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Chapter: Paediatrics: Nephrology

Paediatrics: Bartter’s syndrome

This is a relatively rare form of renal tubular dysfunction.

Bartter’s syndrome

 

This is a relatively rare form of renal tubular dysfunction. The condition is best described as a defect in chloride reabsorption in the ascending loop of Henlé, resulting in:

·  excessive potassium excretion;

 

·  increased prostaglandin synthesis;

 

·  stimulation of the renin–angiotensin–aldosterone system.

 

Clinical features

 

Young children present with:

·  failure to thrive;

 

·  poor growth;

 

·  muscle weakness;

 

·  constipation.

 

Polyuria and polydipsia due to excessive salt and water loss may be evident.

 

Diagnosis

 

Characteristic findings include:

·  hypokalaemia;

 

·  hypochloraemia;

 

·  raised plasma renin and aldosterone levels;

 

·  normal BP.

 

Urine potassium and chloride levels are high.

 

Treatment

 

Goals are to maintain serum potassium levels >3.5mmol/L and to ensure adequate nutrition. Therapy includes a combination of oral potassium sup-plement together with a potassium-sparing diuretic (e.g. spironolactone) and indomethacin (prostaglandin inhibitor).

 

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Paediatrics: Nephrology : Paediatrics: Bartter’s syndrome |


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