Patau and Edwards Syndromes in a University Hospital: beyond palliative care

IF 1.4 Q3 PEDIATRICS

Revista Paulista De Pediatria Pub Date : 2023-12-11 DOI:10.1590/1984-0462/2024/42/2023053

Ligia Marçola, Ivete Zoboli, Rita Tiziana Verardo Polastrini, S. Barbosa, Mário Cícero Falcão, Paula de Vicenzi Gaiolla

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引用次数: 0

Abstract

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30–36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2–304) and 25 days (13–58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

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大学医院的帕陶综合征和爱德华兹综合征：超越姑息关怀

【摘要】目的:对一家综合医院重症监护病房(ICU)的新生儿帕陶综合征(T13)和爱德华兹综合征(T18)先天性心脏病患者进行手术和非手术治疗、姑息治疗和预后分析。方法:对2014年1月至2018年12月在某第四医院ICU住院的T13或T18核型阳性患者的记录进行描述性病例系列分析。采用描述性统计分析。结果:共纳入符合条件的患者33例:T18 27例(82%)，T13 6例(18%);女性占64%，男性占36%。8名是胎龄在30-36周之间的早产儿(24%)，33名婴儿中只有4名出生体重>2500克(12%)。4名患者接受了心脏手术，其中一人死亡。T13组院内死亡率为83%，T18组为59%。大多数患者有其他畸形，并接受了其他手术治疗。54%的患者接受了姑息治疗。T18和T13的中位住院时间分别为29天(2-304天)和25天(13-58天)。结论:T13和T18患者发病率和死亡率高，住院和ICU时间长。需要进行多中心研究，以便分析制定方案的重要方面，寻求治疗的相称性，从而为患者及其家属带来更好的生活质量。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Revista Paulista De Pediatria Medicine-Pediatrics, Perinatology and Child Health

CiteScore

2.30

自引率

0.00%

发文量

100

审稿时长

11 weeks

期刊介绍： The Revista Paulista de Pediatria publishes original contributions, case reports and review of clinical research with methodological approach in the areas of health and disease of neonates, infants, children and adolescents. The objective is to disseminate research with methodological quality on issues that comprise the health of children and adolescents. All articles are freely available online, via SciELO. Its abbreviated title is Rev. Paul. Pediatr., which should be used in bibliographies, footnotes and bibliographical references and strips.