Acute intermittent porphyria: Biochemical and clinical analysis in the Argentinean population

Acute intermittent porphyria (AIP) is the most common type of hepatic acute porphyria. In this work, we have analyzed the biochemical data of all Arge ntinean AIP families studied in the Porphyrins and Porphyrias Research Cent re (CIPYP). We have shown that: (i) the prevalence for this population is a bout 1:125,000; (ii) the disease is more frequent in women than in men (7:3 ); (iii) about 60% are latent carriers; (iv) 15% of patients with symptomat ic AIP died during an acute attack; (v) the most important precipitating fa ctors of acute attacks in our population were the ingestion of therapeutic drugs (25%), anesthetics in surgical interventions (25%) and infections (20 %); (vi) the initial symptom in Argentinean ATP individuals is severe abdom inal pain (100%), and it is often accompanied by constipation (37%), anorex ia (37%) and tachycardia (30%); and (vii) the percentage of recurrence of t he acute attacks is high (81%). (C) 1999 Elsevier Science B.V. All rights r eserved.