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)

Patients with large cutaneous burns are characterized by an elevated metabolic rate and lose up to 25% of their body weight within 3 weeks. A previous study suggested that intravenous supplementation to attain nutritional requirements was of no benefit in patients with cutaneous burns covering greater than 50% of their total body surface area. In this study 39 patients with burns greater than 50% of their total body surface area were randomly assigned to receive intravenous supplementation of enteral calories (n = 16) or enteral calories alone (n = 23). Intravenous supplementation decreased the amount of enteral calories that patients with burns could tolerate. The mortality rate was significantly higher (p less than 0.05) in the intravenously supplemented group at 63% as compared with 26% in the group receiving enteral calories alone. Both groups showed significant decrease in natural killer cell activity when compared with controls at both 0 to 7 and 7 to 14 days after injury. T cell helper/suppressor ratios were depressed in both groups when compared with controls; however, the intravenously supplemented group was significantly depressed at 7 to 14 days after burn. Both groups demonstrated hepatomegaly, moderate fatty infiltration, and cholestasis. It is suggested that intravenous supplementation should be carefully evaluated and used only in patients with total enteral failure.
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PMID:Increased mortality with intravenous supplemental feeding in severely burned patients. 250 48

A 42-year-old woman with multiple hepatic cysts associated with autosomal polycystic kidney disease was evaluated for abdominal discomfort and new liver test abnormalities following blind aspirations of her liver cysts. Tc-99m mebrofenin hepatobiliary imaging revealed a markedly enlarged liver with multiple photon deficient areas, focal retention of isotope in the left hepatic ductal system, no accumulation of radionuclide in cysts, and an unusual medial gallbladder position. Endoscopic retrograde cholangiography confirmed all of these findings. Abdominal discomfort and liver biochemical abnormalities were attributed to cyst compression of nearby structures, including bile ducts. Hepatobiliary imaging is useful to exclude communication of bile ducts with hepatic cysts, to detect incidental abnormalities such as partial bile duct obstruction, and to distinguish the gallbladder from nearby hepatic cysts.
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PMID:Liver cysts associated with polycystic kidney disease: role of Tc-99m hepatobiliary imaging. 259 50

Seventy eight patients with diffuse hepatic lesions were selected for this study. They were classified after liver biopsy histopathologically into four groups. 45 patients with pure bilharzial hepatomegaly (group 1). 12 patients with mixed bilharzial hepatosplenomegaly with hepatitis (group 2). 16 patients with chronic active hepatitis and cirrhosis (group 3) and 5 patients with extrahepatic cholestasis (group 4). Ultrasonography of gall bladder was done for all patients. Group 3 patients showed the highest incidence of gall stones (29.4%) as compared to other groups (P less than 0.05).
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PMID:Pattern of gall bladder sonography in chronic liver diseases. 267 Nov 76

Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11-22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts.
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PMID:Infantile chronic tubulo-interstitial nephritis with cortical microcysts: variant of nephronophthisis or new disease entity? 270 88

One-hundred and fifty-nine patients intoxicated with adulterated oil in the province of Salamanca, Spain, were followed-up for 2 years from the onset of the syndrome, in 1981. Four alcoholic patients were excluded from the study. In the remaining 155 patients the liver involvement at the onset took different forms. It was acute in 12 (7%), with jaundice and/or hepatomegaly associated with cytolysis and/or cholestasis; 21 patients (13%) had evidence of cytolysis and/or cholestasis without clinical symptoms, and 29 (18%) only showed a rise in gamma-glutamyltranspeptidase with normal transaminase and alkaline phosphatase levels. Liver enlargement and/or jaundice subsided within 1 month in the 12 patients with clinical symptoms. However, high gamma-glutamyltranspeptidase levels persisted in 4 of them and in 5 of the 21 patients with laboratory abnormalities only. At the end of the study, this enzyme remained elevated in 6 and 7 patients respectively of these two groups and in 5 of the 29 patients with no other abnormality than a rise in gamma-glutamyltranspeptidase.
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PMID:[Liver disease in toxic oil syndrome. Clinical and enzymatic course]. 286 83

Hypermetabolism with negative nitrogen balance and immune deficiencies characterize the systemic response to major thermal injury. Patients with burns greater than 50% of the total body surface area (TBSA) initially have poor gastrointestinal function, making it difficult to deliver sufficient enteral calories to meet nutritional requirements. Controversy has developed over whether to supplement oral alimentation with total parenteral nutrition (TPN) early in their treatment. This study randomly assigned 28 patients with burns greater than 50% TBSA to receive TPN supplementation or no TPN supplementation in the first 10 days postburn. Patients receiving TPN supplementation had significantly lower T-cell helper-to-suppressor cell ratios than the unsupplemented group. However, there was no difference in mortality between the groups (eight in each). All patients who died developed hepatomegaly associated with fatty infiltration cholestasis and antemortem liver function abnormalities, indicating that this syndrome is the result of burn injury itself, not TPN.
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PMID:Failure of TPN supplementation to improve liver function, immunity, and mortality in thermally injured patients. 310 54

