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Bone remodeling in beta thalassemia patients, does it differ between Thalassemia major and intermedia?

Nagham M. S. El-Beblawy, Dalia Nabil Toaima, N Refaat Mohamed

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Abstract

Background: The management of patients with thalassemia has improved markedly over the past few decades with the use of optimized transfusion programs and chelating therapy. With prolongation in life expectancy, it has been observed that this hemoglobinopathy is associated with a variety of bone disorders like deformities, bone pains, growth failure, pathologic fractures, osteopenia, and osteoporosis. High-dose iron chelating therapy may also contribute to osteopenia and osteoporosis. Osteoporosis is a significant cause of morbidity in these patients. It is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. The amino-terminal pro-peptide of type I procollagen (P1NP) is a recently introduced biochemical turnover marker (BTM) that is considered the most sensitive index of bone formation in patients with bone disease of varying origins. We assessed the level of P1NP and bone mineral density as measured by dual X-ray absorptiometry (DEXA) in β-thalassemic pediatric patients for early detection of signs of bone remodeling and assess their correlation to the efficacy of therapeutic interventions (blood transfusion & chelation therapy).

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Background: The management of patients with thalassemia has improved markedly over the past few decades with the use of optimized transfusion programs and chelating therapy. With prolongation in life expectancy, it has been observed that this hemoglobinopathy is associated with a variety of bone disorders like deformities, bone pains, growth failure, pathologic fractures, osteopenia, and osteoporosis. High-dose iron chelating therapy may also contribute to osteopenia and osteoporosis. Osteoporosis is a significant cause of morbidity in these patients. It is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. The amino-terminal pro-peptide of type I procollagen (P1NP) is a recently introduced biochemical turnover marker (BTM) that is considered the most sensitive index of bone formation in patients with bone disease of varying origins. We assessed the level of P1NP and bone mineral density as measured by dual X-ray absorptiometry (DEXA) in β-thalassemic pediatric patients for early detection of signs of bone remodeling and assess their correlation to the efficacy of therapeutic interventions (blood transfusion & chelation therapy).

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Available abstract

Background: The management of patients with thalassemia has improved markedly over the past few decades with the use of optimized transfusion programs and chelating therapy. With prolongation in life expectancy, it has been observed that this hemoglobinopathy is associated with a variety of bone disorders like deformities, bone pains, growth failure, pathologic fractures, osteopenia, and osteoporosis. High-dose iron chelating therapy may also contribute to osteopenia and osteoporosis. Osteoporosis is a significant cause of morbidity in these patients. It is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. The amino-terminal pro-peptide of type I procollagen (P1NP) is a recently introduced biochemical turnover marker (BTM) that is considered the most sensitive index of bone formation in patients with bone disease of varying origins. We assessed the level of P1NP and bone mineral density as measured by dual X-ray absorptiometry (DEXA) in β-thalassemic pediatric patients for early detection of signs of bone remodeling and assess their correlation to the efficacy of therapeutic interventions (blood transfusion & chelation therapy).

Key concepts: Intermedia, Thalassemia, Beta thalassemia, Bone remodeling, Medicine, BETA (programming language), Internal medicine, Computer science

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Bone remodeling in beta thalassemia patients, does it differ between Thalassemia major and intermedia? — Research Paper | ScholarLens