Bartter Syndrome

Bartter Syndrome
Shane M. Meehan, MBBCh
Key Facts
Terminology
  • Normotensive hyper-reninemic hyperaldosteronism with hypokalemic alkalosis and hyperplasia of juxtaglomerular apparatus
Etiology/Pathogenesis
  • 5 types of loss-of-function mutations of genes affecting ion channels are described in Bartter syndrome
Clinical Issues
  • Growth retardation, polyuria, dehydration
Microscopic Pathology
  • Diffuse enlargement of the JGA with renin and AT II expression
  • Hypertrophy and hyperplasia of medullary interstitial cells
Bartter syndrome shows a hyperplastic and hypertrophic juxtaglomerular apparatus image with prominent macula densa image. This reaction to Na loss is the primary histologic feature in Bartter syndrome.
Hyperplastic JGA with increased cytoplasmic volume and granularity image, and prominence of the macula densa image, is shown in an example of Bartter syndrome.
TERMINOLOGY
Definitions
  • Normotensive hyper-reninemic hyperaldosteronism with hypokalemic alkalosis and hyperplasia of juxtaglomerular apparatus
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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Bartter Syndrome

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