Bachelot Anne, Vialon Magaly, Baptiste Amandine, Tejedor Isabelle, Elie Caroline, Polak Michel, Touraine Philippe
A Bachelot, Endocrinology and reproductive medicine, Hopitaux Universitaires Pitie Salpetriere-Charles Foix, Paris, 75013, France
M Vialon, Endocrinology and reproductive medicine, Hopitaux Universitaires Pitie Salpetriere-Charles Foix, Paris, France.
Endocr Connect. 2017 Aug 9;6(7):422-429. doi: 10.1530/EC-17-0094. Print 2017 Oct.
Health-related quality of life (QoL) in adult patients with congenital adrenal hyperplasia (CAH) has been variously reported. However, there is no study evaluating the impact of transition on quality of life.
Adult patients with classic or non-classic CAH diagnosed during childhood CAH, born between 1970 and 1990, were recruited from the registers of Pediatric departments belonging to the French reference center for endocrine rare disease. Primary end point was the quality of life (WHOQoL -BREF).
Seventy three patients were included in the study, among them 59/73 (81%) were transferred to adult endocrinologist by their pediatricians for transition. WHOQoL -BREF scores were similar between patients with or without transition to specialist adult services, except for environment dimension score, which was slightly higher in CAH patients without transition. However, CAH patients with a regular follow-up had a better physical health, psychological health and environment score and item global quality of life than the group without regular follow-up after transition.
Regular medical follow-up in adulthood is associated with the transition between pediatric and adult care and with better QoL in adults with CAH.
关于成年先天性肾上腺皮质增生症（CAH）患者的健康相关生活质量（QoL）已有多种报道。然而，尚无研究评估过渡对生活质量的影响。
从法国内分泌罕见病参考中心所属儿科部门的登记册中招募1970年至1990年出生、在儿童期被诊断为经典型或非经典型CAH的成年患者。主要终点是生活质量（世界卫生组织生活质量简表-WHOQoL-BREF）。
73名患者纳入研究，其中59/73（81%）由儿科医生转至成人内分泌科医生处进行过渡。无论是否过渡到专科成人服务，患者的WHOQoL-BREF评分相似，但环境维度评分除外，未过渡的CAH患者该评分略高。然而，与过渡后未进行定期随访的组相比，进行定期随访的CAH患者在身体健康、心理健康和环境评分以及总体生活质量方面更好。
成年期定期医学随访与儿科和成人护理之间的过渡相关，且与成年CAH患者更好的生活质量相关。
