Familial Bartter’s Syndrome and the Effect of Indomethacin in One Family Member

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Abstract
A Japanese family in which a father, daughter and son had hypokalemia and hyperreninemia was investigated. Both father and daughter had reduced vascular sensitivity to angiotensin II; the daughter had juxtaglomerular cell hyperplasia and dwarf glomeruli. These features are consistent with Bartter''s syndrome occurring in 2 successive generations in 1 family. The 12 yr old girl had growth retardation in spite of normal growth hormone secretion. No chromosomal abnormalities were found. Indomethacin administration to this patient in doses sufficient to reduce urinary prostaglandin excretion resulted in a marked improvement of polydipsia and polyuria, and an increase in serum Na, K and Cl concentrations. Even though plasma aldosterone concentrations were reduced to within the normal range, serum K concentrations remained low, and plasma renin activity (PRA) remained elevated. Apparently, hypokalemia is not induced only by aldosteronism. Prostaglandins may be the major determinant of polydipsia, polyuria and high plasma aldosterone levels and contribute to the hypokalemia observed in this patient. The failure of complete correction of the hypokalemia and the persistence of the raised PRA with a significant reduction of the prostaglandins suggest the possibility that additional factors are involved in the pathogenesis of Bartter''s syndrome.