Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bile duct obstruction was induced in 6 cats by surgical ligation and transection of the common bile duct. Clinical and laboratory changes were monitored weekly for 25 to 54 days. Clinical signs of obstruction were similar in all cats and included anorexia, pyrexia, lethargy, intermittent vomiting, weight loss, palpable gallbladder, hepatomegaly, and bleeding tendencies. Tissue jaundice and acholic feces were evident grossly as early as postsurgical day (PSD) 4 with a mean onset of jaundice at PSD 5.3 +/- 0.4. Hematologic changes were initially characterized by a mild neutrophilic leukocytosis that increased with the chronicity of bile duct obstruction. Regenerative anemia developed in 4 cats associated with gastrointestinal blood loss. Acute serum biochemical changes were characterized by a marked increase in the mean values of aspartate aminotransferase, alanine aminotransferase, total cholesterol, and copper. Comparatively, only moderate increases in mean serum alkaline phosphatase activity were observed. Mean total bilirubin values increased remarkably at postsurgical week (PSW) 1, reaching a maximal value of 23.1 +/- 4.4 mg/dl at PSW 3 with 71.6 +/- 2.7% direct bilirubin. With chronicity of bile duct obstruction ranging from PSW 3 to PSW 7, the mean serum values of aspartate aminotransferase, alanine aminotransferase, total cholesterol, serum alkaline phosphatase, and total and direct bilirubin stabilized and then declined, whereas the increased mean serum copper values persisted. At PSD 25 to 54, hepatic copper values and serum bile acids were markedly increased. Seemingly, clinicopathologic changes of induced cholestatic hepatic injury depended largely on the duration of biliary obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Hematologic and biochemical abnormalities associated with induced extrahepatic bile duct obstruction in the cat. 663 41

Chlamydiosis was diagnosed in six African clawed frogs (Xenopus laevis) from a tank of laboratory-bred frogs experiencing sudden deaths. Gross findings were hepatomegaly, distention of the gall bladder, and splenomegaly. Hepatic lesions were present in each frog and were characterized by mononuclear cell infiltrates, necrosis of hepatocytes, Kupffer's cell proliferation, and sinusoidal fibrin thrombi. Cholestasis, hemosiderin-laden Kupffer's cells, parenchymal infarction, and multinucleated hepatocytes were less common findings. Other lesions included degenerative and inflammatory changes in the kidneys, epicarditis and myocarditis, and reticuloendothelial cell proliferation, lymphoid depletion, and necrosis in the spleens. Intracytoplasmic inclusions typical of chlamydiae were seen by light microscopy in hepatocytes and reticuloendothelial cells of the spleen. Electron microscopy demonstrated that these inclusions were composed of three distinct types of particles consistent with the life stages described for chlamydiae.
 ...
PMID:Pathology of naturally occurring chlamydiosis in African clawed frogs (Xenopus laevis). 671 Aug 8

4 cases of massive hepatic amyloidosis are reported with special reference to their clinical profiles and histologic features. On the basis of these data, two different clinical and histologic courses of the disease can be distinguished. 2 patients showed marked hepatomegaly without cholestasis, whereas in the other 2 the clinical picture was characterized by much less pronounced hepatomegaly, but by severe and progressive intrahepatic cholestasis. The time course of the disease seems to be different in the two forms, the cholestatic form being more rapidly fatal than the other.
 ...
PMID:Different morphologic aspects and clinical features in massive hepatic amyloidosis. 674 5

