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Bartter syndrome type 4

Disease definition

A form of Bartter syndrome characterized by maternal polyhydramnios, premature delivery, salt loss, polyuria and sensorineural deafness, associated with hypokalemic and hypochloremic metabolic alkalosis, increased levels of plasma renin and aldosterone, and low to normal blood pressure. Urinary calcium excretion rates are variable, and nephrocalcinosis is typically absent.


Classification level: Subtype of disorder
  • Synonym(s):
    • Bartter syndrome type IV
    • Bartter syndrome with sensorineural deafness
    • Bartter syndrome with sensorineural hearing loss
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Antenatal
  • ICD-10: E26.8
  • ICD-11: GB90.43
  • OMIM: 602522  613090
  • UMLS: -
  • MeSH: -
  • GARD: 10508
  • MedDRA: -

Detailed information

General public


Genetic Testing

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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