Conflict of interest The authors have stated explicitly that there are no conflicts of interest in connection with this article.
MAIN RESEARCH ARTICLE
Women with acute intermittent porphyria have a defect in 5α-steroid production during the menstrual cycle
Article first published online: 1 NOV 2012
© 2012 The Authors Acta Obstetricia et Gynecologica Scandinavica© 2012 Nordic Federation of Societies of Obstetrics and Gynecology
Acta Obstetricia et Gynecologica Scandinavica
Volume 91, Issue 12, pages 1445–1452, December 2012
How to Cite
INNALA, E., BÄCKSTRÖM, T., POROMAA, I. S., ANDERSSON, C. and BIXO, M. (2012), Women with acute intermittent porphyria have a defect in 5α-steroid production during the menstrual cycle. Acta Obstetricia et Gynecologica Scandinavica, 91: 1445–1452. doi: 10.1111/j.1600-0412.2012.01536.x
Please cite this article as: Innala E, Bäckström T, Sundström Poromaa I, Andersson C, Bixo M. Women with acute intermittent porphyria have a defect in 5α-steroid production during the menstrual cycle. Acta Obstet Gynecol Scand 2012;91: DOI: 10.1111/j.1600-0412.2012.01536.x.
- Issue published online: 5 DEC 2012
- Article first published online: 1 NOV 2012
- Accepted manuscript online: 24 AUG 2012 05:35AM EST
- Received: 16 March 2012 Accepted: 1 August 2012
- Acute intermittent porphyria;
- menstrual cycle;
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.