Survey of the status of iron and sclerostin in major Thalassemia Patients

Abstract

Background and Aims: Thalassemia is one the most prevalent genetic anemia in the world; homozygote patients usually suffer from severe disturbances. Osteopenia and osteoporosis are of various types of thalassemia complications which are increase in rate in patients with iron overload conditions. Sclerostin is a protein which enhances bone loss by inhibiting osteoblasts. The aim of this study was to measure sclerostin protein and its association with iron overload in major thalassemia patients.
 Materials and Methods: Forty patients with major beta-Thalassemia and 40 healthy control individuals were includedin the study; Sclerostin protein and ferritin were evaluated using ELISA method.
 Results: Mean sclerostin protein was 100.7 pg/ml, in the case group; it was 143.1 pg/ml in the control group.There was a significant differences between sclerostin protein in case and control groups (p= 0.015). The association of sclerostin and ferritin was not significant in the case group (p= 0.7), while it was meaningful in the control individuals (p= 0.037).
 Conclusion: Our findings suggest that sclerostin protein can play an important role in the pathogenesis of osteoporosis.

Associations of serum sclerostin with bone mineral density, markers of bone metabolism and thalassaemia characteristics in adult patients with transfusion-dependent beta-thalassaemia

“…However, our results are not in line with the findings of Voskaridou [8] and Morabito et al [9] who reported a positive relationship in thalassaemia patients with osteoporosis. They are also different from Moinzadeh's study [39], which showed no relation between sclerostin serum levels and bone mineral density in osteoporotic TM patients. We presume that the difference could be due to the fact that in our study the patient group included not only osteoporotic, but also osteopenic and TDßT patients with normal BMD, thus respresenting a wider range of patient characteristics vs. the aforementioned studies which explored the same association in samples that were more homogeneous (restricted range).…”

“…The negative association with pretransfusion haemoglobin was supported by very strong statistical evidence, however the 95%CI showed a rather large range of coefficients between À0.60 and À0.19, cautioning against any hasty conclusions. The uncertainty is coupled by the lack of related research specific to thalassaemia patients (at least to our knowledge), except for that by Moinzadeh et al [39] Yet, our finding can not be compared with the latter study because Moinzadeh et al did not explore the strength and direction of the relation between sclerostin and pretransfusion haemoglobin. The relationship of sclerostin with liver iron overload was significant, however not very strong.…”

“…Voskaridou et al and Morabito et al reported a moderate positive correlation between the levels of sclerostin in serum and BMD in osteoporotic thalassaemia patients [8][9]. Moinzadeh et al found a significant relation between the sclerostin serum levels and pretransfusion haemoglobin in patients with TM [39]. What our study adds to the existing research is a multilayered account of the role of serum sclerostin in transfusion-dependent beta thalassaemia patients, including both TM and TI cases, in relation to a range of thalassaemia-specific parameters and markers of bone metabolism.…”

“…As mentioned earlier, research about serum sclerostin levels in beta-thalassaemia patients is scarce. The few existing studies have involved only osteoporotic thalassaemia cases [8,9,38,39]. Voskaridou et al and Morabito et al reported a moderate positive correlation between the levels of sclerostin in serum and BMD in osteoporotic thalassaemia patients [8][9].…”