Adult Fanconi Syndrome, Amyloidosis and Marked x-Light Chain Proteinuria

Finkel, P.N.; Kronenberg, Katharina; Pesce, A J; Pollak, Victor E.; Pirani, Conrad L.

doi:10.1159/000180174

Cited by 55 publications

(10 citation statements)

References 15 publications

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“…Kappa light-chain deposits were observed in some tubular epithelial cells and casts and we could assess the kappa type protein for the com position of the fibrils observed in our patient. In the majority of cases reported, deposits were cytoplasmic crystalline inclusions described as rectangular, rhomboid or round surrounded by a smooth membrane [4,5,8] and interpreted as phagolysosomes. In some cases [5,10], crystals presented a regular lattice pattern of a peri odicity of 10.4 nm.…”

Section: Discussionmentioning

confidence: 99%

“…Adult Fanconi's syndrome characterized by glycos uria, aminoaciduria and phosphaturia, which are mani festations of an acquired absorptive defect of proximal renal tubules, is a recognized complication of multiple myeloma [ 1 -7], Pathologic renal changes in cases of Fan coni's syndrome associated with multiple myeloma have been reported to show the presence of cytoplasmic crys tals and round or rod-like electron opaque structures [5,[8][9][10][11]. These structures are probably lysosomal inclu sions composed of altered light chains.…”

Section: Introductionmentioning

confidence: 99%

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Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

Orfila

Lepert²,

Modesto³

et al. 1991

Am J Nephrol

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A 59-year-old woman with kappa light-chain myeloma had Fanconi’s syndrome characterized by renal glycosuria, generalized aminoaciduria, bicarbonaturia and decrease of phosphorus and uric acid reabsorption. A bone marrow biopsy showed the presence of 27% of dystrophic plasma cells; the cytoplasm of these cells was intensely stained with anti-kappa light-chain monoclonal antibodies. By light microscopy, the renal biopsy revealed a tubulointerstitial nephritis without glomerular lesions and with intratubular casts. By immunofluorescence, no deposits were observed along the glomerular and tubular basement membranes, but a positivity with anti-kappa light chain was noticed in some tubular epithelia and casts. By electron microscopy, fibrils (35-nm diameter) were observed in the cytoplasm of proximal tubular cells. These fibrils were situated in vesicles (100- to 600-nm diameter) in the luminal side of tubular cells. In the basal pole of the cell, fibrils seemed to group in crystals (120- to 200-nm diameter). Only kappa light-chain protein was demonstrated in these fibrils and crystals by an immunoelectron microscopic technique. These data suggested the pathogenic role of the fibrils and crystals present in tubular epithelium in the tubular proximal syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

Orfila

Lepert²,

Modesto³

et al. 1991

Am J Nephrol

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“…Many factors contribute to the nephrotoxic potential of these light chains, named Bence Jones proteins (3): degree of polymerization, isotype, isoelectric point and glycosylation. Clinical (4)(5)(6)(7)(8) and experimental (9-12) studies have been conducted to elucidate the mechanisms of this toxicity. Bence Jones proteins are highly glycosylated, differing from the pattern of normal secreted immunoglobulins (13).…”

Section: Introductionmentioning

confidence: 99%

The renal and hepatic distribution of Bence Jones proteins depends on glycosylation: a scintigraphic study in rats

Prado¹,

Nicastri²,

Costa³

et al. 1997

Braz J Med Biol Res

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The aim of the present study was to evaluate renal and liver distribution of two monoclonal immunoglobulin light chains. The chains were purified individually from the urine of patients with multiple myeloma and characterized as lambda light chains with a molecular mass of 28 kDa. They were named BJg (high amount of galactose residues exposed) and BJs (sialic acid residues exposed) on the basis of carbohydrate content. A scintigraphic study was performed on male Wistar rats weighing 250 g for 60 min after iv administration of 1 mg of each protein (7.4 MBq), as the intact proteins and also after carbohydrate oxidation. Images were obtained with a Siemens gamma camera with a high-resolution collimator and processed with a MicroDelta system. Hepatic and renal distribution were established and are reported as percent of injected dose. Liver uptake of BJg was significantly higher than liver uptake of BJs (94.3 vs 81.4%) (P<0.05). This contributed to its greater removal from the intravascular compartment, and consequently lower kidney accumulation of BJg in comparison to BJs (5.7 vs 18.6%) (P<0.05). After carbohydrate oxidation, there was a decrease in hepatic accumulation of both proteins and consequently a higher renal overload. The tissue distribution of periodate-treated BJg was similar to that of native BJs: 82.7 vs 81.4% in the liver and 17.3 vs 18.6% in the kidneys. These observations indicate the important role of sugar residues of Bence Jones proteins for their recognition by specific membrane receptors, which leads to differential tissue accumulation and possible toxicity.

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“…Prominent renal tubular dysfunction has rarely been reported in patients with 'idiopathic' BJ proteinuria [10] or amyloidosis [11], and mainly in patients with multiple myeloma. Adult Fanconi syndrome is rarely found in classical myeloma.…”

Section: Light-chain Proteinuria: From Asymptomatic Forms To Renal Tumentioning

confidence: 99%

Proteinuria in Multiple Myeloma and Related Diseases

Ganeval¹,

Lacour

Chopin

et al. 1990

Am J Nephrol

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Proteinuria is frequently found in multiple myeloma and related disorders. Immunofixation electrophoresis is very helpful for the identification and characterization of the monoclonal component. In multiple myeloma, the presence of Bence-Jones (BJ) proteinuria is significantly associated with renal failure. The coexistence of BJ proteinuria and the nephrotic syndrome in myeloma patients should suggest AL amyloidosis or light chain deposition disease. In the latter, BJ proteinuria is absent in 30% of the cases and diagnosis is based on the demonstration of the monoclonal light chain deposits in tissues, predominantly in kidneys.

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Adult Fanconi Syndrome, Amyloidosis and Marked x-Light Chain Proteinuria

Cited by 55 publications

References 15 publications

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

The renal and hepatic distribution of Bence Jones proteins depends on glycosylation: a scintigraphic study in rats

Proteinuria in Multiple Myeloma and Related Diseases

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Adult Fanconi Syndrome, Amyloidosis and Marked x-Light Chain Proteinuria

Cited by 55 publications

References 15 publications

Fanconi&rsquo;s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

Fanconi&rsquo;s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

The renal and hepatic distribution of Bence Jones proteins depends on glycosylation: a scintigraphic study in rats

Proteinuria in Multiple Myeloma and Related Diseases

Contact Info

Product

Resources

About

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium

Fanconi’s Syndrome, Kappa Light-Chain Myeloma, Non-Amyloid Fibrils and Cytoplasmic Crystals in Renal Tubular Epithelium