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IWO Meeting 16 November 2022 - ASBMR young talent: Silvia Storoni (Amsterdam): Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study

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IWO Meeting 16 November 2022 - ASBMR young talent: Silvia Storoni (Amsterdam): Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study

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IWO Meeting 16 November 2022 - ASBMR young talent: Silvia Storoni (Amsterdam): Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study

IWO Meeting 16 November 2022 - ASBMR young talent: Silvia Storoni (Amsterdam): Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study

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IWO Meeting 16 November 2022 - ASBMR young talent: Silvia Storoni (Amsterdam): Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study

  1. 1. Contact: s.storoni@amsterdamumc.nl Prevalence and hospital admissions in patients with Osteogenesis Imperfecta in The Netherlands: a nationwide registry study IWO Webinar 16 November 2022 Silvia Storoni Abstract SAT-676 ASBMR Annual Meeting September 9-12, 2022 Austin, TX Copyright Silvia Storoni
  2. 2. Disclosure I have NO disclosure. I have NO conflicts of interest with the presented material in this presentation. Copyright Silvia Storoni
  3. 3. Osteogenesis Imperfecta Genetic disease: COL1A1 gene COL1A2 gene Copyright Silvia Storoni
  4. 4. Osteogenesis Imperfecta Altered Collagen type 1 Genetic disease: COL1A1 gene COL1A2 gene Copyright Silvia Storoni
  5. 5. Osteogenesis Imperfecta Genetic disease: COL1A1 gene COL1A2 gene Altered Collagen type 1 Copyright Silvia Storoni
  6. 6. Osteogenesis Imperfecta Genetic disease: COL1A1 gene COL1A2 gene Altered Collagen type 1 Copyright Silvia Storoni
  7. 7. Osteogenesis Imperfecta Copyright Silvia Storoni
  8. 8. Classification Copyright Silvia Storoni
  9. 9. Classification https://en.wikipedia.org/wiki/Osteogenesis_imperfecta Copyright Silvia Storoni
  10. 10. Classification Majeet et al. 2019 Copyright Silvia Storoni
  11. 11. Classification What is the treatment for Osteogenesis Imperfecta? - Quora https://tandheelkunde.qualitypractice.nl/tijdschrift/editie/artikel/t/ dentinogenesis-imperfecta-etiologie-diagnostiek-en-behandeling Copyright Silvia Storoni
  12. 12. Classification Tauer et al. 2019 Boric et al 2017 Copyright Silvia Storoni
  13. 13. Osteogenesis Imperfecta Altered Collagen type 1 Genetic disease: COL1A1 gene COL1A2 gene Copyright Silvia Storoni
  14. 14. Osteogenesis Imperfecta Genetic disease: COL1A1 gene COL1A2 gene Altered Collagen type 1 Copyright Silvia Storoni
  15. 15. Osteogenesis Imperfecta Genetic disease: COL1A1 gene COL1A2 gene Altered Collagen type 1 Copyright Silvia Storoni
  16. 16. Amsterdam UMC study To what extent extra skeletal complications impacts OI patients’ life in terms of hospitalization and complications? Copyright Silvia Storoni
  17. 17. Methods Copyright Silvia Storoni
  18. 18. Methods Copyright Silvia Storoni
  19. 19. Methods Copyright Silvia Storoni
  20. 20. Methods • Hospital admissions Information • Length of stay per clinical admission • Medical specialties Copyright Silvia Storoni
  21. 21. Results Copyright Silvia Storoni
  22. 22. OI cohort: 674 patients • 2225 hospital admissions (479 patients) • Mean length of stay of 4.50 days per clinical admission • OI patients showed a hospitalization rate ratio 2.9 times higher than the general Dutch population (2013-2019) Results Copyright Silvia Storoni
  23. 23. 2% 1% 1% 1% 4% 4% 1% 4% 2% 5% 20% 13% 41% 34% 66% Intravenous infusion Other 57% 29% 14% Removal of ostesynthesis material Fracture related surgery Other 40% 10% 11% 24% 14% Removal of ostesynthesis material Fracture related surgery Intravenous infusion Surgical correc ti on of limb deformity Other Widely distribution of hospital admissions across medical specialties Copyright Silvia Storoni
  24. 24. 0% 25% 50% 75% 100% 0-19 Y 20-44 Y 45-64 Y 65-80 Y Pediatrics Surgery Orthopedic surgery Internal Medicine Neurology Otolaryngology Ophtalmology Gynaecology Gastroenterology Cardiology Pulmonology Urology Other Age of admissions by medical specialty Copyright Silvia Storoni
  25. 25. Take home message Osteogenesis Imperfecta patients appear to have almost three-fold higher hospitalization rate, when compared to the general Dutch population. Hospitalizations seem to be due to both skeletal and extraskeletal problems, which could have been due to complications related to OI. Due to the seemingly higher rate of hospital admissions, more awareness about the impact of OI on patients’ life is needed to improve and implement prevention and follow-up guidelines. Copyright Silvia Storoni
  26. 26. Thank you for the attention! Contact: s.storoni@amsterdamumc.nl Acknowledgments Sanne Treurniet, Department of Internal Medicine, Section Endocrinology, Amsterdam Rare Bone Disease Alessandra Maugeri, Department of Human Genetics, Amsterdam Movement Sciences, Amsterdam Rare Bone Disease Gerard Pals, Department of Human Genetics, Amsterdam Movement Sciences, Amsterdam Rare Bone Disease Joost G. van den Aardweg, Department of Respiratory Medicine, Amsterdam University Medical Center Stéphanie L. van der Pas, Department of Epidemiology and Data Science (EDS), Amsterdam University Medical Center Mariet W. Elting, Department of Human Genetics, Amsterdam University Medical Center Peter Kloen, Department of Orthopedic Surgery, Amsterdam Movement Sciences, Amsterdam Rare Bone Disease Dimitra Micha, Department of Human Genetics, Amsterdam Movement Sciences, Amsterdam Rare Bone Disease E.M.W. Eekhoff, Department of Internal Medicine, Section Endocrinology, Amsterdam Rare Bone Disease Copyright Silvia Storoni

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