Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background: Osteoporosis is a notable cause of morbidity in thalassemia major (TM) patients with a complex pathophysiology and multifactorial etiology. Osteoprotegerin (OPG) and osteoprotegerin ligand (OPGL) have been recently implicated in the pathogenesis of osteoporosis. Aims: To measure the level of OPG and OPGL serum levels in patients with Ý thalassemia and to evaluate the relation between the OPG&OPGL level and the bone mineral density (BMD) in children with Thalassemia Major. Patients and methods: In 60 Egyptian thalassemic patients and 60 healthy control children (age = 5-14 year), serum OPG and serum OPGL measured by ELIZA. The patients had recent results of BMD scanning using dual X-ray absorptiometry (DEXA).Results: High prevalence of bone diseases was detected in TM patients, 40% had spine low bone mass, 10% had femur low bone mass, 70% complained from bone pain and 21.7% had history of fracture of long bone. The thalassemic patients showed significantly lower serum levels of OPG (P=0.003) and lower serum levels of OPGL (P=0.002) OPG/ OPGL ratio (P=0.019) as compared with controls. We couldn{u2019}t demonstrate significant association between serum OPG & serum OPGL levels and BMD.Conclusions: Our data underline the important role of the OPG {u2013} OPGL system in the pathogenesis of osteoporosis in thalassemia major, which could be a potential target for novel therapeutic agents

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