1/21. nesidioblastosis, myelodysplastic syndrome and nodular diabetic glomerulosclerosis in an elderly nondiabetic woman: an autopsy report.nesidioblastosis as the cause of hyperinsulinaemic hypoglycaemia in an adult is rare. We report here an additional case of nesidioblastosis, which resulted in fatal hyperinsulinaemic hypoglycaemia in a 72-year-old woman with an underlying myelodysplastic syndrome. The diagnosis of nesidioblastosis was established only after post-mortem examination with a careful exclusion of minute insulinoma. To our surprise, the renal pathology disclosed typical diabetic nodular glomerulosclerosis in the same patient who had no previous history of diabetes mellitus (DM). nesidioblastosis has been reported to cause 'reversal' of Type 1 DM and insulinoma causing 'reversal' of Type 2 disease. We therefore hypothesize that our patient might have had an undiagnosed DM in the past, which resulted in the typical diabetic nodular glomerulosclerosis. The nesidioblastosis caused a 'reversal' of DM and even the ultimate development of hyperinsulinaemic hypoglycaemia.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/21. Enhanced intra-abdominal visceral fat accumulation in patients with Werner's syndrome.OBJECTIVE: Studies were made on the abnormality of glucose and lipid metabolism and its cause in four patients with Werner's syndrome to infer the reason for accelerated atherogenesis in this syndrome. RESULTS: Of these four patients, hypercholesterolemia was found in three, hypertriglyceridemia in four, hypoalphalipoproteinemia in two and hypertension in two. All the patients had insulin-resistant diabetes mellitus and three of them had apparent hyperinsulinemia. Abdominal computed tomography revealed that all of them had visceral fat obesity, namely augumented intra-abdominal adipose tissue. CONCLUSION: The clinical features of these patients resemble those recently designated as insulin resistant syndrome (syndrome X) or visceral fat syndrome. The metabolic abnormality may be one of important factors in the accelerated atherogenesis in this syndrome.- - - - - - - - - - ranking = 81.93279054179keywords = adipose, fat (Clic here for more details about this article) |
3/21. Hyperinsulinism in short-chain L-3-hydroxyacyl-coa dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion.A female infant of nonconsanguineous Indian parents presented at 4 months with a hypoglycemic convulsion. Further episodes of hypoketotic hypoglycemia were associated with inappropriately elevated plasma insulin concentrations. However, unlike other children with hyperinsulinism, this patient had a persistently elevated blood spot hydroxybutyrylcarnitine concentration when fed, as well as when fasted. Measurement of the activity of L-3-hydroxyacyl-coa dehydrogenase in cultured skin fibroblasts with acetoacetyl-CoA substrate showed reduced activity. In fibroblast mitochondria, the activity was less than 5% that of controls. Sequencing of the short-chain L-3-hydroxyacyl-coa dehydrogenase (SCHAD) genomic dna from the fibroblasts showed a homozygous mutation (C773T) changing proline to leucine at amino acid 258. Analysis of blood from the parents showed they were heterozygous for this mutation. Western blot studies showed undetectable levels of immunoreactive SCHAD protein in the child's fibroblasts. Expression studies showed that the P258L enzyme had no catalytic activity. We conclude that C773T is a disease-causing SCHAD mutation. This is the first defect in fatty acid beta-oxidation that has been associated with hyperinsulinism and raises interesting questions about the ways in which changes in fatty acid and ketone body metabolism modulate insulin secretion by the beta cell. The patient's hyperinsulinism was easily controlled with diazoxide and chlorothiazide.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
4/21. A family-based study of hyperinsulinemia and hypertriglyceridemia in heterozygous lipoprotein lipase deficiency.CASE REPORT: A case is presented of predisposing a patient's father with obligate heterozygous lipoprotein lipase (LPL) deficiency to mild hypertriglyceridemia in Japanese I-family members (n=8) with patient DI, who was a compound heterozygote for a novel missense mutation of G154V (GG(716)C-->GTC/Gly(154) Val) in exon 5 and a novel splice mutation (Int8/5'-dss/t( 2)c; a T-to-C transition in the invariant GT at position 2 of the 5' donor splice site (dss)) in intron 8 of the LPL gene. RESULTS: The patient's father and paternal grandmother were heterozygotes for the Int8/5'-dss/t( 2)c allele, while the patient's mother and maternal grandmother were heterozygotes for the G154V allele. These four heterozygous carriers with one defective LPL allele showed 45-57% of the mean LPL activity and mass in the post-heparin plasma (PHP) observed in normal individuals. Among the four heterozygous carriers, the patient's father, who was <40 years old, nonobese and hyperinsulinemia, manifested mild hypertriglyceridemia (type IV hyperlipoproteinemia). The remaining three healthy heterozygous carriers (two were >40 years old and the other was <40 years old) were all normolipidemic state. CONCLUSION: In this family, hyperinsulinemia as a marker of insulin resistance may be a strong determinant of hypertriglyceridemia in the carrier with heterozygous LPL deficiency.- - - - - - - - - - ranking = 3keywords = fat (Clic here for more details about this article) |
