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

Abstract

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.

Keywords:

• α-Klotho
• β-thalassemia major (β-TM)
• parathyroid hormone (PTH)
• pseudoxanthoma elasticum-like (PXL)

Acknowledgments

The authors would like to thank Ms. Cinzia Tonioli, Department of Medical Sciences, Section of Pediatrics, University of Ferrara, Ferrara, Italy, for administrative support.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Additional information

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].