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Pregnancy and the acute porphyrias
British Journal of Obstetrics and Gynaecology
|October 1, 1977
Summary
Pregnancy in women with acute porphyria, including acute intermittent porphyria, has a better prognosis than previously thought. While some attacks occur, maternal mortality is low, though fetal wastage and smaller birth weights are noted in specific cases.
Area of Science:
- Medicine
- Genetics
- Obstetrics
Background:
- Acute porphyrias are rare genetic disorders affecting heme biosynthesis.
- Pregnancy can potentially exacerbate porphyria symptoms.
- Limited data exists on pregnancy outcomes in women with acute porphyria.
Purpose of the Study:
- To analyze obstetric histories of women with acute porphyria.
- To assess maternal and fetal outcomes during pregnancy and puerperium.
- To compare outcomes with existing literature.
Main Methods:
- Retrospective analysis of obstetric histories.
- Inclusion of 50 women with acute porphyria (39 acute intermittent porphyria, 3 variegate porphyria, 8 hereditary coproporphyria).
- Comparison of outcomes between porphyric pregnancies and non-porphyric controls.
Main Results:
- 54% of acute intermittent porphyria patients experienced attacks during pregnancy/puerperium.
- Low maternal mortality (1 death).
- Total fetal wastage was 13%; babies born to mothers with attacks were smaller (P < 0.001).
Conclusions:
- Pregnancy may have some adverse effects in acute porphyria.
- The prognosis for porphyric pregnancies is significantly better than suggested by prior literature.
- Close monitoring is essential for porphyric pregnancies.