FaCD Online Syndrome Fact Sheet

Last updated: 19 Mar 2008

Name: Familial Hyperaldosteronism type II

Synonym: FH-II

Mode of Inheritance: AD

Genes

FH-II#, mapped to 7p22

Tumor features

adrenal adenoma

Non-tumor features

adrenal hyperplasia

Comment

Gordon et al.[1] have reported Familial Hyperaldosteronism type 2, in which there is no sensitivity to dexamethasone (see: dexamethasone sensitive hyperaldosteronism) but there is response to posture and angiotensin II infusion. They could detect no clinical or biochemical differences between FH-2 and sporadic primary hyperaldosteronism.
Adrenal hyperplasia (bilateral) as well as adrenal adenomas have been observed in the patients.

References

[1] Gordon RD, Stowasser M. Familial forms broaden the horizons for primary aldosteronism. Trends.Endocrinol.Metab. 9[6], 220-227. 1998.
[2] Jeske YW, So A, Kelemen L, Sukor N, Willys C, Bulmer B, Gordon RD, Duffy D, Stowasser M. Examination of chromosome 7p22 candidate genes RBaK, PMS2 and GNA12 in familial hyperaldosteronism type II. Clinical and experimental pharmacology & physiology 2008; 35(4):380-5.
[3] So A, Duffy DL, Gordon RD, Jeske YW, Lin-Su K, New MI, Stowasser M. Familial hyperaldosteronism type II is linked to the chromosome 7p22 region but also shows predicted heterogeneity. Journal of hypertension 2005; 23(8):1477-84.
[4] Jackson RV, Lafferty A, Torpy DJ, Stratakis C. New genetic insights in familial hyperaldosteronism. Annals of the New York Academy of Sciences 2002; 970():77-88.
[5] Stowasser M, Gunasekera TG, Gordon RD. Familial varieties of primary aldosteronism. Clinical and experimental pharmacology & physiology 2001; 28(12):1087-90.