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-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.
De Smet
Eric
E
Hôpital du Sacré-Coeur and Université de Montréal, QC, Canada.
Rioux
Jean-Philippe
JP
Ammann
Hélène
H
Déziel
Clément
C
Quérin
Serge
S
D000284:Administration, Oral; D001798:Blood Proteins / Q000378:metabolism*; D003404:Creatinine / Q000097:blood; D005690:Galactose / Q000008:administration & dosage* / Q000627:therapeutic use; D005923:Glomerulosclerosis, Focal Segmental / Q000097:blood / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000097:blood* / Q000188:drug therapy* / Q000209:etiology; D011507:Proteinuria / Q000097:blood / Q000188:drug therapy / Q000209:etiology
D001798:Blood Proteins; D003404:Creatinine; D005690:Galactose