Sustained high plasma 5-aminolaevulinic acid concentration in a volunteer: no porphyric symptoms.
The pathogenesis of the acute porphyric attack is not known. One hypothesis is that porphyrin precursors, especially 5-aminolaevulinic acid (ALA), are toxic for neuronal tissue. This was tested by infusing ALA in a male volunteer after a loading dose at a rate of 50-80 mg h-1 for 92.5 h. During the experiment plasma ALA concentration was 9-11 mumol 1-l and porphobilinogen concentration 3-6 mumol 1-l which are the levels seen during acute attacks. Urinary excretion of these porphyrin precursors was also markedly increased. ALA infusion caused no subjective symptoms and no change in pulse rate, blood pressure, or autonomic nerve function or conduction velocity of peripheral nerves. Photosensitivity was not demonstrable. It is concluded that sustained high plasma ALA concentration does not cause porphyria-like symptoms.
Mustajoki
P
P
Third Department of Medicine, University of Helsinki, Finland.
Timonen
K
K
Gorchein
A
A
Seppäläinen
A M
AM
Matikainen
E
E
Tenhunen
R
R
D000622:Aminolevulinic Acid / Q000097:blood*; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009420:Nervous System / Q000503:physiopathology; D011164:Porphyrias / Q000097:blood* / Q000503:physiopathology; D011166:Porphyrins / Q000378:metabolism
D011166:Porphyrins; D000622:Aminolevulinic Acid