Cystic fibrosis in Canadian Hutterites

Background The Hutterite Brethren are a communal group of Anabaptists who live in the Western regions of North America, predominantly in the Western Canadian provinces. Due to a founder effect, Hutterites have a high rate of cystic fibrosis (CF) with genotypes limited to only two CFTR mutations. One...

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Bibliographic Details
Published in:	Pediatric pulmonology Vol. 55; no. 2; pp. 526 - 532
Main Authors:	Pasterkamp, Hans, Menzies, Kathryn J., Bayomi, Dennis J.
Format:	Journal Article
Language:	English
Published:	United States Wiley Subscription Services, Inc 01-02-2020
Subjects:	Cystic fibrosis epidemiology exocrine pancreatic insufficiency genetic background Mutation phenotype population characteristics Tobacco smoke phenotype epidemiology exocrine pancreatic insufficiency cystic fibrosis genetic background population characteristics
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Summary:	Background The Hutterite Brethren are a communal group of Anabaptists who live in the Western regions of North America, predominantly in the Western Canadian provinces. Due to a founder effect, Hutterites have a high rate of cystic fibrosis (CF) with genotypes limited to only two CFTR mutations. One‐third of Hutterite patients with CF are pancreatic sufficient. Previously we found an unexplained younger age at death in Hutterites compared with nonHutterites homozygous for the common F508del mutation. The present study expanded the data collection and analysis for confirmation and further exploration. Methods Anonymized information, based on Hutterite surnames, was extracted from the Canadian CF Registry. Summary data on nonHutterite patients with CF homozygous for F508del served as control. Statistical analyses explored the effects of genotype within Hutterites and compared nutritional status, lung function, and microbiologic findings between the groups. Results The younger average age at death in Hutterites compared with controls was confirmed, but there was no suggestion of a generally shortened life expectancy. While the nutritional status in Hutterite children was better than that of controls, their lung function was slightly but significantly lower. Staphylococcus aureus was more frequent in Hutterites while there was no difference between the groups regarding Pseudomonas aeruginosa. Conclusions Despite less pancreatic insufficiency, better nutrition, communal life in socioeconomic stability, and without exposure to environmental tobacco smoke, the clinical course of CF appears to be more severe in a significant number of Hutterites. Investigations of gene‐environment interactions and of CF disease gene modifiers may help to explain this conundrum.
Bibliography:	Presented in part at the American Thoracic Society Conference, May 2014, in San Diego, CA
ISSN:	8755-6863 1099-0496
DOI:	10.1002/ppul.24590