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)

From 1976 to 1983, 28 patients (24 male and four female) with unresectable hepatocellular carcinoma (HCC) were treated by intraarterial chemotherapy at the Istituto Nazionale Tumori of Milan, Milan, Italy. Tumors were retrospectively classified by a previously proposed staging system. Two patients were classified as Stage I and 26 as Stage II. Liver cirrhosis was present only in the males (in 50% of them). Nineteen patients were treated with doxorubicin (Adriamycin [Adria Laboratories, Columbus, OH]) and nine with 5-fluorouracil. Systemic toxicity was mild, but the treatment induced hepatic toxicity (ascites, clinical jaundice, or biochemical impairment) in 18% of noncirrhotic and 66% of cirrhotic patients. Clinical reduction of hepatomegaly was observed in 50% of noncirrhotic versus 16% of cirrhotic patients. Doxorubicin was effective in 66% of noncirrhotic patients and 20% of cirrhotic patients, with an overall response rate of 42%. 5-fluorouracil was effective only in patients without cirrhosis, with an overall response rate of 22%. Overall median actuarial survival was 3.5 months, with a significant difference between noncirrhotic and cirrhotic patients (6 versus 2 months, respectively). Overall median survival of patients who responded to the treatment was 13 versus 2 months for nonresponders (P less than 0.001). Liver cirrhosis was the most important prognostic factor in terms of liver toxicity, response rate, and survival. This study emphasized the negative impact of the treatment on cirrhotic patients. Also, the real value of intraarterial administration of doxorubicin was investigated.
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PMID:Intrahepatic chemotherapy for unresectable hepatocellular carcinoma. 283 36

The pathologic features, clinical presentation and natural history of hepatocellular carcinoma (HCC) developing in the noncirrhotic liver were studied in 61 patients against a background of 63 patients seen concurrently with HCC complicating cirrhosis. Noncirrhotic HCC had a bimodal age distribution, with females predominating the first age-clustering (10-50 years) and males predominating the second age-clustering (50-90 years). Cirrhotic HCC had a unimodal age distribution (40-90 years) with male dominance throughout. Estrogen exposure was noted in 57% of the noncirrhotic HCC women overall and in 80% of those in the younger age-clustering. The majority of noncirrhotic HCC presented with a single hepatic mass or a dominant primary with satellite lesions in contrast to the usual multinodular or diffuse disease seen with cirrhosis. Twenty-nine noncirrhotic patients survived complete resection of disease limited to the liver and exhibited a median survival of 2.7 years with a 5-year survival of 25%. Low histologic grade, minimal necrosis, and the absence of hemoperitoneum, hepatomegaly, and adjacent organ involvement were all favorable prognostic variable. Patients with metastatic or locally unresectable noncirrhotic HCC had a median survival of 9 months, and 24% survived in excess of 2 years. This survival experience is significantly more favorable than cirrhotic HCC patients, who had only a 1.2-month median and a 3% 2-year survival. Low histologic grade, mild mitotic activity and the presence of some fibrosis within the specimen were associated with a favorable outcome in advanced noncirrhotic HCC. The favorable prognosis and heterogeneous composition of the noncirrhotic, when compared to the cirrhotic HCC cohort, may be important considerations in the design and evaluation of future clinical trials.
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PMID:Hepatoma in the noncirrhotic liver. 284 80

From October 1982 to June 1985 158 hospitalized patients in the National Hospital of Niamey, Republic of Niger, were selected whenever one of the following signs was found: hepatomegaly, jaundice, ascites, oesophageal varices, abdominal venous pattern, or splenomegaly. Investigations included hepatic echography (158/158), needle liver biopsy (68/158), radioimmunoassays for serum hepatitis B surface antigen (HBsAg; 158/158), anti-HBs (152/158), anti-HBc (129/158) and anti-delta antibody (anti-HD; 158/158). 112 patients with liver diseases comprised 28 with chronic hepatitis, 55 with non-alcoholic hepatic cirrhosis, and 29 with hepatocellular carcinoma (HCC). 46 patients with other diagnoses were used as controls. 71/112 liver disease patients were positive for HBsAg in serum compared with 1/46 controls (P less than 10(-9)). Prevalences of delta superinfection in patients with serum HBsAg (+) and anti-HD (+) were 45/112 (40.2%) in liver disease patients versus 1/46 (2.2%) in controls (P less than 10(-9)). Delta superinfection was very frequent in chronic hepatitis (8/28), non-alcoholic cirrhosis (24/55) and HCC (14/29). In chronic hepatitis, delta superinfection was more frequent in the chronic active form than in the chronic persistent type (not significant). Cirrhosis patients with delta superinfection were younger (10 years in males, 11 years in females) than those without (P less than 0.05).
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PMID:Delta superinfection in patients with chronic hepatitis, liver cirrhosis and hepatocellular carcinoma in a Sahelian area. Study of 112 cases versus 46 controls. 284 9

