title: Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome.
abstract: BACKGROUND
  Idiopathic steroid-resistant nephrotic syndrome (SRNS) has been associated with the presence of a circulating focal sclerosis permeability factor (FSPF) thought to damage the glomerular barrier and increase permeability to albumin. Galactose binds and inactivates FSPF in vitro, but its effect in vivo is uncertain.
  
  
  METHODS
  A prospective clinical trial was conducted to investigate the effect of oral galactose on FSPF and proteinuria in children with SRNS. Seven pediatric subjects with idiopathic SRNS and positive FSPF activity (>0.5) were treated with oral galactose (0.2 gm/kg/dose twice daily) for 16 weeks. Post-treatment FSPF and proteinuria were measured.
  
  
  RESULTS
  Focal sclerosis permeability factor activity of the seven subjects decreased from 0.69 ± 0.11 to 0.35 ± 0.21 (p = 0.009) in response to galactose. The two subjects with post-transplant recurrence of focal segmental glomerulosclerosis (FSGS) demonstrated the most significant improvement in FSPF (p = 0.006). Despite this decrease in FSPF, the pre- and post-treatment urine protein:creatinine ratio remained unchanged and no subject achieved remission.
  
  
  CONCLUSIONS
  Galactose decreases FSPF in children with SRNS, with the most significant improvement in those with post-transplant FSGS recurrence, but it fails to improve proteinuria. At the present time there is no evidence to support the use of galactose in children with FSGS, either pre- or post-transplant. Future studies to investigate the role of galactose as preemptive therapy to decrease the risk of post-transplant FSGS recurrence may be useful.
authors.affiliation: Department of Nephrology, Children's National Medical Center, 111 Michigan Avenue NW, Washington DC, 20010, USA.
authors.forename: Kristen
authors.initials: K
authors.lastname: Sgambat
authors.forename: Mindy
authors.initials: M
authors.lastname: Banks
authors.forename: Asha
authors.initials: A
authors.lastname: Moudgil
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D005260:Female; D005690:Galactose / Q000627:therapeutic use*; D005919:Glomerular Filtration Rate; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy; D006801:Humans; D007678:Kidney Glomerulus / Q000187:drug effects*; D016030:Kidney Transplantation; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000188:drug therapy; D010539:Permeability; D010865:Pilot Projects; D011446:Prospective Studies; D011507:Proteinuria / Q000188:drug therapy* / Q000652:urine; D055815:Young Adult
chemical_list: D003404:Creatinine; D005690:Galactose