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J Pediatr. 2024 Feb;265:113799. doi: 10.1016/j.jpeds.2023.113799. Epub 2023 Oct 23.
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本文引用的文献

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Cleft palate morphology, genetic etiology, and risk of mortality in infants with Robin sequence.Robin 序列婴儿的腭裂形态、遗传病因和死亡率风险。
Am J Med Genet A. 2021 Dec;185(12):3694-3700. doi: 10.1002/ajmg.a.62430. Epub 2021 Jul 22.
2
Impact of Hospital Characteristics on Mandibular Distraction Osteogenesis Outcomes Among Patients With Pierre Robin Sequence Utilizing a National Inpatient Database.利用国家住院患者数据库研究医院特征对 Pierre Robin 序列患者下颌骨牵引成骨结局的影响
Cleft Palate Craniofac J. 2022 May;59(5):622-628. doi: 10.1177/10556656211015007. Epub 2021 May 12.
3
Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review.罗宾序列综合征婴儿上气道梗阻的客观测量指标:我们在测量什么?系统评价。
J Clin Sleep Med. 2021 Aug 1;17(8):1717-1729. doi: 10.5664/jcsm.9394.
4
Associated syndromes in patients with Pierre Robin Sequence.Pierre Robin序列患者的相关综合征。
Int J Pediatr Otorhinolaryngol. 2020 Apr;131:109842. doi: 10.1016/j.ijporl.2019.109842. Epub 2019 Dec 30.
5
International Pediatric ORL Group (IPOG) Robin Sequence consensus recommendations.国际儿科耳鼻喉科小组(IPOG)罗宾序列共识建议。
Int J Pediatr Otorhinolaryngol. 2020 Mar;130:109855. doi: 10.1016/j.ijporl.2019.109855. Epub 2019 Dec 28.
6
A retrospective study of patients with Robin sequence: Patient characteristics and their impact on clinical outcomes.一项关于罗宾序列征患者的回顾性研究:患者特征及其对临床结局的影响。
Int J Pediatr Otorhinolaryngol. 2020 Feb;129:109769. doi: 10.1016/j.ijporl.2019.109769. Epub 2019 Nov 7.
7
Management of Airway Obstruction in Infants With Pierre Robin Sequence.Pierre Robin序列婴儿气道阻塞的管理
Plast Reconstr Surg Glob Open. 2018 May 10;6(5):e1688. doi: 10.1097/GOX.0000000000001688. eCollection 2018 May.
8
Surgical Management and Outcomes of Pierre Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion.Pierre Robin 序列的手术治疗及结果:下颌骨牵引成骨术与舌唇粘连术的比较。
Plast Reconstr Surg. 2018 Aug;142(2):480-509. doi: 10.1097/PRS.0000000000004581.
9
The ontogeny of Robin sequence.罗宾序列的个体发生
Am J Med Genet A. 2018 Jun;176(6):1349-1368. doi: 10.1002/ajmg.a.38718. Epub 2018 Apr 25.
10
Mortality in Robin sequence: identification of risk factors.Robin 序列的死亡率:危险因素的识别。
Eur J Pediatr. 2018 May;177(5):781-789. doi: 10.1007/s00431-018-3111-4. Epub 2018 Feb 28.

先天性小下颌症住院新生儿的疾病谱:北美三级护理重症监护单位 3236 例婴儿队列研究。

Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

机构信息

Division of Neonatology, Children's Hospital of Philadelphia, Philadelphia, PA.

School of Public Health, University of Illinois Chicago, Chicago, IL.

出版信息

J Pediatr. 2024 Feb;265:113799. doi: 10.1016/j.jpeds.2023.113799. Epub 2023 Oct 23.

DOI:10.1016/j.jpeds.2023.113799
PMID:37879601
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10872910/
Abstract

OBJECTIVE

To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers.

STUDY DESIGN

The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge.

RESULTS

Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both).

CONCLUSIONS

Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

摘要

目的

描述多中心三级护理中心住院的先天性小下颌畸形婴儿的疾病谱和护理负担。

研究设计

2010 年至 2020 年,通过儿童医院新生儿数据库查询诊断为小下颌畸形的婴儿。总结人口统计学特征、遗传综合征的存在和唇腭裂情况。结果包括死亡、住院时间、新生儿手术以及出院时的喂养和呼吸支持。

结果

分析包括 3236 例先天性小下颌畸形婴儿。1266 例(39.1%)存在腭裂。256 例(7.9%)在新生儿住院期间诊断出与小下颌畸形相关的遗传综合征。中位(IQR)住院时间为 35(16,63)天。住院期间死亡(n=228,6.8%)与无腭裂(4.4%,P<.001)和母亲黑种人种族(11.6%,P<.001)有关。在新生儿住院期间,1289 例(39.7%)接受了矫正气道阻塞的手术,1059 例(32.7%)接受了胃造口管放置。出院时,1035 例(40.3%)仅经口喂养。各中心之间在住院时间和出院时是否存在喂养管方面存在显著差异(两者 P<.001)。

结论

因先天性小下颌畸形住院的婴儿疾病负担沉重,通常接受手术干预,且大多数在出院时需要进行管饲喂养。我们发现了基于种族和中心的差异。制定基于证据的指南可以改善新生儿护理。