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Women with acute intermittent porphyria have a defect in 5α-steroid production during the menstrual cycle
(Dept of Clinical Science, Obstetrics and Gynecology, Umea University)
(Dept of Clinical Science, Obstetrics and Gynecology, Umea University)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Obstetrics and Gynaecology. (Reproduktiv hälsa/Sundström Poromaa)
Dept of Public Health and Clinical Medicine, Family Medicine, Umea University.
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2012 (English)In: Acta Obstetricia et Gynecologica Scandinavica, ISSN 0001-6349, E-ISSN 1600-0412, Vol. 91, no 12, 1445-1452 p.Article in journal (Refereed) Published
Abstract [en]

Objective.

To measure serum concentrations of progesterone, estradiol and 5α- and 5β-reduced progesterone metabolites in the follicular and luteal phases of the menstrual cycle in women with latent acute intermittent porphyria and manifest acute intermittent porphyria in comparison with healthy control women.

Design.

A descriptive study with repeated measurements during a complete, ovulatory menstrual cycle.

Setting.

University hospital out-patient clinic.

Population.

Thirty-two women with DNA-diagnosed acute intermittent porphyria and 20 healthy control women.

Methods.

Blood samples for serum progesterone, estradiol, allopregnanolone and pregnanolone were drawn on predefined menstrual cycle days, twice in the follicular phase and three times in the luteal phase. Serum levels of estradiol and progesterone were analysed with commercial kits. Allopregnanolone and pregnanolone levels were analysed with radioimmunoassay following diethylether extraction and celite column chromatography.

Main outcome measures.

Changes in serum levels of progesterone, estradiol, allopregnanolone and pregnanolone throughout the menstrual cycle.

Results.

Women with acute intermittent porphyria displayed lower serum concentrations of allopregnanolone in comparison with healthy control women, the difference being most prominent in the luteal phase (p < 0.001). Levels of pregnanolone did not differ significantly between groups. No significant difference was found between women with latent acute intermittent porphyria and manifest acute intermittent porphyria.

Conclusions.

Decreased levels of the 5α-reduced progesterone metabolite allopregnanolone were found in the menstrual cycle of women with acute intermittent porphyria. This has not been reported previously and could indicate a reduced 5α-reductase type 1 capacity in the ovary and liver among these women.

Place, publisher, year, edition, pages
2012. Vol. 91, no 12, 1445-1452 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-187089DOI: 10.1111/j.1600-0412.2012.01536.xISI: 000312032700015PubMedID: 22924787OAI: oai:DiVA.org:uu-187089DiVA: diva2:573751
Available from: 2012-12-03 Created: 2012-12-03 Last updated: 2017-12-07Bibliographically approved

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