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)

The case is reported of a 49-year-old chronic alcoholic woman, who presented with severe pulmonary arterial hypertension (PAH) mimicking as an acute abdomen. She was admitted with right-sided hypochondrial abdominal pain and hepatomegaly, with a moderate jaundice. On admission to intensive care unit, she had an arterial blood pressure of 110/70 mmHg, a heart rate of 100 b.min-1, and a respiratory rate of 36 c.min-1. An electrocardiogram showed sinus rhythm and right-sided heart failure. Whilst breathing 6 l.min-1 oxygen, her arterial blood gases were: PaO2 47 mmHg PaCO2 29 mmHg. Severe PAH was confirmed by measuring her mean pulmonary arterial pressure, which was 46 mmHg, whilst her pulmonary wedge pressure was 7 mmHg. Hepatic function was also altered: total bilirubin 41 mumol.l-1, alkaline phosphatase 145 UI.l-1 and gamma glutamyl transferase 1 340 UI.l-1. She developed arterial hypotension, which did not respond to increasing doses of isoproterenol. She died on the third day. Necropsy confirmed the diagnosis of primary PAH, with acute "cardiac liver".
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PMID:[Pseudosurgical acute abdomen syndrome in primary pulmonary hypertension]. 175 58

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

A review was carried out of 187 previously untreated cases of chronic lymphocytic leukaemia diagnosed between 1969 and 1988. The median age of the patients was 65 years (range, 36-87). There were 118 males (M/F ratio, 1.7). In accordance with Rai's staging system the patients were distributed as follows: 0.29%; I, 20%; II, 25%; III, 13%; IV, 13%, and according to Binet's staging the distribution was: A, 55%; B, 21%; C, 24%. The most frequently found physical findings were lymph node enlargement (55%), splenomegaly (32%) and hepatomegaly (28%). Anaemia was present in 20% of the cases and thrombocytopenia in 13%. The mean lymphocyte count was 62 x 10(9)/L (range, 6-475 x 10(9)/L). Bone marrow infiltration of over 80% was seen in 46% of the patients. Bone marrow biopsy was performed on 97 patients, the diffuse pattern of involvement being most commonly found (44%). Increased BUN (55%), alkaline phosphatase (42%) and LDH (38%) were the most frequent biochemical alterations. Hypogammaglobulinaemia was present in 55% of the patients, IgM being the most commonly affected immunoglobulin (66%). Monoclonal gammopathy was seen in 4% of the cases. LDT, measured in 75 patients, was less than a year in 32%. No antileukaemic drugs were needed in 34% of the patients. When concluding this study, 100 patients had died. The median survival was 57 months and death was related to chronic lymphocytic leukaemia in 53% of such patients.
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PMID:[Chronic lymphatic leukemia. I. Clinico-biological features and survival analysis. Study of 187 patients]. 177 6

Congenital hepatic fibrosis (CHF) is a recognized cause of portal hypertension with oesophageal varices, gastro-intestinal haemorrhage and cholangitis in children without significant impairment of hepatic or renal function. This report describes the varied clinical presentation of CHF as seen at King Faisal Specialist Hospital and Research Centre (KFSH & RC) and emphasizes the clinical patterns that should enable a pediatrician to consider the diagnosis. Fourteen children with CHF were diagnosed between 1981 and 1988. The age at presentation ranged from 1.8-14 years (mean: 7.5 years); clinical manifestations at diagnosis were splenomegaly (12), hepatomegaly (11), failure to thrive (10), marked abdominal distention (4), and fever (4). Liver function tests were normal except for high alkaline phosphatase. Eight patients had polycystic kidneys confirmed on ultrasound examination. Upper gastro-intestinal endoscopy showed oesophageal varices of variable severity in all eight patients examined. Splenoportography revealed splenic vein occlusion in one patient. One patient died within days of admission with convulsions, coma, and aspiration pneumonia. One patient was lost to follow-up. The remaining 12 patients are all alive and receive regular follow-up. Two patients required splenorenal shunt. In view of the prevalence of consanguinity in Saudi Arabia, the diagnosis of CHF should be considered in children with hepatomegaly despite normal liver function tests, and particularly in those with renal abnormalities and/or evidence of portal hypertension.
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PMID:Congenital hepatic fibrosis in Saudi Arabia. 178 58

