FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapy

Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF). Galactose has a high affinity for FSPF, and experimental data suggest that it could reduce its activity. We describe the case of a 48-year-...

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Bibliographic Details
Published in:Nephrology, dialysis, transplantation dialysis, transplantation, 2009-09, Vol.24 (9), p.2938-2940
Main Authors: De Smet, Eric, Rioux, Jean-Philippe, Ammann, Hélène, Déziel, Clément, Quérin, Serge
Format: Article
Language:English
Subjects:
Administration, Oral
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Blood Proteins - metabolism
Creatinine - blood
Emergency and intensive care: renal failure. Dialysis management
focal segmental
galactose
Galactose - administration & dosage
Galactose - therapeutic use
Glomerulonephritis
glomerulosclerosis
Glomerulosclerosis, Focal Segmental - blood
Glomerulosclerosis, Focal Segmental - complications
Humans
Intensive care medicine
Male
Medical sciences
Middle Aged
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
nephrotic syndrome
Nephrotic Syndrome - blood
Nephrotic Syndrome - drug therapy
Nephrotic Syndrome - etiology
permeability factor
Proteinuria - blood
Proteinuria - drug therapy
Proteinuria - etiology
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Summary:Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF). Galactose has a high affinity for FSPF, and experimental data suggest that it could reduce its activity. We describe the case of a 48-year-old male with a nephrotic syndrome found to be resistant to corticosteroids, immunosuppression and plasmaphaeresis. The patient was given oral galactose as a last resort treatment, which was followed by a remission of his nephrotic syndrome that correlated with a reduction of FSPF activity. This case is the first report of a long-standing remission of an FSPF-associated nephrotic syndrome on oral galactose therapy.
ISSN:0931-0509
1460-2385
DOI:10.1093/ndt/gfp278