Correlation between levels of free and protein-bound plasma porphyrin and urinary porphyrins in porphyria cutanea tarda.
Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism that leads to massive overproduction and excretion of uroporphyrin. Most plasma porphyrins are bound to albumin and hemopexin. The aim of this work was to analyze the relationship between the concentrations of serum albumin and hemopexin, the levels of total, free and protein-bound plasma porphyrins and the urinary coproporphyrin and uroporphyrin excretion, in PCT patients at different stages of the disease. Urinary porphyrins showed a stronger correlation with total plasma porphyrin levels (r = 0.863) than with the free fraction of plasma porphyrins (r = 0.608). Patients considered in an active stage of PCT, have a higher mean level of total plasma porphyrins (8.80 micrograms/dl +/- 8.75) and a lower mean percentage of free plasma porphyrins (12.79% +/- 11.21) than those patients on remission (0.71 +/- 0.5 microgram/dl and 44.3 +/- 35.3%, respectively). 30% of patients showed hypohemopexinaemia, presumably due to hepatic damage. Despite the high affinity of this protein for porphyrins, no significant correlation was found between plasma porphyrin levels and hemopexin or albumin. It is concluded that (i) the kidney is not merely a passive filter for free plasma porphyrins and (ii) that the formation of hemopexin-porphyrin complex occurs when plasma porphyrins concentrations are increased (i.e. in those patients in an active stage of PCT).
Moran
M J
MJ
Porphyria Unit, Hospital Universitario Doce de Octubre, Madrid, Spain.
Fontanellas
A
A
Santos
J L
JL
Enriquez de Salamanca
R
R
D003306:Coproporphyrins / Q000652:urine*; D005260:Female; D006466:Hemopexin / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D008297:Male; D017119:Porphyria Cutanea Tarda / Q000378:metabolism*; D011166:Porphyrins / Q000097:blood* / Q000378:metabolism / Q000652:urine*; D012709:Serum Albumin / Q000737:chemistry / Q000378:metabolism; D018709:Statistics, Nonparametric; D014578:Uroporphyrins / Q000097:blood / Q000378:metabolism / Q000652:urine*
D003306:Coproporphyrins; D011166:Porphyrins; D012709:Serum Albumin; D014578:Uroporphyrins; D006466:Hemopexin