Long-term surgical and patient-reported outcomes of Hirschsprung Disease
Long-term surgical and patient-reported outcomes of Hirschsprung Disease
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DOI：
10.1016/j.jpedsurg.2021.01.043
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发表时间：
2021-08-19
影响因子：
2.4
通讯作者：
Curry, Joe
中科院分区：
文献类型：
--
作者：
Davidson, Joseph R.;Kyrklund, Kristiina;Curry, Joe
背景资料：需要关于先天性巨结肠（HSCR）长期功能结局与健康相关生活质量（HRQoL）之间复杂关系的信息。我们描述了多个领域的长期结果，完成了一个核心的结果集，通过adultage.Methods：HSCR患者在一个单一的中心超过35年的时间（1978年至2013年）进行了研究。患者完成了关于肠道和泌尿系统功能以及HRQOL的详细问卷。排除学习障碍（LD）患者。结果与规范性数据进行比较。结果：186名患者（中位年龄28 [18-32]岁; 135名男性）完成了调查。肠功能降低（BFS 17 [14-19] vs. 19 [19-20]，p < 0.0001;eta（2）= 0.22）。粪便污染和粪便意识的患病率和严重程度随着年龄的增长而提高（两者均为p < 0.05）。尿失禁比对照组更常见，大多数发生在13- 26岁女性中（65% vs. 31%，p = 0.003）。在成人中，这与便秘症状独立相关（OR 3.18 [1.4-7.5]，p = 0.008）。HRQoL结局与功能结局密切相关：42%的儿童表现出PedsQL总评分的临床显著降低，肠道不良结局与QOL受损密切相关（B = 22.7 [12.7- 32.7]，p < 0.001）。在成人中，GIQLI评分更常在扩展节段疾病患者中受到影响。SF-36评分降低相对于人口水平的数据，在大多数领域，与大的影响大小注意到女性在一般健康（g = 1.19）和社会福利（g = 0.8）。结论：功能障碍是常见的拉通过后，但肠功能随着年龄的增长而改善。多个领域的不良功能结局的聚集表明需要对这些患者进行早期识别和长期支持。(C)2021爱思唯尔公司All rights reserved.
Background: Information is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe longterm outcomes across multiple domains, completing a core outcome set through to adulthood.Methods: HSCR patients operated at a single center over a 35-year period (1978-2013) were studied. Patients completed detailed questionnaires on bowel and urologic function, and HRQOL. Patients with learning disability (LD) were excluded. Outcomes were compared to normative data. Data are reported as median [IQR] or mean (SD).Results: 186 patients (median age 28 [18-32] years; 135 males) completed surveys. Bowel function was reduced (BFS 17 [14-19] vs. 19 [19-20], p < 0.0001;eta(2) = 0.22). Prevalence and severity of fecal soiling and fecal awareness improved with age ( p < 0.05 for both). Urinary incontinence was more frequent than controls, most of all in 13-26y females (65% vs. 31%, p = 0.003). In adults, this correlated independently with constipation symptoms (OR 3.18 [1.4-7.5], p = 0.008). HRQoL outcomes strongly correlated with functional outcome: 42% of children demonstrated clinically significant reductions in overall PedsQL score, and poor bowel outcome was strongly associated with impaired QOL (B = 22.7 [12.7- 32.7], p < 0.001). In adults, GIQLI scores were more often impacted in patients with extended segment disease. SF-36 scores were reduced relative to population level data in most domains, with large effect sizes noted for females in General Health (g = 1.19) and Social Wellbeing (g = 0.8).Conclusion: Functional impairment is common after pull-through, but bowel function improves with age. Clustering of poor functional outcomes across multiple domains identifies a need for early recognition and long-term support for these patients. (C) 2021 Elsevier Inc. All rights reserved.