Evaluation of bone mineral density in children with hemophilia: Mansoura University children hospital (MUCH) experience, Mansoura, Egypt

Abdelrazik, N.; Reda, M.; El-Ziny, M.; Rabea, Hala

doi:10.1080/10245330701383700

Cited by 38 publications

(43 citation statements)

References 13 publications

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“…Decreased BMD has been previously demonstrated in children with haemophilia, but only using measures of areal BMD assessed by DXA (mean Z‐scores for lumbar spine aBMD −1.6 [4], −0.9 [2] and −0.7 [19]). This areal BMD is a general indicator of the bone mass, while information is lacking about the bone structure and geometry.…”

Section: Discussionmentioning

confidence: 99%

Boys with haemophilia have low trabecular bone mineral density and sarcopenia, but normal bone strength at the radius

et al. 2011

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Although a decreased areal bone mineral density (BMD) has been reported in patients with haemophilia, data are lacking that would reflect the three-dimensional structure of the bone and the muscle-bone relationship. We aimed to assess volumetric BMD, bone geometry and muscle-bone phenotype in boys with haemophilia, and to describe the association between clinical characteristics of haemophilia and bone quality and structure. A cross-sectional study was conducted in 41 boys with haemophilia (mean age 12.4, range 6.6-19.8 years) using peripheral quantitative CT (pQCT) at the nondominant forearm. Results were transformed into Z-scores using previously published reference data. Significant differences were tested by one-sample t-test or sign test. Two-sample t-test and anova were used to compare results between subgroups of patients divided according to the severity of the disease, the fracture history and the number of joint and muscle bleedings. Boys with haemophilia had a decreased trabecular volumetric BMD (mean Z-score -0.5, P < 0.01), while their cortical volumetric BMD was increased (mean Z-score 0.4, P < 0.05). The volumetric bone mineral content and the bone geometry at the radial diaphysis were normal when adjusted for patients' shorter body height. Muscle area was decreased (mean Z-score -1.0, P < 0.001), irrespective of age. No association was observed of bone quality parameters and bone geometry with the disease severity, fracture history or number of bleedings. Bone strength measured at the diaphysis of the radius is not impaired in boys with haemophilia. The finding of the decreased trabecular bone density can be most likely attributed to their sarcopenia.

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Section: Discussionmentioning

confidence: 99%

Boys with haemophilia have low trabecular bone mineral density and sarcopenia, but normal bone strength at the radius

et al. 2011

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“…Patients with haemophilia seem to achieve lower peak bone mineral mass when compared to healthy controls. In favour of this hypothesis, studies showed reduced bone mineral density among children with severe haemophilia (Barnes, 2004, Abdelrazik , 2007., Nair, 2007., Tlacuilo-Parra, 2008. The major impact on bone formation during childhood and adolescents has physical activity and, moreover, weight-bearing exercises.…”

Section: Physical Activitymentioning

confidence: 96%

“…The strong relationship has been established between reduced bone mass and joint status in young patients with haemophilia (Abdelrazik, 2007), as well as among adult haemophiliacs (Nair, 2007., Katsarou, 2009. Joint status was evaluated using clinical score as described by the Orthtopedic Advisory Council of the World Federation of Haemophilia (RodriguezMerchan EC, 2003.…”

Section: Joint Status and Prophylactic Therapymentioning

confidence: 99%

Characteristics of Older Patient with Haemophilia

Šalek¹,

Boban²,

Pulanić³

2012

Hemophilia

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“…Joint disease reduces physical fitness and muscular strength, creates muscle atrophy, and in a vicious cycle exacerbates articular instability and repeated bleeding episodes. Children with hemophilia may have reduced bone mineral density compared to age- and gender-matched controls 27. Hemarthroses begin in the toddler age.…”

Section: Prophylaxis In Nonrandomized Studiesmentioning

confidence: 99%

“…Children with severe hemophilia may have reduced bone mineral density (BMD) compared with age-matched controls,27 but prophylaxis starting in early childhood may preserve normal BMD and reduce the risk of degenerative bone disease, pathologic fractures, and the financial burden that such problems impose 80…”

Section: Consideration Of Cost In Hemophilia Prophylaxismentioning

confidence: 99%

Review of antihemophilic factor injection for the routine prophylaxis of bleeding episodes and risk of joint damage in severe hemophilia A

Rossbach¹

2010

VHRM

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Individuals with severe factor VIII deficiency experience recurrent hemorrhages and develop progressive joint damage. Large retrospective, nonrandomized studies of patient cohorts followed over decades show that factor prophylaxis initiated at an early age before the onset of recurrent bleeding reduces the incidence of hemophilic arthropathy. Two recent prospective, multicenter, randomized trials conducted in Europe (the ESPRIT study) and the USA (the Joint Outcome Study) confirm the efficacy of prophylaxis in the prevention of hemarthroses and arthropathy. Regular prophylaxis initiated in early childhood enhances the quality of life for patients with severe hemophilia and reduces the risk of inhibitor development. The substantial costs of such preventative therapy may be offset by the reduced expenditures that the care of degenerative joint disease in adult hemophilia patients would otherwise require.

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Evaluation of bone mineral density in children with hemophilia: Mansoura University children hospital (MUCH) experience, Mansoura, Egypt

Abstract: (1) Early detection of osteopenic hemophilic children using DEXA scanning, (2) bisphosphonates plus calcium for hemophilic children with reduced BMD, (3) evaluation of the effect of on demand vs. prophylaxis replacement therapy in hemophilic patients on BMD and hemophilic arthropathy.

Cited by 38 publications

References 13 publications

Boys with haemophilia have low trabecular bone mineral density and sarcopenia, but normal bone strength at the radius

Boys with haemophilia have low trabecular bone mineral density and sarcopenia, but normal bone strength at the radius

Characteristics of Older Patient with Haemophilia

Review of antihemophilic factor injection for the routine prophylaxis of bleeding episodes and risk of joint damage in severe hemophilia A

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