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Idiopathic subglottic stenosis in Saskatchewan Hutterite population
C Holmes1, K Aravinthan1, R A Murphy1,2
1College of Medicine, University of Saskatchewan, Saskatoon, Canada.
The Journal of Laryngology and Otology
|February 14, 2022
Summary
Genetics may predispose individuals to idiopathic subglottic stenosis. This rare disease is significantly more common in the Hutterite population, suggesting a genetic link.
Area of Science:
- Genetics
- Epidemiology
- Otolaryngology
Background:
- Idiopathic subglottic stenosis (iSGS) is a rare airway disease.
- A genetic predisposition for iSGS is hypothesized but not fully understood.
- The isolated Hutterite population offers a unique model to study genetic influences on disease development.
Purpose of the Study:
- To investigate the prevalence of iSGS in the Hutterite population.
- To determine if genetic factors contribute to the etiology of iSGS.
- To assess the risk of iSGS development in Hutterites compared to non-Hutterites.
Main Methods:
- Retrospective chart review of adult iSGS patients in Saskatchewan (2008-2018).
- Cases were categorized into Hutterite and non-Hutterite groups.
- Analysis of demographic and familial history data.
Main Results:
- Four of 36 iSGS cases were identified in the Hutterite population.
- Hutterites had 21.89 times higher odds of developing iSGS compared to non-Hutterites.
- A positive family history of iSGS was exclusively reported in the Hutterite cohort.
Conclusions:
- The findings support the hypothesis that genetic factors play a role in iSGS etiology.
- The Hutterite population exhibits a significantly higher risk for developing iSGS.
- This study is the first to identify a specific subpopulation with an elevated risk for iSGS.
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