際際滷

際際滷Share a Scribd company logo
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
Disclosure
I have NO disclosure.
I have NO conflicts of interest with the presented material in this presentation.
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Genetic disease:
COL1A1 gene
COL1A2 gene
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Altered
Collagen type 1
Genetic disease:
COL1A1 gene
COL1A2 gene
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Genetic disease:
COL1A1 gene
COL1A2 gene
Altered
Collagen type 1
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Genetic disease:
COL1A1 gene
COL1A2 gene
Altered
Collagen type 1
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Copyright
Silvia Storoni
Classification
Copyright
Silvia Storoni
Classification
https://en.wikipedia.org/wiki/Osteogenesis_imperfecta
Copyright
Silvia Storoni
Classification
Majeet et al. 2019
Copyright
Silvia Storoni
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
Classification
Tauer et al. 2019
Boric et al
2017
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Altered
Collagen type 1
Genetic disease:
COL1A1 gene
COL1A2 gene
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Genetic disease:
COL1A1 gene
COL1A2 gene
Altered
Collagen type 1
Copyright
Silvia Storoni
Osteogenesis Imperfecta
Genetic disease:
COL1A1 gene
COL1A2 gene
Altered
Collagen type 1
Copyright
Silvia Storoni
Amsterdam UMC study
To what extent extra skeletal complications impacts OI patients life in terms of
hospitalization and complications?
Copyright
Silvia Storoni
Methods
Copyright
Silvia Storoni
Methods
Copyright
Silvia Storoni
Methods
Copyright
Silvia Storoni
Methods
 Hospital admissions Information
 Length of stay per clinical admission
 Medical specialties
Copyright
Silvia Storoni
Results
Copyright
Silvia Storoni
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
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
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
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
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
St辿phanie 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

More Related Content

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