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唇裂和/或腭裂患儿先天性畸形的范围和频率。

Range and Frequency of Congenital Malformations Among Children With Cleft Lip and/or Palate.

机构信息

Clinical Effectiveness Unit, Royal College of Surgeons of England, London, UK.

West of Scotland Centre for Genomic Medicine, Queen Elizabeth University Hospital, Glasgow, UK.

出版信息

Cleft Palate Craniofac J. 2023 Aug;60(8):917-927. doi: 10.1177/10556656221089160. Epub 2022 Apr 5.

Abstract

To assess the range and frequency of additional congenital malformations identified among children born alive with CL/P. Analysis of patient-level data from a national registry of cleft births linked to national administrative data of hospital admissions. National Health Service, England. Children born between 2000 and 2012 receiving cleft care in English NHS hospitals. The proportion of children with codes for additional congenital malformations, according to cleft type. The study included 9403 children. Of these 2114 (22.5%) had CL±A, 4509 (48.0%) had CP, 1896 (20.2%) had UCLP, and 884 (9.4%) had BCLP. A total of 3653 (38.8%) children had additional congenital malformations documented in their hospital admission records. The prevalence of additional congenital malformations was greatest among children with CP (53.0%), followed by those with BCLP (33.5%), UCLP (26.3%), and then CL±A (22.2%) ( < .001). Among those with UCLP, children with right-sided clefts were more likely to have additional malformations than those with left-sided clefts (31.6% vs 23.0%,  < .001). Malformations of the skeletal system and circulatory system were most common, affecting 10.5% and 10.2% of the included children, respectively. A total of 16.8% of children had additional congenital malformations affecting 2 or more structural systems. Congenital malformations are common among children born alive with a cleft, affecting over half of some cleft subgroups. Given the frequency of certain structural malformations, clinicians should consider standardized screening for these children. Establishing good links with pediatric and genetic services is recommended.

摘要

评估伴有唇腭裂(CL/P)存活患儿的其他先天性畸形的发生范围和频率。对英国国民保健署(NHS)全国性唇腭裂出生登记处与全国性医院入院管理数据进行链接的患者水平数据进行分析。英国国民保健署,英格兰。2000 年至 2012 年期间在英格兰 NHS 医院接受唇腭裂治疗的儿童。根据唇腭裂类型,按 CL/P 类型分组,列出伴有其他先天性畸形代码的患儿比例。本研究共纳入 9403 例患儿。其中 2114 例(22.5%)为 CL±A,4509 例(48.0%)为 CP,1896 例(20.2%)为 UCLP,884 例(9.4%)为 BCLP。共有 3653 例(38.8%)患儿的入院记录中记录了其他先天性畸形。CP 患儿的伴发先天性畸形患病率最高(53.0%),其次是 BCLP(33.5%)、UCLP(26.3%),然后是 CL±A(22.2%)( < .001)。在 UCLP 患儿中,右侧裂患儿比左侧裂患儿更易发生伴发畸形(31.6%比 23.0%, < .001)。骨骼系统和循环系统的畸形最为常见,分别影响了 10.5%和 10.2%的患儿。共有 16.8%的患儿存在影响 2 个或更多结构系统的伴发先天性畸形。唇腭裂患儿伴发先天性畸形较为常见,一些唇腭裂亚组患儿中超过一半存在伴发畸形。鉴于某些结构性畸形的发生频率,临床医生应为这些患儿考虑进行标准化筛查。建议与儿科和遗传服务建立良好联系。

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