Light-chain nephropathy. Renal tubular dysfunction associated with light-chain proteinuria.
We observed idiopathic light-chain proteinuria in a patient with multiple abnormalities of proximal-tubule transport mechanisms (Fanconi syndrome), nephrogenic diabetes insipidus, and distal renal tubular acidosis. Seventeen of the 19 urinary amino acid levels measured were elevated. Uric acid and phosphate clearances were greater than 60 per cent and 50 per cent, respectively, of the simultaneous inulin clearance. When water deprivation was coupled with vasopressin administration, the maximum urinary concentration observed was 384 mOsm per kilogram of water. During ammonium-chloride loading, the level of hydrogen-ion concentration in the urine remained less than 100 times that in the blood. Kappa light-chain excretion was 149 mg per 24 hours. It appears that the concurrence of proximal tubular dysfunction, distal tubular dysfunction and light-chain proteinuria represents a distinct syndrome, which we call "combined light-chain nephropathy." Available evidence indicates that excessive light-chain production with subsequent filtration, reabsorption and catabolism, causes the complex tubular dysfunctions observed.
D000141:Acidosis, Renal Tubular / Q000150:complications / Q000175:diagnosis; D000643:Ammonium Chloride; D001536:Bence Jones Protein / Q000032:analysis; D003919:Diabetes Insipidus / Q000150:complications; D005198:Fanconi Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains / Q000652:urine; D007671:Kidney Concentrating Ability; D007674:Kidney Diseases / Q000209:etiology / Q000276:immunology; D007684:Kidney Tubules; D007686:Kidney Tubules, Distal; D008875:Middle Aged; D011507:Proteinuria / Q000150:complications*; D000608:Renal Aminoacidurias / Q000150:complications; D013577:Syndrome; D014667:Vasopressins
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D000643:Ammonium Chloride; D014667:Vasopressins; D001536:Bence Jones Protein