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)

Hepatomegaly is an important clinical finding in patients with argininosuccinic aciduria (a hereditary defect of the urea cycle enzyme, argininosuccinate lyase [argininosuccinase]). A severe degree of liver fibrosis, almost corresponding to cirrhosis, was observed in liver biopsy material obtained from a boy with this disorder. This observation is of interest in light of the fact that liver fibrosis or cirrhosis are hallmarks of many inheritable phenotypes, and especially of inborn errors of metabolism. Variable degrees of liver fibrosis are noted in other inborn defects of the urea cycle, eg, in ornithine transcarbamylase and carbamoylphosphate synthetase deficiencies. These findings appear to indicate that inheritable defects of urea synthesis may form a group of metabolic disorders prone to cause hepatic fibrosis, or even cirrhosis, as shown in our patient.
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PMID:Severe liver fibrosis in argininosuccinic aciduria. 375 45

A few cases of alcohol-like chronic liver disease have recently been described in patients on high daily doses of amiodarone (400-600 mg) for 5-24 months. We report here 3 cases in which similar lesions were observed after low daily doses of amiodarone. Daily dosage was 200 mg in 2 cases, 400 mg then 200 mg in the third one, and duration of therapy ranged between 36 and 60 months. Symptomatology was limited to hepatomegaly and/or mild elevation of serum aminotransferases. Liver biopsy showed alcohol-like lesions with fibrosis in 2 cases and cirrhosis in 1 case. Electron microscopy disclosed hepatic phospholipidosis. These cases indicate that chronic liver disease may be observed even when amiodarone is prescribed at low daily dosage, provided that duration of treatment is long. They also suggest that rather than the daily dosage, the cumulative dose of amiodarone is a major factor in the development of hepatic injury.
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PMID:Chronic liver disease after low daily doses of amiodarone. Report of three cases. 379 3

78 hospitalized patients were selected when presenting with at least one of these signs: hepatomegaly, jaundice, ascites, oesophageal varices, abdominal venous pattern, splenomegaly. All had radioimmunoassays for hepatitis B surface antigen (HBsAg) and antidelta antibody (78/78). Acute or chronic hepatic disease was diagnosed in 56 patients: 7 acute viral hepatitis, 13 chronic hepatitis, 23 non alcoholic hepatic cirrhosis, and 13 hepatocellular carcinoma. Twenty-two patients with other diagnoses served as controls. Serum antidelta was present in each group: acute viral hepatitis (2/7), chronic hepatitis (2/13), non alcoholic hepatic cirrhosis (9/23), hepatocellular carcinoma (3/13), controls (2/22). Every patient with acute or chronic hepatic disease and positive serum anti-delta was positive for serum HBsAg. Amony controls, 2 patients with positive serum antidelta were negative for serum HBsAg but positive for antiHBs. Delta superinfection is present in the sahelian region; Patients with acute viral hepatitis, chronic hepatitis, non alcoholic hepatic cirrhosis, and hepatocellular carcinoma are electively infected. Patients with acute or chronic hepatitis and positive serum antidelta have hepatitis B virus evolutive infection (positive serum HBsAg).
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PMID:[HB virus infection and delta surinfection in Sahelian Africa]. 380 84

To assess the spectrum of hepatic abnormalities in acquired immune deficiency syndrome (AIDS), we reviewed clinical, biochemical, and pathological material in 32 patients with AIDS. Eight-four percent of AIDS cases had a history of intravenous drug abuse. Ninety percent of AIDS patients has some liver biochemical abnormality at the first presentation of illness. During the course of AIDS, significant (p less than 0.05, paired Student's t test) rises in alkaline phosphatase and bilirubin occurred, without rises in aminotransferases. Mean abnormalities were mild, reflecting approximately 2-fold increases over baseline. Liver failure was not believed to contribute to the death of any AIDS patient. Pathological findings in AIDS included specific infectious diagnosis in 26%, granulomas in 16%, hemosiderosis in 26%, nonspecific abnormalities in 39%, cirrhosis in 23%, and chronic active hepatitis in 3%. AIDS cases were also compared to 10 selected age, sex, and epidemiologically similar non-AIDS patients. Although granulomas or infections were not seen in our comparison group, only the incidence of chronic active hepatitis was significantly different between the groups. If only those with intravenous drug abuse were studied, then none of 24 AIDS patients versus four of eight non-AIDS cases (p less than 0.005) had chronic active hepatitis. AIDS patients with specific hepatic infections tended to have a higher alkaline phosphatase and aspartate aminotransferase (p less than 0.05) than noninfected cases. However, substantial overlap existed, and no difference in hepatomegaly was noted. Ninety percent of AIDS patients were ingesting at least one potentially hepatotoxic drug. We conclude that AIDS patients have a high incidence of underlying hepatic abnormalities. However, clinical and biochemical abnormalities are similar in our selected liver biopsy patients with intravenous drug abuse with or without AIDS. As expected, AIDS patients have a higher incidence of hepatic granulomas and infections, but these patients were not clearly distinguishable from other AIDS cases. Histological examination showed a wide array of changes by light microscopy, but no specific lesion of AIDS was noted. The low incidence of chronic active hepatitis in this AIDS population may imply that the altered T lymphocyte function in AIDS could influence the course of liver disease in these patients.
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PMID:The liver in acquired immune deficiency syndrome: emphasis on patients with intravenous drug abuse. 382 29

