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18 三体综合征患儿的监测指南。

Surveillance guidelines for children with trisomy 18.

机构信息

School of Medicine, Creighton University, Omaha, Nebraska, USA.

Munroe-Meyer Institute for Genetics and Rehabilitation, University of Nebraska Medical Center, Omaha, Nebraska, USA.

出版信息

Am J Med Genet A. 2021 Apr;185(4):1294-1303. doi: 10.1002/ajmg.a.62097. Epub 2021 Feb 2.

Abstract

Trisomy 18 is the second most common aneuploidy syndromes in live born infants. It is associated with high mortality rates, estimated to be 75%-95% in the first year of life, as well as significant morbidity in survivors. The low survival is largely due to the high prevalence of severe congenital anomalies in infants with this diagnosis. However, interventions to repair or palliate those life-threatening anomalies are being performed at a higher rate for these infants, resulting in increased rates of survival beyond the first year of life. While it is well documented that trisomy 18 is associated with several cardiac malformations, these patients also have respiratory, neurological, neoplastic, genitourinary, abdominal, otolaryngologic, and orthopedic complications that can impact their quality of life. The goal of this review is to present a comprehensive description of complications in children with trisomy 18 to aid in the development of monitoring and treatment guidelines for the increasing number of providers who will be caring for these patients throughout their lives. Where the evidence is available, this review presents screening recommendations to allow for more rapid detection and documentation of these complications.

摘要

三体 18 是活产婴儿中第二常见的非整倍体综合征。它与高死亡率相关,估计在生命的第一年高达 75%-95%,幸存者也有显著的发病率。低存活率主要是由于患有这种诊断的婴儿中严重先天性异常的高患病率。然而,对于这些婴儿,修复或缓解那些危及生命的异常的干预措施的实施率更高,导致生命第一年以后的存活率增加。虽然已经有充分的文献证明三体 18 与几种心脏畸形有关,但这些患者也有呼吸、神经、肿瘤、泌尿生殖、腹部、耳鼻喉和骨科并发症,这会影响他们的生活质量。本综述的目的是全面描述三体 18 患儿的并发症,以帮助制定监测和治疗指南,为越来越多的将在其一生中照顾这些患者的提供者提供指导。在有证据的情况下,本综述提出了筛查建议,以更快速地发现和记录这些并发症。

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