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.;
Origin ID : 777233947;
Automatic exact mappings (from CISMeF team)
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See also inter- (CISMeF)
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.