Fracture Risk in People With Haemophilia A and B: A Systematic Review and Meta-Analysis
Corresponding Author
Efstathios Divaris
Department of Endocrinology, Hippokration General Hospital, Thessaloniki, Greece
Correspondence: Efstathios Divaris ([email protected])
Search for more papers by this authorIoannis Konstantinidis
Research Team “Histologistas”, Interinstitutional Postgraduate Program “Health and Environmental Factors”, Department of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorParaskevi Karvouni
School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
Search for more papers by this authorEleni Gavriilaki
2nd Propedeutic Department of Internal Medicine, Haematology Unit - Haemophilia Centre of Northern Greece, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorSofia Vakalopoulou
2nd Propedeutic Department of Internal Medicine, Haematology Unit - Haemophilia Centre of Northern Greece, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorDimitrios G. Goulis
Unit of Reproductive Endocrinology, 1st Department of Obstetrics & Gynecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorPanagiotis Anagnostis
Unit of Reproductive Endocrinology, 1st Department of Obstetrics & Gynecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
3rd Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorCorresponding Author
Efstathios Divaris
Department of Endocrinology, Hippokration General Hospital, Thessaloniki, Greece
Correspondence: Efstathios Divaris ([email protected])
Search for more papers by this authorIoannis Konstantinidis
Research Team “Histologistas”, Interinstitutional Postgraduate Program “Health and Environmental Factors”, Department of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorParaskevi Karvouni
School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
Search for more papers by this authorEleni Gavriilaki
2nd Propedeutic Department of Internal Medicine, Haematology Unit - Haemophilia Centre of Northern Greece, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorSofia Vakalopoulou
2nd Propedeutic Department of Internal Medicine, Haematology Unit - Haemophilia Centre of Northern Greece, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorDimitrios G. Goulis
Unit of Reproductive Endocrinology, 1st Department of Obstetrics & Gynecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorPanagiotis Anagnostis
Unit of Reproductive Endocrinology, 1st Department of Obstetrics & Gynecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
3rd Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
Search for more papers by this authorFunding: The authors received no specific funding for this work.
ABSTRACT
Introduction
Haemophilia A and B is a disease consistently associated with reduced bone mineral density, both in adults and children. However, whether haemophilia also increases fracture risk has not yet been proven.
Aim
This systematic review and meta-analysis aimed to synthesize and analyse studies evaluating the association between haemophilia and fracture risk.
Methods
Comprehensive research was conducted in three electronic databases (PubMed, CENTRAL, and Scopus) up to 30 June 2024. Data were expressed as relative risk (RR) with 95% confidence intervals (CI). The I2 index was employed to evaluate heterogeneity.
Results
Fourteen studies were included in the qualitative and four in the quantitative analysis (participants: 13,221, publication years: 2007–2022). Regarding design, five studies were retrospective cohorts, two were case-control, and seven were cross-sectional. Fracture prevalence in people with haemophilia (PWH) was 5.7%, ranging from 1.4% to 27.7% (data from 14 studies), compared with 0.9% in the control group, ranging from 0% to 5.1% (data from 3 studies). In comparison with healthy men, PWH demonstrated increased fracture risk (RR 4.56, 95% CI 1.28–16.25, p = 0.019, I2 90.74%). However, there was insufficient data to categorize fractures according to their location and to compare fracture incidence between patients receiving prophylaxis and those on-demand treatment, as well as according to the type or severity of haemophilia.
Conclusion
This is the first meta-analysis showing a more than 4-fold increased fracture risk in PWH compared with the general population.
Conflicts of Interest
The authors declare no conflicts of interest.
Open Research
Data Availability Statement
The data that support the findings of this study are available from the corresponding author upon reasonable request.
Supporting Information
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| hae70033-sup-0001-TableS1.docx29.2 KB | Supporting Information |
| hae70033-sup-0002-TableS2.docx13.7 KB | Supporting Information |
| hae70033-sup-0003-TableS3.docx16.5 KB | Supporting Information |
| hae70033-sup-0004-TableS4.docx15.3 KB | Supporting Information |
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