Figure 5.

Development of APA or multinodular cortical hyperplasia from zona glomerulosa. In FH-I the hybrid chimeric gene comprising the CYP11B2 coding sequences under control of the CYP11B1 promoter induces hyperproduction of aldosterone controlled by ACTH. Growth and proliferation of stem cells cause multinodular cortical hyperplasia or multinodular lesions with tumors. In the only FH-I pedigree [see Jeunemaitre et al. (163) and Pascoe et al. (172)], two patients had a tumor but did not lateralize on adrenal venous sampling (AVS), suggesting that these tumors were not APAs. In FH-III the excess production of aldosterone is caused by mutations in the KCNJ5 gene, which are located in or near the selectivity filter. Whether growth and proliferation of stem cells cause development of APAs remains to be investigated. [© 2018 Illustration Presentation ENDOCRINE SOCIETY]