Unusual manifestation of acute hepatic porphyria in pregnancy.
A 22-year-old para I/gravida II developed psychiatric symptoms at 8 weeks of gestation. Subsequently neurological symptoms with seizures developed leading to a status epilepticus with continuing seizures at week 14. Anticonvulsive therapy had little effect in alleviating the seizures and the condition of the patient rapidly deteriorated. A sudden reddening of her urine lead to the diagnosis of acute hepatic porphyria confirmed by laboratory tests. After extensive discussion with the patient's family it was decided to terminate the pregnancy at week 16. Within hours after pregnancy termination the seizures stopped and the patient recovered without any neurological deficits. Acute hepatic porphyria can be triggered by pregnancy and usually presents with gastrointestinal symptoms and personality changes. In its rare neurological manifestation it can lead to untreatable convulsions which leave no option but to terminate the pregnancy.
Weinzierl
A
A
Department of Gynecology and Obstetrics, University of Innsbruck, Innsbruck, Austria. anneliese.weinzierl@uibk.ac.at
Brezinka
C
C
Engelhardt
K
K
D000028:Abortion, Induced; D000208:Acute Disease; D000328:Adult; D005260:Female; D006801:Humans; D017118:Porphyria, Acute Intermittent / Q000150:complications / Q000175:diagnosis*; D017094:Porphyrias, Hepatic / Q000150:complications / Q000175:diagnosis; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis*; D013226:Status Epilepticus / Q000209:etiology*