Bartter and Gitelman Syndrome

A detailed comparison of Bartter and Gitelman syndrome inheritance patterns, site of defects in the kidney, clinical features, pathophysiology, and treatment. A description of similarities and differences between both conditions.

Similarities between Bartter and Gitelman Syndrome

Treat both conditions as though the patient is on a diuretic medication. In both conditions, this leads to fluid (low blood pressure and diuretic-induced secondary hyperaldosteronism) and electrolyte loss (low potassium, low hydrogen ions and low magnesium).

Table 1. Details of similarities of both syndromes

Effects on aldosterone and reninElevated plasma aldosterone concentration and plasma renin activity
Blood pressureNormal or Low blood pressure
Serum BicarbonateMetabolic Alkalosis
Treatment with spironolactoneYes
Treatment with amilorideYes
Treatment with liberal salt intakeYes
Treatment with potassium and magnesium supplementationYes

Differences between Bartter and Gitelman Syndrome

Table 2. Key Differences between both syndromes

ParameterBartter SyndromeGitelman Syndrome
Inheritance patternAutosomal recessive or dominantAutosomal recessive
PathophysiologyDefective epithelial transport of sodium and chloride in the thick ascending limb of loop of Henle (TALH) due to mutations in pumps, channels or transportersInactivating mutations in thiazide-sensitive sodium chloride co-transporter in the distal convoluted tubule (DCT)
Diuretic SimulationPresents as though patient is on a loop diureticPresents as though the patient is on a thiazide sensitive diuretic
Site of Defect in the nephronTALHDCT
Age of onsetChildhoodAdolescents/Adults
Polyuria and PolydipsiaYesNo
Urinary calcium handlingHypercalciuriaHypocalciuria
Prostaglandin EHighNormal
Responds to NSAIDsYesNo

Bartter Syndrome Mnemonic

This is a simple memory aid or mnemonic to remember this syndrome. BOTH AGONY RESPONDS TO (THICK ASCENDING LOOP OF HENLE), prostaglandin E and REHYDRATION

LetterMemory Aid
BBoth (Autosomal Recessive or Dominant)
AAgony (renal stones and hypercalciuria)
RTResponds To (NSAIDS)
TThick Ascending Loop of Henle
Eprostaglandin E
RRehydration (patients have polyuria and polydipsia which leads to dehydration)

Gitelman Syndrome Mnemonic

Use the number of syllables to determine the site of this defect. Remember the unique features of Bartter syndrome are absent in Gitelman syndrome. Use the features of the former to tease out the unique findings in the latter.

LettersMemory Aid

Visual Summary

A model of the thiazide sensitive Sodium Chloride symporter in the pathogenesis of Gitelman’s syndrome


Images(s) Courtesy

Nine VAM Knoers and Elena N Levtchenko, CC BY 2.0

Post published on April 4, 2022 and Last Updated on March 16, 2023 by MyEndoConsult

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