Pyrophosphate is an endogenous, non-toxic metabolite inhibiting soft tissue calcification.
The aim of our study is to find optimal dosing and safety of oral disodium-PPi (Na2H2PPi).
Absorption curves (pharmacokinetics), AUC0-t, Cmax and Tmax for PPi and phosphate will be
provided for healthy controls and PXE-patients both fasting and with standard meal intake.