Fractures in Osteogenesis Imperfecta

Pathogenesis, Treatment, Rehabilitation and Prevention

Journal article (2022)

Authors

Wouter Nijhuis University Medical Center Utrecht
Marjolein Verhoef University Medical Center Utrecht
Christiaan van Bergen Amphia Hospital
Harrie Weinans University Medical Center Utrecht, Biomaterials & Tissue Biomechanics - Mechanical, Maritime and Materials Engineering
Ralph J.B. Sakkers University Medical Center Utrecht
Research Group
Biomaterials & Tissue Biomechanics (Mechanical, Maritime and Materials Engineering) (TU Delft)
Copyright: © 2022 Wouter Nijhuis, Marjolein Verhoef, Christiaan van Bergen, Harrie Weinans, Ralph Sakkers
DOI: https://doi.org/10.3390/children9020268
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Published Date

2022

Language

English

Faculty

Mechanical, Maritime and Materials Engineering

Department

Biomechanical Engineering

Research Group

Biomaterials & Tissue Biomechanics

Abstract

Fractures in patients with osteogenesis imperfecta (OI) are caused by a decreased strength of bone due to a decreased quality and quantity of bone matrix and architecture. Mutations in the collagen type 1 encoding genes cause the altered formation of collagen type I, one of the principal building blocks of bone tissue. Due to the complexity of the disease and the high variation of the clinical problems between patients, treatment for these patients should be individually tailored. In general, short immobilization periods with flexible casting material, use of intramedullary implants, and simultaneous deformity correction are preferred. Multidisciplinary care with a broad view of the support needed for the patient and his/her living environment is necessary for the optimal rehabilitation of these patients. Increasing bone strength with exercise, medication, and sometimes alignment surgery is generally indicated to prevent fractures.