Ercan O, Hatemi S, Kutlu E, Turan N
Department of Pediatrics, Division of Pediatric Endocrinology, Istanbul University, Cerrahpasa Faculty of Medicine, Turkey.
Indian J Pediatr. 2000 Nov;67(11):783-9. doi: 10.1007/BF02726219.
In 22 patients with congenital adrenal hyperplasia (CAH), the effect of treatment on growth was evaluated retrospectively. The degree of control with treatment had generally been assessed by measurements of serum 17-hydroxyprogesterone (17-OHP) and/or urinary pregnanetriol levels. In 20 patients, there were no significant differences in both height SDS for bone age (-2.05 +/- 1.89 vs -1.85 +/- 1.30) and height SDS for chronological age (CA) (0.23 +/- 1.94 vs -0.36 +/- 1.81) in a mean uninterrupted treatment period of 5.80 +/- 3.58 years. Further analysis in salt-losers and nonsalt-losers separately showed that height SDS for bone age had decreased in the salt-losers and had increased in the nonsalt-losers. Six patients had signs of early puberty. In 22 patients, there was a negative correlation between the prednisolone dose on one hand and height velocity SDS for CA and bone age velocity on the other. It was also found that the mean serum levels of 17-OHP did not always reflect the degree of long term control measured by auxological parameters. These findings indicate that our treatment in the patients did not result in a significant improvement in height prognosis. We can conclude that growth prognosis in CAH patients can be improved by relying on auxological parameters rather than serum 17-OHP and urinary pregnanetriol levels for monitoring control. Special attention should be paid to salt-losers in this respect.
对22例先天性肾上腺皮质增生症(CAH)患者的治疗效果进行了回顾性评估。治疗的控制程度通常通过测定血清17-羟孕酮(17-OHP)和/或尿孕三醇水平来评估。在20例患者中,平均5.80±3.58年的不间断治疗期间,骨龄身高标准差(-2.05±1.89 vs -1.85±1.30)和实际年龄(CA)身高标准差(0.23±1.94 vs -0.36±1.81)均无显著差异。分别对失盐型和非失盐型患者进行进一步分析显示,失盐型患者骨龄身高标准差下降,而非失盐型患者则上升。6例患者有性早熟迹象。在22例患者中,一方面泼尼松龙剂量与另一方面CA身高速度标准差和骨龄速度之间呈负相关。还发现,17-OHP的平均血清水平并不总是反映通过体格学参数测量的长期控制程度。这些发现表明,我们对患者的治疗并未使身高预后得到显著改善。我们可以得出结论,依靠体格学参数而非血清17-OHP和尿孕三醇水平来监测控制,可改善CAH患者的生长预后。在这方面应特别关注失盐型患者。