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)

Overnight metabolic studies in 39 poorly controlled insulin-treated diabetic patients aged 9 to 66 years showed hypoglycaemia (blood-glucose less than 2 mmol/1) in 22 patients; it lasted 3 h or more in 17. Hypoglycaemic symptoms were very mild or absent, but 19 patients had other features of overtreatment with insulin. These included lethargy, depression, night sweats, morning headaches, fits (3 patients), glycogen-laden hepatomegaly (3), and acquired tolerance to high doses of insulin (mean 1 u/kg/24 h). The best clinical clue to recurrent nocturnal hypoglycaemia was the intermittent occurrence of symptoms, however "mild" and infrequent these appeared to be. Reduction of insulin by a mean of 25% in these patients (without change of species) did not result in loss of overall control; 1 patient with recurrent ketoacidosis was stablished on 40% of his initial dose. It is difficult, sometimes impossible, to achieve good overnight control with conventional once or twice daily insulin therapy. Since patients readily become tolerant of low blood-glucose levels, reliance on urine tests and symptoms of hypoglycaemia as a guide to dosage easily produces a spiral of overtreatment.
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PMID:Unrecognised nocturnal hypoglycaemia in insulin-treated diabetics. 8 75

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

Five patients, 4 men and 1 woman, had adult-onset and slowly progressive weakness. There was distal wasting in 2, hepatomegaly in 3, and congestive heart failure in 2. Electromyography showed a mixed pattern with abundant fibrillations. Serum creatine phosphokinase was increased 5- to 45-fold. Blood glucose failed to respond to epinephrine or glucagon, and venous lactate did not rise after ischemic exercise. Muscle biopsy showed vacuolar myopathy affecting both fiber types. By electron microscopy the vacuoles corresponded to large pools of glycogen not limited by a membrane. Glycogen concentration was 3 to 5 times normal in muscle and 7 to 21 times normal in erythrocytes. In the presence of iodine, muscle glycogen showed a spectrum characteristic of phosphorylase-limit-dextrin. Debrancher activity was measured by a spectrophotometric assay and by a radioactive reverse reaction. The activity was lacking in muscle and erythrocytes of 4 patients according to both assays; in 1 patient the reverse reaction was not impaired. Though previously reported in only 5 patients, debrancher deficiency myopathy may not be rare and should be considered in the differential diagnosis of adult-onset hereditary myopathies.
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PMID:Debrancher deficiency: neuromuscular disorder in 5 adults. 28 18

Supplementation of a closed formula, cereal based stock diet with excess L-histidine at a 5% or 8% level for 4 days reduced growth and induced hepatomegaly and an increase in plasma cholesterole levels in weanling rats. The enlargement of the liver was in part due to glycogen accumulation; plasma glucose concentration was unchanged. Feeding four different amino acids (L-phenylalanine, L-glutamic acid, glycine and L-tryptophan), at levels which caused reduction of growth comparable to the 5% and 8% L-histidine supplementation, did not effect liver weight or plasma cholesterol levels. L-Threonine added, at a 2% level, to the 8% L-histidine diet did not alleviate any of the histidine effects. Rats fed a diet containing 5% urocanic acid, the first metabolite of the histidine degradative pathway, grew at a normal rate but had higher plasma cholesterol levels compared to rats fed stock diet. When rats fed L-histidine-or urocanic acid-supplemented diets were returned to stock diet, a normal growth rate was resumed immediately and plasma cholesterol levels returned to normal within 6 days. These results suggest that L-histidine and/or urocanic acid induce a hypercholesterolemia which disappears several days after the supplementation ceases.
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PMID:Effect of excess dietary L-histidine on plasma cholesterol levels in weanling rats. 65 Feb 95

Hepatic fatty infiltration complicating jejunoileal bypass can be massive and may require restoration of gastrointestinal continuity. This fatty infiltration appears to be caused by protein depletion associated with adequate or high carbohydrate intake. The present study has shown that calorie-free amino acid alimentation can reverse these changes. In three of thirteen patients who underwent 12 inch to 6 inch jejunoileal bypass procedures, symptomatic hepatomegaly developed with near total replacement of hepatocytes by massive fatty infiltration. After undergoing liver scan, liver biopsy, and liver function tests, the patients were started on a peripheral infusion of 2L per day of a 4.25 per cent crystalline amino acid solution, allowing for fat mobilization while preserving body protein stores. All oral intake was withheld except for water. At the end of a fourteen to twenty-one day infusion period, serum albumin levels increased by 1 gm in all patients. Decreases in liver volume of 83, 45, and 40 per cent occurred. During the infusion period ketonuria was 4 plus in all patients indicating active lipolysis. Weight loss was impressive (17, 19, and 40 pounds). All patients showed marked symptomatic improvement, and postinfusion liver biopsy specimens showed a return to near normal architecture. Maintenance of normal liver size by a high-protein, low-carbohydrate diet was observed in a five to seven month follow-up period. In contrast to previous studies using standard hyperalimentation solutions, the use of calorie-free amino acid solutions reverses the hepatic fatty infiltration seen after intestinal bypass by mobilization of fat. This fat mobilization does not occur as readily in the presence of large amounts of glucose.
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PMID:Reversal of severe fatty hepatic infiltration after intestinal bypass for morbid obesity by calorie-free amino acid infusion. 80 74

