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)

In 3 patients with dermatomyositis scintigraphic examination of liver and spleen revealed in every case diseases of the liver such as hepatoma, hepatomegaly and damage of the liver parenchyma. Therefore scintigraphic examination of liver and spleen in patients with dermatomyositis is suggested to exclude primary or secondary affections of these organs.
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PMID:[Scintigraphic findings of liver and spleen in dermatomyositis]. 18 42

Two children are described with congenital abnormalities (microcephaly, nystagmus, deafness, hepatomegaly) and the anomalous feature of triglyceride deposits in peripheral adipose tissue associated with severe malnutrition. Peripheral adipose tissue of one of these children displayed: (a) reduced sensitivity of adenyl cyclase to stimulation by noradrenaline (b) no response in tissue levels of cyclic AMP when stimulated by isoprenaline and (c) impaired release of glycerol following stimulation with isoprenaline. The other child, with similar clinical features, showed abnormal deposits of glycogen in the liver. It is postulated that a primary metabolic defect occurs in peripheral adipose tissue (and possibly at other sites such as the liver) that interferes with triglyceride (and glycogen) mobilization during prolonged malnutrition.
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PMID:Triglyceride storage disease. A report of two affected children associated with neurological abnormalities. 18 5

A fatal case of systemic carnitine deficiency is reported. The patient suffered from slowly progressive muscle weakness since early childhood. After the age of 17 years her weakness progressed more rapidly until her death at the age of 20. A pregnancy during the last year of the patient's life was followed by rapid deterioration in her condition. An episode of renal insufficiency occurred at the age of 17 years and hepatomegaly, increased BSP dye retention and intermittent ketoacidosis were present during the last month of her life. Biopsy and autopsy specimens of muscle showed a lipid storage myopathy. Type 1 fibers were selectively severely affected, and many Type 1 fibers were atrophic. Abundant large mitochondria, some also containing abnormal inclusions, were also present in the muscle fibers. At autopsy there was marked accumulation of sudanophilic lipid deposits in all hepatocytes, in the renal tubular epithelial cells, and a patchy increase of lipid material was found in the myocardial fibers. There was marked carnitine deficiency in the patient's liver as well as muscel, while the carnitine palmityltransferase activities in these tissues were abnormally high. The basic metabolic abnormality is assumed to be a defect in carnitine biosynthesis.
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PMID:Fatal systemic carnitine deficiency with lipid storage in skeletal muscle, heart, liver and kidney. 18 38

Observations on the clinical effects of venesection therapy in 85 treated, as compared with 26 untreated, patients with idiopathic haemochromatosis showed decreased pigmentation and hepatomegaly together with a return to normal tests of liver function in half the patients who had abnormal tests at presentation. Control improved in 28 per cent of those patients with diabetes mellitus, although some patients developed it during the period of observation, despite venesection. Portal hypertension, testicular atrophy and arthropathy were not improved. In only 12 patients was there sufficient reaccumulation of iron after the initial course of venesection to merit further treatment. Rates of iron accumulation in these patients varied between 1-4 mg and 4-8 mg per day and chelatable iron levels were noted to be inappropriately high in relation to body iron stores during the early stages of the reaccumulation period. Life table data shows that the percentage survival five and ten years after diagnosis was 66 and 32 per cent respectively for the treated patients, and 18 and 6 per cent respectively for the untreated patients, both statistically highly significant differences (p less than 0-01). Possible clinical differences such as age of presentation, the presence of diabetes mellitus, cirrhosis, clinical hepatic failure and hepatoma between the treated and untreated groups that might otherwise have weighted survival in favour of the treated group were corrected by the use of covariant analysis. This gave mean log survival values of 4-15 and 2-88 for the treated and untreated patients respectively, equivalent to 63-4 months and 17-8 months, a highly significant difference (p less than 0-01). Ten patients, all of whom had cirrhosis at the time of diagnosis, died of malignant hepatoma between three and 15 years after completing venesection therapy. There was also a high rate of death from neoplasms in a variety of other sites--22 per cent in the venesected group, strikingly higher than that rate predicted for a similarly aged population using national cancer mortality rates.
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PMID:Long term results of venesection therapy in idiopathic haemochromatosis. 18 63

A girl aged 3 years and 11 months, with recurrent episodes of unexplained metabolic acidosis, hepatomegaly, and fasting hypoglycemia unresponsive to glucagon, showed profound falls in blood glucose levels in response to oral fructose and glycerol challenge. In vitro analysis of her hepatic glycolytic and gluconeogenic enzymes demonstrated absent fructose-1,6-diphosphatase activity. A therapeutic trial of orally given folic acid, 30 mg daily, did not improve her tolerance for fructose and glycerol. Over the next two years she showed improvement in tolerance to fasting, and to fructose and glycerol loading on dietary management.
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PMID:Fructose-1,6-diphosphatase deficiency. 19 69

