Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0019209 (hepatomegaly)
5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixteen cases of chronic Q fever are described. In eight there was a history of exposure to infection from farms or farm products. All had valvular heart disease, involving the mitral valve in nine and the aortic valve in seven. Infection occurred on a prosthetic valve in two patients. Arterial embolism was common. Venous thrombosis occured in three patients, and pulmonary embolism occurred in three other patients. Complement fixing antibodies to phase 1 antigen were found in a titre of 1:200 or greater in all except two patients. In one of these post-mortem examination revealed rickettsial bodies in mitral valve vegetations, and in the other Coxiella burneti was isolated from heart valve tissue. The majority presented with infective endocarditis but two presented primarily with liver disease. All patients had evidence of liver involvement and in one this led to death from cirrhosis. Abnormal tests of liver function, particularly hyperglobulinaemia, raised alkaline phsophatase and abnormal bromsulphthalein retention were found in all patients. Hepatic histology was abnormal in all eight patients in whom it was studied. The commonest features were mononuclear cell infiltration of the portal tracts and prominence of the sinusoidal Kupffer cells. Patchy focal necrosis of parenchymal cells, granulomata, fatty change, and eosinophilia of the sinusoidal walls were also noted in several patients and cirrhosis developed in one. Six patients had a purpuric rash, and in 12 there was thrombocytopenia. It is suggested that the presence of hepatomegaly and liver involvement and thrombocytopenia may help to differentiate Q fever endocarditis from bacterial endocarditis. Raised serum IgM and IgA levels occured frequently, but with only a moderate dominance of IgM. Sheep cell agglutination and latex fixation tests for rheumatoid factor were occasionally positive. Several features of the disease suggest the possibility that immune-complex mechanisms may play a role in chronic Q fever. Treatment was with prolonged courses of tetracycline usually combined with lincomycin. Seven patients underwent valve replacement surgery for haemodynamic reasons. Five patients died; two from heart failure, one from cirrhosis, one seven days after valve replacement and one from intraperitoneal haemorrhage following percutaneous liver biopsy. Three patients have survived for more than five years, and another six for more than three and a half years after diagnosis. Of these nine patients, three received medical therapy alone and six required valve replacement as well. Antibiotics have been discontinued in four patients who have had valve surgery and three others. Six patients had received antibiotics for continuous periods varying from 29-62 months. In the period after stopping therapy varying from 15-21 months, no relapse has occured. A seventh patient, who had received antibiotics for four months prior to valve replacement, has survived 43 months after the withdrawal of antibiotics...
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PMID:Chronic Q fever. 94 Sep 18

Liver disease in children with alpha1-antitrypsin deficiency and protease inhibitor type ZZ does not necessarily carry a bad prognosis. Fourteen of our 18 patients presented with the neonatal hepatitis syndrome and four had hepatomegaly without jaundice. Although four patients have died of cirrhosis and its complications, and three have severe liver disease, most of the 11 others, of whom four are over 13 years of age, have relatively little clinical, biochemical, or histologic evidence of liver disease. Persistent elevation of SGOT during the third year of life and renal or pulmonary problems were associated with a poor prognosis. Liver biopsy early in the course of the disease was not helpful prognostically but was useful in assessment of the severity of liver disease and demonstration of alpha1AT storage, alpha1AT deficiency was found in 29% of our patients who presented with the neonatal hepatitis syndrome. One of seven apparently healthy Pi type ZZ sibs of our patients had significant liver disease which had not been suspected previously.
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PMID:Liver disease associated with alpha1-antitrypsin deficiency in childhood. 108 17

This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that alpha1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe alpha1-antitrypsin deficiency is well illustrated by these two infants.
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PMID:alpha1-Antitrypsin deficiency and liver disease in children. 108 88

