-insulin releasing test in liver cirrhosis]. 267 37

Type III glycogen storage disease (GSD) is a disorder of carbohydrate metabolism caused by a deficiency of debranching enzyme. Different subtypes with different clinical pictures have been recognized. During childhood and early adulthood, the symptoms generally regress, and normal adulthood appears possible in most patients without symptoms or signs of cirrhosis. We report on an adult patient with GSD who developed endstage cirrhosis and a small hepatocellular carcinoma. She had GSD subtype IIIb, i.e., there were no signs of cardiomyopathy, myopathy, or neuropathy. She underwent a successful transplantation, representing the first case treated this way for this indication to our knowledge, and she is doing well after 1 year. Debranching enzyme activity was absent both in the liver and in the leukocytes before transplantation. The debranching enzyme activity remained absent in the leukocytes after transplantation. We conclude that patients with GSD type III may develop end-stage cirrhosis and hepatocellular carcinoma and therefore need hepatological follow-up during adulthood.
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PMID:Type IIIb glycogen storage disease associated with end-stage cirrhosis and hepatocellular carcinoma. The Liver Transplant Group. 904 94