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)

This report deals with two sisters who died with eight, respectively ten weeks under the signs of respiratory failure caused by progressive muscular weakness. Only an elevated cerebrospinal fluid protein was suspicious of an additional disturbance of the central nervous system. Muscle biopsy revealed a vacuolar myopathy. Histochemistry showed lipid storage, increased mitochondrial enzyme activity, and to a lower degree, glycogen accumulation especially in type I muscle fibers. Electron microscopy confirmed elevated lipid content in combination with increased, enlarged and abnormally structured mitochondria. Biochemical studies on muscle biopsy, in comparison with normal children, showed a significant decrease of carnitine content and an increased activity of carnitine palmityltransferase. Retrospectively from a clinical point of view this disease is suggestive of "systemic carnitine deficiency", even if some symptoms (hepatomegaly, cardiomyopathy) were not present and serum- and liver carnitine was not measured because the children died before the diagnosis of muscle carnitine deficiency was confirmed. The clinical picture of these two fatal cases is compared with another observation of muscle caritine deficiency. This child shows only a mild course of muscle disorder, but very similar morphological changes in muscle biopsy. Biochemically, there was a clear decrease in muscular carnitine, while the serum levels were in the normal range. The activity of muscular carnitine palmityltransferase was also normal.
Klin Wochenschr 1979 Sep 17
PMID:[Clinical, morphological and biochemical studies on muscle carnitine deficiency (author's transl)]. 50 58

A patient with liver abscess in association with regional enteritis is reported. Liver abscess should be suspected in patients with regional enteritis who present with fever, elevated serum alkaline phosphatase, liver tenderness, right upper quadrant pain or hepatomegaly.
Am J Gastroenterol 1979 Sep
PMID:Liver abscess. A complication of regional enteritis. 50 33

Ethyl-alpha-p-chlorophenoxyisobutyrate), a hypolipidaemic drug which induces hepatomegaly and proliferation of peroxisomes in liver cells of rats and mice, was fed to 15 male F344 rats at a dietary concentration of 0.5% (v/w) for up to 28 months. Hepatocellular carcinomas developed in 10/11 (91%) rats killed between 24 and 28 months. Other tumours included carcinoma of the pancreas (2 rats), leiomyoma of the small intestine (1 rat) and a large dermatofibrosarcoma (1 rat). Clofibrate is the third hypolipidaemic peroxisome proliferator demonstrated to be hepatocarcinogenic in rats. These studies suggest that hypolipidaemic agents which are capable of producing a sustained hepatomegalic and peroxisome-proliferative effect also induce liver tumours.
Br J Cancer 1979 Sep
PMID:Tumorigenicity of the hypolipidaemic peroxisome proliferator ethyl-alpha-p-chlorophenoxyisobutyrate (clofibrate) in rats. 50 72

Alcohol feeding to rats produced hepatomegaly, associated with enlargement of the hepatocytes. The increase in liver dry weight was accounted for not only by fat but also by protein accumulation, primarily in microsomes and cytosol, with a selective increase in export proteins: concentrations of both immunoreactive albumin and transferrin were augmented in liver microsomes and cytosol of ethanol-fed rats. To investigate the mechanism of this protein accumulation, [14C]leucine was injected intravenously and its incorporation into both liver and serum proteins was measured after various time intervals. Rates of synthesis and export were assessed from protein labeling and specific activities of leucyl-tRNA. Synthesis of liver protein and proalbumin were enhanced by chronic ethanol feeding, but this was not associated with a corresponding rise in serum albumin output. Actually, there was a significant retention of the label in liver albumin and transferrin with delayed appearance in the serum of ethanol-fed rats. This indicated that, regardless of the changes in synthesis, the export of protein from the liver into the plasma was impaired. This alteration in export was associated with a decreased amount of polymerized tubulin in the liver of ethanol-treated animals. Thus, both enhanced protein synthesis and defective export contribute to the ethanol-induced accumulation of liver protein, and the decrease in liver microtubules represents a possible site for impairment of protein export.
J Clin Invest 1977 Sep
PMID:Pathogenesis of alcohol-induced accumulation of protein in the liver. 56 Nov 18

Burkitt's lymphoma presented atypically in a six-year-old Nigerian girl with back pain, oliguria and facial oedema following a fall at school. Two weeks later, she developed bilateral ptosis, hepatomegaly and ascites. Burkitt's lymphoma cells were found in both ascitic and cerebrospinal fluids. She was successfully treated with intravenous cyclophosphamide and intrathecal methotrexate but later developed fatal herpes zoster at the same time as the resident doctor developed chicken pox. Chart's review showed that she had been in brief contact with chicken pox during a short stay in a transit ward prior to full admission.
J Trop Med Hyg 1977 Sep
PMID:Fatal herpes zoster in Burkitt's lymphoma following contact with chicken pox. 59 65

