Kidney nontumor
Hereditary renal disease
Bartters syndrome

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 31 October 2017, last major update August 2012

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Bartter's syndrome [title]
Cite this page: Sangle, N. Bartters syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneybartter.html. Accessed November 21st, 2017.
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)
Etiology
  • Due to primary molecular defect in NaCl reabsorption in thick ascending limb of Henleā€™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
Treatment
Microscopic (histologic) description
  • Hyperplasia of juxtaglomerular apparatus
Microscopic (histologic) images

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Neonatal variant

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