Objective: We aim to assess the bone mineral density (BMD) and bone biochemical parameters in Iraqi patients with β-thalassemia major (β-TM).Methods: Dual-energy X-ray absorptiometry scan was used to evaluate bone density and interpreted about Z-score which compares to the BMD of age-, sex-, and ethnicity-matched reference population. Biochemical parameters such as calcium, 25-OH Vitamin D, parathyroid hormone, and serum ferritin (SF) evaluated.Results: No statistical difference in SF between pediatrics and adults was determined; however, 66 patients were having their SF between 1000 and 2500 ng/ml and 122 patients with SF ˃2500 ng/ml. Calcium and Vitamin D levels are low in both adults and pediatrics. The bone status shows high percentages of osteoporosis 62% and 54.5% for pediatrics and adults, respectively, as well as osteopenia 27% and 34.3% for both pediatric and adults and to a lesser extent normal bone status 11% for each. Conclusion: Osteopathy has a high prevalence in Iraqi patients with β-TM and should receive an optimal transfusion and chelation therapy to prevent bone expansion. Calcium and Vitamin D should be routinely determined to prevent deficiency.
Beta thalassemia syndrome (β-TM) syndrome is a group of hereditary blood disorders that are mainly characterized by reduction or absence of β-globin chain synthesis. Without iron chelation therapy (ICT) the regular blood transfusion would increase the iron stores to several times. Endocrine glands are vulnerable to iron overload causing endocrine dysfunction. Iron deposition within the parathyroid gland causes hypoparathyroidism particularly after ten years of age. Pancreatic islets are very susceptible to oxidative damage due to iron overload; their high divalent metal expression makes them highly susceptible to iron-catalyzing oxidative stress. The pathogenicity of osteopathy in β-TM is multifactorial comprising environmental (diet and lifestyle), iatrogenic (medicines), genetic and acquired factors (expansion of bone marrow, hemochromatosis, deficiency of growth hormone, hepatitis and hypogonadism). The increase in blood transfusion and RBCs break down in addition to iron accumulation and deposition are the main factors causing splenomegaly. Liver disease is one of the major complications affecting patients with β-TM.Liver damage is multifactorial with iron overload is considered the main causative factor, as well as hepatitis C (HCV) and hepatitis B (HBV) infections which are acquired on recurrent blood transfusions. The free radicals of deposited iron overcome the cellular antioxidant mechanisms resulting in peroxidative cellular injury. As a result, iron overload is the leading cause of left ventricular cardiomyopathy development.
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