The growth hormone-insulin-like growth factor axis in adult patients with Prader Willi syndrome

摘要: Abstract Objective. Prader Willi syndrome (PWS) is a genetic disorder characterised by short stature, extreme obesity, body composition abnormalities and behavioural problems. Hypothalamic dysfunction with low growth hormone (GH) secretion levels of GH-related factors common. However, the interpretation difficult because concomitant which in itself has important effects on GH-IGF-I-system. We therefore analysed free total IGF-I, IGF-II their binding proteins obese PWS adults before during 12 months GH treatment. Seventeen adults, 9 men 8 women, 17–32 years age mean BMI 35 ± 2.3 kg/m 2 participated. All had clinical PWS. They were randomized to treatment placebo or (Genotropin®, Pharmacia) 0.8 IU (0.26 mg) for one month, then 1.6 (0.53 mg) 5 months. Subsequently doses individually titrated normal age. Overnight fasting IGF-II, GH-binding protein (GHBP) IGF-binding (IGFBP)-1, -2 -3 measured RIA at baseline after 6 Mean levels ± SEM IGF-I 1.02 ± 0.12 μg/L as compared reference value 0.95 ± 0.15 μg/L, while was 128 ± 15 μg/L (212 ± 14 μg/L) 704 ± 45 μg/L (825 ± 34 μg/L). IGFBP-2 158 ± 24 μg/L (764 ± 72 μg/L) GHBP 2.65 nmol/L (1.71 ± 0.31 nmol/L). IGFBP-1 IGFBP-3 normal. Both increased significantly treatment, IGF- well remained unchanged. Conclusion. Low and, relation non-suppressed are consistent concept that patients have partial deficiency, can be corrected replacement.