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)

Forty-one children with liver disease were studied by ultrasound scan at King Khalid University Hospital, Riyadh, Saudi Arabia. Diagnoses were confirmed either by liver biopsy or specific laboratory tests. Sonograms were studied for liver size, beam penetration, echogenicity, vascularity, and biliary tree abnormalities. Different liver diseases, such as chronic hepatitis, biliary cirrhosis, Wilson's disease, familial idiopathic cirrhosis, type III glycogen storage disease, and secondary haemochromatosis revealed non-specific disease patterns. Four cases of biliary cirrhosis and two cases of glycogen storage disease showed periportal fibrosis. Two cases of familial idiopathic cirrhosis and a case of Wilson's disease revealed thickening of the gall bladder wall, which has not been described in the literature.
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PMID:Ultrasound findings in childhood chronic liver parenchymal diseases. An analysis of 41 patients. 332 32

The clinical picture and laboratory data of five symptomatic and two asymptomatic cases of Wilson's disease in four unrelated Saudi families are reported. More than one member was affected in two families. The oldest and youngest patients were twenty and eight years respectively. The two oldest patients were siblings and presented with neurological disturbance only while the youngest two were unrelated and had predominantly hepatic involvement. A mixture of hepatic cirrhosis and extrapyramidal syndrome was encountered in one patient only and Kayser-Fleischer rings in three. The initial presentation of one patient who subsequently developed cirrhosis closely simulated subacute glomerular nephritis. Although only three patients showed clinical or biochemical evidence of liver disease either initially or in later stages, liver biopsy demonstrated a spectrum of morphological changes in all the seven cases. These findings indicate that a positive family history, Kayser-Fleischer rings and simultaneous hepatic and brain involvement need not be present and that subclinical hepatic involvement may be commoner than is generally realised in Wilson's disease. That seven cases were seen at one centre, which takes a largely unselected patient population, in a four-year period only, suggests that this preventable and treatable disease may be frequent in Saudi Arabia.
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PMID:Protean manifestations of Wilson's disease: a review of seven Saudi patients. 340 2

One-hundred-and-ten children between the ages of two months and 14 years with the following liver diseases were studied: 16 with acute viral hepatitis, 8 with persistent chronic hepatitis, 31 with active chronic hepatitis, 5 with hepatic steatosis, 11 with cirrhosis of the liver, 24 with newborn cholestasis, 3 with Wilson's disease, 2 with congenital hepatic fibrosis, 5 with metabolic diseases and 5 due to other causes. These children presented Pi system phenotypes in isoelectric focus using ultrafine polyacrylamide gels according to Kuepper's method, with modifications incorporated to determine Alpha-1-antitrypsin (A1-AT) serum level deficiencies in those presenting the Pi ZZ phenotype, a liver biopsy with P.A.S. coloration on digestion of diastase and a family history of the phenotype. Four (3.6%) of the children with Pi ZZ phenotypes showed a decrease of serum A1-AT and the presence of positive P.A.S. inclusions resistant to diastase in the cytoplasm of hepatocytes. Three had a history of postnatal icterus and the fourth presented hepatomegaly. The phenotypic study of the parents showed their being heterozygous (MZ), while siblings were normal (MM). The importance of the diagnosis of A1-AT deficiency and the diagnostic value of detecting Pi system phenotypes in every case of liver disease in children and adolescents is stressed.
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PMID:[The value of the Pi system phenotype in alpha 1-antitrypsin deficiency]. 349 88

One hundred seventy orthotopic liver transplants were performed under conventional immunosuppression with azathioprine and steroids with 1- and 5-year survivals of 32.9 per cent and 20.0 per cent, respectively. Since the introduction of cyclosporine-prednisone therapy in March 1980, 313 primary orthotopic liver transplants have been performed. Actuarial survivals at 1 and 5 years have improved to 69.7 per cent and 62.8 per cent, respectively. Biliary atresia is now the most common indication for liver replacement. In adults, primary biliary cirrhosis and sclerosing cholangitis have become more common indications for transplantation, and alcoholic cirrhosis and primary liver malignancy as indications have declined. Early enthusiasm for liver transplantation in patients with hepatic cancer has been tempered by the finding that recurrence is both common and rapid. An increasing number of patients with inborn errors of metabolism originating in the liver are receiving transplants, including patients with Wilson's disease, tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage disease, familial hypercholesterolemia, and hemochromatosis. Survival in this group of patients has been excellent (74.4 per cent at 1 and 5 years). A hemophiliac who received a transplant for postnecrotic cirrhosis has survived and may have been cured of his hemophilia. About 20 per cent of patients require retransplantation for rejection, technical failure, or primary graft failure. Only four of the patients receiving retransplants under conventional immunosuppression survived beyond 6 months, and all died within 14 months of retransplantation. Sixty-eight patients have received retransplants under cyclosporine-prednisone. Thirty-one patients are surviving, all for at least 1 year. Six of the twelve patients requiring a third transplant are alive 2 to 3 years after the primary operation. An aggressive approach to retransplantation in the patient with a failed graft is justified.
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PMID:Indications for liver transplantation in the cyclosporine era. 352 Aug 95

