1/14. Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome.Cutis verticis gyrata (CVG) is a rare disorder; it is characterized by thickening of the scalp which becomes raised to form ridges and furrows resembling the cerebral gyri. We report a case of CVG associated with the autosomal dominant insulin resistance syndrome. This syndrome is characterized by obesity, mild mental retardation, delayed puberty, acanthosis nigricans and hyperinsulinaemia. The association of CVG and autosomal dominant insulin resistance has not been previously described.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
2/14. 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 = 1keywords = obesity (Clic here for more details about this article) |
3/14. insulin resistance, hyperleptinemia, and obstructive sleep apnea in Launois-Bensaude syndrome.OBJECTIVE: Launois-Bensaude Syndrome (LBS) is a very rare cause of obesity, characterized by a symmetrical accumulation of a very large number of lipomata in different regions of the body, excluding the face, the forearms, and the shanks. obesity is known to be closely associated with insulin resistance, hyperleptinemia, and obstructive sleep apnea (OSA). We were interested in studying whether these conditions are also present in patients with obesity due to LBS with a similar frequency as in patients with "simple" truncal obesity. research methods AND PROCEDURES: We performed polysomnography and hyperinsulinemic euglycemic clamp studies and measured serum leptin in three patients with LBS and in six patients with "simple" truncal obesity, matched for sex and body mass index (LBS group, 36.39 kg/m(2); controls, 35.82 kg/m(2)). RESULTS: polysomnography revealed severe OSA in one LBS patient with marked "horsecollar lipomata." In the other LBS patients, no OSA could be demonstrated. The leptin levels of the two groups were comparable (LBS group, 36.39 microg/liter; controls, 37.18 microg/liter) and the insulin responsiveness index was also comparable in the two groups (LBS group, 3.47 micromol/kg. minute; controls, 3.79 micromol/kg. minute). DISCUSSION: patients with LBS demonstrated similar metabolic features in terms of insulin sensitivity and hyperleptinemia as patients with "simple" truncal obesity. LBS is not strictly associated with OSA.- - - - - - - - - - ranking = 5keywords = obesity (Clic here for more details about this article) |
4/14. 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 = 1keywords = obesity (Clic here for more details about this article) |
5/14. hyperinsulinism and overgrowth without obesity.We report a 5 year old girl with postnatal overgrowth (height velocity >97th centile), hyperinsulinaemia, and increased insulin-like growth factor 1 for age, without evidence of bioactive or immunoreactive growth hormone excess or pituitary abnormality. Although her overgrowth may be a result of hyperinsulinism, her serum contains a factor (neither insulin nor IGF-1) which is able to stimulate the proliferation of lymphocyte precursors, and this could also account for the overgrowth. Over the course of two years observation she has developed acanthosis nigricans and diabetes mellitus.- - - - - - - - - - ranking = 4keywords = obesity (Clic here for more details about this article) |
6/14. Cohen syndrome with insulin resistance and seizure.Cohen syndrome is a rare, genetic, connective-tissue disorder with the genetic abnormality linked to chromosome 8q22. The diagnosis of Cohen syndrome is based on the recognition of certain clinical findings, which include mental retardation, typical morphologic stigmata (e.g., truncal obesity, hypotonia, short philtrum, prominent frontal incisors, high-arched palate, narrow hands and feet), and characteristic ophthalmologic abnormalities. We report a patient manifesting the typical characteristics of Cohen syndrome with seizure and hyperinsulinemia.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
7/14. Hyperinsulinemic hypoglycemia with nesidioblastosis after gastric-bypass surgery.We describe six patients (five women and one man; median age, 47 years; range, 39 to 54) with postprandial symptoms of neuroglycopenia owing to endogenous hyperinsulinemic hypoglycemia after Roux-en-Y gastric bypass surgery. Except for equivocal evidence in one patient, there was no radiologic evidence of insulinoma. Selective arterial calcium-stimulation tests, positive in each patient, were used to guide partial pancreatectomy. nesidioblastosis was identified in resected specimens from each patient, and multiple insulinomas were identified in one. Hypoglycemic symptoms diminished postoperatively. We speculate that hyperfunction of pancreatic islets did not lead to obesity but that beta-cell trophic factors may have increased as a result of gastric bypass.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
