Immunoreactive insulin and growth hormone responses in patients with Prader-Willi syndrome

Parra, Adalberto; Cervantes, C. Escobar; Schultz, Robert B.

doi:10.1016/s0022-3476(73)80219-7

Cited by 35 publications

(17 citation statements)

References 11 publications

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“…Thyroid hormone, lipid profiles, insulin, glucocorticoid, and amino acid levels are comparable to those in obese individuals, although reduced glucose tolerance is reported in one-fifth of PWS patients [Hall and Smith, 1972;Parra et al, 1973;Holm, 1981;Butler et al, 1989c,dl. In six PWS patients, no abnormalities in fat metabolism or transport were identified in response to norepinephrine, insulin, and glucose [Bier et al, 19771. While fat cells were found to be larger than in control individuals, uptake of fat and fatty acid composition in adipose tissue was normal and the fat cell number not increased [Bier et al, 1977;Ginsberg-Fellner, 1981;Gurr et al, 19821.…”

Section: Discussion Clinical Manifestations Of Pwsmentioning

confidence: 93%

Prader‐Willi syndrome: Current understanding of cause and diagnosis

1990

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Prader-Willi syndrome (PWS) is characterized by hypotonia, obesity, hypogonadism, short stature, small hands and feet, mental deficiency, a characteristic face, and an interstitial deletion of the proximal long arm of chromosome 15 in about one-half of the patients. The incidence is estimated to be about 1 in 25,000, and PWS is the most common syndromal cause of human obesity. DNA abnormalities, usually deletions or duplications of chromosome 15, have been identified in individuals with PWS with or without recognizable chromosome 15 deletions. Paternal origin of the chromosome 15 deletion by cytogenetic and DNA studies has been found in nearly all PWS individuals studied. No cytogenetic evidence for chromosome breakage has been identified, although an environmental cause (e.g., paternal hydrocarbon-exposed occupations) of the chromosome 15 abnormality has been proposed. PWS patients with the chromosome 15 deletion are more prone to hypopigmentation compared with PWS individuals with normal chromosomes, but no other clinical differences are consistently identified between those with and without the chromosome deletion. Anthropometric, dermatoglyphic, and other clinical findings indicate homogeneity of PWS patients with the chromosome deletion and heterogeneity of the nondeletion patients. A review of our current understanding of the major clinical, cytogenetic, and DNA findings is presented, and clinical manifestations and cytogenetic abnormalities are summarized from the literature.

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Section: Discussion Clinical Manifestations Of Pwsmentioning

confidence: 93%

Prader‐Willi syndrome: Current understanding of cause and diagnosis

1990

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“…In GH deficiency GH levels are low, free and total IGF-I, IGF-II and IGFBP-3 are low, while IGFBP-1 and -2 are elevated. In childhood PWS GH, total IGF-I and total IGF-II have been reported to be low, while IGFBP-3 has been reported to be normal [4,[44][45][46][47][48][49][50][51][52]. The relative changes in some of the key parameters of the GH-IGF-I axis regulated in simple obesity, GH insufficiency and PWS are summarised in Table 2. IGF-I circulates bound to a number of different binding proteins of which six high affinity proteins (IGFBP-1-6) have been identified.…”

Section: Baseline Datamentioning

confidence: 99%

Endocrine and metabolic aspects of adult Prader–Willi syndrome with special emphasis on the effect of growth hormone treatment

Höybye

2004

Growth Hormone & IGF Research

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“…Impaired glucose tolerance as well as an early and more frequent manifestation of diabetes mellitus were often described in individuals with Prader-Labhart-Willi syndrome (PWS) [1, 2, 3, 4, 5]. To date, however, the precise aetiology of diabetes in PWS remains unknown, and it is defined neither as type 1 nor type 2 [6].…”

Section: Introductionmentioning

confidence: 99%

Carbohydrate Metabolism Is Not Impaired after 3 Years of Growth Hormone Therapy in Children with Prader-Willi Syndrome

l’Allemand¹,

Eiholzer²,

Schlumpf³

et al. 2003

Horm Res Paediatr

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Background/Aim: In children with Prader-Labhart-Willi syndrome (PWS), the insulin secretion is reduced, despite obesity, being ascribed to the growth hormone (GH) deficiency of hypothalamic origin. Besides, an increased prevalence of diabetes mellitus was described in this syndrome. Hence, we addressed the questions of how body composition and insulin secretion are interrelated and what impact GH therapy has on the carbohydrate metabolism in PWS. Methods: We measured weight, lean and fat mass (by dual-energy X-ray absorptiometry), triglycerides, HbA_1c, and fasting insulin and glucose levels in 17 children (age range 1.5–14.6 years) with PWS to examine whether the carbohydrate metabolism is altered during 36 months of therapy with 8 mg GH/m² body surface/week. In a subgroup of 8 children, the insulin secretion was longitudinally assayed during oral glucose tolerance at 0 and 12 months of therapy. Results: Before therapy, the insulin secretion was lower and markedly delayed as compared with reference data and did not rise during therapy. The glucose tolerance was impaired in 2 of 12 children examined by oral glucose tolerance test before therapy and normalized during therapy. Fasting insulin and insulin resistance being normal at the beginning, significantly increased at 12 months and returned to initial levels at 36 months of GH therapy. Fasting glucose as well as HbA_1c and triglyceride levels were always normal. The fat mass before GH therapy was increased (39.5%) and dropped into the upper normal range (28.3%) during 3 years of therapy, being correlated with fasting insulin concentration and indices of insulin sensitivity before and after 1 year of therapy. Conclusions: Children with PWS are characterized by an intact insulin sensitivity with a decrease and a delay of insulin secretion, regardless of moderate obesity or GH treatment. In the present setting, the carbohydrate metabolism is not impaired by GH therapy, but by the excessively increased fat mass.

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Immunoreactive insulin and growth hormone responses in patients with Prader-Willi syndrome

Cited by 35 publications

References 11 publications

Prader‐Willi syndrome: Current understanding of cause and diagnosis

Prader‐Willi syndrome: Current understanding of cause and diagnosis

Endocrine and metabolic aspects of adult Prader–Willi syndrome with special emphasis on the effect of growth hormone treatment

Carbohydrate Metabolism Is Not Impaired after 3 Years of Growth Hormone Therapy in Children with Prader-Willi Syndrome

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