ABSTRACT
Idiopathic hypogonadotropic hypogonadism(IHH) is a complex oligogenic disease.About 60％ of patients are associated with anosmia or hyposmia,also known as Kallmann syndrome. Based on genetic and molecular biological research,about more than ten IHH responsible genes have been discovered.
ABSTRACT
Objective21 -hydroxylase deficiency ( 21-OHD) patients are at high risk of developing metabolic syndrome.Low dose of glucocorticoid is crucial in the treatment.This study is to investigate the effect of glucocorticoid therapy on potential metabolic disorders.Methods Thirty-two treated and 31 untreated 21-OHD patients were recruited.The components of metabolic syndrome were investigated in both groups.Results Serum testosterone [ (0.61 ±0.12 vs 4.10±0.66) ng/ml,P＜0.01 ],17-(OH) progesterone[ 17-OHP,( 14.83±3.48 vs 48.52±4.72 )ng/ml,P＜0.01 ],dehydroepiandrosterone sulfate[ DHEAS,(55.7±23.6 vs 405.2±65.7 ) μg/dl,P＜0.01 ],and ACTH[ ( 105.8±44.7 vs 617.4± 163.3 ) pg/ml,P＜0.01 ] levels were significantly reduced,whereas body mass index [ ( 23.2±0.9 vs 21.1 ±0.5 ) kg/mz,P＜0.05 ],systolic blood pressure [ ( 120.5 ± 1.3 vs 115.5 ± 1.8 ) mm Hg,P＜0.05,1 mm Hg =0.133 kPa ],serum triglyceride [ ( 1.8±0.2 vs 1.1 ±0.1 ) mmol/L,P＜0.05 ],and homeostasis model assessment for insulin resistance [ HOMA-IR,( 2.07 ± 0.27 vs 1.16 ± 0.12 ),P ＜ 0.01 ] were markedly increased in glucocorticoid treated group.Multivariates regression analysis showed that body mass index was the most important risk factor for HOMA-IR.The correlation of glucocorticoid replacement and HOMA-IR was not observed after adjustment of age and body mass index.ConclusionGlucocorticoid treatment increases body weights,which leads to insulin resistance and metabolic disorders for 21-OHD patients.More attention should be paid to control BMI and metabolic disturbances in 21-OHD patients.