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  • Title: [Efficacy of letrozole in treatment of children with congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency].
    Author: Wang Q, Zhang S, Ma X, Li G, Wang Z, Wang F.
    Journal: Zhejiang Da Xue Xue Bao Yi Xue Ban; 2020 May 25; 49(3):302-307. PubMed ID: 32762167.
    Abstract:
    OBJECTIVE: To assess the efficacy of letrozole in treatment of children with congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency (21-OHD). METHODS: Twenty eight children, including 19 boys and 9 girls aged 4-10y, with CAH due to 21-OHD were enrolled in the study. At the first six months of study, all children received conventional treatment with hydrocortisone or fludrocortisone, then letrozole was added to original regimen. The height velocity (HV), difference between bone age and chronological age (BA-CA), height standard diviation score based on bone age (HtSDS BA), predicted adult height (PAH), Tanner phase, sex hormone, and possible adverse reaction were evaluated and compared between those before and after letrozole treatment. RESULTS: After 6 months of letrozole treatment, there was significant deceleration of HV, but it would recover soon. There was significant increase of HtSDS BA after 12 months of letrozole treatment ( P &lt; 0.05 or P &lt; 0.01), and significant changes in BA-CA after 18 months of letrozole treatment ( P &lt; 0.05). PAH of female children was significantly increased during letrozole treatment ( P &lt; 0.05), whereas PAH of male children was significantly increased 18 months after letrozole treatment ( P &lt; 0.05). Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were significantly increased, but did not meet the diagnostic criteria of central precocious puberty. Estradiol was significantly decreased ( P &lt; 0.01), but no changes in testosterone level was observed. During 24 months letrozole treatment, no hirsutism, severe acne, headache, bone pain, obesity, hypertension, rash and other adverse reactions were observed. CONCLUSIONS: Letrozole can delay bone maturation and improve PAH, which can be used with conventional treatment for children with CAH due to 21-OHD, especially for those with high BA and low PAH. 目的: 探讨来曲唑联合常规方法治疗21-羟化酶缺乏型先天性肾上腺皮质增生症（CAH）患儿的可行性。 方法: 选取2010年12月至2016年1月确诊的4~10岁21-羟化酶缺乏型CAH患儿共28例，其中男性19例，女性9例。入组患儿前6个月为常规治疗（氢化可的松、氟氢可的松），第6个月末开始加用来曲唑联合治疗。通过自身前后对照的方法，对联合治疗前后患儿的骨龄进展速度、预期成年身高、性征发育程度及性激素水平、可能的不良反应等指标进行统计。 结果: 加用来曲唑联合治疗后，患儿身高增长速度在短期（6个月内）有所下降，但很快恢复正常；联合治疗12个月后患儿骨龄别身高标准差分值（HtSDS BA）较入组时及常规治疗均显著改善（ P < 0.05或 P < 0.01）；骨龄与时序年龄差值在联合治疗18个月后较常规治疗显著改善（ P < 0.05）；女性患儿预期成年身高在联合治疗12个月后较常规治疗显著改善（ P < 0.05），男性患儿在联合治疗18个月后预期成年身高亦显著改善（ P < 0.05）；患儿卵泡刺激素、促黄体生成素升高，但未达到中枢性性早熟标准；患儿雌二醇水平降低（ P < 0.01）；患儿睾酮水平无显著改变。来曲唑联合治疗24个月，患儿未见多毛、严重痤疮、头疼、骨痛、肥胖、高血压、皮疹等不良反应。 结论: 来曲唑联合常规治疗的方案可用于21-羟化酶缺乏型CAH患儿长期治疗，特别是骨龄明显超期、预期成年身高受损的患儿，可实现抑制骨龄进展、改善预期成年身高的治疗目的。
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