Bone health in persons with haemophilia: a review

Ghosh, Kanjaksha; Shetty, Shrimati

doi:10.1111/j.1600-0609.2012.01803.x

Cited by 23 publications

(24 citation statements)

References 50 publications

(74 reference statements)

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“…In normal healthy people, BMI and BMD are known to be related. While some studies have also confirmed the existence of such a relationship in haemophilia patients, others have reached the opposite conclusion . Our results indicated a statistically significant positive correlation between BMI and femoral neck, lumbar spine and total hip BMD.…”

Section: Discussioncontrasting

confidence: 51%

Decreased bone mineral density and associated factors in severe haemophilia A patients: A case‐control study

et al. 2019

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Introduction Haemophilia patients may exhibit lower levels of bone mineral density (BMD) than the general population for a variety of reasons. Aim We aimed to investigate decreased BMD in people with severe adult haemophilia A (PWH) living in eastern Turkey, and to evaluate the related potential risk factors. Methods The study included 41 PWH and 40 healthy volunteers. Dual‐energy x‐ray absorptiometry (DXA) was used to measure the BMD. Blood tests and body mass index (BMI) were recorded. The Functional Independence Score in Hemophilia (FISH) test was used to measure functional ability status. Results There was a significant difference between the PWH and control groups with respect to femoral neck and total hip BMD (in g/cm2), but the difference for lumbar spine was not significant (P = .017, P < .001, P = .071, respectively). In PWH, patients under 50 years of age, 19.4% were found to have “lower than expected” BMD levels for their age, while 27.8% showed “low normal” levels. In PWH, osteoporosis was found in 60% of the patients over 50 and osteopenia in 20%. Vitamin D insufficiency and deficiency were present in 63.4% of the PWH, significantly higher than the control group (37.5%; P < .001). Conclusion The results indicated that the decrease in BMD was significantly greater in patients with severe haemophilia A than in the normal healthy population. This reduction was correlated with BMI, vitamin D and low functional ability status. However, in multivariate analysis, none of these was a strong independent risk factor.

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Section: Discussioncontrasting

confidence: 51%

Decreased bone mineral density and associated factors in severe haemophilia A patients: A case‐control study

et al. 2019

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“…Bone disease in haemophilia has been attributed to bleeding sequelae from chronic inflammation and decreased weight‐bearing activity from painful and debilitating haemarthroses (Ghosh & Shrimati, ). Chronic hepatitis C and HIV infections are associated with poor bone health and have a high incidence in the haemophilia population secondary to past exposure to contaminated blood products (Lo et al , ).…”

Section: Haemophilia Cohort Baseline Characteristicsmentioning

confidence: 99%

Increased fracture rates in people with haemophilia: a 10‐year single institution retrospective analysis

et al. 2015

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“…To the editor: Ghoshy et al in their recent review in European Journal of Haematology (1) tried to present data about the prevalence of low bone mineral density (BMD) in patients with haemophilia A and B and analyse the pathophysiology of this entity proposing appropriate management. The authors report data of small studies and refer mainly to the association of low BMD with hepatitis C (HCV) and human immunodeficiency virus (HIV) infections and their treatment (interferona, antiretroviral therapy).…”

Section: Bone Disease In Patients With Haemophilia What Is True?mentioning

confidence: 99%

Bone disease in patients with haemophilia. What is true?

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Garipidou

Kazantzidou

et al. 2013

European J of Haematology

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Haematology (1) tried to present data about the prevalence of low bone mineral density (BMD) in patients with haemophilia A and B and analyse the pathophysiology of this entity proposing appropriate management. The authors report data of small studies and refer mainly to the association of low BMD with hepatitis C (HCV) and human immunodeficiency virus (HIV) infections and their treatment (interferona, antiretroviral therapy). They also refer to the possible role of vitamin K and the interaction between factor VIII-von Willebrand and receptor activator of nuclear factor-jB (RANK) ligand (RANKL), in the induction of osteoclastogenesis (1).In the largest study conducted so far, we found an increased prevalence of bone disease in haemophiliacs of Northern Greece (26.9%), compared with apparently healthy age-and sex-matched controls, a prevalence that was lower than previously reported. Except for the lower prevalence of severe haemophilia in our cohort, this difference is mainly attributed to the fact that in all the previous studies, T-scores were used for all patients irrespectively of their age, instead of using them only for patients > 50 yr old and Z-scores for those < 50 yr old, based on the criteria for the definition of osteoporosis in males by the International Society of Clinical Densitometry (ISCD). According to ISCD, when Z-scores are <-2 SD, BMD is defined as 'below the expected range for age' and not 'osteoporosis'. In a similar way, a BMD with Zscore >-2 and <-1 SD, in a patient <50 yr old, is considered normal and not 'osteopenia' (2). If T-scores were used, the prevalence of low BMD in our series would have been about 50% and about 74% in those with severe haemophilia (3).Several factors contributed to low BMD in our study: severity of haemophilia, level of physical activity, severity of arthropathy, HCV and HIV exposure and vitamin D deficiency, most of them having been reported previously. However, after multiple regression analysis, only the degree of physical activity and vitamin D deficiency independently predicted low BMD. Exercise, especially weight-bearing activity, at childhood and adolescence is essential for acquisition of adequate peak bone mass and stimulates bone formation (4). Haemophilic arthropathy leads to avoidance of weight-bearing activity, due to the patients' fear of inducing a bleeding (5).The most important finding of our study was that low 25-hydroxy-vitamin D [25(OH)D] levels is an independent predictor of low BMD in hip, which was also reported for the first time in the literature. We found a high prevalence of vitamin D deficiency [defined as 25(OH)D levels < 20 ng/ mL] (in 47% of the patients), despite living in a climate that provides adequate sun exposure. We also found that the level of haemophilic arthropathy and the number of affected joints were significantly related to 25(OH)D levels. Although these findings cannot suggest causality, it can be speculated that vitamin D deficiency contributes to a more severe form of haemophilic arthropathy and we regard measuring...

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Bone health in persons with haemophilia: a review

Cited by 23 publications

References 50 publications

Decreased bone mineral density and associated factors in severe haemophilia A patients: A case‐control study

Decreased bone mineral density and associated factors in severe haemophilia A patients: A case‐control study

Increased fracture rates in people with haemophilia: a 10‐year single institution retrospective analysis

Bone disease in patients with haemophilia. What is true?

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