We present our experience with 18 pediatric patients with alpha 1-antitrypsin deficiency of the PiZZ phenotype. Fifteen patients (83%) presented with neonatal cholestatic jaundice at a mean age of 2 +/- 0.6 months (+/- S.D.). The male:female ratio was 15:3, indicating a male predominance. All metabolic, infectious and obstructive causes of jaundice were ruled out by appropriate tests in the patients with neonatal cholestasis. Liver biopsy in 14 patients with neonatal cholestasis showed a histological picture of cholestasis in all biopsies; neonatal giant cell hepatitis appeared in seven, increased fibrosis in appeared five and established liver cirrhosis appeared in two biopsies. Patients were followed for a mean of 3.7 +/- 2.4 years (+/- S.D.). Of the 15 patients with neonatal cholestasis, 3 under went liver transplantation because of decompensated liver cirrhosis at 3, 3 1/2 and 7 years. Two patients died at 4 months and 3 years from complications of liver cirrhosis. Of the remaining 10 patients, 3 had histological evidence of liver cirrhosis, and the remaining 7 patients continue to have enlarged liver and spleen with abnormal liver function tests. Of the three patients without history of neonatal cholestasis, only one had enlarged liver and spleen, and the remaining two are healthy with normal liver function tests. Our experience indicates serious liver disease is highly likely to develop in patients with PiZZ alpha 1-antitrypsin deficiency who present with neonatal cholestatic jaundice. Our experience differs from more recent reports on such patients.
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PMID:Liver disease in children with PiZZ alpha 1-antitrypsin deficiency. 328 87

Cholesterol ester storage disease (CESD) is infrequent in children. Four new cases in two nonrelated families are presented. Acid lipase deficiency in the leukocytes of the patients and reduced activity (50%) in those of parents were demonstrated. Clinical manifestations varied from neonatal cholestasis to asymptomatic hepatomegaly. Hepatic histology showed lipid vacuoles and cholesterol ester storage in hepatocytes and Kupffer cells. Increased levels of cholesterol and hepatomegaly were the first findings. There is as yet no specific treatment for CESD; however, the early detection of cases would make possible the timely control of complications.
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PMID:Cholesterol ester storage disease: clinical, biochemical, and pathological studies of four new cases. 329 Apr 19

One-hundred-and-ten children between the ages of two months and 14 years with the following liver diseases were studied: 16 with acute viral hepatitis, 8 with persistent chronic hepatitis, 31 with active chronic hepatitis, 5 with hepatic steatosis, 11 with cirrhosis of the liver, 24 with newborn cholestasis, 3 with Wilson's disease, 2 with congenital hepatic fibrosis, 5 with metabolic diseases and 5 due to other causes. These children presented Pi system phenotypes in isoelectric focus using ultrafine polyacrylamide gels according to Kuepper's method, with modifications incorporated to determine Alpha-1-antitrypsin (A1-AT) serum level deficiencies in those presenting the Pi ZZ phenotype, a liver biopsy with P.A.S. coloration on digestion of diastase and a family history of the phenotype. Four (3.6%) of the children with Pi ZZ phenotypes showed a decrease of serum A1-AT and the presence of positive P.A.S. inclusions resistant to diastase in the cytoplasm of hepatocytes. Three had a history of postnatal icterus and the fourth presented hepatomegaly. The phenotypic study of the parents showed their being heterozygous (MZ), while siblings were normal (MM). The importance of the diagnosis of A1-AT deficiency and the diagnostic value of detecting Pi system phenotypes in every case of liver disease in children and adolescents is stressed.
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PMID:[The value of the Pi system phenotype in alpha 1-antitrypsin deficiency]. 349 88

A case of secondary syphilitic hepatitis is reported. A 49-year old woman was admitted for weight-loss, fever, hepatomegaly and splenomegaly. Diagnosis of syphilitic hepatitis was based on cholestasis associated with positive serologic tests for syphilis without other immunological disturbances. Biopsy of the liver showed a moderate infiltration in and around the portal tracts. Immunofluorescence study for treponema was negative. Rapid improvement was obtained with penicillin initially associated with steroid therapy. Liver involvement in secondary syphilis is characterized by anicteric cholestasis, an inflammatory syndrome, and periportal infiltrate inconstantly associated with centrilobular necrosis, granulomatous reaction and presence of treponemas in the lesions. Due to the increasing frequency of sexually transmitted diseases, this diagnosis could become more frequent.
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PMID:[Hepatic involvement in secondary syphilis]. 355 64


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