Kidney nontumor
Glomerular disease
Inherited glomerular disease
Bartter syndrome

Topic Completed: 6 August 2012

Minor changes: 26 February 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Bartter's syndrome [title]

Nikhil Sangle, M.D.
Page views in 2019: 229
Page views in 2020 to date: 180
Cite this page: Sangle N. Bartter syndrome. website. Accessed October 20th, 2020.
Definition / general
  • Rare, < 150 cases reported
  • Hypokalemia, metabolic alkalosis, hyperaldosteronism, growth retardation, normal blood pressure but with blunted pressor response to exogenous angiotensin II (eMedicine: Bartter Syndrome)
  • Also polyuria, impairment of concentrating ability, increased renin, angiotensin II and prostaglandins
  • Hyperuricemia and gouty arthritis are common (Saudi J Kidney Dis Transpl 2010;21:1129)
  • Neonatal type: more severe, presents with polyhydramnios due to intrauterine polyuria; also high urinary calcium, nephrocalcinosis, severe failure to thrive and marked growth retardation
  • Classic type: develops during first months or years with failure to thrive, dehydration, growth retardation, at most mild hypercalciuria and no significant nephrocalcinosis
  • Pseudo-Bartter's syndrome: hypokalemic metabolic alkalosis and persistent failure to thrive due to cystic fibrosis (Acta Paediatr 2011;100:e234)
  • Due to primary molecular defect in NaCl reabsorption in thick ascending limb of Henle’s loop
  • Causes increased delivery of NaCl to distal and collecting tubules, which promotes increased potassium and acid secretion in collecting tubules; leads to hypokalemia, metabolic alkalosis
  • Lack of NaCl reabsorption is associated with lack of calcium reabsorption
  • Salt wasting and volume depletion cause renin secretion and increased serum angiotensin II and aldosterone, further stimulating potassium and acid secretion, causing hypokalemia and acidosis
Case reports
Microscopic (histologic) description
  • Hyperplasia of juxtaglomerular apparatus
Electron microscopy description
  • Epithelioid cells associated with afferent and sometimes efferent arterioles, with prominent Golgi complexes and secretory granules and some rhomboidal
  • Also immature glomeruli in children, nephrocalcinosis
Differential diagnosis
  • High dose loop diuretics
  • Conditions causing severe GI potassium chloride loss (have low chloride levels)
  • Cystinosis: Ren Fail 2010;32:277
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