The development of the syndrome of chronic intrahepatic cholestasis in five young, black men who had systemic granulomatous disease and clinical features consistent with those of sarcoidosis is described. Clinical and biochemical aspects, similar to those of primary biliary cirrhosis, included pruritus, jaundice, hepatomegaly and striking elevations of serum levels of alkaline phosphatase and cholesterol. (One patient had skin xanthomas.) Mitochondrial antibodies were not found; and survival of the patients (7 to 18 years) exceeded the usual survival of patients with primary biliary cirrhosis. The histologic abnormalities included noncaseating granulomas, chronic intrahepatic cholestasis, increased copper in hepatocytes, progressive diminution in number of interiobular bile ducts, periportal fibrosis and the eventual development of a micronodular "biliary" cirrhosis. The histologic evolution of the disease suggests a slow, progressive destruction of the bile ducts by granulomas. Although the end stage of this syndrome resembles primary biliary cirrhosis, the characteristic nonsuppurative, destructive cholangitis of primary biliary cirrhosis was not present.
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PMID:Chronic intrahepatic cholestasis of sarcoidosis. 116 46

The non-protein amino acid indospicine, which occurs in the free state in high concentration in the tropical pasture legume Indigofera spicata, causes toxic liver lesions in ruminants. Indospicine is a specific antagonist of arginine and an inhibitor of protein synthesis. The liver lesion was studied in rats at four dose levels by feeding diets containing 96, 48, 24 and 15 per cent. of the seed. The too higher levels caused death of most animals in 2-6 wk. Females were more susceptible than males. The rats fed the 24 per cent. seed diet developed a nodular cirrhosis by 6 wk and survived up to 18 wk. The rats fed the 15 per cent. seed diet developed cirrhosis at 16 wk and survived up to 28 wk. Prior to the onset of cirrhosis the liver showed a characteristic lesion consisting of hepatomegaly, periportal fatty change, portal cellularity due to proliferation of ovoid and cuboidal duct cells, gross enlargement of the hepatocyte cytoplasm nuclei and nucleoli in spite of brisk mitotic activity, and focal centrilobular necrosis. The lesion was interpreted as a restricted hepatic response to a growth stimulus, possibly the mobilisation of tissue amino acids. Improvement occurred in the rats fed the two lower dosage levels after nodular cirrhosis developed, producing a new parenchyma. An attempt is made to relate the lesions to the biochemical derangement induced by a specific amino acid antagonist.
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PMID:Effects on the liver in the rat of ingestion of Indigofera spicata, a legume containing an inhibitor of arginine metabolism. 121 90

The nonerythropoietic component of early labeled bilirubin in plasma and bile was studied in 7 patients with acute infectious hepatitis during the late convalescent stage, in 13 patients with Laennec-type liver cirrhosis, and in 7 control subjects after intravenous injection of a tracer dose (2.5 muCi) of 4-14C-delta-aminolevulinic acid (14C-deltaALA). All subjects were examined during the nonicteric stage. In control subjects, the mean cumulative radioactivity readings in 4 hours were 29.6 +/- 4.7 X 10(3) d.p.m. per milligram times 4 hours in plasma and 27.0 +/- 1.2 X 10(3) d.p.m. per milligram times 4 hours in bile. In acute infectious hepatitis patients, the mean cumulative radioactivity readings for both plasma and bile in 4 hours were approximately twice that found in control subjects. In mild cirrhotic patients with enlarged liver on scintigram, the mean cumulative radioactivity readings for both plasma and bile were approximately 1.4 times that in control subjects. In patients with more advanced cirrhosis and markedly small livers on scintigram, the mean cumulative radioactivity readings for both plasma and bile were as low as approximately 0.5 that of control subjects. These findings seem to indicate the important role of the liver in the production of the nonerythropoietic component of early bilirubin in man.
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PMID:The effects of acute infectious hepatitis and cirrhosis of the liver on the nonerythropoietic component of early bilirubin. 124 90

