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)

Two infants with idiopathic panhypopituitarism presented with severe neonatal hypoglycaemia, hepatomegaly and hyperbilirubinaemia (direct and indirect). Abnormal liver function tests returned to normal over a 5--8 month period. The growth rate in the absence of detectable growth hormone was 50% of normal during the first 6 months. The effect of growth hormone on somatomedin levels and growth rate during the first year of life in one of the infants is described.
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PMID:Hyperbilirubinaemia and idiopathic hypopituitarism in the newborn period. 41 96

The present study determined the composition and nucleic-acid content of livers obtained from 11-wk-old, broiler-strain pullets infused with pituitary-derived chicken growth hormone (p-cGH) in either a pulsatile (Study 1) or continuous (Study 2) manner for 21 days. Pulsatile p-cGH administration resulted in a decrease in ratios for liver DNA to wet weight (P less than .01), DNA to protein (P less than .03), and DNA to RNA (P less than .002), compared with controls. Total RNA-to-liver ratio (P less than .003) and liver weight (P less than .007) were significantly elevated, compared with controls. Total grams of protein (P less than .0007), water (P less than .004), and ash (P less than .01) were also elevated, with no significant differences in the percentage of composition. Continuous growth-hormone (GH) administration resulted in a decrease in the DNA-to-protein ratio (P less than .04) and a modest increase in the mass of liver protein (P less than .11) in comparison with controls, suggestive of cell hypertrophy. On a mass and percentage basis, liver composition was otherwise unaffected by the continuous administration of GH. In summary, pulsatile administration of GH induced hepatomegaly, largely due to cell hypertrophy, with no change in relative tissue composition. The continuous administration of GH had no effect on liver size, tissue composition, or most nucleic-acid-based indicators of cell hypertrophy or hyperplasia. Thus, previously observed differences in overall growth performance due to pattern of GH administration were also reflected in selected target-tissue response.
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PMID:Hepatomegaly induced by the pulsatile, but not continuous, intravenous administration of purified chicken growth hormone in broiler pullets: liver composition and nucleic-acid content. 169 89

Mauriac syndrome (MS) consists of a triad of poorly controlled diabetes, profound growth retardation, and hepatomegaly. The mechanisms involved in the growth retardation of those patients are not well understood. In an attempt to determine whether the growth retardation was secondary to somatroph secretory failure, abnormal pulsatile secretion, deletion of the growth hormone (GH) receptor, inadequate insulin-like growth factor I (IGF-I) generation, or abnormal IGF-I binding proteins (IGFBPs) two patients with MS were studied and their results compared with those of age-matched diabetic boys of similar glycemic control who were growing well. Overnight GH profiles in the MS and normally growing diabetics were analyzed by the CLUSTER program. The mean 12-hour GH concentrations, pulse amplitude, and pulse frequency were not different in either group of patients and did not change during acute normalization of the serum glucose overnight in the MS patients. The GH-binding proteins (GHBPs) relative binding were found to be the same in both groups of patients and did not differ from normal nondiabetic sera (62% +/- 8.0% relative specific binding in MS patients, v 53% +/- 4.3% in diabetic controls). The IGF-I concentrations were normal and comparable in both groups of patients (1.1 +/- 0.1 U/mL MS, v 1.1 +/- 0.3 diabetic controls). The IGFBPs were comparable in both groups of patients as well. One of the patients with MS had no meaningful increase in his growth velocity after 1 year on GH therapy despite good compliance. In conclusion, our data show normal hypothalamic-pituitary function, normal GHBP, IGF-I generation, and IGFBPs in two patients with MS when compared with normally growing diabetic children. These data, and the lack of linear growth in response to exogenous GH therapy in one patient, suggest a GH-resistant state, either secondary to impaired bioactivity of IGF-I, or a defect at or distal to the IGF-I receptor.
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PMID:Function of the growth hormone-insulin-like growth factor I axis in the profoundly growth-retarded diabetic child: evidence for defective target organ responsiveness in the Mauriac syndrome. 171 38

