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Titolo:
Clinical outcomes of newborn screening for cystic fibrosis
Autore:
Waters, DL; Wilcken, B; Irwig, L; Van Asperen, P; Mellis, C; Simpson, JM; Brown, J; Gaskin, KJ;
Indirizzi:
Royal Alexandra Hosp Children, James Fairfax Inst Paediat Nutr, Parramatta, Royal Alexandra Hosp Children Parramatta NSW Australia 2124 , Parramatta, Univ Sydney, Dept Biochem Genet, Sydney, NSW 2006, Australia Univ Sydney Sydney NSW Australia 2006 Genet, Sydney, NSW 2006, Australia Univ Sydney, Dept Resp Med, Sydney, NSW 2006, Australia Univ Sydney Sydney NSW Australia 2006 sp Med, Sydney, NSW 2006, Australia Univ Sydney, Dept Publ Hlth, Sydney, NSW 2006, Australia Univ Sydney Sydney NSW Australia 2006 l Hlth, Sydney, NSW 2006, Australia
Titolo Testata:
ARCHIVES OF DISEASE IN CHILDHOOD
fascicolo: 1, volume: 80, anno: 1999,
pagine: F1 - F7
SICI:
0003-9888(199901)80:1<F1:COONSF>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
PANCREATIC FUNCTION; INFANTS; DIAGNOSIS; PROGNOSIS; PROGRAM; GROWTH;
Keywords:
cystic fibrosis; neonatal screening; early diagnosis; clinical outcomes;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Gaskin, KJ Royalarramatta, Hosp Children, James Fairfax Inst Paediat Nutr,POB 3515, P Royal Alexandra Hosp Children POB 3515 Parramatta NSW Australia 2124
Citazione:
D.L. Waters et al., "Clinical outcomes of newborn screening for cystic fibrosis", ARCH DIS CH, 80(1), 1999, pp. F1-F7

Abstract

Aim-To determine how early diagnosis of cystic fibrosis, using neonatal screening, affects long term clinical outcome. Methods-Fifty seven children with cystic fibrosis born before neonatal screening was introduced (1978 to mid 1981) and a further 60 children born during the first three years of the programme (mid 1981 to 1984), were followed up to the age of 10. The cohorts were compared on measures of clinical outcome, including height, weight, lung function tests, chest x-ray picture and Shwachman score. Results-Age and sex adjusted standard deviation scores (SDS) for height and weight were consistently higher in children screened for cystic fibrosis than in those born before screening. At 10 years of age, average differences in SDS between groups were 0.4 (95% CI -0.1, 0.8) for weight and 0.3 (95%CI -0.1, 0.7) for height. This translates to an average difference of about 2.7 cm in height and 1.7 kg in weight. Mean FEV1 and FVC (as percentage predicted) were significantly higher in the screened cohort at 5 and 10 years of age, with an average difference of 9.4% FEV1 (95% CI 0.8, 17.9) and 8.4% FVC (95% CI 1.8, 15.0) at 10 years. Chest x-ray scores were not different between the groups at any age, but by 10 years screened patients scored an average 5.3 (95% CI 1.2, 9.4) points higher on the Shwachman score. Conclusion-Although not a randomised trial, this long term observational study indicates that early treatment made possible by neonatal screening maybe important in determining subsequent clinical outcomes for children withcystic fibrosis. For countries contemplating the introduction of neonatal screening for cystic fibrosis, its introduction to some areas in a cluster randomised design will permit validation of studies performed to date.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/10/20 alle ore 07:45:53