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Cystic Fibrosis-related Deaths in Infancy and the Effect of Newborn Screening by Iolo J.M. Doull; Henry C. Ryley; Peter Weller; Mary C. Goodchild is a Medicine article available to read on EtoBox.

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## Abstract Although newborn screening for cystic fibrosis (CF) is widely advocated, hard evidence in its favor is difficult to obtain, partly because of a dramatically improved life expectancy. Between 1985–l989 infants, born in Wales and the West Midlands were randomized to newborn CF screening by heel‐prick immunoreactive trypsin (IRT) measurement or diagnosis by clinical presentation. Eligible children with CF who died in the first 5 years of life were identified from the local pediatricians and from the National UK CF Survey. In all, 230,076 infants were randomized to be screened, while 234,510 were unscreened. One hundred seventy‐six CF children were identified, of whom 7 died in the first 5 years of life, 3 having presented with meconium ileus. Median age of diagnosis in the screened group was 8 weeks. On an __intention to treat__ analysis, all 4 nonmeconium ileus‐related deaths occurred in the unscreened group (Fisher's exact test, __P__ < 0.05). However, the clinical presentation of 2 of these infants led to them being diagnosed prior to 8 weeks, i.e., earlier than would have been likely by screening. In conclusion, newborn screening has the potential to decrease infant CF

Who reads Cystic Fibrosis-related Deaths in Infancy and the Effect of Newborn Screening?

Author

Iolo J.M. Doull; Henry C. Ryley; Peter Weller; Mary C. Goodchild

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley (ISSN 8755-6863)

Published

2001

Language

EN

Field

Medicine (Health Sciences)