Effects of growth hormone (GH) overproduction were studied in transgenic mice expressing murine metallothionein I-GH fusion genes. The most obvious consequence was the acceleration of growth, which led to substantial increases in body weight of up to more than twice that seen in controls. Growth of the internal organs was stimulated, with hepatomegaly and nephromegaly as the most prominent features. GH transgene expression was also reflected in increased skeletal growth which affected various bones to different extents. The mean life-span of human GH transgenic mice with serum levels of hGH ranging from 3 x 10(3) to 9 x 10(5) ng/ml was drastically reduced at 160 days in both sexes. Severe renal lesions were the primary cause of the decrease in life expectancy and were characterized by marked nephron atrophy, obsolescence of numerous glomeruli, and a massive cystic dilation of the tubules. Initial changes involved the glomeruli, which showed significant enlargement and sclerotic lesions. The liver exhibited a pronounced hepatocellularmegaly and progressive degenerative as well as hyperplastic changes. One-third of the hGH transgenic animals displayed myocardial fibrosis. Hepatocellular carcinoma was found in bovine GH transgenic mice older than 12 months. Our observations are compared with results of other investigators.
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PMID:Accelerated growth and visceral lesions in transgenic mice expressing foreign genes of the growth hormone family: an overview. 191 Nov 31

Depending on its etiology hypoglycemia appears after short or prolonged periods of fasting and shows different metabolite and hormonal patterns. In children it is caused by a disturbed homoeostasis of blood glucose (hormonal disorders, decreased activity of glycogenolysis or gluconeogenesis), by a primarily decreased ketogenic activity, or by a deranged adjustment of ketogenesis and carbohydrate metabolism. For the diagnostic procedure the age at manifestation, periods of fasting as well as signs and symptoms (f.e. hepatomegaly, growth retardation, somnolence) have to be carefully evaluated. Based on the extent of ketonemia the hypoglycemic syndromes can be classified into ketotic and hypoketotic forms. Hyperinsulinism, defects in fatty acid oxydation, glycogen storage disease I and postprandial hypoglycemias belong to the second category. In diagnosing hypoglycemia analysis of metabolite (glucose, lactate, beta-hydroxybutyrate, free fatty acids, carnitine) and hormonal (insulin, cortisol, growth hormone) patterns during hypoglycemic episodes is of outstanding importance. Urine has to be analysed for abnormal organic acids in order to demonstrate disturbed fatty acid oxydation. Rarely, loading tests with intermediates of carbohydrate metabolism are necessary. Suspected enzyme deficiencies have to be demonstrated in appropriate tissues (liver biopsy, erythrocytes, fibroblasts).
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PMID:[Diagnostic procedures in pediatric hypoglycemias]. 307 Mar 65

The effect of pulsatile versus continuous intravenous administration of exogenous, pituitary-derived chicken growth hormone (cGH) on growth performance and endocrine/metabolite status of broiler-strain pullets was determined. In a first study, 8-week-old pullets, surgically prepared with intravenous catheters and maintained via a fluid swivel/spring tether/harness system, were administered cGH or vehicle (control) over a 10-min period every 90 min (i.e., 90-min pulse pattern) for 21 consecutive days. Feed intake, body weight gain, and carcass yield and composition were determined in conjunction with plasma concentrations of several hormones and metabolites. In a second study, 8-week-old pullets were intravenously administered cGH or vehicle continuously for 21 consecutive days under the same conditions as for Study I. Pulsatile cGH administration improved feed efficiency (P less than 0.02), increased longitudinal bone growth (P less than 0.02) and mass (P less than 0.01), and reduced abdominal fat pad size (P less than 0.05) and total carcass lipid (P less than 0.09) over the 21-day treatment period in comparison to vehicle infusion. Pulsatile cGH administration also resulted in hepatomegaly, a marked elevation in plasma IGF-I (P less than 0.003) and T3 (P less than 0.005) concentrations, and a reduction in plasma T4 levels (P less than 0.04). In contrast to the above responses to pulsatile cGH, continuous intravenous cGH administration significantly impaired feed efficiency (P less than 0.01) and had no significant effect on abdominal fat pad or liver size or on total carcass lipid, but did result in widening of the epiphyseal growth plate (P less than 0.06) and increased bone mass (P less than 0.01) in comparison to vehicle infused controls. These studies demonstrate that in the broiler chicken, for which endogenous plasma GH concentrations are pulsatile at early ages in conjunction with rapid growth, the pattern of exogenous GH administration is clearly a factor influencing the nature of response to the hormone.
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PMID:Effect of pattern of administration on the response to exogenous pituitary-derived chicken growth hormone by broiler-strain pullets. 320 75

