Research Unit for Surgery, Odense University Hospital, Odense, Denmark.
University of Southern Denmark, Odense, Denmark.
Pediatr Surg Int. 2024 Feb 9;40(1):52. doi: 10.1007/s00383-024-05634-y.
Hirschsprung's disease (HD) may result in an impaired quality of life (QoL) due to bowel problems, postoperative complications and other health-related issues. The Hirschsprung and Anorectal Malformation Quality of Life (HAQL) questionnaire is a disease-specific instrument developed in the Netherlands to measure the QoL in patients with HD and anorectal malformations. The aim of this study was to translate, culturally adapt and validate HAQL in a Danish Hirschsprung population.
Translation and cultural adaptation were performed according to international guidelines. Invitations to participate in the validation were sent to 401 patients operated for HD during the period from 1985 to 2012. A total of 156 patients completed the translated and culturally adapted Danish versions of HAQL and 35 parents of children and adolescents completed the corresponding parent questionnaire. Reliability was evaluated in terms of internal consistency using Cronbach's α and test-retest reliability using Intraclass Correlation Coefficient for the retest step. Known groups comparison was performed with comparison of mild HD (defined as recto-sigmoidal HD) and serious HD (defined as more proximal disease).
The internal consistency of the dimensions was overall satisfactory for adults and adolescents but more problematic for children, where Cronbach's α was less than 0.7 in 60% of the dimensions. For both children and adolescents, the α-value was unsatisfactory for social functioning, emotional functioning, and body image. The test-retest reliability was overall good. The known groups comparison was only able to demonstrate a significant difference between mild and severe HD within one dimension.
The translated version of the HAQL questionnaires provides an overall reliable instrument for evaluating disease-specific QoL in a Danish HD population, but it is important to acknowledge the limitations of the questionnaire, especially in children and adolescents.
先天性巨结肠（HD）可能会因肠道问题、术后并发症和其他健康相关问题而导致生活质量（QoL）受损。Hirschsprung 和肛门直肠畸形生活质量（HAQL）问卷是一种在荷兰开发的专门用于测量 HD 和肛门直肠畸形患者 QoL 的疾病特异性工具。本研究的目的是翻译、文化适应并验证丹麦 HD 人群的 HAQL。
翻译和文化适应按照国际指南进行。邀请了 1985 年至 2012 年期间接受 HD 手术的 401 名患者参与验证。共有 156 名患者完成了翻译和文化适应后的丹麦版 HAQL，35 名儿童和青少年的父母完成了相应的家长问卷。采用 Cronbach's α 评估内部一致性，采用 Intraclass Correlation Coefficient 评估重测信度。采用轻度 HD（定义为直肠乙状结肠 HD）和严重 HD（定义为更近端疾病）的比较进行已知组比较。
成人和青少年的各维度的内部一致性总体上令人满意，但对于儿童来说，60%的维度的 Cronbach's α 值低于 0.7。对于儿童和青少年，社会功能、情绪功能和身体形象的 α 值均不理想。重测信度总体良好。已知组比较仅在一个维度上能够证明轻度和重度 HD 之间存在显著差异。
HAQL 问卷的翻译版本为评估丹麦 HD 人群的疾病特异性 QoL 提供了一个总体可靠的工具，但需要注意问卷的局限性，特别是在儿童和青少年中。
