• Chest · Sep 2024

    Risk of pulmonary diseases in osteogenesis imperfecta in Denmark - A register-based cohort study.

    • Jane Dahl Andersen, Marie Louise Lyster, Mette Kathrine Holst, Daniel Pilsgaard Henriksen, Anders Christensen, Christian B Laursen, Antonella Forlino, and Lars Folkestad.
    • Department of Internal Medicine, Lillebaelt Hospital, Kolding, Denmark.
    • Chest. 2024 Sep 17.

    BackgroundOsteogenesis Imperfecta (OI) is a rare hereditary disease mainly resulting in reduced or altered collagen type I. Collagen type I is a major constituent of the respiratory system, and normal collagen type I is vital for the pulmonary tissue function.Research QuestionsDoes patient with OI have increased admission rates due to pulmonary diseases compared to the general population?Study Design And MethodsThis is a register-based, nationwide, cohorts study, including all patients with OI in Denmark and a reference population. From 1st of January 1995 until the 31st of December 2018, we evaluated the rates of admissions due to asthma, chronic obstructive pulmonary disease (COPD) and pneumonia as well as the use of broncodilatator drugs and antibiotics comparing individuals with OI to the reference population.ResultsWe included 862 individuals with OI and 4,283 persons in the reference population covering 15952 and 79471 person years of observation respectively in the two cohorts. Admissions rate (IR) was highest in women with OI aged 65+ years with 56.3 admissions per 1000 person years and 29.4 admissions per 1000 person years in the reference population (amounting to an admissions rate ratio (IRR) 1.91 [95%CI 1.38-2.70]) . The highest admission rate in men with OI was found amongst the participants aged 0-18 years IR 30.4 per 1,000 person years and IR 7.7 per 1,000 person years in the reference population (IRR 4.92 [3.79-6.38]). We found a higher proportion of long and short acting broncodilatator drug users in the OI cohort, but no increased use of antibiotics.InterpretationOverall, the admission rates for respiratory diseases were low in the OI cohort, but a higher relative risk of hospitalizations due to respiratory disease than in the general population. Timely diagnosis and treatment of respiratory complications in individuals with OI is warranted.Copyright © 2024. Published by Elsevier Inc.

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