Ekbom Kerstin, Lajic Svetlana, Falhammar Henrik, Nordenström Anna
Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Department of Paediatric Endocrinology, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Department of Paediatric Endocrinology, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
Endocr Pract. 2023 Apr;29(4):266-271. doi: 10.1016/j.eprac.2023.01.010. Epub 2023 Jan 21.
Congenital adrenal hyperplasia (CAH) refers to a group of genetic disorders that affect cortisol biosynthesis and the need for glucocorticoid treatment is lifelong. The complexities of CAH can greatly affect teenage life and the transition from pediatric to adult care. The aim was to assess transition readiness and the impact on quality of life (QoL) as well as medication adherence rates in adolescents and young adults with CAH.
Prospective assessment of transition readiness was conducted through standardized questionnaires for adolescents and young adults (aged 16-35 years). Four open-ended questions on self-care were summarized in adolescents (aged 18-19 years) and their parents. Transition readiness was assessed using a modified CAH specific questionnaire: "Transition preparation and readiness to transfer from pediatric to adult care" with a cutoff level of >25 defined as good transition readiness. Measurement of QoL was performed using Rand 36. Medication adherence rate was measured using the self-reported questionnaire Adherence Starts with Knowledge.
Thirty-eight adolescents and young adults with CAH were included in the study. Transition readiness was classified as good in 26 (68%) of the participants. Good transition readiness was more frequent in participants with good medication adherence rates. A general linear model analysis showed a good transition readiness affected QoL by increasing QoL scores.
Self-reported transition readiness was found in the majority of adolescents and young adults with CAH. A good medication adherence rate was associated with a better transition readiness and a good transition readiness was associated with increased QoL scores.
先天性肾上腺皮质增生症（CAH）是指一组影响皮质醇生物合成的遗传性疾病，且终生都需要糖皮质激素治疗。CAH的复杂性会极大地影响青少年的生活以及从儿科护理向成人护理的过渡。本研究旨在评估CAH青少年和青年的过渡准备情况、对生活质量（QoL）的影响以及药物依从率。
通过针对青少年和青年（年龄在16 - 35岁）的标准化问卷对过渡准备情况进行前瞻性评估。对青少年（年龄在18 - 19岁）及其父母总结了四个关于自我护理的开放式问题。使用一份经过修改的CAH特定问卷“从儿科护理过渡到成人护理的准备及就绪情况”来评估过渡准备情况，将临界值设定为>25分定义为良好的过渡准备情况。使用兰德36量表测量生活质量。使用自我报告问卷“知识开启依从性”测量药物依从率。
38名患有CAH的青少年和青年纳入本研究。26名（68%）参与者的过渡准备情况被分类为良好。药物依从率良好的参与者中，良好的过渡准备情况更为常见。一般线性模型分析表明，良好的过渡准备情况通过提高生活质量得分来影响生活质量。
大多数患有CAH的青少年和青年自我报告有过渡准备情况。良好的药物依从率与更好的过渡准备情况相关，而良好的过渡准备情况与生活质量得分增加相关。
