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.
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Data di pubblicazione: | 2017 | |
Titolo: | Pseudoxanthoma Elasticum-Like in β-Thalassemia Major, a matter of α-Klotho and Parathyroid Hormone? | |
Autori: | Baldan, Alessandro; Giusti, Andrea; Bosi, Cristina; Malaventura, Cristina; Forni, Gianluca; Borgna-Pignatti, Caterina | |
Rivista: | HEMOGLOBIN | |
Keywords: | parathyroid hormone (PTH); pseudoxanthoma elasticum-like (PXL); α-Klotho; β-thalassemia major (β-TM); Hematology; Genetics (clinical); Clinical Biochemistry; Biochemistry (medical) | |
Abstract in inglese: | 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. | |
Digital Object Identifier (DOI): | 10.1080/03630269.2017.1403927 | |
Handle: | http://hdl.handle.net/11392/2385702 | |
Appare nelle tipologie: | 03.1 Articolo su rivista |