Symptomatic occlusion of hepatic veins, the Budd-Chiari syndrome (BCS), may be on the increase in women taking oral contraceptives. 17 liver scans in 7 patients with confirmed BCS over a 7-year period were studied. 6 of the 7 patients were women. When more than 1 vein is occluded BCS results, with hepatomegaly, abdominal pain, ascites, and hepatic histology showing centrizonal sinusoidal distention, hemorrhage, and necrosis. Mortality has been high. BCS is associated with polycythemia, oral contraceptive use, malignancy, trauma, and congenital abnormalities. The scintigram appearance with radiocolloid is usually characteristic but a similar appearance has rarely been reported in cirrhosis of the liver and in 1 case of contrictive pericarditis. Excessive uptake in the midline with markedly diminished activity at the periphery may be the 1st clue that BCS is present. Confusing conditions with incomplete BCS include partial hepatectomy, radiation injury, fortuitous segmental involvement by diffuse or focal liver disease, and rarely hepatic artery occlusion. Treatment is by the porto-caval shunting operation. Venous obstruction as shown venographically has had good correlation with liver scans. After the shunt procedure, hepatic artery flow to the affected lobes has increased as the pressure falls and underperfusion of the hypertorphied midline section. After 14 months, the midline area has shown no uptake, possibly because of atrophy. Radiocolloid uptake also appears in the ribs, spine, and lung. This uptake recedes when the liver function improves.
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PMID:Liver scan in Budd-Chiari syndrome. 126 39

Amiodarone, a commonly used antiarrhythmic agent, has numerous adverse effects. The purpose of this case report is to highlight its hepatotoxicity, an unusual complication of long term amiodarone therapy. Our patient is a 76-year-old man with underlying ischaemic heart disease and recurrent ventricular tachycardia. Eleven months after commencing amiodarone, he developed asymptomatic raised aminotransferases which resolved following drug withdrawal. Amiodarone was then reintroduced and four years later, the patient developed hepatomegaly, worsening liver biochemistry and histopathological changes consistent with early cirrhosis. His symptoms improved following discontinuation of amiodarone. However, hepatomegaly and a low serum albumin still persist four years later.
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PMID:Hepatotoxicity of amiodarone. 129 29

Investigations included 52 drug-addicts with asymptomatic HIV virus infection. 8 of them suffered some years ago, from virus hepatitis of type B. Physical examinations did not reveal in examined persons any deviations from normal condition except for hepatomegaly. Results of liver biochemical investigations remained within normal limits. In each of them one confirmed presence of serological markers of HBV infection and in 35 of them of HCV and in 5 of them in parallel HDV. In all the examined persons one carried out liver biopsy and routine morphological examinations. In every case one disclosed a liver injury of drug-induced type. Further, in 31 examined persons one detected a coexistence of chronic, active inflammatory process of liver hepatitis minimal--17 hepatitis chronica persistent--12 hepatitis chronica aggressivE--1 cirrhosis hepatis--1 and in four of them changes of the type fibrosis periportal. In HIV infected drug-addicts it comes about to clinically asymptomatic, chronic hepatitis coexisting with morphological changes of drug-induced type liver.
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PMID:[Results of liver examination in drug addicts infected with HIV virus]. 129 40

Alagille's syndrome or arteriohepatic dysplasia has been described in Cuba in nine patients between nine months and 12 years of age (8 males and one female). Among the clinical features we found five major abnormalities: chronic cholestasis with neonatal jaundice (9/9), peculiar facies (9/9), peripheral pulmonary artery hypoplasia associated with cardiac murmur (6/9), butter-fly-like arch defects (4/9), and posterior embryotoxon (6/7). Two children had a severe xanthomatosis. Laparoscopy showed green hepatomegaly depending on the degree of cholestasis, and only one patient had incipient signs of micronodular cirrhosis. Liver histology showed a paucity of interlobular bile ducts. Survival was of 60%. One patient survived more than 30 years. Four patients died of liver carcinoma (unique report in infants), broncho-pneumonia, acute renal failure, and sudden death respectively. Among the minor features were mental retardation (5/9), a peculiar voice (3/9), growth retardation observed in some of our patients. This is the first report on Alagille's syndrome in Latin America, because so far reports have come only from Europe and North America.
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PMID:[Alagille's syndrome in Cuba. A report of 9 cases]. 134 Aug 42


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