GB90.43

ICD-11 MMS 2026-01

Bartter syndrome

Description

Bartter syndrome is a genetic renal tubular disease characterised by the association of hypokalaemic 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), characterised 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.

Index terms

  • Bartter syndrome
  • Bartter syndrome with hypocalcaemia
  • Classic Bartter syndrome
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  • Bartter syndrome type 1
  • Bartter syndrome type 2
  • Bartter syndrome type 3
  • Bartter syndrome type 4
  • Infantile Bartter syndrome with deafness
  • Antenatal Bartter syndrome

Exclusions from above levels

  • http://id.who.int/icd/entity/1746821938 – Urolithiasis
  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • GB90.40 – Hypotonia-cystinuria type 1
  • GB90.41 – Pseudohypoaldosteronism type 1
  • GB90.42 – Fanconi syndrome
  • GB90.44 – Renal tubular acidosis
  • GB90.45 – Renal glycosuria
  • GB90.46 – Tubular disorders of sodium or potassium transport
  • GB90.47 – Aminoaciduria
  • GB90.48 – Disorders of calcium or phosphate excretion
  • GB90.49 – Renal hypocalciuria
  • GB90.4A – Nephrogenic diabetes insipidus
  • GB90.4Y – Other specified renal tubular function disorders
  • GB90.4Z – Renal tubular function disorders, unspecified

Foundation entity

Bartter syndrome – http://id.who.int/icd/entity/777233947

Part of: GB90.4: Renal tubular function disorders › GB90: Certain specified disorders of kidney or ureter › 16: Diseases of the genitourinary system