Natural history of trisomy 13

Title

Natural history of trisomy 13

Creator

Wyllie JP; Wright MJ; Burn J; Hunter S

Publisher

Archives Of Disease In Childhood

Date

1994

Subject

Female; Humans; infant; Male; Trisomy; Pregnancy; Adult; Prenatal Diagnosis; Longitudinal Studies; Incidence; Syndrome; Newborn; Chromosomes; Heart Defects; Human; Fetal Diseases/diagnosis; Congenital/diagnosis/genetics/mortality; England/epidemiology; Neural Tube Defects/diagnosis/genetics/mortality; Pair 13

Description

The poor prognosis of patients with trisomy 13 has long been accepted and has been ascribed to brain and heart malformations. It has been suggested, however, that the long term survival is better than was previously thought and that cardiac surgery may be justified. This population based study reviews the incidence, antenatal diagnosis, spectrum of survival from congenital heart disease, and mode of death for patients with trisomy 13 in the Northern Health Region from 1985 to 1992. There was an observed prevalence at birth of 0.049/1000 live births and an expected prevalence, allowing for antenatal diagnosis, of 0.077. None of the cardiac lesions found would cause early death. The median survival in this series was four days; the longest survival was 3.5 months. The principal mode of death was apnoea in 14 of 16 children, irrespective of the presence of a cranial abnormality. In the light of these findings, cardiac surgery cannot be justified in patients with trisomy 13.
1994

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Citation

Wyllie JP; Wright MJ; Burn J; Hunter S, “Natural history of trisomy 13,” Pediatric Palliative Care Library, accessed March 28, 2024, https://pedpalascnetlibrary.omeka.net/items/show/11907.