A report on 9 cases of infantile hepatopathy and cirrhosis of the liver respectively in cases of hereditary autosome-recessive alpha 1-antitrypsin deficiency (alpha 1-ATM). The genetic variants of the serum-protease-inhibitor (Pi) alpha 1-antitrypsin (alpha 1-AT) were examined by means of iso-electric focusing (Polyacrylamidgelen). The gene incidence was of the allel PiZ 0,0138 in the 868 blood donors from the Tyrol and was therefore within the range of the PiZ-frequencies seen in other Central-European populations. The other alleles PiM1, PiM2, PiM3, and PiS, point to the incidence of 0.7062, 0.1480, 0.1037, and 0.0225. The patients under observation (9) are homozygote PiZZ, the clinically healthy parents heterozygote PiZM. Risk of repetition in siblings of the patients is 25%. Early indicative symptoms are prolonged jaundice, acholic stools and hepatomegaly. Further developments are the fading of the hyperbilirubinaemia, temporary improvement in the pathological liver values, a freedom of symptoms for different lengths of time in each case, in the case of two patients, finally, decompensated cirrhosis of the liver and death in hepatic coma. The histological picture of the liver tissue shows PAS-positive storage granula in hepatozytes, intrahepatic hypoplasia of the bile duct, cholestasis as well as early cell necrobiosis, fibrosis and cirrhotic transformation. Course and severity of the liver complaint differ greatly, and are independent of the quantitative alpha 1-antitrypsin deficiency revealed, treatment is purely symptomatic.
 ...
PMID:[Hereditary alpha 1-antitrypsin deficiency and infantile cirrhosis of the liver]. 676 50

Endoscopic retrograde cholangiopancreatography revealed the association between intra- and extra-hepatic biliary tree hypoplasia in an 8-year-old boy affected by Alagille's syndrome. The patient, in whom chronic cholestasis had been present from birth, presented characteristic facies, retarded growth, hepatomegaly, and splenomegaly. Chromosome studies were normal. HIDA Tc 99m appeared late in the gallbladder and emptied into the bowel after 3 h. Endoscopic retrograde cholangiopancreatography showed marked and diffuse narrowing of the extrahepatic biliary ducts and uniform narrowing of the intrahepatic ducts with reduced arborization. Menghini needle biopsy revealed no interlobular ducts in the portal tracts. The last two techniques confirmed the clinical picture and made surgical diagnosis unnecessary. This is, as far as we know, the first time endoscopic retrograde cholangiopancreatography has been used in the diagnosis of Alagille's syndrome.
 ...
PMID:Endoscopic retrograde cholangiopancreatography study in Alagille's syndrome: first report. 683 50

To determine the life expectancy of patients with primary biliary cirrhosis, we analyzed survival data from 280 patients with either symptomatic (243) or asymptomatic (37) disease. Patients were followed for up to 19 years (mean, 6.9 years). The average length of survival was 11.9 years--nearly twice that reported in other studies. In contrast, over a 12-year period the survival of the asymptomatic patients after diagnosis did not differ from that of a control population matched for age and sex. Jaundice, weight loss, hepatomegaly, splenomegaly, and ascites were each associated with a poor prognosis. Prognosis also correlated with the histologic stages of hepatic fibrosis, cholestasis, and periportal-cell necrosis. A multivariate analysis of clinical features revealed that at the onset of disease, age, hepatomegaly, and elevated levels of serum bilirubin were independent discriminators of a poor prognosis. A histologic finding of fibrosis limited to portal areas improved this discrimination, correlating with prolonged survival. No other factors enchanced the prediction of risk.
 ...
PMID:The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. 684 17

The authors report the case of a 32-year old woman admitted for hepatomegaly, weight loss, and moderate diarrhea. Liver function tests showed anicteric cholestasis with slight increase in serum level of transaminases. Liver biopsy demonstrated massive steatosis. Biological and radiological investigations of the small intestine showed a malabsorption pattern. Stool fat excretion was 54 g per day. Duodenal biopsies disclosed total villous atrophy. A ten-day treatment with metronidazole (1,5 g per day), followed by a gluten-free diet, resulted in rapid improvement of hepatic and intestinal symptoms. This case report shows that: 1) adult celiac disease may be the cause of severe steatosis; 2) anicteric cholestasis with or without hepatomegaly during the course of adult celiac disease may be secondary to steatosis, as well as primary biliary cirrhosis or malignant infiltration of the liver; 3) bacterial overgrowth should be searched and eventually treated in the case of massive fatty liver occurring in adult celiac disease.
 ...
PMID:[Massive hepatic steatosis disclosing adult celiac disease. Study of a case and review of the literature]. 685 13

