A reduced number of insulin receptors in patients with Prader-Willi syndrome

Kousholt, Arne M.; Beck‐Nielsen, Henning; Lund, Hans T.

doi:10.1530/acta.0.1040345

Cited by 15 publications

(2 citation statements)

References 9 publications

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“…Generally, metformin is recommended as a first-line pharmacological treatment for T2DM in children and adolescents (17). Since increased insulin resistance is observed in some PWS patients with DM (18), some experts advocate the use of metformin for these patients (19). However, decreased secretion of insulin is also reported in other patients having DM with PWS (5).…”

Section: Discussionmentioning

confidence: 99%

Characterization of Diabetes Mellitus in Japanese Prader-Willi Syndrome

Tsuchiya

Oto

Ayabe

et al. 2011

Clin Pediatr Endocrinol

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Prader-Willi syndrome (PWS) is frequently associated with marked obesity and diabetes mellitus (DM). Although the overall frequency of DM in PWS ranges from 7–20%, there is only limited data available on Japanese patients. This study evaluated five factors associated with DM in PWS: 1) frequency, 2) age of onset, 3) risk factors, 4) long-term complications and 5) treatment. Sixty-five patients, ranging in age from 10 to 53 yr, were studied retrospectively. The frequency of DM in patients over 10 yr of age was 26.2% (17/65 patients). The age of DM onset ranged from 10 to 29 yr with a median age of 15 yr. The body mass index (BMI) was significantly higher in the DM group in comparison with the non-DM group. The number of patients using growth hormone (GH) in the DM group was significantly lower than the number that did not. Proteinuria (urinary excretion of albumin/creatinine at spot collection: U-Alb/Cr ≥300 mg/gCr) was observed in 1/17 patients (5.9%), microalbuminuria (U-Alb/Cr 30–300 mg/gCr) was observed in 4/17 patients (23.5%) and nonproliferative retinopathy was observed in 2/17 patients (11.8%). Among oral hypoglycemic agents, alpha-glucosidase inhibitors (α-GI) were most often used in our patients (10/17, 58.8%). Eleven out of 17 patients (64.7%) had been treated with insulin.

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Section: Discussionmentioning

confidence: 99%

Characterization of Diabetes Mellitus in Japanese Prader-Willi Syndrome

Tsuchiya

Oto

Ayabe

et al. 2011

Clin Pediatr Endocrinol

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“…Diabetes mellitus similar to the type II form is common in adults with PWS (2). The abnormally low growth hormone (GH) response to stimulation by clonidine, an a 2 -adrenoreceptor agonist, in lean children with PWS indicates that GH deficiency is a primary feature of this syndrome and not secondary to obesity (3).…”

Section: Introductionmentioning

confidence: 99%

Effect of growth hormone treatment on insulin action in adipocytes from children with Prader-Willi syndrome

Kamel¹,

Norgren²,

Lindgren

et al. 1998

European Journal of Endocrinology

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Objective: To study the effect of growth hormone (GH) treatment (2-4 months) on insulin action in adipocytes isolated from children with Prader-Willi syndrome (PWS), in whom GH deficiency appears to be a primary defect. We investigated the complex effects of GH on carbohydrate metabolism, as part of a current clinical trial of GH treatment in children with PWS. Methods: Biopsies of subcutaneous abdominal adipose tissue were obtained from 12 children with PWS before and after 2-4 months of GH treatment. Lipogenesis was determined by the incorporation of radiolabelled glucose into lipids in isolated adipocytes, and glycerol release to the incubation medium was used as an index of lipolysis. GLUT4 RNA was measured by solution hybridization. Results: With low glucose concentrations, at which glucose transport is rate-limiting, maximal insulininduced lipogenesis was increased by 120% after GH treatment (P < 0.05), but the sensitivity to insulin (half-maximum effective hormone concentration) was unchanged. This was not accompanied by a significant change in the RNA expression of GLUT4. Neither responsiveness (maximum effect) nor sensitivity of insulin-induced inhibition of lipolysis was affected by GH treatment. Conclusions: GH treatment of children with PWS results in an upregulation of insulin-induced lipogenesis in isolated adipocytes, with no effect on insulin-induced inhibition of lipolysis. The data suggest that the site of the effect of GH on lipogenesis is distal to the insulin hormone-receptor interaction, but does not involve altered GLUT4 expression.

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Hyperlipidemia, insulin-dependent diabetes mellitus, and rapidly progressive diabetic retinopathy and nephropathy in Prader-Willi syndrome with del(15)(q11.2q13)

et al. 1997

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We report on a white man with Prader-Willi syndrome (PWS) and del(15)(q11.2q13), confirmed by fluorescence in situ hybridization (FISH), who had hyperlipidemia, insulin-dependent diabetes, and the early onset and rapid progression of diabetic retinopathy and nephropathy within 4 years after diagnosis of diabetes. The spectrum of glucose intolerance in patients with PWS is discussed, as well as those references which suggest that the prevalence of hyperlipoproteinemia in this condition may be greater than previously recognized. We suggest the need for clarification of both the prevalence and types of hyperlipoproteinemia, as well as the pathophysiology of glucose intolerance and correlation with molecular cytogenetic findings. We also encourage careful monitoring for diabetic complications to further clarify the prevalence and possible accelerated course of microvascular lesions.

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A reduced number of insulin receptors in patients with Prader-Willi syndrome

Cited by 15 publications

References 9 publications

Characterization of Diabetes Mellitus in Japanese Prader-Willi Syndrome

Characterization of Diabetes Mellitus in Japanese Prader-Willi Syndrome

Effect of growth hormone treatment on insulin action in adipocytes from children with Prader-Willi syndrome

Hyperlipidemia, insulin-dependent diabetes mellitus, and rapidly progressive diabetic retinopathy and nephropathy in Prader-Willi syndrome with del(15)(q11.2q13)

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