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 preceding discussions outline the various forms of cirrhosis that may be encountered in the elderly population. Cirrhosis is not uncommon in older patients. Although it has been stated that most cirrhosis in the elderly is due to alcohol, these assumptions are perhaps overestimations. In the authors' experience, many older patients are inappropriately labeled with alcoholic liver disease--presumed guilty until proven otherwise--and have subsequently been shown to have nonalcoholic liver disease. Careful investigation is required. Hepatotoxic drug exposure (e.g., to alpha methyldopa, nitrofurantoin, or isoniazid) should be ruled out, and hepatitis B and hepatitis C serology obtained. Primary biliary cirrhosis may occur in both sexes, and thus antimitochondrial antibody should be assayed. Severe heart disease may result in cardiac cirrhosis in the elderly, with ascites and hepatomegaly. Alpha 1-antitrypsin deficiency, primary sclerosing cholangitis, idiopathic hemochromatosis, and chronic autoimmune hepatitis may result in advanced cirrhosis in the elderly; appropriate serum studies should be obtained. If questions remain and if therapy may be changed, liver biopsy can be performed. A recent study suggested, however, that the risk of hemorrhage from liver biopsy in the elderly may be increased, especially if malignancy is present. The era of treatment for liver diseases has arrived. Colchicine, methotrexate, ursodeoxycholic acid, and others have shown promise in the treatment of PBC, primary sclerosing cholangitis, and alcoholic liver disease. Corticosteroids may be lifesaving in autoimmune liver disease. Phlebotomy remains the treatment of choice for hemochromatosis in any age group. Interferons and other antiviral agents are being used in chronic type B and type C hepatitis. Treatment of the complications of cirrhosis in the elderly may be safely accomplished. Advanced age is not a contraindication to variceal sclerotherapy. Vasopressin, however, may be contraindicated in the elderly patient if there is an underlying history of atherosclerotic coronary or peripheral vascular disease. Large-volume paracentesis and peritoneal venous shunting can afford symptomatic relief of ascites, even in the geriatric population. Finally, as noted previously, advanced age is no longer to be considered an absolute contraindication for liver transplantation. The evaluation of liver disease in the elderly may be diagnostically challenging, and its treatment rewarding.
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PMID:Liver diseases in the elderly. 185 64

Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. We report the clinical course of the liver disease, histologic progression of the hepatic lesions, and etiologic investigations in five patients (four girls, three kinships). All had grown and developed normally until seen at 6 to 36 months of age (mean 20 months), with vomiting (n = 5), progressive hypotonia (n = 3), or seizures (n = 2). All had been given anticonvulsants, including valproic acid in three. Liver disease was noted at a mean age of 35 months (range 9 to 67 months), with hepatomegaly (two patients), abnormal hepatic synthetic function (three) or transaminase values (three), and cirrhosis in one. Patients survived for a mean of 4.6 weeks (range 1 to 8 weeks) after the identification of liver disease; all died of hepatic failure. Results of evaluation for infectious and metabolic causes of liver disease and causes of degenerative neuromuscular disease were negative in all patients. Premortem liver biopsy specimens (n = 3) demonstrated an early lesion consisting of lobular disarray, microvesicular steatosis, periportal acute and chronic inflammation, and individual hepatocyte necrosis. Autopsy findings (n = 5) consisted of macrovesicular steatosis, massive hepatocyte dropout, and proliferation of bile ductular elements, with almost complete replacement of hepatocytes by proliferating bile ductular elements in two patients. Brain showed characteristic neuronal degeneration. We conclude that Alpers disease can be a cause of rapidly progressive liver failure in early childhood. Although the cause of this autosomal recessive disease is not known, it does not appear to be related to peroxisomal dysfunction.
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PMID:Liver involvement in Alpers disease. 186 Dec 11

