Drugs in porphyria: From observation to a modern algorithm-based system for the prediction of porphyrogenicity

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“…Low porphobilinogen deaminase activity can be detected during times of disease remission [13]. The mainstay of AIP management involves the avoidance of suspected precipitants [14]. Well-known precipitants include sulfonamide antibiotics, anti-epileptics, Oral Contraceptive Pills (OCPs), alcohol, decreased caloric intake, stress, and smoking [15].…”

“…Well-known precipitants include sulfonamide antibiotics, anti-epileptics, Oral Contraceptive Pills (OCPs), alcohol, decreased caloric intake, stress, and smoking [15]. In addition to symptomatic management, glucose or intravenous heme (heme arginate in Europe or panhematin in the USA) can be administered to reduce starvation ketosis and ALA synthesis, respectively, in those patients with known or highly suspected cases of AIP [8,9,14].…”

Variegate Porphyria

“…51 Porphyrin production in VP is clearly increased by drugs which increase ALA synthase activity. 135 Porphyrin excretion patterns are highly variable, even within the individual. The extent to which this is due to variability in the biliary and gastrointestinal excretion of porphyrins, or to actual changes in in vivo porphyrin production, has proved difficult to determine and the extent to which intracellular porphyrin production is disturbed in the presence of a PPOX deficiency is unknown.…”

“…A number of articles have extensively reviewed the mechanisms whereby drugs induce an exacerbation of the acute porphyrias. 135,213,214 Principally these are drugs which induce the hepatic cytochrome P450 system. Affinity of a drug for cytochromes CYP 3A4 and 2C9 is strongly associated with 149/Variegate Porphyria the induction of porphyria.…”

Fifty years of porphyria at the University of Cape Town

“…One of us (RJH) collaborates with the Scandinavian authors of this database in a state-of-the-art review of the science of drug porphyrogenicity. 13 …”