Abstract: Hereditary tubular disorders play an important role in the ion transport mechanism of kidney. Hypokalemic salt-losing tubulopathies (SLTs) are a set of rare hereditary diseases, which accompany with hypokalaemic metabolic alkalosis, normo or hypotension, and are associated with high plasma renin activity and hyperaldosteronemia. Bartter syndrome and Gitelman syndrome, characterizedthe disability of the thick ascending limb of Henles loop and/or the distal convoluted tubule, respectively, are two common types of SLTs. Some types of SLTs share similar clinical manifestations, making them difficult to diagnose. Besides, with the development of molecular genetics, new disease-causing genes have been discovered. Its inconvenient for clinicians to refer to the old classification of SLTs. The review mainly covered the newly discovered pathogenic genes of SLTs and the corresponding pathogenic mechanisms. In addition, a new system for classification of SLTs based on physiology and pharmacology was introduced.

Key words: Bartter syndrome, Gitelman syndrome, salt-losing tubulopathy