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Неамілоїдна хвороба відкладення моноклональних імуноглобулінів

ORPHA:86861· ICD-10 D89.8· Non-amyloid monoclonal immunoglobulin deposition disease

Визначення(English summary)

A rare, secondary glomerular disease characterized by proteinuria, dysproteinemias, nephrotic syndrome, and nodular glomerulopathy leading to renal failure, with or without extra-renal manifestations. The renal biopsy shows typical deposits of monoclonal immunoglobulins that do not show a fibrillar organization and are negative for Congo red staining. Associated signs and symptoms depend on the involvement of other organs, liver, heart, nerve fibers, gastrointestinal tract, or skin.

Вік початку
Adult