In 5 years, 64 solid hepatic lesions have been referred for diagnosis and management which have been found unexpectedly on organ imaging in well patients. We have called this lesion a "dystychoma". Patients have undergone a two phase investigation programme which allows a diagnosis without admission to hospital in about 50% of cases. About three quarters of patients (47/64) have had non-neoplastic lesions, and about half (33/64) have had haemangiomas. About one patient in four (17/64) has had a neoplasm, and the neoplasm has been malignant in about one in six (11/64) of all patients. We stress the need to pursue the diagnosis in these patients. There were no reliable clinical, biochemical or imaging characteristics which individually distinguished benign from malignant lesions. Age over 55 years, an enlarged liver or a palpable liver mass and a raised serum alkaline phosphatase were all significantly more frequent with malignant tumours. The risk of malignancy rose with the number of risk factors, and all patients with all three risk factors had malignant tumours. Only 11 of the 64 patients were judged to have benefited by significant increase in quality or quantity of life as a result of what was frequently inappropriate organ imaging. There is no strong argument for replacing history taking and physical examination by CT scanning, ultrasound examination or other organ imaging.
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PMID:Hepatic dystychoma: a five year experience. 181 Mar 71

The clinical features at the time of presentation and the outcome in 126 patients with primary sclerosing cholangitis were studied to clarify the natural history and prognosis in symptomatic and asymptomatic individuals. The median age of the patients at the time of presentation was 36 years, 62% were male, and 16% were asymptomatic. The median follow-up from time of presentation was 5.8 years. There were more patients who had liver transplants (21%) than patients who died of liver-related disease (16%); the estimated median survival to these end points was 12 years. Cholangiocarcinoma was found in 8 patients and in 23% of those undergoing liver transplantation. Asymptomatic patients had milder disease than symptomatic patients, but in a univariate analysis the presence of symptoms was not prognostically significant. On multivariate analysis, the following independent prognostic factors were found: hepatomegaly, splenomegaly, serum alkaline phosphatase, histological stage, and age. These features were combined to produce a prognostic model that should be valuable in the stratification of patients in clinical trials and in the timing of liver transplantation, particularly in those patients seen soon after presentation.
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PMID:Natural history and prognostic variables in primary sclerosing cholangitis. 185 Mar 76

Eleven patients with acute non-lymphocytic leukemia developed hepatosplenic candidiasis following the termination of chemotherapy. Five who did not receive amphoteicin B (AMPH) died of liver failure, whereas the other six who received AMPH and/or miconazole or fluconazole were cured. The features of hepatosplenic candidiasis included prolonged high fever not responsive to antibiotics and hepatomegaly with right-sided hypochondrial pain developing after severe neutropenia. Even if the neutropenia recovered, these symptoms did not subside. In addition, elevation of the serum alkaline phosphatase and total bilirubin levels were observed at onset. CT scan revealed multiple low-density areas of varying size that showed no contrast enhancement. Ultrasonography also demonstrated hypoechoic or hyperechoic lesions, and a so-called "target sign appearance" in the liver and spleen. The clinical diagnosis for hepatosplenic candidiasis is not so difficult because of the uniform symptoms, signs and laboratory abnormalities. The importance of the early administration of antifungal agents to obtain a cure is discussed in this article.
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PMID:[Hepatosplenic candidiasis in acute non-lymphocytic leukemia]. 191 11

