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Gitelman Syndrome Pathophysiology
Gitelman Syndrome is a rare autosomal recessive salt-losing tubulopathy with a prevalence of 1-
Gitelman Syndrome ... Gitelman Syndrome ... H+ secretion → Hypokalemia ... Metabolic alkalosis ... #Syndrome #Pathophysiology
Primary Metabolic Alkalosis - Differential Diagnosis Algorithm
Volume Depleted 2° hyperaldosteronism
 • Low Urine Cl-: Vomiting, NGT
- Differential ... hyperaldosteronism • Low Urine Cl ... NGT suction, Low Cl ... • High Urine Cl ... , Gitelman Syndrome
Gitelman Syndrome Overview

What?
• Inherited (AR) hypokalemic salt-losing tubulopathies affecting the thiazide-sensitive sodium chloride cotransporter
• Gitelman's syndrome
Gitelman Syndrome ... Hypocalciuria (spot ... ratio 0.5%) Differential ... #nephrology #management ... #pathophysiology
Hypokalemia Differential Diagnosis Algorithm
Hypertensive:
 • High Al, Low R - Primary aldosteronism
 • High Al, High
Hypokalemia Differential ... Normotensive: • UCl ... Post-hypercapnia • UCl ... , Gitelman's syndrome ... #Hypokalemia #
Primary Metabolic Alkalosis - Differential Diagnosis Algorithm
Volume Depleted - 2° hyperaldosteronism
 • Low Urine Cl-: Vomiting,
- Differential ... hyperaldosteronism • Low Urine Cl ... NGT suction, Low Cl ... • High Urine Cl ... , Gitelman Syndrome
Metabolic Alkalosis - Differential Diagnosis Algorithm - Urinary Chloride
 • Cl- responsive metabolic alkalosis
Urinary Chloride • Cl ... Kidney loss of Cl ... - Loss of Cl ... Cystic fibrosis) • Cl ... , Gitelman syndrome
Algorithm for patients with hypokalemia on the basis of the potassium-to-creatinine ratio in the urine
Urine K+
patients with hypokalemia ... • Low Urine Cl ... • High Urine Cl ... , Gitelman syndrome ... urine #potassium #differential
Metabolic Alkalosis - Urinary Chloride Algorithm
Cl- responsive metabolic alkalosis
 • Kidney loss of Cl-
Chloride Algorithm Cl ... Kidney loss of Cl ... • Loss of Cl- ... Cystic fibrosis Cl ... , Gitelman syndrome
Gitelman Syndrome - Management
Treatment includes
 - Electrolyte replacement - K & Mg, preferably Cl salts
Gitelman Syndrome ... - Management Treatment ... Mg, preferably Cl ... In persistent hypokalemia ... #Syndrome #Management
Primary Metabolic Alkalosis - Differential Diagnosis Algorithm
Volume Depleted - 2° hyperaldosteronism
 • Low Urine Cl-: Vomiting,
- Differential ... hyperaldosteronism • Low Urine Cl ... NGT suction, Low Cl ... • High Urine Cl ... , Gitelman Syndrome