5/21. Hyperinsulinemia induced by highly active antiretroviral therapy in an adolescent with polycystic ovary syndrome who was infected with human immunodeficiency virus.OBJECTIVE: To report a case of polycystic ovary syndrome (PCOS) in an adolescent infected with human immunodeficiency virus (hiv) and lipodystrophy whose insulin resistance was induced by highly active antiretroviral therapy (HAART). DESIGN: Case report. SETTING: Academic department of pediatrics. PATIENT(S): A 14-year-old vertically hiv-infected adolescent receiving HAART. INTERVENTION(S): Clinical observation, metabolic and endocrinologic assessment, imaging of ovaries by ultrasound, and of intra-abdominal adipose tissue content by magnetic resonance imaging. MAIN OUTCOME MEASURE(S): lipodystrophy, insulin resistance, PCOS. RESULT(S): After 34 months of HAART this adolescent showed lipodystrophy with central obesity and insulin resistance after oral glucose tolerance test (OGTT). Ovaries were normal at ultrasonography. After 56 weeks of HAART, the adolescent showed more severe evidence of lipodystrophy and insulin resistance, and she developed acne, hirsutism, and amenorrhea. The following hormone levels were elevated: FSH 5.9 mUI/mL, LH 15.4 mUI/mL, LH/FSH ratio >2.5, free T 5.6 pg/mL, DHEAS 2,070 ng/mL, androstenedione (A) 3.42 ng/mL; whereas 17-beta-E(2), P, PRL, and free T(4) values were within the normal range. ultrasonography demonstrated a typical polycystic echographic architecture of the ovaries. CONCLUSION(S): lipodystrophy and insulin resistance are well-recognized side effects of HAART. polycystic ovary syndrome might be an additional side effect secondary to insulin resistance and lipodystrophy.- - - - - - - - - - ranking = 75.93279054179keywords = adipose (Clic here for more details about this article) |
6/21. Metabolic effects of growth hormone therapy in an alstrom syndrome patient.AIM: To investigate the metabolic effects of recombinant human growth hormone (rhGH) in an alstrom syndrome patient with growth hormone deficiency. methods: A 15-year-old alstrom syndrome boy with growth hormone deficiency received rhGH therapy for 1 year. Biochemical parameters, including hepatic enzyme levels, lipid profiles, and insulin sensitivity, were measured. body composition analysis and computed tomography scans of the liver were performed. RESULTS: After 1 year of rhGH treatment, body fat mass, fat infiltration in the liver, and serum lipid profiles had all decreased. Insulin sensitivity and acanthosis nigricans improved. CONCLUSION: rhGH therapy might have beneficial effects on body composition, liver fat content, lipid profiles, and insulin resistance in alstrom syndrome patients, with improvement of the glucose homeostasis.- - - - - - - - - - ranking = 3keywords = fat (Clic here for more details about this article) |
7/21. Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation.Inappropriately elevated insulin secretion is the hallmark of persistent hyperinsulinemic hypoglycemia of infancy (PHHI), also denoted congenital hyperinsulinism. Causal mutations have been uncovered in genes coding for the beta-cell's ATP-sensitive potassium channel and the metabolic enzymes glucokinase and glutamate dehydrogenase. In addition, one hyperinsulinemic infant was recently found to have a mutation in the gene encoding short-chain 3-hydroxyacyl-coa dehydrogenase (SCHAD), an enzyme participating in mitochondrial fatty acid oxidation. We have studied a consanguineous family with severe neonatal hypoglycemia due to increased insulin levels and where well-established genetic causes of hyperinsulinism had been eliminated. A genome-wide, microsatellite-based screen for homozygous chromosomal segments was performed. Those regions that were inherited in accordance with the presupposed model were searched for mutations in genes encoding metabolic enzymes. A novel, homozygous deletion mutation was found in the gene coding for the SCHAD enzyme. The mutation affected rna splicing and was predicted to lead to a protein lacking 30 amino acids. The observations at the molecular level were confirmed by demonstrating greatly reduced SCHAD activity in the patients' fibroblasts and enhanced levels of 3-hydroxybutyryl-carnitine in their blood plasma. urine metabolite analysis showed that SCHAD deficiency resulted in specific excretion of 3-hydroxyglutaric acid. By the genetic explanation of our family's cases of severe hypoglycemia, it is now clear that recessively inherited SCHAD deficiency can result in PHHI. This finding suggests that mitochondrial fatty acid oxidation influences insulin secretion by a hitherto unknown mechanism.- - - - - - - - - - ranking = 6keywords = fat (Clic here for more details about this article) |