Hepatoma is a rare disease in Natal Indians. It occurs in male patients in the fifth decade. They have no history of alcohol intake. The main presenting feature is abdominal pain, weight loss and hepatomegaly. Blood tests reveal a raised alkaline phosphatase, hypoalbuminaemia, hypergammaglobulinaemia and markedly raised gamma glutamyl transferase. The tumour is a single large expanding mass in the right lobe. The patient usually presents in a late stage of the illness and shows a progressive downhill course. Hepatitis B virus infection is emerging as the likeliest carcinogen.
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PMID:Hepatocellular carcinoma in South African Indians resident in Natal. 198

Patients with the acquired immune deficiency syndrome (AIDS) frequently develop hepatic dysfunction. Although hepatic injury may indirectly result from malnutrition, hypotension, administered medications, sepsis, or other conditions, the hepatic injury is frequently due to opportunistic hepatic infection, directly related to AIDS. Infection with Mycobacterium avium intracellulare typically occurs in patients with advanced immunocompromise and with systemic symptoms due to widely disseminated infection. In contrast, hepatic tuberculosis often occurs with less advanced immunocompromise. Cytomegaloviral infection may produce a hepatitis. Cytomegaloviral and cryptosporidial infections have been implicated as causes of acalculous cholecystitis and of a secondary sclerosing cholangitis. About 10-20% of patients with AIDS have chronic hepatitis B infection. These patients tend to develop minimal hepatic inflammation and necrosis. The clinical findings in patients with hepatic cryptococcal infection are usually due to concomitant extrahepatic infection. Hepatic histoplasmosis usually develops as part of a widely disseminated infection with systemic symptoms. Hepatic involvement by Kaposi's sarcoma is rarely documented ante mortem because an unguided liver biopsy is an insensitive diagnostic procedure. Patients with non-Hodgkin's lymphoma of the liver typically have lymphadenopathy, hepatomegaly, and systemic symptoms. As a pragmatic approach, patients with liver dysfunction and HIV-related disease should have a sonographic or computerized tomographic examination of the liver. Patients with dilated bile ducts should undergo endoscopic retrograde cholangiopancreatography because opportunistic infection may produce biliary obstruction. Patients with a focal hepatic lesion should be considered for a guided liver biopsy. Patients with a significantly elevated serum alkaline phosphatase level should be considered for a percutaneous liver biopsy. When performed for these indications, liver biopsy will demonstrate a significant disease involving the liver in about 50% of patients with AIDS and in about 25% of patients who are HIV seropositive but who are not known to have AIDS. The clinical impact of a diagnostic biopsy is blunted by a lack of efficacious therapy for many opportunistic infections.
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PMID:Hepatobiliary manifestations of the acquired immune deficiency syndrome. 198 33

An attempt was made to estimate noninvasively portal pressure (PP) in patients with chronic liver disease, using the theory of quantification, a kind of multivariate analysis. Forty-one patients with liver cirrhosis and 22 patients with chronic hepatitis in whom hepatic venous catheterization had been performed were studied. Seventeen parameters (age, sex, mean blood pressure, red blood cell count, platelet count, prothrombin time, lactate dehydrogenase, alkaline phosphatase, total bilirubin, albumin, gamma-globulin, indocyanine green retention at 15 min, blood urea nitrogen, hepatomegaly, splenomegaly, ascites and edema) were selected for the estimation of PP. The estimated PP correlated significantly with the data obtained by hepatic venous catheterization with a high correlation coefficient of 0.835 (p less than 0.01). An investigation using the theory of quantification was also undertaken to determine which of the 17 parameters selected above was most useful in estimating PP. Among the 17 parameters indocyanine green retention at 15 min, red blood cell count, prothrombin time, hepatomegaly and splenomegaly seemed to contribute significantly to the estimation of PP. When the formula was applied to 31 successive patients with chronic liver disease (external samples), the correlation between the estimated and measured PP was 0.455 (p less than 0.01). These results indicate that the formula is clinically useful in estimating PP in patients with chronic liver disease.
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PMID:[Estimation of portal pressure using the theory of quantification]. 201 41


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