TY - JOUR
AR - SCR-2019-4-102
TI - Pyrophosphate Treatment in Pseudoxanthoma Elasticum (PXE)-Preventing ReOcclusion After Surgery for Critical Limb Ischaemia
AU - Suvi, Väärämäki
AU -  Hannu , Uusitalo
AU -  Natália , Tőkési
AU -  Saku , Pelttari
AU - András , Váradi
AU - Pasi I, Nevalainen
JO - Surgical Case Reports
PY - 2019
DA - Tue 16, Jul 2019
SN - 2613-5965
DO - http://dx.doi.org/10.31487/j.SCR.2019.04.02
UR - https://www.sciencerepository.org/pyrophosphate-treatment-in-pseudoxanthoma-elasticum-pxe-preventing-re-occlusion-after-surgery-for-critical-limb-ischaemia_SCR-2019-4-102 
KW - Pseudoxanthoma elasticum, PXE, pyrophosphate, critical limb ischemia
AB - 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.