As our understanding of the physiology of the aldosterone-sensitive distal nephron (ASDN) advanced in light of novel acquisitions, mainly pertaining the regulation of key ion channels and transporters by with-no-lysine kinases, the pathophysiology of a variety of conditions affecting this segment of the nephron was partly or fully elucidated as well. The pathophysiology of tubulopathies affecting the ASDN or strictly related nephron segments, and disorders causing aldosteronism, pseudoaldosteronism and pseudohypoaldosteronism are here reviewed. The clinical features, with a strong emphasis on pathophysiology, of a variety of disorders are discussed, including: Liddle, Gordon (and calcineurin inhibitor-related hypertension), and Geller syndrome; apparent mineralocorticoid excess; Bartter and Gitelman syndromes; primary aldosteronism, including familial forms; generalized glucocorticoid resistance (Chrousos syndrome). Moreover, the pharmacological translational potential of such novel acquisitions is briefly discussed.
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