Haemophilia and low bone mass. Ok, but what about fracture risk?

Anagnostis, Panagiotis; Karras, Spyridon Ν.; Vakalopoulou, Sofia; Terpos, Evangelos

doi:10.1111/hae.12833

Cited by 8 publications

(17 citation statements)

References 24 publications

(47 reference statements)

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“…Nevertheless, we found no association between history of fractures and circulating sclerostin. In general, fracture history in PWH is quite complex, because fractures have been reported both after major and minor trauma, even from childhood …”

Section: Discussionmentioning

confidence: 99%

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The role of sclerostin/dickkopf‐1 and receptor activator of nuclear factor kB ligand/osteoprotegerin signalling pathways in the development of osteoporosis in patients with haemophilia A and B: A cross‐sectional study

et al. 2017

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Section: Discussionmentioning

confidence: 99%

“…Other contributory factors leading to bone loss in patients with haemophilia (PWH) are infections from hepatitis C (HCV) and human immunodeficiency virus (HIV), vitamin D deficiency and the degree of haemophilic arthropathy . Nevertheless, data on fracture risk in PWH are not robust …”

Section: Introductionmentioning

confidence: 99%

The role of sclerostin/dickkopf‐1 and receptor activator of nuclear factor kB ligand/osteoprotegerin signalling pathways in the development of osteoporosis in patients with haemophilia A and B: A cross‐sectional study

et al. 2017

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“…3 Studies showed that haemophilic subjects have a lower bone mineral density (BMD), measured by dual energy X-ray absorptiometry (DXA), according to World Health Organization (WHO) criteria for the diagnosis of osteoporosis, compared with the normal population, 4 and show an increased risk of fragility fractures. 5 Two metaanalyses 6,7 confirmed that adults with severe haemophilia have lower lumbar BMD than controls, being unclear if due to failure in reaching the peak bone mass, to premature bone loss or to a combination of both. Reduced physical activity for recurrent hemarthrosis determines increased bone resorption, low peak bone mass in adolescence and osteoporosis in adulthood.…”

Section: Introductionmentioning

confidence: 99%

Usefulness of bone microarchitectural and geometric DXA‐derived parameters in haemophilic patients

et al. 2018

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Introduction Haemophilia is a recessive X‐linked inherited bleeding disorder, whose typical symptom is spontaneous intra‐articular haemorrhage leading to joint damage, which can be quantified by the Haemophilia Joint Health Score (HJHS). Arthropathy and other characteristics of haemophilic patients may reduce bone mineral density (BMD), increasing the risk for fragility fractures, which also may occur due to bone quality impairment. Aim To evaluate bone quantity by BMD and bone quality by Trabecular Bone Score (TBS), bone strain (BS) and hip structural analysis (HSA) in a haemophilic population, and to relate these parameters to general and specific risk factors for osteoporosis and to HJHS. Methods Seventy haemophilic patients ≥18 years were enrolled. Densitometric derived lumbar spine and femoral BMD with TBS, BS and HSA were performed. Data regarding risk factors for osteoporosis, presence of arthroprosthesis or arthrodesis were collected, and HJHS was calculated. A Z‐score ≤−2.0 defined a low bone mass. Results Overall, a reduced bone mass was present in 52 patients at the femur and in 38 at the lumbar spine. Lumbar spine BMD, TBS and BS did not correlate with HJHS. HSA bone geometric parameters correlated negatively with HJHS. BMD and HSA correlated with some risk factors for osteoporosis, namely HIV and its therapy, hepatitis C and smoking. Conclusions Haemophilic patients showed a reduced BMD at lumbar spine and/or femur. Femoral bone density and geometry correlated with HJHS. The microarchitecture of the trabecular vertebral bone seemed to be not influenced by the haemophilic joint damage.

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“…Despite the evidence on the association between haemophilia and low BMD, insufficient and inconsistent evidence exists regarding fractures in PWH . The reported prevalence of fractures in studies (mostly from cross‐sectional and retrospective studies) among PWH ranges from 4% to 37% . In a single institutional and 10‐year retrospective cohort study, a markedly increased rate of bone fractures was observed in PWH as compared to that in the general population, with a risk ratio of 10.7 (95% CI 8.2‐14.11).…”

Section: Discussionmentioning

confidence: 98%

“…Osteoporotic fractures is the one of the major complications of lower BMD that can lead to chronic pain and disability, conditions for which PWH are already at a significant risk. Despite the evidence on the association between haemophilia and low BMD, insufficient and inconsistent evidence exists regarding fractures in PWH . The reported prevalence of fractures in studies (mostly from cross‐sectional and retrospective studies) among PWH ranges from 4% to 37% .…”

Section: Discussionmentioning

confidence: 99%

Risk of osteoporotic fractures as a consequence of haemophilia: A nationwide population‐based cohort study

Tuan

Sun

et al. 2019

Haemophilia

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Aim: Low bone mineral density occurs more commonly in patients with haemophilia (PWH) than the general population. However, the risk of haemophilia-related osteoporotic fractures has not been well established. We aim to explore the relationship between haemophilia and the development of osteoporotic fractures following haemophilia. Methods:This was a nationwide population-based cohort study based on the data in the Taiwan National Health Insurance Research Database (TNHIRD). Patients who were diagnosed with haemophilia were selected. A comparison cohort was formed of patients without haemophilia who were matched according to age and sex. The incidence rate and the hazard ratios (HRs) of new-onset osteoporotic fractures were calculated for both cohorts. Results:The haemophilia cohort consisted of 75 patients, and the comparison cohort comprised 300 matched control patients without haemophilia. The risk of osteoporotic fractures was higher in the haemophilia cohort than in the comparison cohort (HR = 5.41, 95% confidence interval [CI] = 2.42-12.1, P < 0.001). After adjustments for age, sex, comorbidities, urbanizations and socio-economic status, PWH were 4.37 times more likely to develop osteoporotic fractures (95% CI = 1.88-10.17, P = 0.001) as compared to matched cohort. In addition, the incidence of newly diagnosed osteoporotic fractures was significantly increased after 5-year follow-up durations.Conclusion: Though our study by TNHIRD presented methodologic flaws by its design nature, we observed that haemophilia may increase the risk of osteoporotic fractures and the cumulative incidence was significantly higher for PWH diagnosed more than 5 years. Clinicians should pay particular attention to osteoporotic fractures following haemophilia in PWH as they age. K E Y W O R D S cumulative incidence, haemophilia, National Health Insurance Research Database, osteoporotic fracture | 877 TUAN eT Al.

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Haemophilia and low bone mass. Ok, but what about fracture risk?

Cited by 8 publications

References 24 publications

The role of sclerostin/dickkopf‐1 and receptor activator of nuclear factor kB ligand/osteoprotegerin signalling pathways in the development of osteoporosis in patients with haemophilia A and B: A cross‐sectional study

The role of sclerostin/dickkopf‐1 and receptor activator of nuclear factor kB ligand/osteoprotegerin signalling pathways in the development of osteoporosis in patients with haemophilia A and B: A cross‐sectional study

Usefulness of bone microarchitectural and geometric DXA‐derived parameters in haemophilic patients

Risk of osteoporotic fractures as a consequence of haemophilia: A nationwide population‐based cohort study

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