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)

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na+ 108 mEq/L, Cl- 85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.
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PMID:Secretory diarrhea with protein-losing enteropathy, enterocolitis cystica superficialis, intestinal lymphangiectasia, and congenital hepatic fibrosis: a new syndrome. 308 May 72

To identify prognostic factors in cirrhotic patients admitted to the hospital for the treatment of an episode of ascites, a survival analysis was performed in a series of 139 patients hospitalized in our Unit between 1980 and 1985. Mean follow-up was 12.8 +/- 14.2 mo (mean +/- SD). A total of 38 variables based on history, physical examination, hepatic biochemical tests, renal function tests, and endogenous vasoactive systems were analyzed for prognostic value. Eighteen of these variables had prognostic value in the univariate analysis. A multivariate analysis (Cox's regression method) disclosed that 7 of these 18 variables had independent prognostic value. Of these independent predictors of survival, mean arterial pressure and plasma norepinephrine concentration were the variables that best predicted prognosis. Two other variables that independently correlated with survival were urinary sodium excretion and glomerular filtration rate. The remaining three independent predictors of survival were nutritional status, hepatomegaly, and serum albumin concentration. Therefore, these findings indicate that, in patients with cirrhosis and ascites, parameters estimating systemic hemodynamics and renal function are better predictors of survival than those routinely used to estimate hepatic function.
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PMID:Prognostic value of arterial pressure, endogenous vasoactive systems, and renal function in cirrhotic patients admitted to the hospital for the treatment of ascites. 333 20

The antiarrhythmic agent amiodarone is associated with numerous adverse effects, but clinically significant liver disease is rare. A patient is described who presented with muscle weakness, hepatomegaly, and ascites following 28 months of amiodarone usage. His condition deteriorated despite discontinuation of amiodarone therapy. A postmortem liver biopsy demonstrated necrosis, fibrosis, hyalin, and phospholipid-laden lysosomal lamellar bodies. Resolution of hepatic dysfunction may not necessarily occur on withdrawal of amiodarone if irreversible damage is already established. We speculate as to the reasons for the reportedly low incidence of overt liver disease, and suggest that hepatic enzyme levels, as well as other indicators of hepatic function, such as the serum albumin concentration, be monitored indefinitely in all patients while taking amiodarone.
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PMID:Fatal amiodarone hepatoxicity. 334 40

Abdominal ultrasonography was performed on 35 adult patients with proven cystic fibrosis. Thirty-three patients showed pancreatic abnormalities. The most marked ultrasonographic features were increased parenchymal echogenicity, atrophy, non-visualisation of the duct and cyst formation. There was no correlation between these features and the severity of the pulmonary disease, the age of the patient, weight or glucose intolerance. Abnormalities of the biliary tract were demonstrated in nine (26%) patients and were associated with poor nutritional status. Multivariate analysis revealed a significant association between the following: hepatomegaly, increased liver echogenicity, splenomegaly, biliary disease; secondly, between lung function and serum albumin. Ultrasonography is useful in showing organ morphology but not in assessing disease severity.
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PMID:Cystic fibrosis: ultrasonographic findings in the pancreas and hepatobiliary system correlated with clinical data and pathology. 390 96

We evaluated praziquantel for therapy of active Schistosoma mansoni infection in 15 rural Egyptian males with hepatosplenic schistosomiasis. Criteria for inclusion in this study were two pre-treatment S. mansoni egg counts with a mean of greater than 100 eggs g-1 faeces and an enlarged spleen. Fourteen of 15 patients had hepatomegaly, five had ascites, and six had serum albumin below 3 g dl-1. Schistosoma haematobium infection (less than 10 eggs ml-1 urine) was present in three patients. Praziquantel was administered in a single oral dose of 30 mg kg-1 body weight. Eight of the 15 patients (53%) had mild and transient reactions in the form of fever (usually one day), gastrointestinal symptoms, headache and skin rash. Criteria for parasitological cure were the absence of live eggs in two stool samples and a negative rectal snip biopsy three months after therapy. Ten patients ceased to pass live eggs (cure rate 67%). For the five who were still passing live eggs there was a mean egg reduction of 95%. The three patients with S. haematobium demonstrated parasitological cures. We conclude that praziquantel is an effective and well tolerated drug for treatment of S. mansoni infection in patients with advanced hepatosplenic schistosomiasis, and it is the drug of choice for patients with coexisting S. haematobium infection.
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PMID:Praziquantel for treatment of schistosomiasis in patients with advanced hepatosplenomegaly. 393 36