A 54-year-old woman with obesity, type II diabetes mellitus, hyperlipidemia, and massive hepatomegaly was found to have severe steatosis and cirrhosis on liver biopsy. Complete evaluation led to the diagnosis of fatty cirrhosis associated with obesity and diabetic mellitus. She underwent four months of fasting with a protein-carbohydrate and vitamin-mineral liquid supplement to control her weight and metabolic abnormalities and to evaluate the effect of this diet on her liver disease. She lost 40 pounds to ideal body weight, normalized her serum glucose and lipids, and decreased total liver height by one third. Liver biopsy at the completion of her diet showed inactive cirrhosis and complete resolution of steatosis. Supplemented fasting with only modest weight loss can safely resolve fatty liver in obese diabetics with nonalcoholic steatosis and cirrhosis. Aggressive dietary approaches to achieve long-term weight loss deserve study in this subgroup of diabetics with unexplained chronic liver disease.
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PMID:Steatosis and cirrhosis in an obese diabetic. Resolution of fatty liver by fasting. 382 84

Alpha-1-antitrypsin is a blood glycoprotein synthesized in the liver. It is a protease inhibitor of the serpine group and has a specific action for elastase. Alpha-1-antitrypsin electrophoresis shows about 20 phenotypes, the normal one being PiM. The allele PiZ is usually responsible for liver or lung disease in children or adults, respectively. Eleven per cent of PiZZ infants present with prolonged neonatal cholestasis. Twenty-five of 45 PiZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 PiZZ children with cirrhosis died during childhood.
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PMID:[Alpha 1-antitrypsin deficiency in childhood]. 387 73

This prospective study was carried out in order to assess the accuracy of ultrasound in the diagnosis of cirrhosis. One hundred and twenty eight alcoholic patients were included. A careful ultrasonographic examination of the liver was performed before liver biopsy (100 patients). In 15 cases, liver histology was normal, steatosis and/or fibrosis, cirrhosis were diagnosed in 13 and 72 cases respectively. Ultrasonic patterns were classified by the same examiner, according to several criteria: volume, irregular outline, coarse and fine bright echo pattern, attenuation of the ultrasound beam, splenomegaly, ascite, portal hypertension. Cirrhosis was diagnosed in 58 out of 72 patients (80.5%). Specificity was 78.5%, positive and negative predictive values were 90.6% and 61% respectively, and global efficacy was 80%. Irregular outline (0.66), hepatomegaly (0.66) and attenuation of the ultrasound beam (0.64) were the best signs. In case of fine bright echo pattern, the diagnosis of cirrhosis would be missed. The results suggest that ultrasonography is a good test for screening alcoholics for cirrhosis. Therefore, it is useful when liver biopsy is contra indicated or refused or when liver is not detected at the clinical examination.
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PMID:[Value of ultrasonography in the diagnosis of cirrhoses. Prospective study of 128 patients]. 391 96

An outbreak of polychlorinated biphenyl (PCB) poisoning from the consumption of contaminated rice oil, covering four counties in central Taiwan, was investigated. There were 1843 cases by the end of 1980. The highest frequency of incidence occurred during the period from March to July 1979. The severity of clinical manifestations varied. Most patients showed symptoms of mild or moderate severity. The major age group affected was between 11 and 20 years old. Most of the victims were students and factory workers. The amount of PCB intake in each victim was estimated to be 0.7 to 1.84 g and the latent period from the time of intake to the onset of clinical manifestations was approximately 3 to 4 months. The patients' blood PCB concentrations ranged from 3 ppb to 1156 ppb; 44.27% of 613 patients had levels of 51 to 100 ppb and 27.6% PCB blood levels over 100 ppb. In the course of 3.5 years, 2061 persons were determined to be PCB poisoning victims. Now, except for a few severe cases, their skin symptoms are very much improved. Thirty-nine babies showing hyperpigmentation were born from PCB-poisoned mothers. The fatality rate was high: eight of them died. Another 24 deaths were reported among the PCB-poisoned group, almost half of them (12) from hepatoma, liver cirrhosis or liver diseases with hepatomegaly.
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PMID:Discovery and epidemiology of PCB poisoning in Taiwan: a four-year followup. 392 64

In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.
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PMID:Liver disease associated with anti-liver-kidney microsome antibody in children. 395 Aug 19

Amiodarone has proved very effective in the treatment of otherwise resistant cardiac tachyarrhythmias. The use of amiodarone has, however, been limited due to its serious side-effects. A patient with cholestatic hepatitis due to amiodarone treatment is presented below and a review of the hepatotoxicity of amiodarone is given. It is concluded that solid evidence exists of hepatic injury due to amiodarone treatment, including steatosis, alterations resembling alcoholic hepatitis, cholestatic hepatitis and micronodular cirrhosis of the liver. Patients receiving amiodarone should be regularly screened with respect to hepatic enzyme levels. Therapy should be discontinued on the suspicion of cholestatic injury or hepatomegaly.
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PMID:Hepatotoxicity of amiodarone. 396 37


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