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 Argentinean AIP families studied in the Porphyrins and Porphyrias Research Centre (CIPYP). We have shown that: (i) the prevalence for this population is a...

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Bibliographic Details
Published in:Clinica chimica acta 1999-10, Vol.288 (1), p.63-71
Main Authors: De Siervi, Adriana, Rossetti, Marı́a V, Parera, Victoria E, Mendez, Manuel, Varela, Laura S, del C. Batlle, Alcira M
Format: Article
Language:English
Subjects:
Acute intermittent porphyria
Adult
Argentina - epidemiology
Argentinean population
Biochemical analysis
Biological and medical sciences
Female
Humans
Male
Medical sciences
Metabolic diseases
Middle Aged
Other metabolic disorders
Pigments (porphyrias, hyperbilirubinemias...)
Porphyria, Acute Intermittent - epidemiology
Porphyria, Acute Intermittent - metabolism
Porphyria, Acute Intermittent - mortality
Porphyria, Acute Intermittent - pathology
Porphyrinogenic agents
Prevalence
Sex Factors
Tropical medicine
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Summary:Acute intermittent porphyria (AIP) is the most common type of hepatic acute porphyria. In this work, we have analyzed the biochemical data of all Argentinean AIP families studied in the Porphyrins and Porphyrias Research Centre (CIPYP). We have shown that: (i) the prevalence for this population is about 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 symptomatic AIP died during an acute attack; (v) the most important precipitating factors 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 AIP individuals is severe abdominal pain (100%), and it is often accompanied by constipation (37%), anorexia (37%) and tachycardia (30%); and (vii) the percentage of recurrence of the acute attacks is high (81%).
ISSN:0009-8981
1873-3492
DOI:10.1016/S0009-8981(99)00139-4