Acquired monoclonal Ig light chain-associated Fanconi syndrome

Orpha code: 91136OMIM code:

Definition

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.

Disease data
Classification

Disease

Synonyms
Acquired Fanconi syndrome secondary to monoclonal gammopathy
Zespół Fanconiego ze szpiczakiem
Zespół Fanconiego związany z lekkimi łańcuchami kappa
Acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome
ORPHA code
91136
OMIM code
-
ICD10 code
E72.0
ICD11 code
-

No additional description.

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