Fatty liver and kidney syndrome, a disorder of young chicks, was studied under laboratory conditions. Affected chicks had enlarged livers (hepatomegaly), an increased content of lipid in the liver, and an increased level of palmitoleic acid in the liver lipids. The disorder was observed mainly in chicks from young parent flocks, and was associated either with commerical diets which were subsequently found to be low in biotin, or with specially formulated low-biotin diets. A third factor, imposition of stress, was required to initiate the disorder. There was evidence of increased lipogenesis causing an increase of triacylglycerols in the liver lipids and an increased production of saturated fatty acids, particularly palmitic acid. Increased levels of palmitoleic acid resulted from an increased desaturation of palmitic acid. Under stress, affected chicks had low blood glucose levels, suggesting that gluconeogenesis was impaired. Since biotin-dependent enzymes are involved in both gluconeogenesis and lipogenesis, it would appear that the relevant enzymes respond differently to a deficiency of biotin.
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PMID:Fatty liver and kidney syndrome in chicks. I. Effect of biotin in diet. 102 58

The effect of 1-(m-trifluoromethylphenyl)-2-(beta-benzoyloxyethyl)-amino- propane hydrochloride (780 SE) on serum lipids, blood glucose and liver weight was studied in 4 experimental models, and compared with that of clofibrate and tiadenol. When rats were given a daily oral dose of 25 mg/kg or 50 mg/kg of 780 SE for 5 days a marked reduction of serum triglycerides and liver weight was observed. The decreases were more pronounced than those in rats treated with 50 mg/kg or 100 mg/kg of clofibrate or tiadenol. On the other hand, a reduction of serum cholesterol was only observed in the groups given clofibrate and tiadenol. These differences could be explained on the basis of the mechanism of action of the different drugs. Only 780 SE induced a decrease in blood sugar level, a reduction of plasma insulin concentration and restored the insulin sensitivity to a normal value in obese animals. There was a significant decrease in liver weight of 780 SE treated rats, whereas clofibrate and tiadenol cause hepatomegaly.
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PMID:780 SE: a new type of hypolipemic agent. Comparative assays in rats. 107 95

A familial syndrome of partial lipodystrophy inherited as a dominant trait is reported. Subcutaneous fat loss was confined to the extremities and trunk. Diabetes mellitus, hyperlipidemia, hepatomegaly and renal disease were very prevalent in this family. Metabolic studies were performed on 3 members. In vivo tests suggested that the remaining fat tissue responded normally to stimulators and inhibitors of lipolysis. In vitro incubation of the dystrophic fat tissue of one patient suggested that the intracellular pathways of lipid and glucose metabolism were normal. The pattern of subcutaneous loss of adipose tissue observed in this family may be due to sympathetic nervous system overactivity of certain non-contiguous dermatomes.
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PMID:Metabolic studies in familial partial lipodystrophy of the lower trunk and extremities. 120 25

Chemopreventive agents benzyl selenocyanate (BSC) and 1,4-phenylenebis(methylene)selenocyanate (p-XSC) were fed in NIH-07 diet to male and female F344 rats (4, 2, and 0.5 mg/kg/day for BSC and 20, 10, and 5 mg/kg/day for p-XSC) for 13 weeks. Weight gains were depressed for male and female rats fed 4 and 2 mg/kg/day BSC, females fed 0.5 mg/kg/day BSC, and male rats fed 20 and 10 mg/kg/day p-XSC. At necropsy, no clear treatment-related lesions were noted, but dose-dependent hepatomegaly was observed in both sexes of BSC and p-XSC groups. Plasma transaminases AST and ALT were elevated in the higher dose groups, while hemoglobin, HCT, and RBC were reduced in most BSC and some p-XSC treatment groups. Plasma glucose was reduced in BSC-treated males. Significant histologic findings included moderate to severe hepatic centrilobular hypertrophy with fatty change in all males and females in the 4 mg/kg/day BSC groups and in 9/15 males and 3/15 females in the 2 mg/kg/day BSC groups. Dose-dependent, mild centrilobular hypertrophy with minimal fatty change was observed in the mid- and low-dose BSC groups and in all p-XSC groups. Mild to moderate renal tubular and interstitial nephritis occurred in the 4 mg/kg/day male BSC group. Dietary maximum tolerated dose levels for chemoprevention studies are 0.5 mg/kg/day (3.0 ppm Se) for BSC and 5 mg/kg/day (32.5 ppm Se) for p-XSC, compared to literature values of 2-3 ppm Se for Na2SeO3.
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PMID:Subchronic toxicity of benzyl selenocyanate and 1,4-phenylenebis(methylene)selenocyanate in F344 rats. 142 15

A 14-month-old baby weighing 4300 g was a giant infant with macroglossia. Exomphalos was not present, but diastasis recti abdominis was observed. The patient was therefore diagnosed as having Beckwith-Wiedemann syndrome (EMG syndrome). Other characteristic signs such as neonatal hypoglycemia, hemihypertrophy, and a small ventricular septal defect were also recognized, but nephromegaly or hepatomegaly was not present. Tongue reduction by wedge resection was performed under general anesthesia. Some of the problems associated with anesthetic management in this syndrome are hypoglycemia, airway obstruction and cardiovascular status. After induction with increasing concentration of halothane (0.5-4.0%) and 66% nitrous oxide in oxygen, a nasotracheal tube was inserted. Endotracheal intubation was easy without using a neuromuscular blocking agent. Anesthetic maintenance was accomplished with nitrous oxide 66% in oxygen and halothane 0.5-1.0% and no neuromuscular blocking agent was used. The plasma glucose level was kept within normal ranges during and after the operation by infusion of acetate Ringer's solution with 5% glucose. The postoperative progress was uneventful.
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PMID:[Anesthetic management for partial tongue resection in a patient with Beckwith-Wiedemann syndrome]. 160 68


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