Effects of treatment model on bone metabolism in severe hemophilia patients

Introduction: Osteoporosis is defined as; systemic skeletal disorder that is characterized by low bone mass and deterioration of microarchitecture of bone tissue. Osteoporosis is the commenest metabolic disorder of bone and now is commonest public health issue in all over the world. Unlike women, osteoporosis is less evaluated in men and because of that being less treated. Hemophilia ; is a Xlinked inherited bleeding disorder, that affects male gender. In recently performed studies; osteoporosis has been shown as an important comorbidity factor in patients with hemophilia. Aim: In this study; we aimed to compare biochemical markers of bone and bone mineral density between patients with prophylactic treatment and those without. Therewith ; the effect of prophylactic treatment on bone mineral metabolism and osteoporosis will be evaluated. And besides that, the bone metabolism and bone mineral density of patients with hemophilia will be compared to the healthy population. Proceeding from the results, we aim to ensure more effective therapeutic approaches for hemophilia and long term reduction of social costs. Hypothesis: Bone metabolism of patients with hemophilia is affected by the treatment modality. Bone turnover markers and bone mineral density of the group of profilactic treatment is better than the group of epizodic treatment. Method: A total of 36 patients with hemophilia was evaluated about bone turnover markers and bone mineral density. These paatients are being followed by Ege University Hematology Oncology Department and 24 of them are taking prophylactictreatment while the other 12 are taking epizodic treatment. All the variables were analyzed with SPSS software (Statistical Package fort he Social Sciences, version 20,Chicago,IL,USA). Results: The mean age of patients in the prophylaxis group was found 17.46 ± 6.1 (8-30) and the mean age of the patients in epizodic group was 16 ± 8,6 (9-30). In the assessment of body weight, the weight of the prophylaxis group were found to be significantly higher than other group (p <0.05). Similarly, the body mass index vi (BMI) assessment was found to be significantly higher in the prophylaxis group (p <0.05). In the bone mineral density assessment of prophylaxis group, 5 cases (%20.8) had osteoporotic and 6 cases (%25) had osteopenic Z scores of L1-L4 bone. 13 of the cases (%54.2) were found to have normal bone mineral density of L1-L4. When femoral neck bone mineral density results are discussed, 4 patients (%16.7) found to be osteoporotic and 11 patients (%45.8) found to be osteopenic. Also 9 cases (%37.5) were found to have normal femoral neck bone mineral density scores. In the bone mineral density assessment of epizodic treatment group, considering the L1-L4 Z scores, 3 cases (%25) were found to be osteoporotic and 3 of them (%25) were osteopenic. 6 of the cases (%50) were found to have normal bone mineral density scores in L1-L4. When femoral neck bone mineral density results are discussed, 1 patient (%8.3) found to be osteoporotic and 3 patients (%25) found to be osteopenic. Also 8 cases (%66.7) were found to have normal femoral neck bone mineral density scores. Discussion: Bone mineral density increases during childhood and adolescence, then at twenties the peak bone mass is reached with drawing a plateau. Ther are so many factors that affect peak bone mineral mass. In studies which have done with healthy children and adolescents, normal body weight and weight-related physical activity have been revealed as the most powerful determinants. In our study, when the physical symptoms of the patients were evaluated; body weight was significantly higher in the prophylaxis group compared to the epizodic treatment group. This is also true for the body mass index. But in terms of bone mineral density and vitamin D levels were not significantly different two groups. Considering the body mass index, the avarage body mass index of epizodic treatment group was found 18.4 kg/m2 which is classified as poor. In previous studies, an association between low body mass and increased risk of fracture has been reported. On the other hand, avarage body mass index of prophylaxis group have been found 22.4 kg/m2 and this value is classified to be normal. Hence, the prophylaxis group have higher body mass index, considered as reduced risk of fractures. The comparison of 25(OH)vitamin D3 levels between two groups has no significant difference. Because of the timing may affect the result of vitamin D levels, all the paatients were taken care of within the same climatic conditions. In the vii prophylaxis group only 8 patients (%33.8) and in the epizodic group only 6 patients (%50) have vitamin D levels within the normal limits. When the vitamin D levels are taken "deficient + insufficient", in the prophylaxis group 16 patients (%66.7) and in the epizodic treatment group 6 patients (%50) have the vitamin D levels of deficient or insufficient. Vitamin D is one of the three main hormones that regulate calcium homeostasis. Other hormones are calcitonin and parathormone. Vitamin D has high effectiveness in achieving peak bone mass. In our patient groups , in both groups, the vitamin D levels was found to be deficient or insufficient almost >%50 of the patients. These cases , even the bone mineral density levels are normal, must be followed closely for the risk of osteoporosis and osteopenia development. Key words: Hemophilia, prophylactic treatment, epizodic treatment, osteoporosis