Mulibrey nanism (MUL) is an autosomal recessive disorder with unknown basic metabolic defect. It is characterized by growth failure of prenatal onset, characteristic dysmorphic features, constrictive pericardium, hepatomegaly as a consequence of constrictive pericardium, yellowish dots in the ocular fundi, and J-shaped sella turcica. Hypoplasia of various endocrine glands, causing hormone deficiencies, is common. Here we report the assignment of the MUL gene, by linkage analysis in Finnish families, to a 7-cM region flanked by D17S1799 and D17S948 on chromosome 17q. Multipoint linkage analysis gave a maximum LOD score of 5.01 at loci D17S1606-D17S1853 and at D17S1604. The estimate of the critical MUL region was further narrowed to within approximately 250 kb of marker D17S1853 by linkage disequilibrium analysis. Positional candidate genes that belong to the growth hormone and homeobox B gene clusters were excluded. These data confirm the autosomal recessive inheritance of MUL and allow highly focused attempts to clone the gene.
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PMID:Assignment of the mulibrey nanism gene to 17q by linkage and linkage-disequilibrium analysis. 910 36

A 17 year old boy was admitted because of short stature and hepatomegaly. He was diagnosed with panhypopituitarism and fatty liver. The fatty liver improved, not with hydrocortisone or levothyroxine treatment, but with growth hormone administration. The fatty liver in this patient was attributable to a growth hormone deficient state.
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PMID:Effect of growth hormone on fatty liver in panhypopituitarism. 924 56

A series of studies was conducted to determine whether growth hormone (GH) exerts effects on adult female chickens. Recombinant chicken GH (rcGH) was administered continuously via osmotic minipumps. No consistent effects of rcGH treatment were observed on reproductive indices. Hens receiving rcGH treatment for 10 days exhibited hepatomegaly and showed a tendency (P < 0.1) for increased spleen and thymus weights. Moreover, there were increases in the circulating concentrations of insulin-like growth factor-I (IGF-I) and IGF-binding proteins (IGF-BPs) (22-kDa IGF-BP after 2, 5, and 10 days; 28-kDa IGF-BP after 5 and 10 days; and 36-kDa IGF-BP after 10 days) with rcGH treatment. To determine whether the changes in IGF-BPs were due directly to GH or indirectly via IGF-I, the effects of the continuous administration of rcGH or recombinant human IGF-I (rhIGF-I) were compared. While rcGH again elevated the circulating levels of 28- and 36-kDa IGF-BPs, no such effect was observed with rhIGF-I treatment. However, both treatments exerted similar effects in depressing pituitary GH mRNA levels and elevating plasma concentrations of IGF-I. It is concluded that GH directly elevates circulating concentrations of IGF-I and IGF-BPs, but the negative feedback effect on GH synthesis is mediated via IGF-I.
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PMID:Influence of continuous growth hormone or insulin-like growth factor I administration in adult female chickens. 1033 19

The "cobalt" variant of rainbow trout (Oncorhynchus mykiss) lacks most of the pars intermedia of the pituitary, and shows significant obesity with an enlarged liver and a fat accumulation in the abdominal cavity. Plasma levels of growth hormone, prolactin, and somatolactin were significantly lower in the cobalt variant than those in the normal trout. In contrast, plasma insulin level was four times higher than that in the normal. Plasma levels of total protein, free cholesterol, and triacylglycerol were higher in the cobalt, while those of glucose and fatty acids were not different from the normal levels. In the white muscle, red muscle, liver, and mesenteric fat, the cobalt showed higher contents of triacylglycerol than the normal fish. There was no significant difference in tissue contents of phosphatidylcholine between the two groups of the trout, except for that in the mesenteric fat, exhibiting significantly lower content than in the normal fish. Activity of triacylglycerol lipase in the liver in vivo was lower in the cobalt than that in the normal trout, while there was no significant difference between the two in the cultured liver slices. Desacetyl-alpha-MSH stimulated lipolysis of triacylglycerol similarly in the cultured liver slices from the normal trout and from the cobalt variant. Results from this study suggest that the lack of pars intermedia and the increased plasma level of insulin are involved in a depression of lipid mobilization and obesity in this variant of rainbow trout.
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PMID:Relationships between obesity and metabolic hormones in the "cobalt" variant of rainbow trout. 1227 Jul 86


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