期刊文献+

即使采用现代疗法,新生儿囊性纤维化筛查依然大有裨益

Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment
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摘要 Objectives: To determine whether early identification of babies with cystic fibrosis (CF) improves outcome in the current environment of new improved treatments, considering the criticism that there may be only marginal benefit gained by CF newborn screening (NBS). Study design: We tested whether CF NBS in the setting of modern CF center care still afforded benefit using the UK CF Database (UKCFD; www.cystic-fi-brosis.org.uk) to compare clinical outcomes in infants who underwent NBS and control subjects who were clinically diagnosed (CD). With Mann-Whitney rank tests, 184 patients who underwent NBS aged 1 to 9 years in 2002 (excluding meconium ileus) were compared with matched patients who were CD in 3-year age groups (950 control subjects). Results: Patients as old as 6 years who underwent NBS had significantly greater median height z-scores, less severe Northern chest radiography scores, better Shwachman-Kulczycki scores, and lower rates of chronic Pseudomonas aeruginosa infection. No difference was found for weight z-score or % predicted forced expiratory value in 1 second or forced volume capacity. Nutritional benefit was demonstrated in patients who underwent NBS and were homozygous for the Δ F508 mutation. Conclusions: NBS segregates with better outcomes in patients as old as 6 years compared with age and gene-matched control subjects who are CD. This cross-sec-tional study shows that infants who undergo screening derive nutritional benefit in improved median height and reduced morbidity. Objectives: To determine whether early identification of babies with cystic fibrosis (CF) improves outcome in the current environment of new improved treatments, considering the criticism that there may be only marginal benefit gained by CF newborn screening (NBS). Study design: We tested whether CF NBS in the setting of modern CF center care still afforded benefit using the UK CF Database (UKCFD; www.cystic-fi-brosis.org.uk) to compare clinical outcomes in infants who underwent NBS and control subjects who were clinically diagnosed (CD). With Mann-Whitney rank tests, 184 patients who underwent NBS aged 1 to 9 years in 2002 (excluding meconium ileus) were compared with matched patients who were CD in 3-year age groups (950 control subjects). Results: Patients as old as 6 years who underwent NBS had significantly greater median height z-scores, less severe Northern chest radiography scores, better Shwachman-Kulczycki scores, and lower rates of chronic Pseudomonas aeruginosa infection. No difference was found for weight z-score or % predicted forced expiratory value in 1 second or forced volume capacity. Nutritional benefit was demonstrated in patients who underwent NBS and were homozygous for the Δ F508 mutation. Conclusions: NBS segregates with better outcomes in patients as old as 6 years compared with age and gene-matched control subjects who are CD. This cross-sec-tional study shows that infants who undergo screening derive nutritional benefit in improved median height and reduced morbidity.
出处 《世界核心医学期刊文摘(儿科学分册)》 2006年第A03期59-60,共2页
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