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Pseudoxanthoma Elasticum-Like in β-Thalassemia Major, a matter of α-Klotho and Parathyroid Hormone?

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journal contribution
posted on 2017-12-11, 11:30 authored by Alessandro Baldan, Andrea Giusti, Cristina Bosi, Cristina Malaventura, Gianluca Forni, Caterina Borgna-Pignatti

Pseudoxanthoma elasticum-like (PXL) condition is one of the complications faced by patients with β-thalassemia major (β-TM). Histopathological features include abnormal, mineralized and fragmented elastic fibers in skin, eyes and arterial blood vessels (elastorrhexia). The pathogenesis of PXL lesions in β-TM is not yet completely understood. This study was aimed at analyzing a possible implication of α-Klotho in the clinical manifestation of PXL in patients with β-TM (30 with and 78 without PXL). A significant correlation was observed between Klotho, parathyroid hormone (PTH) and serum calcium (Ca). Our analysis seems to indicate α-Klotho and PTH as factors that can affect the development of PXL.

Funding

This study was supported by the Trans2Care Project funded under the Cross-Border Cooperation Programme Italy-Slovenia 2007-2013 by the European Regional Development Fund and national funds [code CB 101].

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