Pseudoxanthoma Elasticum-Like in ß-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 ß-thalassemia major (ß-TM). Histopathological features include abnormal, mineralized and fragmented elastic fibers in skin, eyes and arterial blood vessels (elastorrhexia). The pathogenesis of PXL lesions in ß-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 ß-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.