Intermittent hepatic porphyria in pregnancy with good perinatal outcome

Domagoj Vidosavljević1, Siniša Sijanović, Mirjana Rubin

  • 1Department of Obstetrics and Gynaecology, Vukovar General Hospital, Vukovar, Croatia. d-vidosavljevic@inet.hr

Porphyrias are rare metabolic diseases caused by enzymatic defects of the haeme biosynthesis. Association of pregnancy and acute porphyria is rare, but mortality rate among pregnant women from acute attack has been reported up to 42%. This paper presents a patient with pregnancy complicated by intermittent hepatic porphyria with good perinatal outcome. The pattern of the attack in pregnancy varies individually and it makes porphyric pregnancies a challenge. Previously diagnosed porphyria patients should be closely monitored during pregnancy and diagnosis of acute porphyria must be also considered in all pregnant women with unexplained abdominal pain.

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