GB90.42

ICD-11 MMS 2026-01

Fanconi syndrome

Description

Disorder associated with generalised dysfunction of the proximal tubule expressed as amino-aciduria, low molecular weight proteinuria, polyuria with sodium and potassium wasting, hyper-phosphaturia (hence bone disease), renal tubular acidosis, glycosuria and hypercalciuria.

Index terms

  • Fanconi syndrome
  • De Toni-Debre-Fanconi Syndrome
  • Fanconi renotubular syndrome
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  • Fanconi-de Toni syndrome
  • Lignac-Fanconi syndrome
  • Lowe-Bickel syndrome
  • Renal Fanconi Syndrome
  • congenital Fanconi syndrome
  • Fanconi syndrome associated with familial or genetic disorders elsewhere classified
  • Primary Fanconi Syndrome
  • Dent disease
  • Dent disease type 1
  • Dent disease type 2
  • Acquired Fanconi syndrome associated with causes classified elsewhere

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

Foundation children elsewhere

  • 5C60.0 – Oculocerebrorenal syndrome

Sibling codes

  • GB90.40 – Hypotonia-cystinuria type 1
  • GB90.41 – Pseudohypoaldosteronism type 1
  • GB90.43 – Bartter 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

Fanconi syndrome – http://id.who.int/icd/entity/788002727

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