Department of Pediatric Gastro-enterology, University Hospital, Bordeaux.
Department of Pediatrics.
J Pediatr Gastroenterol Nutr. 2020 Feb;70(2):238-242. doi: 10.1097/MPG.0000000000002564.
This multicentric study aimed to evaluate the quality of life (QOL) in children with Hirschsprung's disease (HD).
HD patients aged from 6 to 18 years and followed-up in 2 French pediatric surgery centers were included in this study. QOL was assessed using the HAQL questionnaires according to age (6-11 and 12-18), filled by patients and their parents (proxy reports) and correlated with initial disease characteristics, nutritional status, and functional score of Krickenbeck.
Sixty-three patients were included. The acquisition of satisfactory voluntary bowel movements was found in only 50% of the 6 to 11 years old and 68% of the teenagers. Seventy percentage of the children and 55% of teenagers had soiling issues. The overall HAQLproxy6--11 score was 528/700; best scores were found for "fecal continence" (94/100), "social functioning" (94/100), and "urinary continence" (92/100) whereas the worst scores were for "general well-being" (64/100) and "diurnal fecal continence" (58/100). The overall HAQLproxy12--16 score was 607/700; best scores were for "urinary continence" (96/100) and "social functioning" (93/100). In a multivariate analysis, soiling was the only factor significantly associated with low QOL (P = 0.03).
Soiling remains frequent in children operated on for HD and negatively affects their QOL. Assessment and treatment of soiling should be the priority for medical teams in the follow-up of these children.
本多中心研究旨在评估先天性巨结肠症（HD）患儿的生活质量（QOL）。
本研究纳入了在法国 2 个儿科外科中心接受随访的年龄在 6 至 18 岁的 HD 患儿。采用 HAQL 问卷根据年龄（6-11 岁和 12-18 岁）评估 QOL，由患儿及其家长（代理报告）填写，并与疾病初始特征、营养状况和 Krickenbeck 功能评分相关联。
共纳入 63 例患儿。6-11 岁组仅 50%、青少年组仅 68%的患儿获得满意的自主排便。70%的患儿和 55%的青少年存在粪便污染问题。患儿 HAQLproxy6--11 总分 528/700，其中“粪便控制”（94/100）、“社会功能”（94/100）和“尿控”（92/100）得分最高，“总体健康状况”（64/100）和“日间粪便控制”（58/100）得分最低。青少年 HAQLproxy12--16 总分为 607/700，其中“尿控”（96/100）和“社会功能”（93/100）得分最高。多因素分析显示，粪便污染是唯一与 QOL 显著相关的因素（P = 0.03）。
HD 术后患儿仍经常出现粪便污染，这会对其 QOL 产生负面影响。粪便污染的评估和治疗应成为这些患儿随访中医疗团队的首要任务。
