Background: Beta-thalassemia is a genetic disorder characterized by either a reduced or deficiency of β-globin chain which results in imbalance of α-globin chains and red cell hemolysis. It is a chronic hemolytic anemia that constitutes a global health problem in our country. It presents early in life necessitating blood transfusion from the first year of life with its sequelae of iron overload, oxidative stress and complications of blood transfusion. In the recent years, osteopenia and osteoporosis in thalassaemic patients has attracted much attention because it is a major cause of morbidity despite adequate blood transfusion and iron chelation therapy. Bone mass density is maintained by the equilibrium between bone formation and bone resorption under the control of multiple systemic and local factors such as hormones, Vitamin-D and proinflammatory cytokines. The etiology of osteoporosis in thalassemia is multifactorial and is only partially understood.
Objective: The aim of the present work was to evaluate the level of serum TRAP-5b in patients with β-thalassemia and its relation to iron overload and oxidative stress.
Subjects and methods: The present study included 90 subjects; 60 patients with β- thalassemia major and 30 healthy individuals served as control. Thalassemic patients were then divided into, unsplenectomized patients group (n=30) and splenectomized patients group (n=30). Blood samples were collected from all subjects and the level of serum tartrate resistant acid phosphatase 5b (TRACP-5b) activity was measured by enzyme linked immunossorbent assay (ELISA).
Results: The median values of serum (TRACP-5b) (U/l) in unsplenectomized and splenectomized patients groups were significantly higher than in normal control group, notably the unsplenectomized group reflecting a state of bone resorption. A statistically significant higher serum ferritin was evident in patients versus the control especially in the splenectomized. A significantly higher MDA as a marker of oxidative stress was found in patients compared to the control especially in the splenectomized.
Conclusion: Estimating the serum tartrate resistant acid phosphatase 5b (TRACP-5b) activity could be considered as informative diagnostic and prognostic markers of oxidative stress and bone disease in thalassaemic patients especially in the early stages of bone resorption.
Samia Abd El-Moneim Ebied , Nadia Aly Sadek, Samir Ali Abd El-Kaream* and Hamed Ahmed Elsawy
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