Liver cirrhosis is relatively rare in children as compared to adults; frequently it is diagnosed too late. Biliary cirrhosis of early childhood is often the result of neonatal cholestatic syndromes. Beyond infancy, cirrhosis as a consequence of chronic active autoimmune hepatitis or of Wilson's disease may be prevented, if causal therapy is begun in time. Hence paediatricians should exclude both diseases in all children with elevated transaminases and clinical features of a liver disorder.
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PMID:[Liver cirrhosis in childhood--etiology, diagnosis and conservative therapy]. 353 72

Although copper is believed to be hepatotoxic in Wilson's disease and Indian Childhood Cirrhosis (ICC), the rat shows only minimal hepatic damage on copper-loading. To investigate the possibility that copper deposition may potentiate the effects of a superimposed hepatitis, D-galactosamine (GalN) was given to copper-loaded and control rats. In the non-copper-dosed rats, GalN 0.85 g/kg i.p. produced elevated serum AST (3731 +/- 545 IU/l; normal 64.8 +/- 2.1), ALT (2090 +/- 190 IU/l; normal 18.0 +/- 0.7), and OCT (16.7 +/- 2.6 mmol/min/ml; normal 0.12 +/- 0), and liver cell necrosis with portal infiltration. In rats whose liver copper was elevated to 1298 +/- 169 micrograms/g (control 18.7 +/- 1.7) by oral copper supplementation, GalN produced much smaller increases in AST (825 +/- 122 IU/l), ALT (103 +/- 15 IU/l) and OCT (0.27 +/- 0.02 mmol/min/ml) and minimal histological damage. Viable bacterial cell counts from faecal homogenates showed that the anaerobically cultured bacteria were reduced on copper-dosing of rats. Therefore the protective effect of copper may be due to a decrease in gut-derived endotoxin acting on the liver, or to an impaired prostaglandin synthesis or perhaps to synthesis of acute phase reactants.
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PMID:Copper protects against galactosamine-induced hepatitis. 365 8

Consumption of the hepatotoxin arsenic is very common in certain geographical areas of India and occurs as a result of the intake of arsenic contaminated water, vegetables, adultered opium, ayurvedic and indigenous medicines, and "home made brew". Arsenic levels were estimated in livers obtained after autopsy from patients of idiopathic cirrhosis, alcoholic cirrhosis, Indian childhood cirrhosis, non-cirrhotic portal fibrosis, fulminant hepatitis and Wilson's disease. Significantly increased levels of arsenic were found in all diseased livers investigated when compared with values obtained from control groups. The study suggests that elevated levels of arsenic may be associated with liver disease.
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PMID:Arsenicosis in India. 366 14

Thirty-seven children with beta-thalassaemia major, eight children with liver cirrhosis, and 20 matched controls were enrolled in this study. Serum ferritin was determined in each subject by radio-immunoassay and liver enzymes by standard methods. The liver, spleen, kidney and pancreas densities were obtained by computed tomography using a Siemens Somatom 2 Scanner with 8-mm slice thickness. The iron content of liver biopsies from 10 patients was graded by staining. The mean serum ferritin of the thalassaemic patients was significantly higher than that of the control group (p = 0.0001). The ferritin of patients with cirrhosis and Wilson's disease was similar to that of the control group. The liver density of the thalassaemic patients was significantly higher than that of the control group (p less than 0.0001) while that of patients with liver cirrhosis and Wilson's disease was similar to the control group. The liver iron content of patients with liver cirrhosis was within the normal range. The spleen and kidney densities of patients with thalassaemia were higher than that of the control group with p values of 0.02 and 0.056, respectively. The density of the pancreas in patients with thalassaemia was not significantly different from that of the control group, (p = 0.52). There was correlation between the liver density and serum ferritin in patients with thalassaemia (r = 0.432, p less than 0.01) while there was no correlation between spleen, pancreas and kidney densities with serum ferritin.
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PMID:Comparison between serum ferritin and computed tomographic densities of liver, spleen, kidney and pancreas in beta-thalassaemia major. 368 72

The clinical and biochemical findings in two siblings with Wilson's disease are described. One of them, an 11-year-old girl, developed acute liver failure terminating in death within a few weeks. Prior to her terminal illness she had been in good health without symptoms suggestive of Wilson's disease. Copper contents of urine, liver, kidney and brain were 20-100 times above the upper normal limits. The liver showed extensive micronodular cirrhosis with nonbile pigment deposits. Her 15-year-old brother had abnormal liver function tests with urinary copper excretion 20 times above the upper normal limit. Treatment with penicillamine was started. Following a short period of deterioration his condition has steadily improved.
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PMID:Wilson's disease in two siblings--one with fatal outcome. 370 7

A 22-year-old female with Wilson's disease became pregnant and subsequently delivered a normal infant. At the age of 17 the patient presented evidence of liver cirrhosis and was diagnosed as having Wilson's disease. A regimen of d-penicillamine was started at this time and continued, although irregularly for the 10 months prior to her 9th week of pregnancy. At the 14th week of pregnancy she was started on 500 mg of D-penicillamine a day. She was stable during the remainder of her pregnancy and after delivery. Fetal development was normal and a normal infant was delivered at 35 weeks. It is suggested that pregnancy is not contraindicated in well treated Wilson's disease, and that a regimen of D-penicillamine during pregnancy can control the illness without harming the fetus.
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PMID:Pregnancy and penicillamine treatment in a patient with Wilson's disease. 371 63


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