18三体与选择的可能性:围产期安宁疗护支持的重要性。

IF 3 3区 医学 Q1 PEDIATRICS European Journal of Pediatrics Pub Date : 2025-01-16 DOI:10.1007/s00431-025-05970-8
D Visconti, V Esposito, F Brugnoli, V Gallitelli, B Corsano, P Papacci, M Pellegrino, M De Santis, A Lanzone, G Noia
{"title":"18三体与选择的可能性:围产期安宁疗护支持的重要性。","authors":"D Visconti, V Esposito, F Brugnoli, V Gallitelli, B Corsano, P Papacci, M Pellegrino, M De Santis, A Lanzone, G Noia","doi":"10.1007/s00431-025-05970-8","DOIUrl":null,"url":null,"abstract":"<p><p>Trisomy 18 is a severe aneuploidy associated with multiple malformations and a poor prognosis. The diagnosis is typically made prenatally, leading to a high rate of pregnancy terminations. The aim of this study is to demonstrate that even though the prognosis is heterogeneous, prolonged survival is possible and these children are an enrichment for their families after all. A retrospective, descriptive and monocentric study was conducted on fetuses diagnosed with trisomy 18, evaluated between March 2017 and June 2023 at our Institution. The objective was to investigate the natural history of trisomy 18 and the psychological impact on parents who choose to carry the pregnancy on, through a retrospective data collection and the use of a questionnaire. Sixteen couples with a diagnosis of trisomy 18 were cared for within the Perinatal Hospice Pathway during the study period. Cardiac defects were identified in 93.7% of cases, structural abnormalities in 71.4%, respiratory defects in 14.3% of the fetuses, while genitourinary defects affected nearly half of the study population. The survival rate was typically less than one day, however two babies survived for more than four years. All couples reported being satisfied with their decision to continue the pregnancy and would do so again if given the opportunity.Conclusions: despite the severity of the diagnosis, couples may choose to continue the pregnancy and give birth. Our study shows that trisomy 18 is not merely a lethal condition and the Perinatal Hospice plays a crucial role in supporting these families.</p>","PeriodicalId":11997,"journal":{"name":"European Journal of Pediatrics","volume":"184 2","pages":"141"},"PeriodicalIF":3.0000,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11739207/pdf/","citationCount":"0","resultStr":"{\"title\":\"Trisomy 18 and the possibility of choice: The importance of Perinatal Hospice's support.\",\"authors\":\"D Visconti, V Esposito, F Brugnoli, V Gallitelli, B Corsano, P Papacci, M Pellegrino, M De Santis, A Lanzone, G Noia\",\"doi\":\"10.1007/s00431-025-05970-8\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Trisomy 18 is a severe aneuploidy associated with multiple malformations and a poor prognosis. The diagnosis is typically made prenatally, leading to a high rate of pregnancy terminations. The aim of this study is to demonstrate that even though the prognosis is heterogeneous, prolonged survival is possible and these children are an enrichment for their families after all. A retrospective, descriptive and monocentric study was conducted on fetuses diagnosed with trisomy 18, evaluated between March 2017 and June 2023 at our Institution. The objective was to investigate the natural history of trisomy 18 and the psychological impact on parents who choose to carry the pregnancy on, through a retrospective data collection and the use of a questionnaire. Sixteen couples with a diagnosis of trisomy 18 were cared for within the Perinatal Hospice Pathway during the study period. Cardiac defects were identified in 93.7% of cases, structural abnormalities in 71.4%, respiratory defects in 14.3% of the fetuses, while genitourinary defects affected nearly half of the study population. The survival rate was typically less than one day, however two babies survived for more than four years. All couples reported being satisfied with their decision to continue the pregnancy and would do so again if given the opportunity.Conclusions: despite the severity of the diagnosis, couples may choose to continue the pregnancy and give birth. Our study shows that trisomy 18 is not merely a lethal condition and the Perinatal Hospice plays a crucial role in supporting these families.</p>\",\"PeriodicalId\":11997,\"journal\":{\"name\":\"European Journal of Pediatrics\",\"volume\":\"184 2\",\"pages\":\"141\"},\"PeriodicalIF\":3.0000,\"publicationDate\":\"2025-01-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11739207/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European Journal of Pediatrics\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00431-025-05970-8\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Pediatrics","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00431-025-05970-8","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0

