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ALL / Endocrinology
Bartter's syndrome
Bartter's syndrome
Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl- cotransporter (NKCC2) in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine causes of hypokalaemia such as Conn's, Cushing's and Liddle's syndrome which are associated with hypertension).Loop diuretics work by inhibiting NKCC2 - think of Bartter's syndrome as like taking large doses of furosemide
Features
- usually presents in childhood, e.g. Failure to thrive
- polyuria, polydipsia
- hypokalaemia
- normotension
- weakness
+PassMedicine Notes
Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl- cotransporter (NKCC2) in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine causes of hypokalaemia such as Conn's, Cushing's and Liddle's syndrome which are associated with hypertension).
Loop diuretics work by inhibiting NKCC2 - think of Bartter's syndrome as like taking large doses of furosemide
Features- usually presents in childhood, e.g. Failure to thrive
- polyuria, polydipsia
- hypokalaemia
- normotension
- weakness