Report on a case of porphyria in Surinam

ABSTRACT

A male adult Amerindian was after several disgnostic pitfalls recognized as a patient with acute intermittent porphyria. Because of an autosomal congenital transmitted error of metabolism a deficiency of the enzyme uroporphyrinogen I-synthetase (UPG I S) exists resulting in increased levels of delta-aminolevulinic acid (ALA) and porphobilogen(PBG). The Chinese powder containing pyrazolon together with an infectious disease might have been the provocative factors by inducing the enzyme delta-aminolevulinic acid-synthetase(ALA-S). Luminal is leading the long list of drugs to be avoided because of their precitating effect. General practioners, internists as well as surgeons, neurologists, psychiatrists, dermatologists and gynecologists should be aware of the different ways in which the porphyria group of diseases may present. The Watson-Schwartz test is a simple screening method to detect PBG in freshly voided urine when suspecting acute intermittent porphyria. (Au).