1. Acute hepatic porphyria and maternal health: Clinical and biochemical follow‐up of 44 pregnancies
- Author
-
Eliane Sardh and Daphne Vassiliou
- Subjects
Acute hepatic porphyria ,medicine.medical_specialty ,Maternal Health ,Heme ,Urine ,Preeclampsia ,chemistry.chemical_compound ,Pregnancy ,Internal Medicine ,medicine ,Humans ,Sweden ,Fetus ,Obstetrics ,business.industry ,Pregnancy Outcome ,Porphobilinogen Synthase ,medicine.disease ,Porphyrias, Hepatic ,Porphyria ,chemistry ,Cohort ,Female ,business ,Follow-Up Studies - Abstract
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 forty-four pregnancies resulting in forty-four single births. Seven of thirty-three 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 preeclampsia. There were no maternal or fetal pre- or postnatal deaths. One infant had a congenital cardiac anomaly. In thirty-two of the thirty-eight pregnancies in which we measured heme precursors in the urine during pregnancy, the levels were 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. This article is protected by copyright. All rights reserved.
- Published
- 2021