Aycan Zehra, Akbuğa Sami, Cetinkaya Ergun, Ocal Gönül, Berberoğlu Merih, Evliyaoğlu Olcay, Adiyaman Pelin
Departments of Pediatric Endocrinology, Ankara Dişkapi Children's Hospital, Ankara, Turkey.
Turk J Pediatr. 2009 Nov-Dec;51(6):539-44.
The management of children with congenital adrenal hyperplasia (CAH) remains a challenge, especially with regard to their growth potential. We aimed to determine the correlation of the final height of Turkish children with classical CAH to their genetic height potential and to determine the effect of hydrocortisone replacement therapy on the final height. A total of 24 CAH (16 simple virilizing and 8 salt-wasting form) were included in this retrospective longitudinal study. The final height (FH), final height standard deviation score (FHSDS), target height (TH), target height standard deviation score (THSDS), corrected height for target height (CHSDS), weight, and body mass index (BMI) were calculated for all patients. We evaluated the adult height taking into consideration the correlation with the genetic height potential and the country standards. The average follow-up time was 14.2 +/- 3.1 years and the average daily hydrocortisone dose was 19.7 +/- 2.9 mg/m2. The mean FH and FHSDS were 152.2 +/- 7.2 cm and -1.0 +/- 1.1 SD, respectively, in females and 163.1 +/- 6.6 cm and -1.2 +/- 1.0 SD, respectively, in males. The CHSDS was found to be -0.73 +/- 0.9 SD. FH was below the TH in 79.1% of our cases. In 20.8% of our patients, FH was less than the third percentile for the standard height for our country. Interestingly, the FH showed no correlation with the dosage of hydrocortisone. Thirteen of our cases (54.2%) reaching FH were obese/overweight. A positive correlation was detected between hydrocortisone treatment and the BMI. The observations that 79.1% of our classical CAH cases receiving an average daily hydrocortisone dose of 19.7 +/- 2.9 mg/m2 ended up with an adult height below the TH and that 54.2% of the cases were overweight/obese lead us to believe that we should be using the lowest possible dose for treatment.
先天性肾上腺皮质增生症（CAH）患儿的管理仍然是一项挑战，尤其是在其生长潜力方面。我们旨在确定土耳其经典型CAH患儿的最终身高与其遗传身高潜力之间的相关性，并确定氢化可的松替代疗法对最终身高的影响。这项回顾性纵向研究共纳入了24例CAH患儿（16例单纯男性化型和8例失盐型）。计算了所有患者的最终身高（FH）、最终身高标准差评分（FHSDS）、靶身高（TH）、靶身高标准差评分（THSDS）、相对于靶身高的校正身高（CHSDS）、体重和体重指数（BMI）。我们在考虑与遗传身高潜力和国家标准相关性的情况下评估了成人身高。平均随访时间为14.2±3.1年，氢化可的松平均每日剂量为19.7±2.9mg/m²。女性的平均FH和FHSDS分别为152.2±7.2cm和-1.0±1.1SD，男性分别为163.1±6.6cm和-1.2±1.0SD。发现CHSDS为-0.73±0.9SD。在我们的病例中，79.1%的患者FH低于TH。在我们20.8%的患者中，FH低于我国标准身高的第三百分位数。有趣的是，FH与氢化可的松剂量无关。我们的病例中有13例（54.2%）达到FH时肥胖/超重。氢化可的松治疗与BMI之间存在正相关。我们观察到，平均每日接受19.7±2.9mg/m²氢化可的松剂量的经典型CAH病例中有79.1%最终成人身高低于TH，且54.2%的病例超重/肥胖，这使我们认为治疗时应使用尽可能低的剂量。