8/14. A nonsense mutation in the Arg345 of the insulin receptor gene in a Japanese type A insulin-resistant patient.Defects in insulin receptor function have been associated with insulin resistant states such as obesity and type 2 diabetes mellitus. Several types of mutations in the insulin receptor gene have been identified in patients with genetic syndromes of extreme insulin resistance. We have studied a 10-year-old Japanese girl with type A insulin resistance with hirsutism and hyperinsulinemia but without the dysmorphic features characteristic of leprechaunism or Rabson-Mendenhall syndrome. Despite the presence of severe insulin resistance, the patient did not develop overt diabetes mellitus at the time of investigation. Using direct sequencing, we identified a nonsense mutation causing premature termination after amino acid 345 in the alpha subunit of the insulin receptor.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
9/14. Post-gastric bypass hyperinsulinism with nesidioblastosis: subtotal or total pancreatectomy may be needed to prevent recurrent hypoglycemia.Symptomatic hyperinsulinemic hypoglycemia and pancreatic nesidioblastosis have recently been described in a small series of patients after gastric bypass surgery for morbid obesity. In the limited published reports of patients with this condition, hyperinsulinism and nesidioblastosis have been managed with distal or subtotal pancreatectomy, with the extent of resection guided by calcium angiography. However, nesidioblastosis may involve the pancreas diffusely, and limited pancreatic resections may predispose patients to further hypoglycemic episodes. We have treated two patients with refractory hyperinsulinism and symptomatic hypoglycemia after successful gastric bypass surgery. One patient underwent an approximately 80% pancreatectomy with good results but subsequently experienced recurrent drop attacks and fainting from hyperinsulinism; a completion pancreatectomy via a pancreaticoduodenectomy was then required. A second patient had profound hyperinsulinemic hypoglycemia and was treated successfully with a subtotal (95%) pancreatectomy. Our experience, the third published report of post-gastric bypass nesidioblastosis, suggests that the risk of recurrent symptomatic hyperinsulinism after limited pancreatectomy is significant and relative euglycemia may be achieved with subtotal or total pancreatectomy.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
10/14. Reduced urinary insulin clearance in patient with abnormal insulinemia.We recently reported a new case of abnormal insulinemia with LeuA3 insulin. Herein, we measured urinary insulin clearance during oral glucose tolerance tests in proband with abnormal insulinemia (44-yr-old female), three affected family members, two unaffected family members, two other hyperinsulinemic patients with obesity, five non-insulin-dependent diabetic patients, and five normal control subjects. Urinary insulin-to-creatinine clearance ratio in the proband and her affected family members was 0.22 X 10(-3) /- 0.07 (mean /- SD, n = 4) and was markedly reduced compared with those of other groups: 1.73 X 10(-3) in two unaffected family members, 2.77 X 10(-3) in two other hyperinsulinemic patients with obesity, 2.99 X 10(-3) /- 1.48 in five non-insulin-dependent diabetic patients, and 2.54 X 10(-3) /- 0.67 in five normal control subjects. In contrast, urinary c-peptide clearance in these groups was not significantly different from controls. Binding of immunopurified insulins extracted from urine of the patients with abnormal insulinemia to guinea pig kidney membrane was slightly decreased (71% of standard insulin), in contrast with the observation that serum insulin of the proband had much less receptor-binding activity. Reverse-phase HPLC analysis of the immunopurified insulin of the proband revealed that the ratios of normal insulin to abnormal insulin were 8:3 in urine and 1:7 in serum, respectively. These results suggest that excretion of abnormal insulin in urine is much less than that of normal insulin.- - - - - - - - - - ranking = 2keywords = obesity (Clic here for more details about this article) |
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