Cystic fibrosis-related cirrhosis in Europe

cystic fibrosis survival cirrhosis

dr. F.A.J.A. Bodewes

Nature of the research:
Analysis of european Cystic fibrosis patient registry data on the subject of cirrhosis

Fields of study:
epidemiology and statistics pediatrics pulmonology

Background / introduction
~10% of patients of cystic fibrosis develop cirrhosis. In a recent Dutch cohort study, as part of a student project (see reference 1), we found that the presence of cirrhosis in CF patients has a severe impact on patients survival.
We acquired patient data from the European Cystic fibrosis registry. This data set includes approximately 60.000 CF patients. The first analysis of these data has been performed as part of another recently finished student project (stage wetenschap). The same impact of the presence of cirrhosis on the survival of CF patients as seen in the Dutch patient study was found in the European cohort.
Research question / problem definition
Additional data analysis and interpretation concerning the role of age cohort and genetics on cirrhosis related survival in european CF patients
Prepare a research strategy
Statistical analysis of extensive SPSS database.
Prepare a report with the option of publication
Pals, F. H., et al. "Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands." Journal of Cystic Fibrosis 18.3 (2019): 385-389.

Boëlle, Pierre‐Yves, et al. "Cystic fibrosis liver disease: outcomes and risk factors in a large cohort of French patients." Hepatology 69.4 (2019): 1648-1656.

Debray, Dominique, et al. "Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease." Journal of Cystic Fibrosis 10 (2011): S29-S36.

Rowland, Marion, et al. "Outcome in cystic fibrosis liver disease." The American journal of gastroenterology 106.1 (2011): 104-109.
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Laatst gewijzigd: 23 februari 2012