Polybrominated biphenyl (PBB), structurally similar to polychlorinated biphenyl (PCB), affects feed intake, egg production, and hatchability of fertile eggs in chickens and quail. Subcutaneous edema of the neck and shoulder are common abnormalities of hatched chicks from PBB-fed dams. No characteristic teratogenicity is evident. Offspring hatched are less viable during their first 3 weeks of life. PBB does not produce thinner egg shells or lower egg weights. The ratio of PBB egg content to dietary level is 1.3-1.5:1.0. The biological half-life of PBB in eggs is calculated to be 17 days. Tissue residues are higher in males than females because egg production is a major excretory route for PBB. Polybrominated biphenyl feeding causes tissue changes in size, structure and/or function in the liver, thyroid, testes, comb, heart, spleen, bursa of Fabricus, and blood. Liver enlargement, porphyria and microsomal enzyme induction results from PBB administration. The spleen, bursa and comb are reduced in size, whereas the thyroid increases after PBB feeding. A characteristic of PBB and PCB toxicity is hydropericardium and general edema.
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PMID:The biological effects of polybrominated biphenyls in avian species. 19 4

We report new operative approaches to the treatment of hepatic vein occlusion due to malignant tumors in the liver and their results in four patients. Two patients had hepatoma, one had metastatic melanoma, and one had metastatic leiomyosarcoma. All of them had abdominal pain, abdominal distention secondary to ascites, and massive hepatomegaly. The right lobe and medial segment of the left lobe of the liver were involved in three patients, and the involvement was diffuse throughout the liver in one. Hepatic veins were occluded completely in one patient, and two of three veins were occluded in the others. Two patients were treated by hepatic resection and removal of tumor thrombus from the hepatic vein under isolation-perfusion technique. They lived 18 and six months, respectively, without recurrence of Budd-Chiari syndrome. Tumors in the other patients were diffuse and could not be resected. The hepatic artery was ligated and chemotherapy was given postoperatively. Ascites and abdominal pain disappeared completely in one, who survived 17 months. The other patient had significant palliation and lived nine months.
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PMID:Surgical management of hepatic vein occlusion by tumor: Budd-Chiari syndrome. 19 61

This paper gives, in detail, the causes of either liver disease or hepatomegaly in 100 patients, mostly adults, admitted to the medical wards of Angau Memorial Hospital, Lae, during 1968 and 1969. The major findings included liver cell carcinoma, cirrhosis (often with chronic active hepatitis), tropical splenomegaly, pericholangitis and hepatitis. There were 27 with miscellaneous findings including ten with normal, or almost normal, livers despite the definite enlargement. Patients with liver cell carcinoma presented late in the course of their illness and had a poor prognosis. Others, with pericholangitis, had clinical features of portal hypertension indistinguishable from that complicated cirrhosis. There was an unexpected number with chronic active hepatitis and a liver biopsy is essential for such a diagnosis. Hepatic sinusoidal lymphocytosis is almost invariably found in patients with TS but may occasionally be found in those with a non-palpable spleen. Patients with right heart failure of chronic respiratory disease, and jaundice of acute pneumonia were excluded from the study.
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PMID:Liver disease in Papua New Guinea. 19 19

Epithelial cells and not fibroblasts from human liver and amniotic fluid contain inducible glucose-6-phosphatase (G-6-Pase) activity. The diagnosis of Von Gierke's disease has been made in a patient with hepatomegaly utilizing cultured epithelial cells grown from a liver biopsy. G-6-Pase activity in epithelial cells from this patient could not be induced by dibutyryl cyclic AMP and theophylline. This is the first use of epithelial cells for diagnosis of a metabolic disease. G-6-Pase activity in cloned epithelial cells from amniotic fluid increases 2- to 3-fold after 24-hr exposure to dibutyryl cyclic AMP and theophylline. The prenatal diagnosis of Von Gierke's disease may be possible in a laboratory experienced with these techniques if epithelial cell growth is obtained from amniotic fluid.
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PMID:Epithelial cells and Von Gierke's disease. 19 49

Daily, for 14 days, rabbits of one group were injected with corticotropin, i.e. ACTH-zinc-phosphate (10 units/kg), whereas rabbits of another group were given (in addition) sodium ribonucleate (40 mg/kg) through a tube into the stomach. Formation of lysyl-tRNA, leucyl-tRNA, and alanyl-tRNA in the liver and the skeletal muscles proved to be significantly greater in the animals which received ACTH together with sodium ribonucleate, as compared to that in the animals given the hormone alone. Hyperglycemia, hepatomegaly, and emaciation were less pronounced in the animals given both the preparations.
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PMID:[Effect of enteral administration of sodium ribonucleate on the synthesis of amino acyl t RNA in the liver and skeletal muscles of rabbits in experimental hypercorticism]. 19 79


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