IMR Press / CEOG / Volume 37 / Issue 4 / pii/1630630917172-744812090

Clinical and Experimental Obstetrics & Gynecology (CEOG) is published by IMR Press from Volume 47 Issue 1 (2020). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with S.O.G.

Review
Acute intermittent porphyria in pregnancy: A common misdiagnosis
Show Less
1 Department of Gynecology, General Hospital of Athens, G. Gennimatas
2 Department of Clinical Therapeutics, Alexandra Hospital, Athens
3 Department of Surgery, General Hospital of Athens, G. Gennimatas
4 Department of Cytology, General Hospital of Athens, Sotiria (Greece)
Clin. Exp. Obstet. Gynecol. 2010, 37(4), 256–260;
Published: 10 December 2010
Abstract

Acute intermittent porphyria (AIP) is inherited in an autosomal dominant fashion. Only 10% to 15% of the gene carriers have the clinical syndrome. The prevalence of AIP in Europe is 1/20,000. Pregnancy represents an essential risk factor in patients suffering from AIP. The clinical syndrome in AIP presents mainly with acute attacks, especially during the first trimester. Misdiagnosis of AIP unfortunately is very common. Pregnancy in women with AIP is associated with higher rates of spontaneous abortion, hypertension, low birth weight infants and considerable mortality (2-42%). Pregnancy, despite the major hormonal alterations it causes, is seldom associated with porphyric symptoms. There are only limited reports supporting the use of hemin during pregnancy, but experience indicates that it can be safely administered in pregnant women. Until clinical improvement is achieved, symptomatic treatment is recommended. Despite the fact that pregnancy in women suffering from AIP is related to higher rates of morbidity and complications, close management throughout the pregnancy could ensure a good outcome.
Keywords
Acute intermittent porphyria
Pregnancy
Hemin
Treatment
Acute attacks
Share
Back to top