A German Shepherd Dog was examined because of ascites and hepatomegaly. The finding of proteinaceous ascitic fluid and hepatic histologic features of centrizonal sinusoidal congestion suggested hepatic venous outflow obstruction. Diagnostic tests eliminated heart failure from the differential diagnosis and thus indicated an obstruction between the right atrium and hepatic sinusoids. Vascular manometry and angiography established the site of the lesion in the right atrium and confirmed portal vein hypertension. Surgical exploration revealed an unresectable right atrial tumor occluding the caudal vena cava.
J Am Vet Med Assoc 1978 Sep 15
PMID:Portal hypertension secondary to a right atrial tumor in a dog. 71 96

Hydatid disease in southern Ontario is becoming more frequent because of infection in the large immigrant population. Review of 25 cases of hydatid disease of the liver has clarified the clinical features, diagnostic approach and methods of treatment. The commonest presenting symptoms were pain, mass or hepatomegaly, and jaundice. Diagnosis was confirmed, and cysts were localized, by Casoni skin testing, hepatic scanning and angiography. Surgical treatment consisted either of resection, excision or enucleation, and evacuation of the cyst. Two patients were treated by left lateral segmental resection, 6 by either wedge excision of the cyst or enucleation, and 14 by evacuation of cyst contents with or without drainage with the aid of a technique designed to prevent spillage of cyst contents at operation. None of the 14 treated by means of this technique developed recurrent disease, but in 4 of the patients treated by wedge excision or enucleation the cyst contents spilled at operation, resulting in recurrent disease in 2. Controlled cyst evacuation, with tube drainage for infected cavities and those communicating with the biliary tract is the recommended form of treatment in the management of hepatic hydatid disease.
Can J Surg 1976 Sep
PMID:Surgical treatment of hydatid disease of the liver. 78 76

The hepatic manifestations were studied in 65 patients having uncomplicated primary attacks of vivax and falciparum malaria. Hepatomegaly due to a "non-specific reactive hepatitis" occurred in 57% of cases. Jaundice occurred in 15% of patients and was invariably associated with hepatomegaly. The clinical syndromes of jaundice and hepatomegaly in uncomplicated primary attacks of malaria have to be distinguished from those related to disorders like viral hepatitis, hepatic amoebiasis, typhoid hepatitis, infectious mononucleosis and Q fever. The causes for the jaundice and the pathogenesis for the hepatic lesions have been discussed.
J Trop Med Hyg 1976 Sep
PMID:Jaundice and hepatomegaly in primary malaria. 79 14

We describe nine Spanish-American children from five families with an unusual hereditary lipid storage disease. The family origins were in two small southern Colorado towns. The clinical course varied, but all of the children were found to bruise easily and to have splenomegaly, while most had hepatomegaly. Post-natal jaundice and hepatitis occurred in four. Impairment of vertical gaze and intellectual and neurologic deterioration occurred in most of the patients, with the onset of the disease, usually in childhood. The bone marrow in all patients examined contained both foamy and sea-blue histiocytes. Sphingomyelinase levels in skin fibroblast cultures were greatly decreased in seven of the eight cases evaluated. It is believed that these patients have a sphingomyelin lipidosis and represent a variant of the Niemann-Pick disease. Clinical and enzymatic findings are compared with those of other cases in the literature.
Am J Dis Child 1977 Sep
PMID:Nine cases of sphingomyelin lipidosis, a new variant in Spanish-American Children. Juvenile variant of Niemann-Pick Disease with foamy and sea-blue histiocytes. 90 82

A study of liver abnormalities in 36 patients with mixed cryoglobulinemia in the absence of underlying infectious, connective tissue, or lymphoproliferative disorders revealed clinical or biochemical evidence of liver dysfunction in 84%. Hepatomegaly was detected in 77%, splenomegaly in 54%, and abnormalities in bilirubin, alkaline phosphatase, or serum glutamic oxalacetic transaminase in 77%. Only four of the patients had overt liver disease. Of 15 biopsies from 12 patients, there was normal tissue structure in two, minimal nonspecific changes in one, portal fibrosis in three, chronic persistent hepatitis in one, chronic active hepatitis in two, chronic active hepatitis with cirrhosis in four, and postnecrotic cirrhosis in two. These findings, together with the previously reported high incidence of serologic evidence of hepatitis B virus (HBV) infection, support the view that the syndrome of purpura, arthritis, and nephritis is often a consequence of immune-complex vasculitis secondary to HBV infection.
Ann Intern Med 1977 Sep
PMID:Liver involvement in the syndrome of mixed cryoglobulinemia. 90 Jun 72


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