CAJ | 학술논문

目的单纯男性化型和非经典型21-羟化酶缺陷症(21-OHD)患者常常合并糖脂代谢异常,本文研究接受糖皮质激素替代治疗的21-OHD患者的糖脂代谢的变化.方法收集2004年至2010年期间接受糖皮质激素治疗的21-OHD患者32例,同时收集到未接受糖皮质激素治疗的新诊断的21-OHD患者31例,均经21-羟化酶(CYP21)基因测序突变分析证实为CYP21 A2基因突变.检测人体测量学指标和空腹血糖、血脂、胰岛素、性激素水平,以及口服葡萄糖耐量试验(OGTT)和胰岛素释放试验.结果糖皮质激素治疗组睾酮[(0.61±0.12对4.10±0.66)ng/ml,P＜0.01]、17-羟孕酮[17-OHP,(14.83±3.48对48.52±4.72) ng/ml,P＜0.01]、硫酸脱氢表雄酮[DHEAS,(55.7±23.6对405.2±65.7)μg/dl,P＜0.01]、促肾上腺皮质激素[ ACTH,(105.8±44.7对617.4± 163.3)pg/ml,P＜0.01]水平明显下降,而体重指数[(23.2±0.9对21.1±0.5)kg/m2,P＜0.05]、收缩压[(120.5±1.3对115.5±1.8)mm Hg,P＜0.05,1mm Hg=0.133 kPa]和甘油三酯[(1.8±0.2对1.1±0.1)mmol/L,P＜0.05]却明显增高,并且糖皮质治疗组的稳态模型评估的胰岛素抵抗指数(HOMA-IR)也明显高于未治疗组[(2.07±0.27对1.16±0.12),P＜0.01].多因素回归分析表明体重指数与HOMA-IR的相关性最强.结论糖皮质激素治疗增加21-OHD患者体重指数、血清甘油三酯水平、收缩压和降低胰岛素敏感性.其中体重指数增加是导致胰岛素抵抗的主要原因,因此21-OHD患者的糖皮质激素治疗需注意代谢紊乱问题.
목적 단순남성화형화비경전형21-간화매결함증(21-OHD)환자상상합병당지대사이상,본문연구접수당피질격소체대치료적21-OHD환자적당지대사적변화.방법 수집2004년지2010년기간접수당피질격소치료적21-OHD환자32례,동시수집도미접수당피질격소치료적신진단적21-OHD환자31례,균경21-간화매(CYP21)기인측서돌변분석증실위CYP21 A2기인돌변.검측인체측량학지표화공복혈당、혈지、이도소、성격소수평,이급구복포도당내량시험(OGTT)화이도소석방시험.결과 당피질격소치료조고동[(0.61±0.12대4.10±0.66)ng/ml,P＜0.01]、17-간잉동[17-OHP,(14.83±3.48대48.52±4.72) ng/ml,P＜0.01]、류산탈경표웅동[DHEAS,(55.7±23.6대405.2±65.7)μg/dl,P＜0.01]、촉신상선피질격소[ ACTH,(105.8±44.7대617.4± 163.3)pg/ml,P＜0.01]수평명현하강,이체중지수[(23.2±0.9대21.1±0.5)kg/m2,P＜0.05]、수축압[(120.5±1.3대115.5±1.8)mm Hg,P＜0.05,1mm Hg=0.133 kPa]화감유삼지[(1.8±0.2대1.1±0.1)mmol/L,P＜0.05]각명현증고,병차당피질치료조적은태모형평고적이도소저항지수(HOMA-IR)야명현고우미치료조[(2.07±0.27대1.16±0.12),P＜0.01].다인소회귀분석표명체중지수여HOMA-IR적상관성최강.결론 당피질격소치료증가21-OHD환자체중지수、혈청감유삼지수평、수축압화강저이도소민감성.기중체중지수증가시도치이도소저항적주요원인,인차21-OHD환자적당피질격소치료수주의대사문란문제.
Objective 21 -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.Conclusion Glucocorticoid 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.