Abstract
The notion of hereditary tubulopathy includes various disorders of renal transport of electrolytes. These disorders are summed up under the name Bartter´s or Gitelman´s syndrome (6, 7, 9, 14, 15, 17, and 22).
Both the variants are autosomally recessively hereditary and have similar characteristic clinical signs. Bartter´s syndrome is classified into 3 genetical groups (3, 8. 18): 1st - mutation of the gene coding the renal furosemid-sensitive Na+/K+/2Cl- contransporter (NKCC2, SLC12A1), 2nd ? mutation of the gene coding ATP-sensitive channel K (ROMK, KCNJ),3rd .deletion/ mutation of the gene coding renal chloride channel (CIC-Kb, CLCNKB), also designated as the so-called classical Bartter´s syndrome (17).