Набутий синдром Фанконі, асоційований з легким ланцюгом моноклонального Ig
ORPHA:91136· ICD-10 E72.0· Acquired monoclonal Ig light chain-associated Fanconi syndrome
Визначення(English summary)
A rare monoclonalgammopathy 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.
- Поширеність
- <1 / 1 000 000
- Успадкування
- Not applicable
- Вік початку
- Adult, Elderly