Clinical features of severe opisthorchiasis were studied in 88 patients. The ratio of males to females was 6.3 : 1; 75% were over 40 years old. The presenting symptoms were obstructive jaundice, 25% associated with secondary infection of biliary system; cholangitis and cholecystitis; intraabdominal mass, which was enlarged liver; 18% had palpable gallbladder; 18% had adenocarcinoma of the bile duct. High bilirubin was found in 46% of cases, high alkaline phosphatase in 80%; elevation of serum transaminase in 78% and low serum albumin in 62% of patients. There was no correlation between severity of the disease and the faecal egg output. The obstructive jaundice patients had low or no egg output. Two patients had no eggs in stool, but numerous Opisthorchis viverrini eggs and flukes were found in the gallbladder and bile ducts at operation.
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PMID:Clinical features in severe opisthorchiasis viverrini. 409 5

A comparison was made of the clinical findings of 59 patients with liver cirrhosis (LC) accompanied with hepatocellular carcinoma (HCC) (of which 35 had ascites and 24 did not at the time of admission) and 164 patients with LC, but without HCC (of which 39 had ascites and 125 did not). HCC patients were older and more often had hepatomegaly, vascular spider and pleural effusion than LC patients. Ascites was more frequently observed in HCC than in LC patients when the serum albumin level and the indocyanine green disappearance rate were relatively well maintained and when peripheral edema was absent. There was no difference in the ascitic protein concentration between LC and HCC patients. Malignant cells were detected in ascites only in 14% of the HCC patients. These facts indicate the presence of ascites-inducing factors in HCC patients which have no direct relation to serum colloid osmotic pressure and effective hepatic blood flow. Almost all of the HCC patients with ascites (96%) died with ascites, whereas 54% of the LC patients with ascites recovered from the ascitic condition.
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PMID:Clinical studies of hepatocellular carcinoma with liver cirrhosis and ascites. 608 21

Two children with congenital hepatic fibrosis presented atypically: one with prolonged fever and hepatomegaly associated with a giant intrahepatic biliary cyst and a second with ascites at an early age despite normal serum albumin concentrations.
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PMID:Congenital hepatic fibrosis--unusual presentations. 639 92

The nutritional efficacy and hepatic changes in rats given a total parenteral nutrition (TPN) solution consisting of 4.25% amino acids and 25% dextrose by intragastric, intravenous, or prehepatic routes were studied over a 4-day period. Rats fed Purina Chow or given intragastric TPN maintained body weight and showed no appreciable fatty change of liver. In contrast, weight loss, hepatomegaly, and a 37% increase in liver lipid content were observed in rats given intravenous TPN and a 60% increase after prehepatic TPN. Approximately half the rats given intravenous or prehepatic TPN developed fatty changes in the liver. Serum albumin concentration and hepatic protein content were not improved after prehepatic TPN. Serum SGOT and SGPT were elevated in rats given prehepatic TPN only. Prehepatic TPN may result in hepatic injury and offers no apparent benefit over conventional intravenous TPN in rats.
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PMID:Nutritional efficacy and hepatic changes during intragastric, intravenous, and prehepatic feeding in rats. 641 63

From this comparison of 37 black children with hepatic schistosomiasis (HS) and 53 with intestinal Schistosoma mansoni (IS) living in an endemic area, we propose easily identifiable clinical features of mild HS. These patients were generally well nourished school-age children who seldom complained of dysentery but who had a firm hepatomegaly with predominant enlargement of the left lobe and a firm splenomegaly. They were also mildly anaemic (9.4 +/- 2.2 g/dl) and had low serum albumin (30 +/- 7 g/l), raised aspartate transaminase (36 +/- 31 u/l) and high globulins (53 +/- 15 g/l). The implications of the absence of severe hepatosplenic schistosomiasis in many of these children are discussed.
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PMID:Clinical recognition of mild hepatic schistosomiasis in an endemic area. 671 May 66


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