Porphobilinogen and porphyrin synthesis in reticulocytes from uraemic patients.

Since no information exists concerning porphyrin metabolism in uraemic patients we have measured the activities of delta-aminolaevulinic acid dehydrase (D-ALA-D) and porphobilinogen desaminase (PBG-D) in reticulocytes from uraemic patients, anaemic patients without uraemia and in healthy subjects. Despite a severe anaemia uraemic patients had the same amount of reticulocytes/mul blood. In uraemic patients D-ALA-D activity was reduced to about 20% compared to healthy subjects, PBG-D in uraemia was decreased to about 70%. It is concluded that porphyrin metabolism is altered in uraemic patients.