Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients.
Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients.
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DOI：
10.1210/jc.2010-0917
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发表时间：
2010-11
期刊：
影响因子：
--
通讯作者：
United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHASE)
中科院分区：
文献类型：
--
作者：
Arlt W;Willis DS;Wild SH;Krone N;Doherty EJ;Hahner S;Han TS;Carroll PV;Conway GS;Rees DA;Stimson RH;Walker BR;Connell JM;Ross RJ;United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHASE)
背景：由于缺乏有意义的队列数据，对于成人先天性肾上腺增生症 (CAH) 的治疗尚未达成共识。目的：我们的目的是确定患有 CAH 的成人的健康状况。设计和设置：我们对英国各地专门内分泌中心的患有 CAH 的成人进行了一项前瞻性横断面研究。患者：参与者包括 203 名 CAH 患者（199 名患有 21-羟化酶缺乏症）：138 名女性，65 名男性，中位年龄 34（范围 18-69）岁。主要结果指标：评估人体测量、代谢和主观健康状况。将人体测量数据与英国健康调查数据进行比较，并将心理测量数据与适当的参考队列进行比较。结果：糖皮质激素治疗包括氢化可的松（26%）、泼尼松龙（43%）、地塞米松（19%）或联合用药（10%），41%的患者采用逆昼夜节律给药。雄激素的控制变化很大，只有 36% 的患者血清雄烯二酮水平正常，而 38% 的患者雄激素水平受到抑制，表明糖皮质激素过度治疗。与英国健康调查参与者相比，CAH 患者明显身材矮小，体重指数更高，患有经典 CAH 的女性舒张压升高。代谢异常很常见，包括肥胖（41%）、高胆固醇血症（46%）、胰岛素抵抗（29%）、骨质减少（40%）和骨质疏松（7%）。主观健康状况显着受损，生育能力受到损害。结论：目前，少数英国成年 CAH 患者似乎正在接受内分泌专科护理。在研究的患者中，糖皮质激素替代通常是非生理性的，并且雄激素水平控制不佳。这与不良的代谢状况以及生育能力和生活质量受损有关。需要改进成人 CAH 的临床管理。先天性肾上腺增生症（CAH）成年患者主观健康状况较差，只有少数CAH成人接受内分泌专家定期复查。
Context: No consensus exists for management of adults with congenital adrenal hyperplasia (CAH) due to a paucity of data from cohorts of meaningful size. Objective: Our objective was to establish the health status of adults with CAH. Design and Setting: We conducted a prospective cross-sectional study of adults with CAH attending specialized endocrine centers across the United Kingdom. Patients: Participants included 203 CAH patients (199 with 21-hydroxylase deficiency): 138 women, 65 men, median age 34 (range 18–69) years. Main Outcome Measures: Anthropometric, metabolic, and subjective health status was evaluated. Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts. Results: Glucocorticoid treatment consisted of hydrocortisone (26%), prednisolone (43%), dexamethasone (19%), or a combination (10%), with reverse circadian administration in 41% of patients. Control of androgens was highly variable with a normal serum androstenedione found in only 36% of patients, whereas 38% had suppressed levels suggesting glucocorticoid overtreatment. In comparison with Health Survey for England participants, CAH patients were significantly shorter and had a higher body mass index, and women with classic CAH had increased diastolic blood pressure. Metabolic abnormalities were common, including obesity (41%), hypercholesterolemia (46%), insulin resistance (29%), osteopenia (40%), and osteoporosis (7%). Subjective health status was significantly impaired and fertility compromised. Conclusions: Currently, a minority of adult United Kingdom CAH patients appear to be under endocrine specialist care. In the patients studied, glucocorticoid replacement was generally nonphysiological, and androgen levels were poorly controlled. This was associated with an adverse metabolic profile and impaired fertility and quality of life. Improvements in the clinical management of adults with CAH are required. Adult patients with congenital adrenal hyperplasia (CAH) have poor subjective health status and only a minority of CAH adults receives regular review by endocrine specialists.
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影响因子：
5.8
作者：
CAMERON, FJ;KAYMAKCI, B;WARK, JD
通讯作者：
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影响因子：
3.2
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影响因子：
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作者：
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影响因子：
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作者：
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通讯作者：
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