Fürst-Recktenwald S, Dörr H G, Rosanowski F
Department of Pediatrics, University of Erlangen, Germany.
J Pediatr Endocrinol Metab. 2000 Jul-Aug;13(7):959-62. doi: 10.1515/jpem.2000.13.7.959.
Vocal disturbances in women with congenital adrenal hyperplasia and androgen excess should be extremely rare today since effective substitution with glucocorticoids is available. We present a 17 year-old female with congenital adrenal hyperplasia due to 21-hydroxylase deficiency and severe virilization because of long-term insufficient therapy. Laboratory data showed elevated serum levels of testosterone, 17-hydroxyprogesterone, plasma ACTH and a high excretion of urinary pregnanetriol. The phoniatric aspect showed a masculine voice. We discuss the different effects of androgens on the pubertal larynx and various hormonal disturbances that may cause voice changes as well as therapeutic options of voice therapy. From the pediatric point of view it might be important to perform a phoniatric examination in girls with congenital adrenal hyperplasia during puberty in order to monitor androgen effects.
如今，对于患有先天性肾上腺皮质增生症且雄激素分泌过多的女性来说，由于有有效的糖皮质激素替代疗法，嗓音障碍应该极为罕见。我们报告一名17岁女性，因21-羟化酶缺乏患有先天性肾上腺皮质增生症，且由于长期治疗不足导致严重男性化。实验室数据显示血清睾酮、17-羟孕酮、血浆促肾上腺皮质激素水平升高，尿孕三醇排泄量高。嗓音方面表现为男性化嗓音。我们讨论了雄激素对青春期喉部的不同影响、可能导致嗓音变化的各种激素紊乱以及嗓音治疗的选择。从儿科角度来看，在青春期对患有先天性肾上腺皮质增生症的女孩进行嗓音检查以监测雄激素的影响可能很重要。
