Pyrophosphate Treatment in Pseudoxanthoma Elasticum (PXE)-Preventing ReOcclusion After Surgery for Critical Limb Ischaemia

Author Info

Suvi Väärämäki Hannu Uusitalo Natália Tőkési Saku Pelttari András Váradi Pasi I Nevalainen

Corresponding Author

Suvi Väärämäki
Centre for Vascular Surgery and Interventional Radiology, Tampere University Hospital and University of Tampere, Tampere, Finland

A B S T R A C T

Pseudoxanthoma elasticum (PXE) is a rare metabolic disease characterized by reduced plasma pyrophosphate (PPi) concentration, causing progressive soft tissue calcification represented by skin lesions, central vision lost and peripheral artery disease. PXE is currently incurable. Previous reports have shown early high failure after revascularization by unknown mechanism. Reports of oral PPi administration have shown to decrease tissue calcification in a murine model of PXE. We report the outcome of one patient treated with oral PPi and further operated for critical limb ischemia. During the one-year follow-up the operated area has not re-occluded and there have been no significant side effects.

Article Info

Article Type

Case Report

Publication history

Received: Wed 12, Jun 2019
Accepted: Sat 29, Jun 2019
Published: Tue 16, Jul 2019

Copyright

© 2023 Suvi Väärämäki. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository.
DOI: 10.31487/j.SCR.2019.04.02