Prevalence, number of fractures, and hospital characteristics among the pediatric population with osteogenesis imperfecta: results from the nationwide registry of Türkiye.


Journal

Journal of pediatric orthopedics. Part B
ISSN: 1473-5865
Titre abrégé: J Pediatr Orthop B
Pays: United States
ID NLM: 9300904

Informations de publication

Date de publication:
17 Jun 2024
Historique:
medline: 20 6 2024
pubmed: 20 6 2024
entrez: 20 6 2024
Statut: aheadofprint

Résumé

Osteogenesis imperfecta is a rare connective tissue disorder with an estimated number of 4-20 cases per 100 000 inhabitants. Although the prevalence differs among regions, there are only a few number of national registry studies published previously. The aim of this study was to determine the prevalence of osteogenesis imperfecta among the pediatric age group in Türkiye, together with the patient and hospital characteristics. Via the e-health database of the Turkish Ministry of Health, we collected and retrospectively evaluated the medical records of the patients who were under 18 years of age with the diagnosis of osteogenesis imperfecta between 2016 and 2022. Total number of fractures, treatment modalities, and the hospital characteristics were also recorded. Two thousand seven hundred forty patients were extracted with a mean age of 9.77 ± 4.81 years. The prevalence of osteogenesis imperfecta in Türkiye among the pediatric population was calculated as 11.6 per 100 000 individuals. The median annual incidence was 31.5 per 100 000 live births between 2016 and 2022. There were 17.4 hospital admissions per patient per year. The mean age at the time of in-hospital mortality was 4.08 ± 5.03 years. The fracture rate per patient per year was 0.56 and conservative treatment was the most commonly preferred modality for all ages. This is the first registry-based nationwide study of osteogenesis imperfecta patients in Türkiye, providing important characteristics of the disease. Together with the help of the ongoing development of national health database systems, precision in patient identification would yield substantial benefits in terms of management of osteogenesis imperfecta.

Identifiants

pubmed: 38900105
doi: 10.1097/BPB.0000000000001192
pii: 01202412-990000000-00205
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.

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Auteurs

Bariş Görgün (B)

Ortopediatri Istanbul, Academy of Pediatric Orthopaedics, Istanbul.

Niyazi Erdem Yaşar (NE)

Department of Orthopaedics and Traumatology, Ankara Bilkent City Hospital, Health Sciences University.

İzzet Bingöl (İ)

Department of Orthopaedics and Traumatology, Faculty of Medicine, Ankara Oncology Training and Research Hospital, Health Sciences University.

Ebru Dumlupinar (E)

Department of Biostatistics, Faculty of Medicine, Ankara University.

Naim Ata (N)

Ministry of Health, Department of General Directorate for Health Information Systems.

M Mahir Ülgü (MM)

Ministry of Health, Department of General Directorate for Health Information Systems.

Şuayip Birinci (Ş)

Ministry of Health, Ankara, Türkiye.

Sinem Bayram (S)

Ministry of Health, Department of General Directorate for Health Information Systems.

Güzelali Özdemir (G)

Department of Orthopaedics and Traumatology, Ankara Bilkent City Hospital, Health Sciences University.

Classifications MeSH