The purpose of our study was to assess the pattern of liver diseases diagnosed by ultrasonography (US) in Yaounde (Cameroon). From a total of 1,007 consecutive US studies performed over a period of 14 months in subjects over the age of 15 years, we found 322 patients with a sonographic diagnosis of liver disease. 8 diagnoses comprised 83% of the patients. Apart from non specific homogeneous hepatomegaly these diagnoses consisted of: cirrhosis, primary liver malignancies, liver abscess, congestive hepatomegaly, secondary liver malignancies, diffuse steatosis and nodular calcifications. 30 liver abscess drainages were performed with sonographic guidance. We conclude that in our environment where Computed Tomography is scarce and expensive, US is a useful tool in the study of some of the most frequent liver diseases.
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PMID:[Pattern of hepatic pathology diagnosed with echography in Yaounde (Cameroon)]. 192 48

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

We report the diagnostic, prognostic and therapeutic features of non-Hodgkin's lymphoma in eight patients in whom the disease was seen as a primary tumor of the liver. This series illustrates the variety of situations in which lymphoma might be diagnosed: (a) abdominal pain and hepatomegaly (three cases), (b) incidental finding at evaluation of a patient with cirrhosis (two cases), (c) secondary neoplasm after treatment for Hodgkin's disease (one case) and (d) complication of AIDS (two cases). In most cases, clinical and/or radiological features were nonspecific. However, the combination of the following features must be considered as suggestive: occurrence of an apparently primary hepatic tumor in an immunocompromised patient, absence of the usual serum tumor markers and increased serum lactic dehydrogenase activity. The final diagnosis was based on histological examination of specimens obtained by ultrasonically guided liver biopsies or at surgery. All cases belonged to unfavorable histological subtypes. Immunohistochemical findings on paraffin-embedded sections demonstrated the B-lymphocyte lineage of the seven tumors available for study. In the three patients without coexisting disease, complete remission was obtained by surgery alone or combined with chemotherapy. In the two patients with coexisting cirrhosis, outcome was rapidly unfavorable, with death occurring less than 3 mo after diagnosis. Among the three immunocompromised patients, two experienced a rapid unfavorable outcome, and the remaining one was in complete remission after surgery and chemotherapy. In conclusion, primary non-Hodgkin's lymphoma of the liver arising in patients without coexisting disease has a slow progression and might be successfully treated by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Non-Hodgkin's lymphoma presenting as a primary tumor of the liver: presentation, diagnosis and outcome in eight patients. 202 91

Cholesterol ester storage disease is a rare disorder characterized by an hereditary deficiency of lysosomal acid lipase that induces an accumulation of cholesterol ester in most tissues of the body, particularly in liver. The diagnosis is usually made during childhood. The aim of this article is to report two new cases diagnosed in adult age. Two patients, 25 and 20 years old, respectively, presented with hepatomegaly, a slight to moderate increase in serum transaminases, and esophageal varices. In both cases, diagnosis was based on the presence of hypercholesterolemia, fatty infiltration of the liver with lipid droplets in hepatic parenchymal cells, foamy macrophages, hepatic storage of cholesterol esters, and low activity of lysosomal acid lipase. Histological abnormalities were associated with portal and periportal fibrosis in one patient and a micronodular cirrhosis in the other; these lesions were probably the cause of portal hypertension. Fibrosis of varied degrees has been previously reported in cholesterol ester storage disease. Its mechanism remains unclear.
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PMID:[Hepatic cholesterol ester storage disease. Two new cases diagnosed in adults]. 207 Sep 66

Although rather uncommon and multifactorial in etiology, liver cirrhosis is a severe and often rapidly fatal disease in pediatrics. In our institution, during the last 15 years, 22 children with liver cirrhosis have been followed. The underlying predisposing condition was HBV infection (8 cases), CMV perinatal infection (2 cases), Wilson's disease (4 cases), chronic cholestasis (2 cases) and alcohol abuse (2 cases); in 4 cases no predisposing condition was evident. In all cases the histological examination of the liver was the diagnostic cornerstone. The mean age at diagnosis was 6 years and 8 months, with an early onset especially in the posthepatitis cirrhosis. In 10 out of 22 patients, cirrhosis was not preceded by an history of chronic liver disease. Poor subjective symptomatology was present in 13 of the cases, hepatomegaly in all, splenomegaly in 18 cases, signs of hepatic failure in 13 cases. In all patients various impairments of hepatocellular synthesis were detectable, especially during the period preceding the development of hepatic insufficiency. The mean time to cirrhosis was 5 years. The average duration of the follow up was 3 years and 4 months: during the follow up 6 patients improved, 5 patients showed no clinical or functional modifications of their hepatic disease, 3 patients worsened and 8 died. In order to perform suitable treatment of liver cirrhosis the need of early diagnosis and etiological definition should be emphasized.
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PMID:[Liver cirrhosis in childhood. Considerations on 22 cases with different etiology]. 217 33