8/21. The beneficial effect of effective control of anemia on hyperinsulinemia and hypoxemia in a hemodialysis patient with corrected transposition of the great arteries.We report the beneficial effect of control of anemia on hyperinsulinemia and hypoxemia in a hemodialysis patient with corrected transposition of the great arteries. The patient's hemoglobin (Hb) level of 10.3 g/dl on admission represents good control for hemodialysis (HD) patients, but it was too low for this patient with secondary polycythemia because of a right-to-left shunt. Control of anemia for a 10-month period was followed by a marked increase in Hb level (from 10.3 g/dl to 13.9 g/dl) and in aerobic work capacity, while the fasted insulin level decreased from 36.7 microU/ml to 8.0 microU/ml, without changes in leptin level, body mass index (BMI), fat mass, Kt/V, or protein catabolic rate (PCR). Additionally, hypoxemia was ameliorated, from PO2 33.1 mmHg to PO2 56.2 mmHg, and the hyperdynamic cardiac state was improved. The degree of anemia, together with deteriorating tissue oxygenation, may have predisposed this patient to developing insulin resistance and consequent hyperinsulinemia. The most appropriate target Hb concentration should be tailored for the clinical condition of each individual patient, bearing in mind an insulin-resistance state, especially in hemodialysis patients with hypoxemia. A more complete understanding of what regulates insulin resistance and consequent hyperinsulinemia in endstage renal disease (ESRD) awaits the elucidation of carbohydrate and insulin metabolism.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
9/21. Reduction of visceral adiposity after operation in a subject with insulinoma.Generally, it is considered that visceral fat brings insulin resistance and hyper-insulinemia, in the mechanisms of metabolic syndromes. However, whether hyperinsulinemia brings about accumulation of visceral fat is not clear. We followed a case of insulinoma that caused primary hyperinsulinemia, and measured the change in visceral fat and insulin resistance before and after surgical resection of the insulinoma. A 58-year-old woman was admitted to investigate the cause of spontaneous hypoglycemia. An oral glucose tolerance test (OGTT) showed hyperinsulinemia with a high basal level and a glucagon infusion test showed an abnormally high insulin level. Abdominal computed tomography (CT) scan showed an accumulation of visceral fat. Selective celiac angiography showed a pancreatic tumor shadow. Under a diagnosis of insulinoma, the pancreatic body and tail were removed. At 3 months after the operation, the visceral fat area had decreased from 132.6 to 64.2 cm(2). The fasting serum total cholesterol and triglyceride were also reduced. In addition, high-density lipoprotein cholesterol and preheparin serum lipoprotein lipase mass had increased. The midband on the polyacrylamide gel disc electrophoresis of lipoproteins, which appeared before operation, had disappeared completely. An OGTT showed a non-diabetic pattern after the operation. These results suggest that hyperinsulinemia might be one of the factors that enhance visceral adiposity and insulin resistance.- - - - - - - - - - ranking = 5keywords = fat (Clic here for more details about this article) |
10/21. Fatal self-induced hyperinsulinaemia: a delayed post-mortem analytical detection.The inconspicuous number of cases of self-induced hyperinsulinaemia reported in the literature may suggest that many are obscure enough to escape their detection. A case of fatal suicidal hyperinsulinaemia in a non-diabetic is reported here, and in whom only a retrospective biochemical analysis provided an explanatory cause of death. A quantitative radioimmuno assay (RIA) estimation of the refrigerated postmortem blood sample stored at 4 degrees C for three weeks gave a positive insulin yield. It reiterates the need, in forensic cases, for a very low threshold of suspicion and a good back-up for the appropriate body fluid analysis or tissue microexamination, especially when full details of the circumstances surrounding the death are not available at the autopsy. A brief resume on insulin is presented as a background to the current forensic interest in the apparent increase in sudden deaths in young diabetics amidst the controversy about the bio-designed 'human' insulin and subjective unawareness of severe hypoglycaemia.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
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