Acute hepatic porphyria and maternal health: Clinical and biochemical follow-up of 44 pregnancies

J Intern Med. 2022 Jan;291(1):81-94. doi: 10.1111/joim.13376. Epub 2021 Sep 23.


Background: Pregnancy 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.

Methods: Thirty-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.

Results: Four 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.

Conclusion: Our 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.

Keywords: acute hepatic porphyria; heme precursors; porphyria and pregnancy.

MeSH terms

  • Female
  • Follow-Up Studies
  • Heme
  • Humans
  • Maternal Health*
  • Porphobilinogen Synthase / deficiency
  • Porphyrias, Hepatic* / epidemiology
  • Pregnancy
  • Pregnancy Outcome*
  • Sweden / epidemiology


  • Heme
  • Porphobilinogen Synthase

Supplementary concepts

  • Porphyria, Acute Hepatic