GB90.42ICD-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
Foundation children elsewhere
5C60.0– Oculocerebrorenal syndrome
Sibling codes
GB90.40– Hypotonia-cystinuria type 1GB90.41– Pseudohypoaldosteronism type 1GB90.43– Bartter syndromeGB90.44– Renal tubular acidosisGB90.45– Renal glycosuriaGB90.46– Tubular disorders of sodium or potassium transportGB90.47– AminoaciduriaGB90.48– Disorders of calcium or phosphate excretionGB90.49– Renal hypocalciuriaGB90.4A– Nephrogenic diabetes insipidusGB90.4Y– Other specified renal tubular function disordersGB90.4Z– Renal tubular function disorders, unspecified
Foundation entity
Part of: GB90.4: Renal tubular function disorders › GB90: Certain specified disorders of kidney or ureter › 16: Diseases of the genitourinary system