在患有APECED的儿童中,青春期前生长的渐进性损伤
Viivi Saari1,2, Venla Alanko1,2, Elina Holopainen2,3
1Children's Hospital and Paediatric Research Centre, Helsinki University Hospital, Helsinki 00290, Finland.
The Journal of clinical endocrinology and metabolism
|April 5, 2024
概括
患有自身免疫性多核多角膜病变 - 候群病症 - 皮膜外 (APECED) 的儿童从出生到青春期前经历了渐进的生长障碍. 疾病严重程度和生长激素缺乏症等危险因素需要及时干预,以达到最佳身高.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 遗传学和罕见疾病
- 增长和发展 增长和发展
背景情况:
- 自身免疫性多发性肌病 - - 候选症 - - 皮肤外 (APECED) 与非正常的成年人身高有关.
- 几种风险因素可能会导致APECED儿童的身高低于最佳.
研究的目的:
- 研究诊断为APECED的儿童的生长模式.
- 确定影响小儿APECED患者身高增长的因素.
主要方法:
- 来自芬兰国家APECED队列的59名儿童的回顾性纵向研究.
- 对长度/身高和体重z分数的分析,从出生到青春期结束.
主要成果:
- 患有APECED的儿童从出生到青春期前的身高z-score逐渐下降 (中位数z-score -1.95).
- 与最终身高z-score负相关的因素包括青春期前的年龄,APECED表现的数量,葡萄糖皮质激素治疗持续时间和生长激素缺乏.
- 12%的患者出生时比妊娠年龄小,64%的患者在整个青春期前的高度z分数低于0.
结论:
- 患有APECED的儿童在童年时期表现出显著的,渐进的生长障碍.
- 疾病的严重程度和生长激素缺乏是不高身高的关键风险因素.
- 迫切需要及时干预和新的治疗策略,以改善APECED的增长结果.
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