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

Abstract

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.

Detection and evaluation of haemophilic arthropathy: Which tools may be considered more reliable

“…These results are expected, as bleeds history cannot give information regarding subclinical bleeds and subclinical disease progression; these results are also in line with the literature. 21,22 All measures of diagnostic accuracy were the highest for US among the methods (Table 4) 30,31 Partially, these levels could be attributed to the time of research, which was performed from late fall till spring, which is known to affect the levels of 25-OH vitamin D in the general population. 32 To better evaluate this finding, a study including a control group would have been required.…”

“…Interestingly, 70% of patients in our study group had serum levels of 25‐OH vitamin D below the normal values and another 27% only slightly above the normal. Low levels of vitamin D have been previously described in patients with haemophilia 30,31 . Partially, these levels could be attributed to the time of research, which was performed from late fall till spring, which is known to affect the levels of 25‐OH vitamin D in the general population 32 .…”

Osteoporosis asociada a enfermedades hematológicas

“…8 Es un trastorno genético ligado a la herencia del cromosoma X que afecta las articulaciones que soportan el peso corporal, causando daño del cartílago e hipertrofia sinovial, reduciendo la actividad física en estos pacientes. 8,9 Se ha demostrado que el inicio temprano de terapia profiláctica prolonga la salud articular en estos pacientes. 8 Algunos estudios han demostrado que los pacientes jóvenes que sufren de artropatía hemofílica tienen mayor susceptibilidad de padecer osteoporosis.…”

“…8 Algunos estudios han demostrado que los pacientes jóvenes que sufren de artropatía hemofílica tienen mayor susceptibilidad de padecer osteoporosis. 9 Se ha evidenciado que cerca del 27 % de los pacientes con hemofilia sufren de OP en algún momento de su vida; además, se ha mencionado que el uso de terapia profiláctica en estos pacientes mediante el reemplazo de factores, suplemento con vitamina D y calcio junto con actividad física constante, disminución en el tabaquismo y en consumo de alcohol, disminuyen la aparición de osteoporosis. 8 En el estudio realizado por Ekinci et al, no se encontró relación entre el tabaquismo y la aparición de OP en pacientes con hemofilia A, tampoco fueron relevantes los resultados de los pacientes que tenían el antecedente de consumo de licor.…”

The Bone Microarchitecture Deficit in Patients with Hemophilia Is Influenced by Arthropathy, Hepatitis C Infection, and Physical Activity

“…1 General risk factors such as low body mass index (BMI) and vitamin D deficiency have also been identified as risk factors for low BMD in PWHs. 3,5,6 The gold standard for measuring BMD is dual-energy Xray absorptiometry (DXA), which represents a planar technology measuring areal BMD (aBMD), and most of the studies in PWHs have been performed using this method. High-resolution peripheral quantitative computed tomography (HR-pQCT) is a noninvasive method to evaluate volumetric total BMD (Tt.BMD), trabecular BMD (Tb.BMD), and cortical BMD (Ct.BMD) as well as bone microarchitecture of the peripheral bone at the distal radius and tibia.…”