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Adrenarche in Prader Willi syndrome appears not related
to insulin sensitivity and serum adiponectin


Nancy Unanue
1*, Germán Iñiguez 1, Fanny Cortés 2, Alejandra Avila 1 and Verónica Mericq 1.

1 Institute of Maternal and Child Research, University of Chile, Santiago, Chile, and
2 Institute of Nutrition and Food Technology, University of Chile, Santiago, Chile.
P.O. Box:226-3 Santiago, Chile

Introduction

Prader Willi Syndrome (PWS) is a genetic disorder characterized by short stature, obesity, mental retardation and hypogonadism. Obesity has been linked to insulin resistance and the latter is reported to be associated with premature adrenarche.

Methods

To assess whether prepubertal patients with confirmed PWS (Fish/Karyotype/Methylation) develop premature adrenarche and its relationship with markers of insulin sensitivity we studied 14 PWS (8 F/6 M) and 10 paired obese matched controls (OC) (5 F/5 M) mean ages 7.1+0.5 and 8.3+0.4 years respectively. We performed a complete physical exam (acne, axillary and pubic hair) and withdraw fasting blood samples to determine DHEAS, 17 hydroxyprogesterone (17OHP), androstenedione (A), SHBG, testosterone (T), T4, TSH, glucose, insulin, leptin, IGFBP1, lipid profile, adiponectin . Thereafter an oral glucose tolerance test was performed with blood samples insulin and glucose at 120 minutes. Statistical analysis was performed using non paired T test (parametric) or Mann Whitney (non parametric).

Results: PWS patients were shorter than OC. Axilary hair was present in 1/14 PWS and no OC and pubic hair in 5/14 PWS and 1/10 OC. Eight out of 14 PWS and 2/10 OC patients were small for gestational age. No differences in T4, TSH, BMI, lipid profile, A, T, leptin, HOMA, SHBG, IGFBP1 and free androgen index were observed between PWS patients and OC.

 

PWS n=14

OC n=10

Non paired t test/Mann Whitney

Height (SDS)

-1.27+ 0.35

0.62+0.52

<0.01

BMI (SDS)

2.9+0.4

3.2+0.2

ns

Fasting glucose (mg/dl)

67.9+1.2

74.4+1.8

<0.01

Fasting insulin (mU/l)

8.7+1.6

11.6+1.3

ns

17 OH prog (ng/ml)

1.62+0.2

0.87+0.16

<0.03

DHEAS (ng/ml)

627.4+106.4

246.7+63.2

<0.002

Adiponectin (ug/ml)

13.9+1.0

13.6+0.9

ns

Discussion: Significantly lower blood glucose and higher 17 OH prog and DHEAS levels were found in PWS patients. Serum Adiponectin levels were similar in PWS and OC therefore it is unlikely that differences in insulin sensitivity and adiponectin are related to the development of premature adrenarche in these patients.

July 2003

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