The patient, a 29-year-old female, was hospitalized because of clouding of consciousness, fever and right hemiplegia 4 days after the onset. On first examination she was found having fever in the 37 degree range, positive CRP, hepatomegaly, anemia and hepatic function impairment. Neurological examination revealed somnolence, conjugate deviation to the left and stiff neck. The muscular power measured about 3+ for the upper extremities and 0 for the lower extremities. Babinski sign was present on the right side. The spinal fluid showed an increase in cell counts, especially the neutrophil count. CT scans showed diffuse white-matter hypodensity in the left hemisphere. Soon after admission the patient fell into coma and died 6 days after admission. Autopsies led to a diagnosis of Hurst's encephalitis complicated by hepatoma with liver cirrhosis. It was reported that the immune complex was found in 25.9% of liver cirrhosis patients. From this fact it is suggested that Hurst's encephalitis might be elicited by some immunological mechanism.
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PMID:[A case of Hurst's encephalitis complicated by hepatoma]. 217 57

We describe three patients with cholesteryl ester storage disease. Diagnosis was confirmed by demonstrating a deficiency in lysosomal acid cholesteryl hydrolase activity in cultured skin fibroblasts from each of these patients. All had hepatomegaly, elevated serum aminotransferase activities and hyperlipoproteinemia. Histological examination of liver biopsy specimens before treatment revealed accumulation of fat within hepatocytes, bile duct epithelium and endothelial and Kupffer cells. Cholesterol crystals were recognized by their birefringence in frozen sections. A striking feature was the presence of markedly hypertrophied Kupffer cells and portal macrophages with foamy, tan-colored cytoplasm that stained readily with the periodic acid-Schiff reagent and aldehyde fuchsin. Periportal fibrosis was noted in all cases; incomplete cirrhosis was present in one case. Distinctive and hitherto undescribed lysosomal accumulations of triglyceride and cholesterol crystals were noted. The patients were treated with lovastatin, a cholesterol-lowering agent, for at least 12 mo. No significant changes were seen in serum lipoprotein concentrations or liver histopathology after therapy. Thus lovastatin did not have an obviously beneficial effect on abnormal lipid metabolism in these patients.
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PMID:Cholesteryl ester storage disease: hepatopathology and effects of therapy with lovastatin. 234 51

Liver biopsies were done on 20 patients with histiocytosis X (HX) as part of pretreatment evaluation prior to entry on two Childrens Cancer Study Group protocols. Seventeen patients had hepatomegaly, and seven had one or more abnormal laboratory parameters using Lahey's criteria for liver dysfunction. Nineteen of 20 specimens showed various abnormalities of the portal triads. A single biopsy revealed normal liver. Among the changes were triaditis, bile duct proliferation, variable fibrosis with histiocytic infiltrates, and cirrhosis. One patient had typical granulomas of HX within the liver parenchyma in addition to portal triaditis. Patients with larger livers and dysfunction tended to show more marked histologic abnormalities in the portal triads. However, correlations among liver size, function, and pathology showed considerable overlap. Early death among these patients was more likely to be associated with progressive HX in other sites and/or infection. Death from cirrhosis and liver failure per se occurred in one patient 4 years after initial biopsy, but five other children had evidence of cirrhosis on biopsy or at autopsy. The majority of patients with triaditis initially did not have clinical evidence of progressive liver disease although four expired with other manifestations of HX or infection. Conversely, patients showing fibrohistiocytic changes or cirrhosis initially were likely to have continuing or progressive liver disease. Although the liver histology was not diagnostic of HX, the types of portal changes usually predicted the subsequent course of liver disease.
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PMID:Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathologic correlation. 240 53


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