Cause of death in neonates with inconclusive or abnormal T-cell receptor excision circle assays on newborn screening. J Clin Immunol 2011 Dec;31(6):962-7
Date
10/01/2011Pubmed ID
21956495DOI
10.1007/s10875-011-9591-xScopus ID
2-s2.0-84857044933 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
INTRODUCTION: During the first 2 years of newborn screening (NBS) for severe combined immunodeficiency (SCID), 39 infants with an abnormal or inconclusive newborn screening test for SCID died prior to assessment of immune function. We investigated if SCID or primary T-cell lymphopenia likely contributed to the death of these neonates.
METHODS: This study is a detailed retrospective chart review.
RESULTS: Medical records were available in all 39 infants. Three neonates were full-term infants whose deaths were due to congenital anomalies. Thirty-three infants were born <33 weeks estimated gestational age, and the majority of these infants died from complications of prematurity. Six infants died from sepsis: two due to maternal chorioamnionitis, two due to necrotizing enterocolitis, one due to early onset group B strep sepsis, and one from a likely nosocomial infection.
CONCLUSIONS: There was no evidence that SCID contributed to the cause of death in neonates with an abnormal of inconclusive NBS test for SCID.
Author List
Accetta DJ, Brokopp CD, Baker MW, Verbsky J, Routes JMAuthor
James Verbsky MD, PhD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Cause of DeathChorioamnionitis
DNA
Enterocolitis, Necrotizing
Female
Genes, T-Cell Receptor
Humans
Infant
Infant Mortality
Infant, Newborn
Lymphopenia
Male
Medical Records
Neonatal Screening
Pregnancy
Premature Birth
Retrospective Studies
Sepsis
Severe Combined Immunodeficiency