The clinical features of osteogenesis imperfecta in Vietnam
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Osteogenesis imperfecta (OI) has not been studied in a Vietnamese population before. The aim of this study was to systematically collect epidemiological information, investigate clinical features and create a clinical database of OI patients in Vietnam for future research and treatment strategy development.
Participants underwent clinical and physical examinations; also medical records were reviewed. Genealogical information was collected and family members’ phenotypical manifestations recorded. Cases were classified according to the Sillence classification.
In total, 146 OI patients from 120 families were studied: 46 with OI Type I, 46 with Type III and 54 with Type IV. Almost patients had skeletal deformations. One hundred and forty-two had a history of fractures, 117 blue sclera, 89 dentinogenesis imperfecta and 26 hearing loss. The total number of fractures was 1,932. Thirty-four patients had intra-uterine fractures and nine had perinatal fractures. Surgery was performed 163 times in 58 patients; 100 osteosyntheses and 63 osteotomies. Bisphosphonate treatment was used in 37 patients. The number of affected individuals and predominance of severe forms of OI indicate that the disease is under diagnosed in Vietnam, especially in cases without a family history or with mild form of OI. Deformities appeared in all patients with different severity and localisation, affecting mostly the lower limbs. OI medical and surgical treatment rates are low and in most cases surgery was performed due to fractures.
Compared to previous studies, our results indicate a lower OI prevalence and greater severity of symptoms in the Vietnamese population when compared with other areas. Further investigation, improved diagnosis and treatment are needed to increase the patients’ quality of life.
KeywordsOsteogenesis imperfecta OI Skeletal deformations Bone fractures Dentinogenesis imperfecta Vietnam
We would like to thank the following people and organisations for their help and support with data collection, including the National Hospital of Pediatrics, Hanoi OI Center and Hue University Hospital. We deeply appreciate OI Booming Diamond Center in Ho Chi Minh City for their assistance with OI research. This research would not have been possible if not for the support, teaching and cooperation of members of Tartu University and Hue University of Medicine and Pharmacy.
Compliance with ethical standards
Conflict of interest
The authors declare no conflict of interest.
This work was supported by institutional research funding IUT20-46 of the Estonian Ministry of Education and Research, and the European Union’s European Regional Development Fund Programme “Supporting international cooperation in R&D” projects “EVMED” and “DIOXMED”.
Ethics approval and consent to participate
The study was conducted in accordance with the Helsinki Declaration and received approval from the ethical review board of Hue University Hospital (approval no. 75/CN-BVYD) and the Ethical Review Committee on Human Research of the University of Tartu (permit no. 221/M-34).
This article does not contain any studies with human participants or animals performed by any of the authors.
Informed consent from the patients or their legal representatives was obtained prior to inclusion to the study.
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