Improved survival in cystic fibrosis patients diagnosed by newborn screening compared to a historical cohort from the same centre

Background Newborn screening (NBS) for cystic fibrosis (CF) is associated with improved early nutritional outcomes and improved spirometry in children. The aim of this study was to determine whether early diagnosis and treatment of CF with NBS in New South Wales in 1981 led to better clinical outcom...

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Veröffentlicht in:Archives of disease in childhood 2011-12, Vol.96 (12), p.1118-1123
Hauptverfasser: Dijk, F Nicole, McKay, Karen, Barzi, Federica, Gaskin, Kevin J, Fitzgerald, Dominic A
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Sprache:eng
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Zusammenfassung:Background Newborn screening (NBS) for cystic fibrosis (CF) is associated with improved early nutritional outcomes and improved spirometry in children. The aim of this study was to determine whether early diagnosis and treatment of CF with NBS in New South Wales in 1981 led to better clinical outcomes and survival into early adulthood. Methods Retrospective observational study comprising two original cohorts born in the 3 years before (‘non-screened cohort’, n=57) and after (‘screened’; n=60) the introduction of NBS. Patient records were assessed at transfer from paediatric to adult care by age 19 years and survival was documented to age 25 years. Results Non-screened patients (n=38) when compared with screened patients (n=41) had a higher rate and lower age of Pseudomonas aeruginosa acquisition at age 18 years (p≤0.01). Height, weight and body mass index (BMI) z scores (all p
ISSN:0003-9888
1468-2044
DOI:10.1136/archdischild-2011-300449