Bartter’s syndrome

Bartter’s syndrome is an autosomal recessive condition which results in severe hypokalaemia.

It is caused by defective chloride ion reabsorption in the Na-K-2Cl channels in the loop of Henle

It presents in early childhood with:
  • Failure to thrive 
  • Polyuria 
  • Polydipsia 

Note blood pressure is normal (in contrast to Liddle’s syndrome, in which it is high)

Biochemically there is: 
  • Metabolic alkalosis 
  • Hypokalaemia 
  • High urinary potassium and sodium
  • Hypercalciuria 
  • Hyperreninaemia 

  • Potassium replacement 
  • NSAIDs – usually indomethacin 

Patients who are untreated may have a slightly lowered IQ.

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