摘要

18三体是一种严重的非整倍体,伴有多种畸形和预后不良。诊断通常是在产前做出的,导致高终止妊娠率。这项研究的目的是证明,即使预后是不同的,延长生存是可能的,这些孩子是丰富了他们的家庭毕竟。对2017年3月至2023年6月在我院诊断为18三体的胎儿进行了回顾性、描述性和单中心研究。目的是通过回顾性数据收集和问卷调查,调查18三体的自然史以及对选择继续怀孕的父母的心理影响。在研究期间,16对诊断为18三体的夫妇在围产期临终关怀途径内得到照顾。心脏缺陷占93.7%,结构异常占71.4%,呼吸缺陷占14.3%,而泌尿生殖系统缺陷影响了近一半的研究人群。存活率通常不到一天,但有两个婴儿存活了四年多。所有夫妇都对自己继续怀孕的决定感到满意,如果有机会,他们还会继续怀孕。结论:尽管诊断严重,夫妻仍可选择继续妊娠并分娩。我们的研究表明,18三体不仅仅是一种致命的疾病,围产期临终关怀在支持这些家庭方面起着至关重要的作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Trisomy 18 and the possibility of choice: The importance of Perinatal Hospice's support.

Trisomy 18 is a severe aneuploidy associated with multiple malformations and a poor prognosis. The diagnosis is typically made prenatally, leading to a high rate of pregnancy terminations. The aim of this study is to demonstrate that even though the prognosis is heterogeneous, prolonged survival is possible and these children are an enrichment for their families after all. A retrospective, descriptive and monocentric study was conducted on fetuses diagnosed with trisomy 18, evaluated between March 2017 and June 2023 at our Institution. The objective was to investigate the natural history of trisomy 18 and the psychological impact on parents who choose to carry the pregnancy on, through a retrospective data collection and the use of a questionnaire. Sixteen couples with a diagnosis of trisomy 18 were cared for within the Perinatal Hospice Pathway during the study period. Cardiac defects were identified in 93.7% of cases, structural abnormalities in 71.4%, respiratory defects in 14.3% of the fetuses, while genitourinary defects affected nearly half of the study population. The survival rate was typically less than one day, however two babies survived for more than four years. All couples reported being satisfied with their decision to continue the pregnancy and would do so again if given the opportunity.Conclusions: despite the severity of the diagnosis, couples may choose to continue the pregnancy and give birth. Our study shows that trisomy 18 is not merely a lethal condition and the Perinatal Hospice plays a crucial role in supporting these families.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
CiteScore
5.90
自引率
2.80%
发文量
367
审稿时长
3-6 weeks
期刊介绍: The European Journal of Pediatrics (EJPE) is a leading peer-reviewed medical journal which covers the entire field of pediatrics. The editors encourage authors to submit original articles, reviews, short communications, and correspondence on all relevant themes and topics. EJPE is particularly committed to the publication of articles on important new clinical research that will have an immediate impact on clinical pediatric practice. The editorial office very much welcomes ideas for publications, whether individual articles or article series, that fit this goal and is always willing to address inquiries from authors regarding potential submissions. Invited review articles on clinical pediatrics that provide comprehensive coverage of a subject of importance are also regularly commissioned. The short publication time reflects both the commitment of the editors and publishers and their passion for new developments in the field of pediatrics. EJPE is active on social media (@EurJPediatrics) and we invite you to participate. EJPE is the official journal of the European Academy of Paediatrics (EAP) and publishes guidelines and statements in cooperation with the EAP.
期刊最新文献
Development and validation of the PACPN: Parents' Attitudes Towards Crying Pediatricians and pediatric Nurses questionnaire. Quality of life trajectories in children aged 2-4 years with acute lymphoblastic leukemia. Bioethical issues in neonatal care: the case of CARPEDIEM. Enhanced mortality prediction in pediatric sepsis using NGAL: A comparison with PRISM III scores in critical care settings. Bosentan as adjunctive therapy in neonates with congenital diaphragmatic hernia-associated pulmonary hypertension: a case series.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1