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1 Dept of Paediatrics, Medical Centre Vrije Universiteit, Amsterdam, 2 Dept of Epidemiology and Statistics, University of Groningen, 3 Dept of Paediatrics, University Hospital Groningen, the Netherlands
CORRESPONDENCE: J.E. Dankert-Roelse, Dept of Paediatrics, Medical Centre Vrije Universiteit, PO Box 7057, 1007 MB, Amsterdam, the Netherlands. Fax: 31 204440849
Keywords: centre treatment, cystic fibrosis, follow-up study, lung function, neonatal screening
Received: September 12, 1999
Accepted April 17, 2001
This study was supported by ZorgOnderzoekNederland (ZON) grants 28.566, 28.566-1 and 28.566-2.
After an experimental neonatal screening program for cystic fibrosis (CF) from 1973–1979, a follow-up study took place from 1980–1997. Patients were treated at specialized centres (C) or at local hospitals (non-C). Aims of the study were: 1) to determine whether the previously reported benefits from screening persisted with time and after adjustment for confounding variables; and 2) to investigate whether centre treatment was associated with improved prognosis of CF patients.
Prognosis of patients detected by screening (S; n=24) was compared with patients detected clinically, born during (non-S; n=29) and after the screening programme (post-S; n=39). In addition, prognosis was compared between 45 C and 47 non-C patients. Multivariable regression analysis was used to compare survival and mixed-effects model regression analysis was used to compare clinical outcome between patients. The analyses included the variables screening, centre treatment, sex, meconium ileus and genotype.
S patients had a significantly smaller decline in forced expiratory volume in one second (FEV1) (difference +2.74% predicted) and significantly lower immunoglobulin-G (IgG) levels (difference –473.69 mg·dL–1) than non-S patients until 12 yrs of age. At 12 yrs of age, vital capacity was significantly higher in S patients than in non-S patients (difference +362.79 mL). Survival seemed to be best for S patients compared to both non-S and post-S patients. Post-S patients were significantly heavier (difference in sd weight +0.77), had a significantly smaller decline in FEV1 (difference +2.80% pred) and lower IgG levels (difference –453.04 mg·dL–1) than non-S patients until 12 yrs of age. C patients had a significantly improved survival (relative risk (RR) 0.18, 95% confidence interval 0.05–0.57) than non-C patients.
Early diagnosis through neonatal screening leads to better preservation of lung function in the long term in cystic fibrosis patients. Management of cystic fibrosis patients in specialized centres improves survival.
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