Suvi Väärämäki, Hannu  Uusitalo, Natália  Tőkési, Saku  Pelttari,András  Váradi,Pasi I Nevalainen,
Pyrophosphate Treatment in Pseudoxanthoma Elasticum (PXE)-Preventing ReOcclusion After Surgery for Critical Limb Ischaemia
Surgical Case Reports
2019
2613-5965
http://dx.doi.org/10.31487/j.SCR.2019.04.02
https://www.sciencerepository.org/pyrophosphate-treatment-in-pseudoxanthoma-elasticum-pxe-preventing-re-occlusion-after-surgery-for-critical-limb-ischaemia_SCR-2019-4-102 
Abstract: 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.Keywords: Pseudoxanthoma elasticum, PXE, pyrophosphate, critical limb ischemia