PO-0070 Peculiarities Of The Growth Hormone And Insulin-like Growth Factor (igf-1, Igfbp-3) Secretion In Genetically Determined Types Of Short Stature In Uzbekistan
Abstract
<h3>Goal</h3> To study the growth hormone (GH) and insulin-like growth factor (IGF-1, IGFBP-3) secretion in genetically determined types of short stature (GDSS) in Uzbekistan. <h3>Materials and methods</h3> We examined 92 patients with GDSS [11 - Russel-Silver syndrome (RSS), 16 - Noonan syndrome (NS), 10 - Sekkel syndrome (SS), 9 - Prader-Willi syndrome (PWS), 8 - Cornelius de Lange syndrome (CLS), 38 girls with TS] at the age of 7–18. A level of GH, IGF-1, IGFBP-3, anthropometry (SDS) was studied. <h3>Results</h3> Stunting of various degrees of expression was observed in all patients with GDSS but it was most expressed in patients with RSS (-5.16 ± 1.18 SDS), SS (-4.18 ± 1.12 SDS) and CLS (-6.10 ± 1.14 SDS). A reliably low level of GH vs. the control was found in patients with CLS (0.64 ± 0.05 ng/ml, p < 0.05), RSS (0.7 ± 0.04 ng/ml, p < 0.05), SS (1.02 ± 0.07 ng/ml, p < 0.05) on the background of a low level of IGF-1 and IGFBP-3. Patients with NS, TS and PWS had a level of GH within the lower limit norm, 12 girls with TS had a GH level which was reliably low (0.04 ± 0.05 ng/ml, p < 0.05) but IGF-1 and IGFBP-3 rates corresponded to the lower limit of the age norm. <h3>Conclusions</h3> In Uzbek patients with GDSS pronounced stunting was found in patients with RSS, SS and CLS which was associated with disturbances of a direct and reverse relation in the GH-IGF-IGFBP-3 system. A low GH level and relative deficiency of IGF-1 and IGFBP-3 in girls with TS was related with disturbances in the pituitary-ovarian system.