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新生儿囊性纤维化筛查的益处:加拿大的经验。

The benefits of newborn screening for cystic fibrosis: The Canadian experience.

作者信息

Mak D Y F, Sykes J, Stephenson A L, Lands L C

机构信息

Cystic Fibrosis Canada, 2323 Yonge Street Suite 800, Toronto, M4P 2C9, Canada.

Department of Respirology, St. Michael's Hospital, 30 Bond Street, Toronto, M5B 1W8, Canada; Keenan Research Centre, Li Ka Shing Knowledge Institute of St. Michael's Hospital, 209 Victoria Street, Toronto, Ontario, M5B 1T8, Canada.

出版信息

J Cyst Fibros. 2016 May;15(3):302-8. doi: 10.1016/j.jcf.2016.04.001. Epub 2016 Apr 21.

DOI:10.1016/j.jcf.2016.04.001
PMID:27118577
Abstract

BACKGROUND

The impact of newborn screening (NBS) for cystic fibrosis (CF) on early indicators of long-term health was evaluated in the context of government-sponsored healthcare and access to current therapies.

METHODS

Using data from the Canadian CF Registry between 2008 and 2013, we compared the rates of respiratory infections and markers of nutritional status in those diagnosed through NBS to those who were diagnosed clinically within the same time period using Mann-Whitney and Fischer's exact test as appropriate.

RESULTS

The study included 303 subjects, 201 in the NBS group and 102 in the non-NBS group. NBS patients were diagnosed earlier and had their first clinic visit at a younger age. Pancreatic insufficiency was less common in NBS patients. The incidence of Pseudomonas aeruginosa and Staphylococcus aureus were lower in NBS patients. After adjusting for age at clinic visit, gender, pancreatic status, and Pseudomonas aeruginosa infection status, mean z-scores for weight-for-age and height-for-age were higher in NBS patients, with no differences in BMI-for-age.

CONCLUSIONS

NBS programs for CF lead to improved long-term health outcomes for the CF population.

摘要

背景

在政府资助的医疗保健以及获取当前治疗方法的背景下,评估了新生儿筛查(NBS)对囊性纤维化(CF)长期健康早期指标的影响。

方法

利用2008年至2013年加拿大CF登记处的数据,我们使用曼-惠特尼检验和费舍尔精确检验(视情况而定),比较了通过NBS诊断的患者与同期临床诊断的患者的呼吸道感染率和营养状况指标。

结果

该研究纳入了303名受试者,NBS组201名,非NBS组102名。NBS患者诊断较早,首次就诊年龄较小。NBS患者中胰腺功能不全较少见。NBS患者中铜绿假单胞菌和金黄色葡萄球菌的发生率较低。在对就诊年龄、性别、胰腺状况和铜绿假单胞菌感染状况进行调整后,NBS患者年龄别体重和年龄别身高的平均z评分较高,年龄别BMI无差异。

结论

CF的NBS项目可改善CF人群的长期健康结局。

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