Balducci R, Toscano V
Istituto Clinica Pediatrica-II University, Rome, Italy.
Clin Endocrinol (Oxf). 1990 Sep;33(3):407-14. doi: 10.1111/j.1365-2265.1990.tb00506.x.
Prepubertal simple hypertrichosis is characterized by excessive growth of vellus hair in children without other signs of endocrinopathies. The aetiology is unknown and it is not clear if it is an abnormal entity or an extreme form of the normal range of hair growth. Sixteen girls (aged 7 +/- 1.9 years, mean +/- SD) with prepubertal simple hypertrichosis and, as controls, 12 normal age-matched girls were studied. All patients were in preadrenarchal age and in all patients an ACTH test was performed to exclude non-classical forms of congenital adrenal hyperplasia. Testosterone (T), androstenedione (A), dehydroepiandrosterone sulphate (DHA-S), dihydrotesterone (DHT), 3 alpha-androstanediol (3Ad) and its glucuronide (3AG), and sex hormone binding globulin (SHBG) were evaluated and free testosterone (FT) and T/SHBG ratio were calculated in all subjects. In all patients we found T, A and DHA-S plasma levels comparable to controls, excluding an increased glandular androgen secretion. No significant differences, compared to controls, were observed for SHBG, FT and T/SHBG ratio, suggesting a normal T bioavailability. DHT plasma levels were significantly increased with respect to controls (0.42 +/- 0.04 vs 0.11 +/- 0.03 nmol/l; P less than 0.002) whereas 3Ad and 3AG were comparable to controls. The very significant increase in DHT plasma levels, without a parallel increase in 3Ad and 3AG found in our cases with prepubertal simple hypertrichosis, is difficult to explain. The clinical and biochemical significance of the high DHT plasma values needs more investigation.
青春期前单纯性多毛症的特征是儿童的毳毛过度生长,且无其他内分泌疾病迹象。病因不明,尚不清楚它是一种异常病症还是正常毛发生长范围的极端形式。我们对16名患有青春期前单纯性多毛症的女孩(年龄7±1.9岁,均值±标准差)以及12名年龄匹配的正常女孩作为对照进行了研究。所有患者均处于肾上腺功能初现前期,对所有患者进行了促肾上腺皮质激素(ACTH)试验以排除非典型形式的先天性肾上腺皮质增生。评估了睾酮(T)、雄烯二酮(A)、硫酸脱氢表雄酮(DHA-S)、双氢睾酮(DHT)、3α-雄烷二醇(3Ad)及其葡糖苷酸(3AG)以及性激素结合球蛋白(SHBG),并计算了所有受试者的游离睾酮(FT)和T/SHBG比值。在所有患者中,我们发现血浆中T、A和DHA-S水平与对照组相当,排除了腺体雄激素分泌增加的情况。与对照组相比,SHBG、FT和T/SHBG比值未观察到显著差异,表明T的生物利用度正常。与对照组相比,DHT血浆水平显著升高(0.42±0.04 vs 0.11±0.03 nmol/l;P<0.002),而3Ad和3AG与对照组相当。在我们患有青春期前单纯性多毛症的病例中,DHT血浆水平非常显著升高,而3Ad和3AG却未平行升高,这难以解释。DHT血浆高值的临床和生化意义需要更多研究。