Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Eiholzer, Urs; Stutz, K.; Weinmann, Claudia; Torresani, Toni; Molinari, Luciano; Prader, A.

doi:10.1007/s004310050961

Cited by 93 publications

(75 citation statements)

References 25 publications

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“…While fasting or post-prandial insulin levels have sometimes been reported as low in human PWS children, 44,58 this has not been found in the current study and others. 28,39,41,45,48 In addition low fasting and postprandial insulin levels in PWS children and adults may be related more to preserved insulin sensitivity than reduced insulin secretion, as supported by reduced hypertriglyceridaemia and altered levels of related adipocytokines, such as increased plasma adiponectin in PWS.…”

Section: Discussioncontrasting

confidence: 87%

Appetite hormones and the transition to hyperphagia in children with Prader-Willi syndrome

et al. 2012

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OBJECTIVE: Prader --Willi syndrome (PWS) is a genetic neurodevelopmental disorder with several nutritional phases during childhood proceeding from poor feeding, through normal eating without and with obesity, to hyperphagia and life-threatening obesity, with variable ages of onset. We investigated whether differences in appetite hormones may explain the development of abnormal eating behaviour in young children with PWS. SUBJECTS: In this cross-sectional study, children with PWS (n ¼ 42) and controls (n ¼ 9) aged 7 months --5 years were recruited. Mothers were interviewed regarding eating behaviour, and body mass index (BMI) was calculated. Fasting plasma samples were assayed for insulin, leptin, glucose, peptide YY (PYY), ghrelin and pancreatic polypeptide (PP). RESULTS: There was no significant relationship between eating behaviour in PWS subjects and the levels of any hormones or insulin resistance, independent of age. Fasting plasma leptin levels were significantly higher (mean±s.d.: 22.6±12.5 vs 1.97 ± 0.79 ng ml À1 , P ¼ 0.005), and PP levels were significantly lower (22.6 ± 12.5 vs 69.8 ± 43.8 pmol l À1 , Po0.001) in the PWS group compared with the controls, and this was independent of age, BMI, insulin resistance or IGF-1 levels. However, there was no significant difference in plasma insulin, insulin resistance or ghrelin levels between groups, though PYY declined more rapidly with age but not BMI in PWS subjects. CONCLUSION: Even under the age of 5 years, PWS is associated with low levels of anorexigenic PP, as in older children and adults. Hyperghrelinaemia or hypoinsulinaemia was not seen in these young children with PWS. Change in these appetite hormones was not associated with the timing of the transition to the characteristic hyperphagic phase. However, abnormal and/or delayed development or sensitivity of the effector pathways of these appetitive hormones (for example, parasympathetic and central nervous system) may interact with low PP levels, and later hyperghrelinaemia or hypoinsulinaemia, to contribute to hyperphagia in PWS.

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Section: Discussioncontrasting

confidence: 87%

Appetite hormones and the transition to hyperphagia in children with Prader-Willi syndrome

et al. 2012

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“…46 All of these factors were controlled for in the present study. Although IGF-I levels in patients with PWS have been reported to range from normal to subnormal, 37,47,48 as was confirmed in the present study, it may still be possible, arguably, that the GH function is diminished because of a loss of sensitivity for GH metabolites.…”

Section: Discussionsupporting

confidence: 66%

Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: Relationship to physical activity and growth hormone function

Mil¹,

Westerterp²,

Gerver³

et al. 2001

The Journal of Pediatrics

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“…Surprisingly, however, premature adrenarche may be observed in affected children, 55 eventhough their insulin and IGF-1 levels are below the normal average because of insufficient GH secretion. 56 In addition, the mean adrenal androgen levels in patients with PWS are elevated above the normal range, 57 like in healthy obese children. The typically delayed gonadal maturation in PWS may be contrasted by elevated androgen levels and premature adrenarche.…”

Section: Obesity and Pubertymentioning

confidence: 99%

Role of obesity and leptin in the pubertal process and pubertal growth—a review

2003

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The prevalence of obesity is increasing alarmingly to epidemic proportions in children and adolescents, especially in industrialized countries. The finding that overweight children, especially girls, tend to mature earlier than lean children has led to the hypothesis that the degree of body fatness may trigger the neuroendocrine events that lead to the onset of puberty. Obese children have high leptin levels, and these may play a role in their earlier onset of puberty. Leptin receptors have been identified in the hypothalamus, gonadotrope cells of the anterior pituitary, and ovarian follicular cells, as well as Leydig cells. Leptin accelerates gonadotropin-releasing hormone (GnRH) pulsatility in hypothalamic neurons, and it has a direct effect on the anterior pituitary. Leptin administration at low doses may have a permissive, threshold effect on the central networks that regulate gonadotropin secretion. However, at high levels, such as those in obese people, it can have an inhibitory effect on the gonads. Children with obesity also have increased adrenal androgen levels, which may be involved in the accelerated growth of these children before puberty. Recent data indicate that leptin has a specific role in stimulating the activity of enzymes essential for the synthesis of adrenal androgens. Children with exogenous obesity frequently show an increase in height velocity with tall stature for age despite low growth hormone levels. Our group has shown that leptin acts as a skeletal growth factor, with a direct effect on skeletal growth centers, in the mice mandibular condyle, a model of endochondral ossification. In summary, obesity is associated with early puberty. Elevated leptin levels might have a permissive effect on the pubertal process and pubertal growth.

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Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Cited by 93 publications

References 25 publications

Appetite hormones and the transition to hyperphagia in children with Prader-Willi syndrome

Appetite hormones and the transition to hyperphagia in children with Prader-Willi syndrome

Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: Relationship to physical activity and growth hormone function

Role of obesity and leptin in the pubertal process and pubertal growth—a review

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