Early diagnosis from newborn screening maximises survival in severe cystic fibrosis.

التفاصيل البيبلوغرافية
العنوان:	Early diagnosis from newborn screening maximises survival in severe cystic fibrosis.
المؤلفون:	Tridello G; Centre for Cystic Fibrosis, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Castellani C; Centre for Cystic Fibrosis, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.; Cystic Fibrosis Centre, Gaslini Institute, Genoa, Italy., Meneghelli I; Centre for Cystic Fibrosis, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Tamanini A; Clinical Chemistry and Haematology Laboratory, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Assael BM; Centre for Cystic Fibrosis, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.; Internal Medicine Dept, Respiratory Unit and Cystic Fibrosis Adult Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
المصدر:	ERJ open research [ERJ Open Res] 2018 Apr 20; Vol. 4 (2). Date of Electronic Publication: 2018 Apr 20 (Print Publication: 2018).
نوع المنشور:	Journal Article
اللغة:	English
بيانات الدورية:	Publisher: European Respiratory Society Country of Publication: England NLM ID: 101671641 Publication Model: eCollection Cited Medium: Print ISSN: 2312-0541 (Print) Linking ISSN: 23120541 NLM ISO Abbreviation: ERJ Open Res Subsets: PubMed not MEDLINE
أسماء مطبوعة:	Original Publication: Sheffield : European Respiratory Society, [2015]-
مستخلص:	Newborn screening (NBS) for cystic fibrosis (CF) has been gradually established in several countries, but scant data are available on its long-term effects on survival. Our objective was to evaluate the long-term effects of CF NBS on survival. 586 patients, diagnosed and followed between 1971 and 2014 at the Verona CF Centre were analysed. Eligibility was confirmed in 342 cases diagnosed by NBS, 101 with meconium ileus and 143 through symptoms (44 out of 143 were NBS false negatives). The primary end-point was the 30-year overall survival in patients diagnosed by NBS. Patients were grouped according to the number of hospitalisations for respiratory or nutritional symptoms in the first 3 years of life: 0 (mild), 1-2 (moderate) and ≥3 (severe). Survival in NBS and symptoms groups was compared. The 30-year survival probability of the NBS group was 80.1% (95% CI 71.4-86.4%); in the symptoms group it was 71.0% (95% CI 62.2-78.2%). The 20-year survival was significantly higher in the NBS versus symptoms group in the severe (85% versus 64%, p=0.007) and moderate (94% versus 86%, p=0.016) groups. An adjusted Cox-model estimation confirmed differences in both the groups. Poor outcome associated with early severe presentation of CF is tempered by NBS. Competing Interests: Conflict of interest: None declared.
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تواريخ الأحداث:	Date Created: 20180426 Latest Revision: 20201001
رمز التحديث:	20221213
مُعرف محوري في PubMed:	PMC5909061
DOI:	10.1183/23120541.00109-2017
PMID:	29692998
قاعدة البيانات:	MEDLINE

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تدمد:	2312-0541
DOI:	10.1183/23120541.00109-2017