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Pregnancy normalized familial hyperaldosteronism type I: a novel role for progesterone?

Journal of Human Hypertension volume 29, pages 138–139 (2015)Cite this article

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Familial hyperaldosteronism type I (FH-I), also known as glucocorticoid remediable aldosteronism, is caused by an unequal crossing‐over of the genes that encode the steroid 11β‐hydroxylase (CYP11B1) and aldosterone synthase (CYP11B2) enzymes. This event results in the generation of a chimeric CYP11B1/CYP11B2 gene with aldosterone synthase activity that is regulated by adrenocorticotropic hormone (ACTH) instead of angiotensin II1 causing hypertension, variable hyperaldosteronism and low plasma renin activity (PRA).

FH-I is a rare disease. There are few published studies describing the management and outcomes of FH-I during pregnancy.2, 3, 4 Owing to ACTH concentration increases during pregnancy5 and aldosterone production in FH-I being under the regulation of ACTH, which differs from traditional adenomatous primary aldosteronism, it is plausible that hypertension is exacerbated in patients with FH-I during pregnancy. However, this disorder does not seem to occur in the majority of reported cases of FH-I and pregnancy. Wyckoff2 reported that only 6% of 35 pregnancies in 16 women positive for the chimeric CYP11B1/CYP11B2 gene showed a worsening of hypertension and Mulatero3 analyzing a five-generation pedigree did not find aggravation of hypertension in 8 FH-I-positive mothers during 29 pregnancies. The hormonal profiles of these women during pregnancy were not analyzed in any of these studies.

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References

  1. Ogishima T, Shibata H, Shimada H, Mitani F, Suzuki H, Saruta T et al. Aldosterone synthase cytochrome P-450 expressed in the adrenals of patients with primary aldosteronism. J Biol Chem 1991; 266: 10731–10734.

    CAS  PubMed  Google Scholar 

  2. Wyckoff JA, Seely EW, Hurwitz S, Anderson BF, Lifton RP, Dluhy RG . Glucocorticoid-remediable aldosteronism and pregnancy. Hypertension 2000; 35: 668–672.

    Article  CAS  Google Scholar 

  3. Mulatero P, di Cella SM, Williams TA, Milan A, Mengozzi G, Chiandussi L et al. Glucocorticoid remediable aldosteronism: low morbidity and mortality in a four-generation Italian pedigree. J Clin Endocrinol Metab 2002; 87: 3187–3191.

    Article  CAS  Google Scholar 

  4. Hamilton E, O'Callaghan C, O'Brien RM, Stowasser M, Gordon R, Zajac J et al. Familial hyperaldosteronism type 1 in pregnancy. Intern Med J 2009; 39: 135–136.

    Article  CAS  Google Scholar 

  5. Goland RS, Conwell IM, Warren WB, Wardlaw SL . Placental corticotropin-releasing hormone and pituitary-adrenal function during pregnancy. Neuroendocrinology 1992; 56: 742–749.

    Article  CAS  Google Scholar 

  6. Quinkler M, Diederich S . Difference of in vivo and in vitro antimineralocorticoid potency of progesterone. Endocr Res 2002; 28: 465–470.

    Article  CAS  Google Scholar 

  7. Vecchiola A, Lagos CF, Fuentes CA, Allende F, Campino C, Valdivia C et al. Different effects of progesterone and estradiol on chimeric and wild type aldosterone synthase in vitro. Reprod Biol Endocrinol 2013; 11: 76–87.

    Article  Google Scholar 

  8. Carvajal CA, Stehr CB, González PA, Riquelme EM, Montero T, Santos MJ et al. A de novo unequal cross-over mutation between CYP11B1 and CYP11B2 genes causes familial hyperaldosteronism type I. J Endocrinol Invest 2011; 34: 140–144.

    Article  CAS  Google Scholar 

  9. Valdes G, Kaufmann P, Corthorn J, Erices R, Brosnihan KB, Joyner-Grantham J . Vasodilator factors in the systemic and local adaptations to pregnancy. Reprod Biol Endocrinol 2009; 7: 79.

    Article  Google Scholar 

  10. Defaye G, Piffeteau A, Delorme C, Marquet A . Specific inhibition of the last steps of aldosterone biosynthesis by 18-vinylprogesterone in bovine adrenocortical cells. J Steroid Biochem Mol Biol 1996; 57: 141–147.

    Article  CAS  Google Scholar 

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Acknowledgements

This work was supported by Chilean grants: FONDECYT 1130427, FONDEF IDeA CA12i10150, Millennium Institute of Immunology, Immunotherapy IMII P09/016-F (ICM), CORFO 13CTI-21526-P1 and Sociedad Chilena de Endocrinología y Diabetes. CA Carvajal is a CONICYT (Comisión Nacional de Investigación Científica y Tecnológica de Chile, www.conicyt.cl) fellow.

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Authors and Affiliations

  1. Departamento Endocrinología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile

    C Campino, P Trejo, C A Carvajal, A Vecchiola, C Valdivia, C A Fuentes, J F Delgado, C F Lagos, C Carrasco & C E Fardella

  2. Instituto Milenio de Inmunología e Inmunoterapia, Santiago, Chile

    C Campino, C A Carvajal, C Valdivia & C E Fardella

  3. División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile

    M Aglony, A Martinez-Aguayo, H García & C Loureiro

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  1. C Campino
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  2. P Trejo
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  3. C A Carvajal
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  4. A Vecchiola
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Corresponding author

Correspondence to C E Fardella.

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Campino, C., Trejo, P., Carvajal, C. et al. Pregnancy normalized familial hyperaldosteronism type I: a novel role for progesterone?. J Hum Hypertens 29, 138–139 (2015). https://doi.org/10.1038/jhh.2014.49

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