Acquired monoclonal Ig light chain-associated Fanconi syndrome
ORPHA:91136· ICD-10 E72.0
Definition
A rare monoclonal gammopathy characterized by renal proximal tubule dysfunction secondary to monoclonal kappa light chain deposits in proximal tubular cells. Clinical presentation is with variable chronic kidney disease, low molecular weight proteinuria, aminoaciduria, hyperphosphaturia, uricosuria, bicarbonaturia, and non-diabetic glycosuria. Renal phosphate and urate wasting may cause hypophosphatemia and hypouricaemia.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly