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)

An eleven year old boy was referred because of sudden loss of consciousness, muscular weakness, poor general health, severe hypoglycemia with seizures and hepatomegaly. Response to oral glucose and galactose increased blood lactic acid and glucose at different times. Fasting values of blood lactic was normal, but glucose was found at 33 mg/dl. Similar test made up two hours after feeding revealed hyperlactatemia (35-50 mg/dL) and hyperglycemia (129 mg/dL). Glucagon did not result in a rise of glucose at fasting or feeding. Hepatic glycogen content was found 15 gm/100 mg of tissue. The enzyme activities revealed a deficiency of the liver debranching enzyme while leukocytes had normal enzyme activity. Hepatic biopsy showed liver fibrosis. The present case had the clinical characteristics of severe form of glycogen storage disease. A low carbohydrate and high protein diet was indicated in order to increase the gluconeogenic precursors. Although debranching enzyme deficiency is almost always benign a high carbohydrate diet induced a more severe expression of the disease.
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PMID:Diet therapy in severe clinical expression of debrancher deficiency. 184 14

Twenty-eight Zairean patients with Schistosoma mansoni infection were investigated and treated with praziquantel. Of these, 22 were re-examined 18 months later and 13 were found to be re-infected. Eighteen uninfected Zaireans were monitored concurrently to control for variations unrelated to schistosomiasis. Pathophysiological changes related to liver fibrosis were assessed by the determination of serum cholylglycine and procollagen-III-peptide. Circulating T-cell subsets were quantitated, and shedded T-cell antigens were measured in sera. In patients initially presenting with hepatomegaly, the biochemical indicators for egg-induced immunopathology became normal after therapy and remained normal even after re-infection, when the parasite load attained about 50% of the pretreatment level. Among T-cell phenotypes, CD4+ cells transiently increased by three months after treatment, but after 18 months the CD4/CD8 ratios both in patients then re-infected and in those not re-infected had reverted to the respective balances which had been observed at the start of the investigation. Both soluble CD8 antigen and interleukin 2 receptor in patients' sera were significantly elevated throughout the study period. The results indicate a dissociation of factors regulating fibrogenesis and immunomodulation after treatment and re-infected.
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PMID:Re-infection in human schistosomiasis mansoni: a prospective field study 18 months after praziquantel therapy. 212 97

Human hepatic capillariasis is a rare disease, which predominantly affects children aged 1 to 4 years and has a poor prognosis in extensive infections. This is the first case observed in Germany. The main symptoms are those of severe parasitosis: persistent high fever, hepatomegaly, and excessive hypereosinophilia. Diagnosis is confirmed by the finding of intracellular typical eggs in liver biopsy. There is a lack of established therapy; our 18 months old girl developed liver fibrosis. She survived under a prolonged treatment with high doses of Thiabendazole and two single doses of Ivermectine. Decortin was given to inhibit further granulomatous processes in the liver.
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PMID:[Severe liver involvement by Capillaria hepatica]. 229 Apr 36

A rare chronic course of Budd-Chiari syndrome associated with thrombosis of the portal vein was observed in a 30-year-old male patient suffering from postmyocarditic cardiosclerosis. At the age of 24 the patient had infectious allergic myocarditis, was hospitalized and rehospitalized for circulatory insufficiency. Upon 3 years since the disease onset the patient was admitted to a hematological department for progressive enlargement of the spleen. The diagnosis on discharge was idiopathic myelofibrosis with portal hypertension. The treatment included prednisolone, blood transfusions, myelosan. In 1987 the patient presented with enlarged liver and spleen, ascites, gastric and esophageal varicosis, augmenting hepatic insufficiency clinically evaluated as hepatic cirrhosis. Postmortem examination revealed macrofocal cardiosclerosis, splenomegaly, ascites, portal varicosis, enlarged nutmeg liver with smooth surface. Microscopically there was phlebosclerosis and phlebothrombosis varying in duration and involving predominantly medial branches of the hepatic and portal veins, liver fibrosis. The findings provided evidence for the final diagnosis of Budd-Chiari syndrome running an uncommon chronic course.
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PMID:[The chronic form of the Budd-Chiari syndrome]. 297 4

A case report is presented of a 43-year-old woman with generalized peliosis hepatitis that developed during longterm use of oral contraceptives (OCs). The patient had been in good health until the last 2 years when she began to experience vague epigastric pains and a feeling of abdominal distension. Several months prior to admission, she had started to complain of itching and fatigue. There was no history of dark urine, white stools, or hepatitis. On physical examination, no jaundice or cutaneous stigmata of chronic liver disease were observed. Laboratory studies showed a normal erythrocyte sedimentation rate and hematological blood count. A radionuclide study of the liver showed hepatomegaly; especially the left lobe was enlarged. A computerized tomographic scan of the liver showed multiple areas of decreased density in both of the enlarged lobes. There was no evidence of a tumor. Selective transfemoral angiography of the celiac artery also showed hepatic enlargement but no signs of a space-occupying lesion. At laparoscopy, the liver was grossly enlarged and had a lumpy appearance, but again there were no signs of a tumor. No evidence of veno-occlusive disease or hepatocellular adenoma was found. The diagnosis was peliosis hepatitis. The OCs were withdrawn, and the patient was discharged. Regular follow-up in the outpatient department showed no decrease in the size of the liver. The alkaline phosphatase level rose. The fatigue became worse, and cholestyramine was prescribed for progressive itching. In September 1980, the patient was admitted for reevaluation. A repeated CT scan and angiography of the liver again yielded no evidence of a tumor. Esophagoscopy showed the presence of varices grade 2. The liver at laparoscopy had the same appearance as it had in 1976. Histological examination of a biopsy specimen showed occasional dilated sinusoids and locally marked periportal and intralobular fibrosis. No regeneration nodules were found. The diagnosis was liver fibrosis. The patient's condition deteriorated gradually in the following years. She experienced increasing fatigue. Steatorrhea developed, and the patient lost weight. She needed increasing doses of cholestyramine and oral supplementation of vitamins A, D, and K. She was admitted for a 3rd time in February 1985. Esophagoscopy revealed varices grade 4. A CT scan of the liver showed no change. The patient successfully underwent an orthotopic liver transplantation in January 1987. The diagnosis of peliosis hepatis was well documented in this patient.
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PMID:Generalized peliosis hepatis and cirrhosis after long-term use of oral contraceptives. 312 33

Circulating immune complexes (CIC), adult schistosome antibody, and total immunoglobulin concentrations were estimated in sera from 35 chronic Schistosoma mansoni patients with different infection intensities and different pathological complications. High CIC levels were present in about one-third (10/35) of the sera. Most of the patients (9/10) with elevated CIC levels also had hepatomegaly or hepatosplenomegaly. This finding is significant in the pathogenesis of schistosomal liver fibrosis and may also apply to other liver diseases, especially cirrhosis. No correlation was found between infection intensity as judged by stool egg counts and CIC levels. A reverse relationship was observed between the level of anti-adult worm IgG and CIC levels. CIC levels were elevated within 7 and 28 days after treatment in most patients. Hypergammaglobulinaemia was detected in most sera.
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PMID:Circulating immune complex levels in patients with schistosomiasis and complications. 313 Jun 85

Ultrasound diagnosis of chronic schistosomiasis japonica was assessed by comparison with the results of serological tests using enzyme-linked immunosorbent assay (ELISA) with egg and adult worm antigen, and the circumoval precipitation test. The subjects were persons resident in the Chikugo River Basin in Kyushu, southwest Japan, where schistosomiasis used to be highly prevalent. Of 93 individuals with a past history of infection, 37 (39.8%) presented the characteristic echo pattern of the disease (network, sieve, mottled or mixed pattern). In the serological tests, the ultrasound (US)-positive subjects showed a significantly higher IgG antibody-positive rate in ELISA (81.1% for egg-ELISA), than the US-negative subjects (37.5% for egg-ELISA). Individuals bearing dead Schistosoma japonicum eggs proven by tissue biopsy showed a similar antibody-positive rate (76.5% for egg-ELISA) to that of the US-positive subjects. Sera of a group which had no schistosomiasis characteristic echo pattern, but had liver fibrosis, hepatomegaly or liver cirrhosis as shown by US, were also highly positive (71.4% for egg-ELISA). The present serological studies thus confirmed the usefulness of ultrasound diagnosis for chronic schistosomiasis japonica. Furthermore, we were able to determine certain indications of liver abnormality currently undefined in the normally utilized US classifications.
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PMID:Serological evaluation of ultrasound examination for chronic schistosomiasis japonica in a previously endemic area--the Chikugo River Basin, Japan. 315 43

Hepatic glycogenosis, heterogeneous in their type, appear in children as an hepatomegaly discovered during manifestations of hypoglycemia and/or growth disorders, sometimes in the course of a systematic physical examination. A usually late puberty determines a transient aggravation of the height insufficiency. Persistence of a marked hepatomegaly and the development of lever adenomas are characteristic of type I glycogenosis. There, the metabolic imbalance (hyperlipoproteinemia and hyperuricemia, especially), lead to severe vascular and renal complications. Haematologic and sometimes infectious disorders may be added. In type III glycogenosis, the danger depends less on the liver fibrosis, usually minimal, than on the frequently associated cardio-vascular involvement. Type VI glycogenosis, usually have a favorable course. Current therapeutic progresses and a better care should result in a marked improvement of the evolution in type I and probably type III.
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PMID:[Clinical aspects of hepatic glycogenoses]. 316 8

Hepatomegaly is an important clinical finding in patients with argininosuccinic aciduria (a hereditary defect of the urea cycle enzyme, argininosuccinate lyase [argininosuccinase]). A severe degree of liver fibrosis, almost corresponding to cirrhosis, was observed in liver biopsy material obtained from a boy with this disorder. This observation is of interest in light of the fact that liver fibrosis or cirrhosis are hallmarks of many inheritable phenotypes, and especially of inborn errors of metabolism. Variable degrees of liver fibrosis are noted in other inborn defects of the urea cycle, eg, in ornithine transcarbamylase and carbamoylphosphate synthetase deficiencies. These findings appear to indicate that inheritable defects of urea synthesis may form a group of metabolic disorders prone to cause hepatic fibrosis, or even cirrhosis, as shown in our patient.
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PMID:Severe liver fibrosis in argininosuccinic aciduria. 375 45

Sixty-seven cases of alcoholic liver disease were histologically classified into 4 groups: alcoholic liver cirrhosis (ALC), alcoholic hepatitis (AH), alcoholic liver fibrosis (ALF) and alcoholic fatty liver (AFL). They were statistically reclassified by the likelihood method using age, total alcohol intake, hepatomegaly and 12 liver function tests. A score table for likely diagnosis was constructed from the incidences of each range. The cases were re-evaluated using the score table, with an overall correct diagnosis rate of 73%. The best combination of 5 parameters included the indocyanine green plasma disappearance rate, total alcohol intake, cholesterol, choline esterase and glutamic oxaloacetic transaminase/glutamic pyruvic transaminase ratio. A correct diagnosis rate of 75% was attained using these 5 parameters, and 94% of patients were correctly diagnosed by the first or the second likelihood diagnosis. Differential diagnosis of alcoholic liver diseases was easily and confidently obtained with the likelihood score table.
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PMID:Comparative diagnosis of alcoholic liver diseases by multivariate and histological analysis. 398 78


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