Fabio Pellegrino1, Maria Chiara Zatelli2, Marta Bondanelli2, Aldo Carnevale1, Corrado Cittanti3, Monica Fortini4, Maria Rita Gamberini4, Melchiore Giganti1, Maria Rosaria Ambrosio5. 1. Department of Morphology, Surgery and Experimental Medicine, Section of Radiology, University of Ferrara, Ferrara, Italy. 2. Department of Medical Sciences, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy. 3. Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara, Italy. 4. Unit of Thalassaemia and Haemoglobinopathies Day Hospital, Regional HUB Centre, Department of Medicine, Azienda Ospedaliero Universitaria S. Anna, Cona - Ferrara, Italy. 5. Department of Medical Sciences, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy. mbrmrs@unife.it.
Abstract
BACKGROUND: Low mineral mass and reduced bone strength with increased fracture risk are the main causes of morbidity in Thalassemia Major (TM). The pathogenesis is multifactorial and includes ineffective erythropoiesis with medullary expansion, multiple endocrine dysfunctions, direct iron bone deposition, deferoxamine-induced bone dysplasia, and reduced physical activity associated with disease complications. Dual-energy X-ray absorptiometry (DXA) is the "gold standard" for bone mineral density (BMD) assessment and for bone strength and quality evaluation. This method identifies patients at greater risk of fragility fractures, guiding treatment and monitoring response to therapy. In TM, DXA shows limitations concerning BMD calculation accuracy and fracture risk prediction. One of the main challenges in the assessment of bone health in patients with TM is the accurate interpretation of densitometric results. PURPOSE: This review investigates the major pitfalls in DXA implementation and interpretation in TM. METHODS: Available literature has been assessed. CONCLUSIONS: DXA shows limitations in assessing bone mineral "status" in TM, especially in the paediatric population, due to the peculiar characteristics of bone architecture and deformities associated with the disease. A radiological technique adjustment in this population is mandatory.
BACKGROUND: Low mineral mass and reduced bone strength with increased fracture risk are the main causes of morbidity in Thalassemia Major (TM). The pathogenesis is multifactorial and includes ineffective erythropoiesis with medullary expansion, multiple endocrine dysfunctions, direct iron bone deposition, deferoxamine-induced bone dysplasia, and reduced physical activity associated with disease complications. Dual-energy X-ray absorptiometry (DXA) is the "gold standard" for bone mineral density (BMD) assessment and for bone strength and quality evaluation. This method identifies patients at greater risk of fragility fractures, guiding treatment and monitoring response to therapy. In TM, DXA shows limitations concerning BMD calculation accuracy and fracture risk prediction. One of the main challenges in the assessment of bone health in patients with TM is the accurate interpretation of densitometric results. PURPOSE: This review investigates the major pitfalls in DXA implementation and interpretation in TM. METHODS: Available literature has been assessed. CONCLUSIONS: DXA shows limitations in assessing bone mineral "status" in TM, especially in the paediatric population, due to the peculiar characteristics of bone architecture and deformities associated with the disease. A radiological technique adjustment in this population is mandatory.
Authors: John A Shepherd; John T Schousboe; Susan B Broy; Klaus Engelke; William D Leslie Journal: J Clin Densitom Date: 2015 Jul-Sep Impact factor: 2.617
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