Romo Karina G, Shu Sharon W, Iqbal Qasim Z, Uwaifo Gabriel I
Division of Endocrinology, Cedars-Sinai, Los Angeles, CA 90048, USA.
University of Queensland-Ochsner Clinical School, Brisbane, QLD 4072, Australia.
JCEM Case Rep. 2024 Feb 23;2(3):luae018. doi: 10.1210/jcemcr/luae018. eCollection 2024 Mar.
Nonclassic congenital adrenal hyperplasia (NCCAH) is characterized by mild cortisol deficiency, excess androgens and adrenocorticotropin (ACTH) production, and often with various features of dysmetabolic syndrome. Elective bariatric surgery is one of the most effective long-term management strategies for severe obesity. Our case presents a 34-year-old woman with symptomatic NCCAH and class III obesity who status post Roux-en-Y gastric bypass (RYGB) had significant weight loss with metabolic resolution of NCCAH, and no longer required glucocorticoid (GC) therapy. At 11 months post operation and off GC therapy, she had a weight deficit of approximately 160 pounds (72.57 kg) with continued metabolic resolution of NCCAH markers including ACTH, 17-hydroxyprogesterone, and androstenedione. Presently, GC therapy remains one of the few available treatments for symptomatic NCCAH; however, long-term GC therapy has the potential for various complications and side effects. Our case presents elective bariatric surgery as a potential and unique treatment option for patients with NCCAH with associated class III obesity. The exact pathophysiologic basis for this effect and its potential role in long-term management of appropriate NCCAH patients requires further study.
非经典型先天性肾上腺皮质增生症（NCCAH）的特征为轻度皮质醇缺乏、雄激素和促肾上腺皮质激素（ACTH）分泌过多，且常伴有各种代谢综合征特征。择期减肥手术是重度肥胖最有效的长期治疗策略之一。我们的病例报告了一名34岁有症状的NCCAH和Ⅲ级肥胖女性，其接受 Roux-en-Y胃旁路术（RYGB）后体重显著减轻，NCCAH的代谢问题得到解决，且不再需要糖皮质激素（GC）治疗。术后11个月且停用GC治疗时，她体重减轻了约160磅（72.57 kg），NCCAH标志物包括ACTH、17-羟孕酮和雄烯二酮的代谢问题持续得到解决。目前，GC治疗仍是有症状NCCAH少数可用的治疗方法之一；然而，长期GC治疗可能会引发各种并发症和副作用。我们的病例表明，择期减肥手术对于伴有Ⅲ级肥胖的NCCAH患者是一种潜在且独特的治疗选择。这种效应的确切病理生理基础及其在合适的NCCAH患者长期管理中的潜在作用需要进一步研究。
