International Orthopaedics

, Volume 41, Issue 1, pp 21–29 | Cite as

The clinical features of osteogenesis imperfecta in Vietnam

  • Ho Duy Binh
  • Katre Maasalu
  • Vu Chi Dung
  • Can T. Bich Ngoc
  • Ton That Hung
  • Tran V. Nam
  • Le N. Thanh Nhan
  • Ele Prans
  • Ene Reimann
  • Lidiia Zhytnik
  • Sulev Kõks
  • Aare Märtson
Original Paper

Abstract

Purpose

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.

Method

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.

Results

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.

Conclusions

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.

Keywords

Osteogenesis imperfecta OI Skeletal deformations Bone fractures Dentinogenesis imperfecta Vietnam 

Notes

Acknowledgements

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.

Funding

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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Copyright information

© SICOT aisbl 2016

Authors and Affiliations

  • Ho Duy Binh
    • 1
    • 2
    • 3
  • Katre Maasalu
    • 2
    • 3
  • Vu Chi Dung
    • 4
  • Can T. Bich Ngoc
    • 4
  • Ton That Hung
    • 5
  • Tran V. Nam
    • 6
  • Le N. Thanh Nhan
    • 1
  • Ele Prans
    • 7
  • Ene Reimann
    • 7
  • Lidiia Zhytnik
    • 2
  • Sulev Kõks
    • 7
  • Aare Märtson
    • 2
    • 3
  1. 1.Hue University of Medicine and Pharmacy, Hue UniversityHueVietnam
  2. 2.Clinic of Traumatology and OrthopaedicsUniversity of TartuTartuEstonia
  3. 3.Clinic of Traumatology and OrthopaedicsTartu University HospitalTartuEstonia
  4. 4.National Hospital of PediatricsDong DaVietnam
  5. 5.OI Booming Diamond CenterHo Chi Minh CityVietnam
  6. 6.Traditional Medicine InstituteHo Chi Minh CityVietnam
  7. 7.Pathophysiology DepartmentUniversity of TartuTartuEstonia

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