Bone density in haemophilia: a single institutional cross‐sectional study

Kempton, Christine L.; Antun, Ana G.; Antoniucci, Diana M.; Carpenter, Walter; Ribeiro, Maria J.A.; Stein, Sidney F.; Slovensky, L.; Elon, Lisa

doi:10.1111/hae.12240

Cited by 48 publications

(80 citation statements)

References 34 publications

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“…In a Mediterranean population of 104 haemophilic adults, it was found that low BMD (Z‐score ≤−2 or T‐score ≤−1) was present in 32%, 43% and 20% of patients with severe, moderate and mild disease, respectively . Another study showed that in 61 patients under 50 years, there were 29%, 20% and 8% of patients with low BMD, in severe, moderate and mild cases, respectively, while the percentages of reduced bone mass in the three severity classes in 26 patients over the age of 50 were 44%, 25% and 39%, respectively . In the normal male population, the prevalence of osteoporosis is 3% between 50 and 59 years and 10% in the men over 80 .…”

Section: Discussionmentioning

confidence: 49%

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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Section: Discussionmentioning

confidence: 49%

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

et al. 2018

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“…A significant negative correlation was found between DEXA z‐score and HJHS in children with severe haemophilia A ( R = −.315) and ( P = .026). Similarly, Kempton et al reported that HJHS was significantly correlated to decreased BMD, accounting for 23% of the variation seen in BMD in adults with haemophilia. For each one‐point increase in the radiographic HJHS, there was a decrease in BMD of 0.006 g/cm 2 .…”

Section: Discussionmentioning

confidence: 99%

Effect of low‐dose factor VIII prophylaxis therapy on bone mineral density and 25(OH) vitamin D level in children with severe haemophilia A

et al. 2019

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Background Decreased bone mineral density (BMD) is a significant morbidity in haemophilia. Vitamin D is important for the bone health of people with haemophilia. Regular factor VIII prophylaxis can prevent bleeding and arthropathy. Aim To determine the 25(OH) vitamin D level in severe haemophilia A patients and correlate it to their Hemophilia Joint Health Score (HJHS) and dual‐energy X‐ray absorptiometry (DEXA). We also compared the 25(OH) vitamin D and DEXA in haemophilia A and healthy children and in haemophilia A children on prophylaxis versus on‐demand therapy. Methods Fifty severe haemophilia A patients were compared to 50 age‐matched healthy boys. Patients were recruited from the Pediatric Hematology Clinic, Ain Shams University from May 2017 to April 2018. Full medical history was taken with emphasis on frequency of bleeding episodes, duration and amplitude of pain assessed by the pain score. Weight, height, body mass index and HJHS were assessed. 25(OH) vit‐D3, calcium, phosphorus and alkaline phosphatase were measured. BMD was assessed using Lunar DEXA, paediatric software. Results People with haemophilia had significantly lower 25(OH) vit‐D3 (P < .001) and DEXA z‐score (P < .001) than controls. Seventy per cent of patients were on factor VIII prophylaxis twice weekly (15U/kg/dose). Significant difference was found regarding DEXA z‐score (P = .012), 25(OH) vit‐D3 (P = .033) and HJHS (P = .022) among patients on prophylaxis and on‐demand therapy. Conclusion Severe haemophilia A patients showed significantly lower 25(OH) vit‐D3 and DEXA than controls. Hence, vitamin D deficiency should be tested in all people with haemophilia for early diagnosis and treatment. Low‐dose prophylaxis in severe haemophilia preserves BMD and increases vitamin D. Further studies are required to evaluate the effect of different prophylaxis protocols on BMD and haemophilic arthropathy.

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“…After approval by the Emory University Institutional Review Board, patients with haemophilia A or B aged ≥ 25 years were recruited from the Emory/Children’s Health Care of Atlanta (CHOA) Comprehensive Haemophilia Treatment Center (HTC) to participate in a study of bone density ([20]. Originally, 163 patients were eligible for inclusion based on age and receipt of treatment at the Emory/CHOA HTC.…”

Section: Methodsmentioning

confidence: 99%

The role of disease severity in influencing body mass index in people with haemophilia: a single‐institutional cross‐sectional study

2013

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Summary Aim The aim of this study is to evaluate the effect of haemophilia disease severity and potential intermediaries on body mass index (BMI) in patients with hemophilia. Methods A secondary analysis of a cross-sectional study eighty-eight adults with haemophilia was undertaken. Results On bivariate analysis, persons with severe haemophilia had 9.8% lower BMI (95% CI −17.1, −3.0) than persons with non-severe hemophilia. The effect of haemophilia severity on BMI varied significantly by HIV status. Among HIV positive subjects, haemophilia severity was not associated with BMI (+5.0%, 95% CI −22.4, 41.9). Among HIV negative subjects, severe haemophilia was associated with 15.1% lower BMI (95% CI, −23.6, −5.7). Older (>41 years) HIV negative subjects with severe haemophilia had a BMI that was 24.8% lower (95% CI −39.1, −7.0) than those with non-severe haemophilia. No statistically significant association was detected between BMI and severe versus non-severe haemophilia for younger HIV negative subjects. Although joint disease, as measured by the WFH joint score, did not influence the association between haemophilia disease severity and BMI, adjustment for the atrophy component of the WFH score reduced the association between haemophilia severity and BMI by 39.1–69.9%. This suggested that muscle atrophy mediated at least part of the relationship between haemophilia severity and BMI. Conclusion Haemophilia disease severity is associated with BMI and appears to be mediated by muscle atrophy of surrounding joints. This association appears to be possibly modified by HIV status and age.

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Bone density in haemophilia: a single institutional cross‐sectional study

Cited by 48 publications

References 34 publications

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

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

Effect of low‐dose factor VIII prophylaxis therapy on bone mineral density and 25(OH) vitamin D level in children with severe haemophilia A

The role of disease severity in influencing body mass index in people with haemophilia: a single‐institutional cross‐sectional study

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