A rare chronic course of Budd-Chiari syndrome associated with thrombosis of the portal vein was observed in a 30-year-old male patient suffering from postmyocarditic cardiosclerosis. At the age of 24 the patient had infectious allergic myocarditis, was hospitalized and rehospitalized for circulatory insufficiency. Upon 3 years since the disease onset the patient was admitted to a hematological department for progressive enlargement of the spleen. The diagnosis on discharge was idiopathic myelofibrosis with portal hypertension. The treatment included prednisolone, blood transfusions, myelosan. In 1987 the patient presented with enlarged liver and spleen, ascites, gastric and esophageal varicosis, augmenting hepatic insufficiency clinically evaluated as hepatic cirrhosis. Postmortem examination revealed macrofocal cardiosclerosis, splenomegaly, ascites, portal varicosis, enlarged nutmeg liver with smooth surface. Microscopically there was phlebosclerosis and phlebothrombosis varying in duration and involving predominantly medial branches of the hepatic and portal veins, liver fibrosis. The findings provided evidence for the final diagnosis of Budd-Chiari syndrome running an uncommon chronic course.
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PMID:[The chronic form of the Budd-Chiari syndrome]. 297 4

In this prospective study of 240 black patients with liver enlargement admitted to the medical wards of King Edward VIII Hospital, Durban, a cause for the hepatomegaly was found in 92.5% of cases (63.8% without recourse to biopsy, 28.7% after liver biopsy). The commonest cause was congestive heart failure (36.7%), followed by amoebic liver abscess (7.1%), hepatocellular carcinoma (5.8%) and cirrhosis (5.4%). Liver biopsy provided the diagnosis in 90.8% of patients with initial unexplained hepatomegaly. The diagnostic yield of liver biopsy was increased by submitting 3 biopsy specimens for histological examination. The 3 specimens are obtained using a single intercostal entry site and redirecting the biopsy needle, without increasing the risk of complications. Hepatic tuberculosis was present in 9.2% of patients who underwent biopsy. There were no consistent clinical findings in these patients. Therefore, in communities in which tuberculosis is endemic, all patients with unexplained hepatomegaly require liver biopsy since it provides the only means of making this diagnosis.
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PMID:Causes of hepatomegaly at King Edward VIII Hospital, Durban. A prospective study of 240 black patients. 300 36

Liver disease, although usually asymptomatic, is a frequent accompaniment of AIDS. Hepatomegaly and macrosteatosis are prevalent but non-specific findings. Evidence of remote hepatitis B virus infection is extremely common; however, the HBsAg carrier state, chronic active hepatitis, or cirrhosis occur no more frequently in AIDS patients than in the general population. Opportunistic intrahepatic infections (such as MAI, fungi, and CMV) or neoplasms (such as lymphoma or KS) usually reflect a disseminated process; liver involvement generally does not directly cause morbidity or result in death. Although biochemical liver tests are commonly elevated in the AIDS population, alkaline phosphatase has proved to be the most specific enzyme for infiltrative processes. Percutaneous liver biopsy has a high diagnostic yield, although the treatment options are currently limited. Acalculous cholecystitis and biliary tract obstruction have been recently described and probably result from CMV and/or cryptosporidial infection. Radiologic features of papillary stenosis and/or sclerosing cholangitis have been demonstrated. In contrast to hepatic parenchymal disease, these entities may be amenable to surgical or endoscopic therapeutic maneuvers.
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PMID:Hepatobiliary abnormalities of AIDS. 304 66

Circulating immune complexes (CIC), adult schistosome antibody, and total immunoglobulin concentrations were estimated in sera from 35 chronic Schistosoma mansoni patients with different infection intensities and different pathological complications. High CIC levels were present in about one-third (10/35) of the sera. Most of the patients (9/10) with elevated CIC levels also had hepatomegaly or hepatosplenomegaly. This finding is significant in the pathogenesis of schistosomal liver fibrosis and may also apply to other liver diseases, especially cirrhosis. No correlation was found between infection intensity as judged by stool egg counts and CIC levels. A reverse relationship was observed between the level of anti-adult worm IgG and CIC levels. CIC levels were elevated within 7 and 28 days after treatment in most patients. Hypergammaglobulinaemia was detected in most sera.
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PMID:Circulating immune complex levels in patients with schistosomiasis and complications. 313 Jun 85

