Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio; Center for Surgical Outcomes Research, Department of Surgery, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio.
Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio.
J Surg Res. 2020 Jan;245:564-568. doi: 10.1016/j.jss.2019.06.086. Epub 2019 Aug 30.
Children with Hirschsprung disease (HD) and Down syndrome (DS) are reported to experience a higher incidence of Hirschsprung-associated enterocolitis (HAEC) than those without DS, although whether their individual episodes are more severe is unknown.
A single-institution retrospective cohort study was performed on all patients with HD admitted with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of DS, and demographics, clinical characteristics at presentation, and clinical outcomes were compared.
Of 86 admissions for HAEC over the study period, 12 (14%) were for patients with DS. Patients admitted with and without DS were similar in terms of age (3.0 versus 2.6 y, P = 0.72), male gender (58% versus 77%, P = 0.17), and proportion with disease proximal to the left colon (9% versus 12%, P = 0.70). Patients admitted with DS were more likely to be tachycardic (75% versus 19%, P ≤ 0.001) and hypotensive (33% versus 7%, P = 0.01) at presentation and presented for treatment after a longer duration of symptoms than those without DS (84 versus 24 h, P = 0.001). Patients admitted with DS were more likely to require intensive care unit admission (58% versus 12%, P = 0.006) and received a longer course of antibiotics (13 d versus 7 d, P = 0.02). The length of stay was similar (6 versus 4 d, P = 0.34).
The results of our study suggest that patients with DS experience more severe episodes of HAEC. Ongoing study is needed to identify preventive strategies to decrease the morbidity of HAEC in children with HD and DS.
患有先天性巨结肠（HD）和唐氏综合征（DS）的儿童比没有 DS 的儿童更易发生先天性巨结肠相关性结肠炎（HAEC），尽管他们各自的发作是否更严重尚不清楚。
对 2009 年至 2017 年间因 HAEC 入院的所有 HD 患者进行了单机构回顾性队列研究。根据是否伴有 DS 进行分组，比较两组患者的人口统计学资料、发病时的临床特征和临床结局。
在研究期间，共发生 86 例 HAEC 住院，其中 12 例（14%）为 DS 患者。伴有和不伴有 DS 的患者在年龄（3.0 岁比 2.6 岁，P=0.72）、男性比例（58%比 77%，P=0.17）和左半结肠近端病变比例（9%比 12%，P=0.70）方面无显著差异。与不伴有 DS 的患者相比，伴有 DS 的患者入院时更易出现心动过速（75%比 19%，P≤0.001）和低血压（33%比 7%，P=0.01），且症状持续时间更长后才开始治疗（84 小时比 24 小时，P=0.001）。伴有 DS 的患者更有可能需要入住重症监护病房（58%比 12%，P=0.006），且接受的抗生素疗程更长（13 天比 7 天，P=0.02）。两组患者的住院时间相似（6 天比 4 天，P=0.34）。
本研究结果表明，DS 患者发生 HAEC 的病情更严重。需要进一步研究以确定预防策略，从而降低 HD 和 DS 患儿 HAEC 的发病率。
