What is the pathophysiology of familial hyperaldosteronism type II (FH-II)?

Updated: Oct 19, 2018
  • Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London); Chief Editor: Robert P Hoffman, MD  more...
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Answer

FH type II (FH-II) is a non–glucocorticoid-suppressible inherited form of hyperaldosteronism that was first recognized as a distinct entity by Gordon et al, though cases had previously been described in the 1980s. Like FH-I, it is inherited in an autosomal dominant manner. In contrast to FH-I, some FH-II kindreds exhibit a high rate of adenoma formation.

The mechanism and gene locus have not yet been identified, though CYP11B and the renin and angiotensin II receptor genes have been excluded. However, linkage has been established for a number of families to band 7p22. [7, 8] It has also been speculated that FH-II is not a single disorder.


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