Ultrasound diagnosis of chronic schistosomiasis japonica was assessed by comparison with the results of serological tests using enzyme-linked immunosorbent assay (ELISA) with egg and adult worm antigen, and the circumoval precipitation test. The subjects were persons resident in the Chikugo River Basin in Kyushu, southwest Japan, where schistosomiasis used to be highly prevalent. Of 93 individuals with a past history of infection, 37 (39.8%) presented the characteristic echo pattern of the disease (network, sieve, mottled or mixed pattern). In the serological tests, the ultrasound (US)-positive subjects showed a significantly higher IgG antibody-positive rate in ELISA (81.1% for egg-ELISA), than the US-negative subjects (37.5% for egg-ELISA). Individuals bearing dead Schistosoma japonicum eggs proven by tissue biopsy showed a similar antibody-positive rate (76.5% for egg-ELISA) to that of the US-positive subjects. Sera of a group which had no schistosomiasis characteristic echo pattern, but had liver fibrosis, hepatomegaly or liver cirrhosis as shown by US, were also highly positive (71.4% for egg-ELISA). The present serological studies thus confirmed the usefulness of ultrasound diagnosis for chronic schistosomiasis japonica. Furthermore, we were able to determine certain indications of liver abnormality currently undefined in the normally utilized US classifications.
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PMID:Serological evaluation of ultrasound examination for chronic schistosomiasis japonica in a previously endemic area--the Chikugo River Basin, Japan. 315 43

Type IV glycogenosis is due to branching enzyme deficiency and is usually manifested clinically by progressive liver disease with cirrhosis and hepatic failure between the second and fourth years of life. We describe a 5-year-old boy who, following an acute febrile illness at 2 years of age, was first noted to have hepatomegaly with mildly elevated serum transaminase levels. Liver biopsy revealed hepatic fibrosis with periodic-acid Schiff-positive, diastase-resistant inclusions in hepatocytes and fibrillar inclusions characteristic of amylopectin by electron microscopy. Enzymatic assay revealed deficient hepatic branching enzyme activity with normal activity of glucose-6-phosphatase, debranching enzyme and phosphorylase activities. During the succeeding 3 years, he grew and developed normally with apparent resolution of any clinical evidence of liver disease and only intermittent elevation in serum transaminase levels associated with fever and prolonged fasting. Repeat liver biopsy at 4 years of age showed persistence of scattered hepatocellular periodic-acid Schiff-positive, diastase-resistant inclusions, but no progression of hepatic fibrosis in spite of persistent deficiency of hepatic branching enzyme activity. Skeletal muscle and skin fibroblasts from the patient also showed deficient enzyme activity. Skin fibroblasts from both parents exhibited half the normal control activity, suggesting a heterozygote state. This is the first documented patient with deficiency of branching enzyme but without evidence of progressive hepatic disease. This patient, coupled with reports of other patients with late onset hepatic or muscle disease with branching enzyme deficiency, suggests that the defect resulting in Type IV glycogen storage disease is more heterogenous and possibly more common than previously suspected.
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PMID:A new variant of type IV glycogenosis: deficiency of branching enzyme activity without apparent progressive liver disease. 316 25

A retrospective study was designed to analyse the mode of presentation, clinical signs, haematological, biochemical and histological features in 46 Indian patients admitted with cirrhosis to R. K. Khan and King Edward VIII Hospitals, Durban, between 1977-1981. The commonest presenting feature was swelling of the body followed by pain in the right upper quadrant, most patients had hepatomegaly, jaundice and ascites, and splenomegaly was detected in one-third of cases. Biochemical investigations indicated that most patients had a high globulin and low albumin concentration. Liver function tests revealed raised bilirubin and gamma-glutamyltransferase values in most cases. On histological examination, micronodular cirrhosis predominated (95%) with a high incidence of fat and iron deposition. Changes consistent with alcoholic hepatitis were superimposed in one-third of cases while immunological and viral markers were absent. This study suggests that alcohol is the predominant cause of cirrhosis in Natal Indians.
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PMID:Patterns of cirrhosis in Natal Indians. 320 19


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