Lampela Hanna, Pakarinen Mikko P, Jahnukainen Timo, Jalanko Hannu, Kosola Silja
*Gastrointestinal Surgery, HUH Abdominal Center †Pediatric Liver and Gut Research Group, Children's Hospital ‡Pediatric Surgery, Children's Hospital §Pediatric Nephrology and Transplantation, Helsinki University and Helsinki University Hospital ||School and Student Healthcare, City of Helsinki, Finland.
J Pediatr Gastroenterol Nutr. 2017 Jun;64(6):883-887. doi: 10.1097/MPG.0000000000001516.
The aim of the study was to evaluate health-related quality of life (HRQoL) and parental distress in a national cohort of children with biliary atresia (BA) with their native livers in relation to BA complications and HRQoL of normal population controls.
We invited all Finnish children with BA surviving with their native livers at age 2 to 18 years to participate in 2009 and in 2014. Parents filled the Pediatric Quality of Life Inventory (PedsQL) proxy questionnaire, a survey of their child's health and evaluated parental distress on a visual-analog scale from 0 to 7. Overall participation rates were 80% (12/15) for the longitudinal and 83% (20/24) for the cross-sectional assessment. A control population of 324 children matched for age and sex was randomly picked, and 108 (33%) participated.
Overall, patients and controls had comparable HRQoL. Patients reported significantly lower scores for school functioning (P = 0.004) as depicted by missing school or day care due to hospital visits. Eighty-five percent of parents reported extreme worry (7.0) when hearing their child's BA diagnosis. At 6 years after diagnosis, parents reported significantly less worry: median score 3.8 (interquartile range 3.0-5.4, P < 0.001 for difference). Parents of patients with optimal health were less worried than parents whose children's health was suboptimal: median worry score 3.3 (3.0-4.8) versus 5.3 (3.8-5.9), P = 0.05.
BA patients' HRQoL was comparable to matched peers in general but reduced by missing school days due to frequent hospital visits. At diagnosis, parents experienced considerable worry that diminished over the years after successful portoenterostomy, especially if the child's health was optimal.
本研究旨在评估全国队列中患有先天性胆道闭锁（BA）且肝脏为自身肝脏的儿童的健康相关生活质量（HRQoL）和家长的苦恼程度，并将其与BA并发症以及正常人群对照组的HRQoL进行比较。
我们邀请了所有2009年和2014年时年龄在2至18岁且肝脏为自身肝脏的芬兰BA患儿参与研究。家长填写了儿童生活质量量表（PedsQL）代理问卷，对其孩子的健康状况进行调查，并使用0至7的视觉模拟量表评估家长的苦恼程度。纵向评估的总体参与率为80%（12/15），横断面评估的参与率为83%（20/24）。随机抽取了324名年龄和性别匹配的儿童作为对照人群，其中108名（33%）参与了研究。
总体而言，患者和对照组的HRQoL相当。患者因就医而缺课或缺课日托所导致的学校功能得分显著较低（P = 0.004）。85%的家长在听到孩子的BA诊断时表示极度担忧（7.0）。诊断后6年，家长表示担忧明显减少：中位数得分3.8（四分位间距3.0 - 5.4，差异P < 0.001）。健康状况最佳的患者家长比孩子健康状况欠佳的家长担忧程度更低：担忧中位数得分3.3（3.0 - 4.8）对5.3（3.8 - 5.9），P = 0.05。
一般来说，BA患者的HRQoL与匹配的同龄人相当，但因频繁就医缺课而有所降低。在诊断时，家长经历了相当大的担忧，而在成功进行肝门空肠吻合术后的几年里，这种担忧有所减轻，尤其是如果孩子的健康状况良好。
