" /> Bartter syndrome - CISMeF





ICD-11 code : GB90.43;

Preferred Label : Bartter syndrome;

ICD-11 definition : Bartter syndrome is a genetic renal tubular disease characterised by the association of hypokalemic alkalosis, increased levels of plasma renin and aldosterone, low blood pressure and vascular resistance to angiotensin II. Two forms of the disease can be distinguished according to clinical criteria: an antenatal or infantile Bartter syndrome (most patients with genotypes I, II and IV), characterized by polyhydramnios, premature delivery, polyuria, dehydration, hypercalciuria and nephrocalcinosis; and classical Bartter syndrome (mostly patients with genotype III, but also some type IV patients), manifesting as polyuria-polydipsia in infancy-childhood through to adulthood, dehydration and a variable delay in the height-weight growth curve.;

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Bartter syndrome is a genetic renal tubular disease characterised by the association of hypokalemic alkalosis, increased levels of plasma renin and aldosterone, low blood pressure and vascular resistance to angiotensin II. Two forms of the disease can be distinguished according to clinical criteria: an antenatal or infantile Bartter syndrome (most patients with genotypes I, II and IV), characterized by polyhydramnios, premature delivery, polyuria, dehydration, hypercalciuria and nephrocalcinosis; and classical Bartter syndrome (mostly patients with genotype III, but also some type IV patients), manifesting as polyuria-polydipsia in infancy-childhood through to adulthood, dehydration and a variable delay in the height-weight growth curve.

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18/06/2025


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