A case of alpha-1-antitrypsin deficiency in an 11 month-old Pi ZZ infant is reported. The patient exhibited intrahepatic cholestasis in the first months of life, with jaundice and hepatomegaly. Liver biopsy confirmed the diagnosis and showed the presence of hepatic fibrosis. Pi phenotypes, serum levels of alpha-1-antitrypsin, and symptoms in the family of the patient are reported. Clinical, biochemical and pathologic findings in liver disease in alpha-1-antitrypsin deficiency are discussed.
 ...
PMID:[Neonatal intrahepatic cholestasis and alpha-1-antitrypsin deficiency (author's transl)]. 697 42

In order to assess the prevalence of venocclusive disease in autopsied recipients of bone marrow transplantation, we reviewed coded liver histology from 204 consecutive autopsied recipients transplanted for leukemia (142), other malignancies (5), or aplastic anemia (57). Twenty-seven patients with leukemia, 2 with carcinoma, and 3 with aplasia had venocclusive disease and survived 2-86 days post-transplant. Early lesions showed subintimal edema and hemorrhage within small central venules and centrilobular congestion with hepatocyte degeneration. Later lesions showed subtotal to complete fibrous obliteration of the central venule lumina and centrilobular sinusoidal fibrosis. Thirteen patients had a subclinical course, and 19 were symptomatic. Venocclusive disease was life-threatening or lethal in 13. Typical symptoms developed 1-3 wk post-transplant and consisted of sudden weight gain, hepatic enlargement, ascites, high bilirubin, and encephalopathy. Statistical analyses showed a significantly higher prevalence of venocclusive disease associated with transplantation for leukemia (P = 0.014), pretransplant conditioning with more rigorous chemoradiotherapy regimens (P < 0.001) and three- to fourfold increase of venocclusive disease in patients whose conditioning included dimethyl busulfan (P < 0.005). Abnormal liver tests before transplant were also more prevalent among patients with venocclusive disease. No factors predicted the clinical outcome of established venocclusive disease. Venocclusive disease showed no association with hepatic graft-versus-host disease even among prolonged cases with severe periportal hepatitis and cholestasis. Other centrilobular lesions (hepatocyte degeneration, sinusoidal fibrosis, and phlebosclerosis) were identified in 23 patients. These non-specific changes may occur with viral hepatitis, graft-versus-host disease or chemoradiotherapy effects.
 ...
PMID:An analysis of hepatic venocclusive disease and centrilobular hepatic degeneration following bone marrow transplantation. 700 4

A 27-year-old patient, originally from Martinique, presented with a progressive hepatic granulomatosis with hepatomegaly, splenomegaly, and non-icteric cholestasis, associated with bronchial granulomatosis lesions. The sarcoidosis regressed rapidly after high doses (60 mg/day) of prednisone. Portal hypertension developed later and provoked a severe hematemesis from rupture of esophageal varices. Signs of pulmonary arterial hypertension were then observed, and the diagnosis confirmed by pressure tests after catheterization, and angiography. A portocaval shunt caused the esophageal varices to subside, but the pulmonary arterial hypertension, resistant to corticotherapy, was rapidly fatal. In the case reported, the pulmonary arterial hypertension, independent of any parenchymatous lesion, was attributed to fibrosis of the arterial walls. The association of portal and pulmonary arterial hypertension with sarcoidosis is a very rare occurrence, and the pathogenesis of this association remains a debatable subject.
 ...
PMID:[Sarcoidosis and portal and pulmonary arterial hypertension: a case report (author's transl)]. 722 52


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>