Acute Porphyria Biomarkers for Disease Activity

Inclusion criteria

• ✓. For AIP: Elevation in urine PBG, with normal or only slight increases in plasma and fecal porphyrins. Most (\~90%) will have deficient activity of erythrocyte PBGD.
• ✓. For HCP: Elevation in urine PBG, with substantial increases in fecal porphyrins (almost entirely coproporphyrin III). In the absence of skin photosensitivity, most will have normal or only slight increases in plasma porphyrins.
• ✓. For VP: Elevation in PBG, with substantial increases in fecal porphyrins (mostly coproporphyrin III and protoporphyrin), increased plasma total porphyrins and a fluorescence emission maximum of diluted plasma at neutral pH near 626 nm.