Bartter syndrome

Last revised by Daniel J Bell on 25 Feb 2021

Bartter syndrome is a rare inherited renal disorder.

Bartter syndrome is characterized by hyperplasia of the juxtaglomerular cells along with:

  • hypokalemia
  • metabolic alkalosis
  • hypotension/normotension
  • elevated plasma renin
  • elevated aldosterone
  • antenatal polyhydramnios

There are two subtypes of Bartter syndrome: 

  • classic: presents in early childhood with polyuria, polydipsia with a predisposition to dehydration
  • neonatal: presents earlier

First described in 1962 by American endocrinologist Frederic Crosby Bartter (1914-1983) 3.

  • Gitelman syndrome: a closely associated disorder, milder than both subtypes of Bartter syndrome
  • Schwartz-Bartter syndrome: a separate entity characterized by hypertonicity of urine, hyponatremia and inability to excrete water load

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