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Bartter, Liddle, Gitelman, Gordon syndrome (Ref. Hari. 18th ed., Ed., Pg - 2361)

  1. Bartter's syndrome and Gitelman’s’ syndrome are characterized by Hypokalemia without hypertension
    1. Renal potassium wasting (Can lead to periodic paralysis)
    2. Metabolic alkalosis
    3. Polyuria.
    4. Blood pressure usually is normal or reduced
    5. Renin and aldosterone levels are very high.
Genetics and Pathogenesis of Bartter’s syndrome and Gitelman’s syndrome
  1. Bartter's syndrome
    1. In Bartter's syndrome, the primary defect lies in one of the epithelial transport proteins involved in the reabsorp-tion of sodium chloride, most commonly the furosemide-sensitive Na + -K+ -2CIcotransporter in the ascending limb of Henle's loop.
    2. There is NaCl wasting, hypercalciuria, and mild hypomagnesemia.
    3. The clinical syndrome mimics a state induced by chronic ingestion of a loop diuretic.
  2. Gitelman’s syndrome
    1. It is due to mutations in the thiazide-sensitive Na-Cl co-transporter (NCCT) in the DCT.
    2. Defects in NCCT in Gitelman’s syndrome impair sodium and chloride reabsorption in the DCT and, thus, resemble the effects of thiazide diuretics.
    3. Hypomagnesemia & Hypocalciuria is a feature.
Rx Bartter’s Syndrome and Gitelman’s syndrome
  1. Both conditions require lifelong therapy with potassium and magnesium supplements and liberal salt intake.
  2. High doses of spironolactone or amiloride are necessary to treat the hypokalemia, alkalosis, and magnesium wasting.
  3. NSAIDs reduce the polyuria and salt wasting in Bartter’s syndrome but are ineffective in Gitelman’s syndrome. They may be lifesaving in hyper prostaglandin E syndrome.
  4. In Gitelman’s syndrome magnesium repletion is essential to correct the hypomagnesemia and control muscle weakness, tetany, and metabolic alkalosis;

Extra Edge


Hyperprostaglandin E syndrome is a particularly severe form of Bartter's syndrome in which neonates present with pronounced volume depletion and failure to thrive, as well as fever, vomiting, and diarrhea from PGE2 overproduction. (Ref. Hari. 18th ed., Pg- 2361)


Hereditary tubular disorders causing hypertension is due to salt retention
  1. Liddle syndrome
  2. Gordon syndrome
  1. Liddle’s syndrome (AIIMS Nov 10)
    1. This disorder is due to unregulated sodium reabsorption by an overactive EnaC in the cortical collecting duct. It is characterized by:
      1. The presence of early and severe hypertension
      2. Hypokalemia and metabolic alkalosis,
      3. Plasma aldosterone and renin levels are low.
    2. Increased potassium and hydrogen ion secretion follow the lumen-negative electrical potential that result from chloride independent sodium reabsorption.
    3. Amiloride or triamterene block ENaC (Amiloride sensitive epithelial Na channel) and, combined with salt restriction, provide effective therapy for hypertension and hypokalemia.
  2. Familial hyperkalemic hypertension (FHHt) (Pseudohypoaldosteronism type II; Gordon syndrome) (AIIMS May 10)
    FHHt is a rare autosomal dominant disease that manifests early adulthood with
    1. Thiazide-responsive, low-renin hypertension
    2. hyperkalemia
    3. Metabolic acidosis
    4. Normal renal function.
Enhanced salt reabsorption in the DCT and impaired distal secretion of potassium and hydrogen ion,
Rx: Thiazide diuretics
K Metabolic BP S. Aldosterone
Bartter Alkalosis N ­
Gitelman Alkalosis N ­
Liddle Alkalosis High
Gordon ­ Acidosis High
Summary of “the Summary” in just two lines !!!
  1. Hyperkalemia, Metabolic acidosis occur in Gordon.
  2. High BP and low level of serum aldosterone occur in Gordon and Liddle

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