Anterior Pituitary Function in the Prader-Labhart-Willi (PLW) Syndrome

Tolis, G.; Lewis, William D.; Verdy, M; Friesen, Henry G.; Solomon, Samuel; Pagalis, G.; Pavlatos, F.; Fessas, Phaedon; Rochefort, J. G.

doi:10.1210/jcem-39-6-1061

Cited by 52 publications

(9 citation statements)

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“…To date, growth hormone (GH) insufficiency and hypogonadism are well described, and the gonadal development at puberty is typically delayed or incomplete. Surprisingly, in this context, premature adrenarche may be observed in children affected by PWS [11, 12, 13, 14, 15], although the insulin [16]and IGF-I [17]levels are below the normal average due to insufficient GH secretion [18]. The issue of accelerated adrenal development against a background of hypothalamic-pituitary insufficiency in PWS has not yet been explained.…”

Section: Introductionmentioning

confidence: 99%

Increased Adrenal Androgen Levels in Patients with Prader-Willi Syndrome Are Associated with Insulin, IGF-I, and Leptin, but Not with Measures of Obesity

l’Allemand¹,

Eiholzer²,

Rousson

et al. 2002

Horm Res Paediatr

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Background/Aim: Since hyperandrogenism in simple obesity is assumed to arise from hyperinsulinism and/or increased insulin-like growth factor I (IGF-I) or leptin levels, we examined how in patients with Prader-Willi syndrome (PWS), the most frequent form of syndromal obesity, the accelerated adrenarche can be explained despite hypothalamic-pituitary insufficiency with low levels of insulin and IGF-I. Methods: In 23 children with PWS and a mean age of 5.6 years, height, weight, fat mass, fasting insulin concentration, insulin resistance (by HOMA-R; see text), and leptin and IGF-I levels were determined to test whether they explain the variance of the levels of dehydroepiandrosterone (DHEA) and its sulfate (DHEAS), of androstenedione, and of cortisol before and during 42 months of therapy with growth hormone. Results: The baseline DHEAS, DHEA, and androstenedione concentrations were increased as compared with age-related reference values, whereas the cortisol level was always normal. During growth hormone treatment, the DHEA concentration further rose, and the cortisol level decreased significantly. The insulin and IGF-I concentrations were low before therapy, while fat mass and leptin level were elevated. The hormonal covariates provided alone or together between 24 and 60% of the explanation for the variance of adrenal androgen levels, but the anthropometric variables did not correlate with them. Conclusions: In children with PWS, elevated androgen levels correlate with hormones that are usually associated with adiposity. However, the lack of direct correlations between disturbed body composition and androgen levels as well as the increased sensitivity to insulin and IGF-I are abnormalities specific to PWS, potentially caused by the underlying hypothalamic defect.

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Section: Introductionmentioning

confidence: 99%

Increased Adrenal Androgen Levels in Patients with Prader-Willi Syndrome Are Associated with Insulin, IGF-I, and Leptin, but Not with Measures of Obesity

l’Allemand¹,

Eiholzer²,

Rousson

et al. 2002

Horm Res Paediatr

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“…In PWS the GH response to insulin, arginine, clonidine and dopa is reported to be low-normal or blunted [9, 11, 12, 13, 14, 15], as are sleep-induced GH secretion [16]and 24-hour integrated GH concentrations [12]. However, simple obesity is also associated with a decreased circulating concentration of GH, and spontaneous 24-hour GH secretion has been shown to be low and similar in both PWS and healthy obese controls [17].…”

Section: Introduction and Scientific Backgroundmentioning

confidence: 99%

Is There Growth Hormone Deficiency in Prader-Willi Syndrome?

Eiholzer¹,

Bachmann²,

l’Allemand³

2000

Horm Res Paediatr

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Prader-Labhart-Willi syndrome (PWS) is the most frequent form of syndromal obesity. Its main features are associated with hypothalamic dysfunction, which has not yet been comprehensively described. The aim of this review is to present arguments to define the presence of genuine growth hormone (GH) deficiency (GHD) in these patients. Decreasing growth velocity despite the onset of obesity, reduced lean body mass in the presence of adiposity, small hands and feet, relatively low insulin-like growth factor-I and low insulin levels, as well as the dramatic effect of GH treatment on growth, support the presence of hypothalamic GHD in PWS. Even though it might be difficult to ultimately prove GHD in PWS because of the obesity-induced counterregulation, the hormonal situation differs from that in simple obesity. The effects of long-term therapies with GH on body composition in these patients are summarized. GH therapy dramatically changes the phenotype of PWS in childhood: height and weight become normal and there is a sustained impact on the net loss of body fat. We conclude that GHD may account for several features of PWS.

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“…Growth hormone (GH) response to insulin, arginine, clonidine and dopa are reported to be low-normal or blunted [1,4,7,8,16,28], as are sleep-induced GH secretion [9] and 24-h integrated GH concentrations [1]. In contrast to simple obesity, insulin-like growth factor (IGF)-I [2,8,15,16] and insulin-like growth factor binding protein-3 [16] were reported to be low or in the low-normal range.…”

Section: Introductionmentioning

confidence: 99%