• Daphne Vassiliou and Eliane Sardh.
• Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden.
• J. Intern. Med. 2022 Jan 1; 291 (1): 81-94.

BackgroundPregnancy in women with acute hepatic porphyria (AHP) has historically been associated with significant morbidity. Clinical outcomes have been the focus of previous reports on porphyria and maternal health, with little data available on the levels of heme precursors during pregnancy. We present the results of a follow-up program for women with AHP in the Swedish cohort who were pregnant between 2001 and 2020.MethodsThirty-three women with AHP were monitored during 44 pregnancies resulting in 44 single births. Seven of 33 women had a clinical history of acute attacks that required hospitalization.ResultsFour women experienced acute porphyria attacks during pregnancy and one during the puerperium. Seven women developed hypertension and four pregnancies ended with pre-eclampsia. There were no maternal or fetal pre- or postnatal deaths. One infant had a congenital cardiac anomaly. In 32 of the 38 pregnancies in which we measured heme precursors in the urine during pregnancy, the levels increased.ConclusionOur observations align with contemporary reports that pregnancy in patients with AHP is frequently uncomplicated. Excretion of heme precursors increased during pregnancy, but this did not manifest as a higher frequency of clinical porphyria manifestations. The involvement of porphyria specialists in the patients' maternal care is recommended for reducing risk and improving the probability of good pregnancy outcomes.© 2021 The Authors. Journal of Internal Medicine published by John Wiley & Sons Ltd on behalf of Association for Publication of The Journal of Internal Medicine.

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