改善先天性肾上腺皮质增生症患者终身高的药物治疗进展

The advances in medical treatment of improving the final height in patients with congenital adrenal hyperplasia

先天性肾上腺皮质增生症(congenital adrenal hyperplasia,CAH)在治疗过程中会面临终身高矮的问题,原因可能为未充分抑制的雄激素促进骨骺融合,在治疗过程中出现中枢性性早熟(central precocious puberty,CPP)加速骨骺融合,糖皮质激素的过量应用,以及盐皮质激素补充不足等。研究提示,某些药物包括促性腺激素释放激素类似物(gonadotrophin releasing hormone analogue,GnRHa)、重组人生长激素(recombinant human growth hormone,rhGH)、芳香化酶抑制剂及雄激素受体拮抗剂等可以通过不同作用机制改善CAH患者的终身高。本文对上述药物改善CAH患者终身高的情况进行综述,以期为临床工作提供依据。

Congenital adrenal hyperplasia(CAH)will face impaired final height during the treatment.Final height may be impaired due to insufficient inhibition of androgens which promoted epiphyseal fusion,occurrence of central precocious puberty(CPP)during the treatment which accelerated epiphyseal fusion,excessive of glucocorticoid and deficit of mineralocorticoid,etc.There are some medications that could improve final height to some extent.Gonadotrophin releasing hormone analogue(GnRHa),recombinant human growth hormone(rhGH),aromatase inhibitor,and androgen receptor antagonists could improve the final height through different mechanisms.The improvement of final height of CAH patients with the above drugs were reviewed in order to provide evidence for clinical management in such conditions.