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Acute intermittent porphyria is a rare inherited disease that is caused by the deficiency of one of the enzymes in the heme synthesis chain. Together with the accelerated metabolism of heme it causes accumulation of neurotoxic precursors. The presentation of acute intermittent porphyria attacks varies as it reflects the impairment of the autonomic, somatic peripheral and central nervous systems. The most common symptoms are abdominal pain, impaired consciousness and neuropathy. Attacks of the disease are caused by several triggering factors, such as infections, stress, starvation, certain medications and drugs and female sex hormones. Due to hormonal changes and other triggering factors, acute intermittent porphyria more frequently deteriorates during pregnancy. Such deteriorations are associated with high maternal and fetal morbidity and mortality. Prognosis can be significantly improved with careful avoidance of triggering factors and early diagnosis and treatment in case of attacks. We present an example of pregnancy management in a patient with known acute intermittent porphyria.