Comparison of the US and Australian Cystic Fibrosis Registries: The impact of newborn screening

Martin, Bradley, Schechter, Michael S., Jaffe, Adam, Cooper, Peter, Bell, Scott C. and Ranganathan, Sarath (2012) Comparison of the US and Australian Cystic Fibrosis Registries: The impact of newborn screening. Pediatrics, 129 2: E348-E355. doi:10.1542/peds.2011-0567

Author Martin, Bradley
Schechter, Michael S.
Jaffe, Adam
Cooper, Peter
Bell, Scott C.
Ranganathan, Sarath
Title Comparison of the US and Australian Cystic Fibrosis Registries: The impact of newborn screening
Journal name Pediatrics   Check publisher's open access policy
ISSN 0031-4005
Publication date 2012-02-01
Sub-type Article (original research)
DOI 10.1542/peds.2011-0567
Open Access Status
Volume 129
Issue 2
Start page E348
End page E355
Total pages 8
Place of publication Grove Village, IL, United States
Publisher American Academy of Pediatrics
Language eng
Formatted abstract
National data registries for cystic fibrosis (CF) enable comparison of health statistics between countries. We examined the US and Australian CF data registries to compare demographics, clinical practice and outcome measures.

We compared the 2003 US and Australian registries. Differences in pulmonary and growth outcomes were assessed by creating models controlling for differences in age, gender, genotype, and diagnosis after newborn screening.

Data on 12 994 US and 1220 Australian patients aged #18 years were analyzed. A significant difference was noted in the proportion who had been diagnosed after newborn screening (Australian 65.8% vs United States 7.2%; P < .001). Australian children had significantly greater mean height percentile (41.0 vs 32.6; P < .001) and weight percentile (43.5 vs 36.1; P = .028) than US children. Mean forced expiratory volume in 1 second (FEV 1) percent predicted adjusted for age, gender, and genotype was similar in the 2 countries (P = .80). Patients diagnosed after newborn screening had higher mean FEV 1 (5.3 [95% confidence interval (CI): 3.6-7.0]) percent predicted and BMI (0.26 [95% CI: 0.09-0.43]). Mean FEV 1 of Australian patients diagnosed after newborn screening was lower by 5.2 (95% CI: 2.8-7.6) percent predicted compared with US children.

Children diagnosed with CF after newborn screening benefited from better lung function and BMI than those diagnosed clinically. The benefit of newborn screening on lung function was significantly less in Australian children compared with US children. Statistical comparisons between CF registries are feasible and can contribute to benchmarking and improvements in care.
Keyword Cystic fibrosis
Lung function
Newborn screening
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ
Additional Notes Published online 16 January 2012.

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2013 Collection
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