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  with prominent macula densa  . This reaction to Na loss is the primary histologic feature in Bartter syndrome. |
 Hyperplastic JGA with increased cytoplasmic volume and granularity  , and prominence of the macula densa  , is shown in an example of Bartter syndrome. |
TERMINOLOGY
Definitions
Normotensive hyper-reninemic hyperaldosteronism with hypokalemic alkalosis and hyperplasia of juxtaglomerular apparatus
ETIOLOGY/PATHOGENESIS
Autosomal Recessive Inheritance
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