Reisch Nicole, Flade Linda, Scherr Michael, Rottenkolber Marietta, Pedrosa Gil Francesco, Bidlingmaier Martin, Wolff Hans, Schwarz Hans-Peter, Quinkler Marcus, Beuschlein Felix, Reincke Martin
Endocrinology and Metabolism, University Hospital München, D-80336 München, Germany.
J Clin Endocrinol Metab. 2009 May;94(5):1665-70. doi: 10.1210/jc.2008-1414. Epub 2009 Mar 3.
Testicular adrenal rest tumors (TARTs) are regarded to contribute to the high prevalence of subfertility in males with congenital adrenal hyperplasia (CAH).
Our objectives were to evaluate reduced fecundity and its possible causes in well-controlled adult males with CAH, and to investigate diagnostic tools for improved treatment monitoring with respect to fertility outcomes.
In a cross-sectional study at the Department of Endocrinology at the University Hospital München, Germany, 22 adult male CAH patients (15 salt wasting and seven simple virilizing, age 19-48 yr) were clinically assessed according to their hormonal control. We performed testicular ultrasound (22 of 22), magnetic resonance imaging (18 of 22), and a semen analysis (19 of 22) in the participants.
All patients had a pathological semen analysis. TART prevalence was 10 of 22 (eight salt wasting, two simple virilizing). Poor therapy control was present in five patients, and all five had TARTs. Of the other 17 well-controlled patients with normal or suppressed adrenal androgens and 17-hydroxyprogesterone levels, five presented with TARTs. There was a significant correlation between sperm concentration and functional testicular volume (r = 0.70; P = 0.002), TART volume (r = -0.70; P = 0.036), as well as inhibin B levels (r = 0.75; P < 0.0001), respectively. In several men, hormonal control parameters suggested hypogonadism, with glucocorticoid overtreatment as a relevant factor for poor semen quality.
Poor semen parameters are common in male CAH patients. TARTs, most likely reflecting undertreatment, as well as inhibin B are important indicators of fecundity. On the other hand, long-term glucocorticoid overtreatment also seems to contribute to low semen quality.
睾丸肾上腺残余瘤（TARTs）被认为是导致先天性肾上腺皮质增生症（CAH）男性患者生育力低下患病率高的原因。
我们的目的是评估病情得到良好控制的成年CAH男性患者生育力降低情况及其可能原因，并研究改善生育结局治疗监测的诊断工具。
在德国慕尼黑大学医院内分泌科进行的一项横断面研究中，根据激素控制情况对22例成年男性CAH患者（15例失盐型和7例单纯男性化型，年龄19 - 48岁）进行了临床评估。我们对参与者进行了睾丸超声检查（22例中的22例）、磁共振成像（22例中的18例）和精液分析（22例中的19例）。
所有患者精液分析均异常。TARTs患病率为22例中的10例（8例失盐型，2例单纯男性化型）。5例患者治疗控制不佳，且这5例均有TARTs。在其他17例肾上腺雄激素和17 - 羟孕酮水平正常或被抑制的病情得到良好控制的患者中，5例有TARTs。精子浓度与功能性睾丸体积（r = 0.70；P = 0.002）、TART体积（r = -0.70；P = 0.036）以及抑制素B水平（r = 0.75；P < 0.0001）之间分别存在显著相关性。在一些男性中，激素控制参数提示性腺功能减退，糖皮质激素过度治疗是精液质量差的一个相关因素。
精液参数不佳在男性CAH患者中很常见。TARTs很可能反映治疗不足，以及抑制素B是生育力的重要指标。另一方面，长期糖皮质激素过度治疗似乎也导致精液质量低下。
