{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T23:17:17Z","timestamp":1774567037735,"version":"3.50.1"},"reference-count":27,"publisher":"European Respiratory Society (ERS)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2001,4,17]]},"published-print":{"date-parts":[[2001,8]]},"abstract":"<jats:p>After an experimental neonatal screening program for cystic fibrosis (CF) from 1973\u20131979, a follow-up study took place from 1980\u20131997. 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.<\/jats:p><jats:p>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.<\/jats:p><jats:p>S patients had a significantly smaller decline in forced expiratory volume in one second (FEV<jats:sub>1<\/jats:sub>) (difference +2.74% predicted) and significantly lower immunoglobulin-G (IgG) levels (difference \u2212473.69\u2005mg\u00b7dL<jats:sup>\u22121<\/jats:sup>) than non-S patients until 12\u2005yrs of age. At 12\u2005yrs of age, vital capacity was significantly higher in S patients than in non-S patients (difference +362.79\u2005mL). 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 FEV<jats:sub>1<\/jats:sub>(difference +2.80% pred) and lower IgG levels (difference \u2212453.04\u2005mg\u00b7dL<jats:sup>\u22121<\/jats:sup>) than non-S patients until 12\u2005yrs of age. C patients had a significantly improved survival (relative risk (RR) 0.18, 95% confidence interval 0.05\u20130.57) than non-C patients.<\/jats:p><jats:p>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.<\/jats:p>","DOI":"10.1183\/09031936.01.00080101","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T12:36:48Z","timestamp":1027773408000},"page":"306-315","source":"Crossref","is-referenced-by-count":86,"title":["Influence of neonatal screening and centralized treatment on long-term clinical outcome and survival of CF patients"],"prefix":"10.1183","volume":"18","author":[{"given":"M.E.","family":"M\u00e9relle","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.P.","family":"Schouten","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Gerritsen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.E.","family":"Dankert-Roelse","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"81","published-online":{"date-parts":[[2001,8,1]]},"reference":[{"key":"2024101808523272000_18.2.306.1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.310.6994.1571"},{"key":"2024101808523272000_18.2.306.2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.156.4.96-11058"},{"key":"2024101808523272000_18.2.306.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199710023371403"},{"key":"2024101808523272000_18.2.306.4","doi-asserted-by":"publisher","DOI":"10.1136\/adc.66.1_Spec_No.29"},{"key":"2024101808523272000_18.2.306.5","unstructured":"M\u00e9relle ME, Nagelkerke AF, Lees CM, Dezateux C. 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