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Published in: Pediatric Nephrology 11/2013

01-11-2013 | Original Article

Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome

Authors: Kristen Sgambat, Mindy Banks, Asha Moudgil

Published in: Pediatric Nephrology | Issue 11/2013

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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.
Literature
1.
go back to reference Fine RN (2007) Recurrence of nephrotic syndrome/focal segmental glomerulosclerosis following renal transplantation in children. Pediatr Nephrol 22:496–502PubMedCrossRef Fine RN (2007) Recurrence of nephrotic syndrome/focal segmental glomerulosclerosis following renal transplantation in children. Pediatr Nephrol 22:496–502PubMedCrossRef
2.
go back to reference Greenbaum LA, Benndorf R, Smoyer WE (2012) Childhood nephrotic syndrome–current and future therapies. Nat Rev Nephrol 8:445–458PubMedCrossRef Greenbaum LA, Benndorf R, Smoyer WE (2012) Childhood nephrotic syndrome–current and future therapies. Nat Rev Nephrol 8:445–458PubMedCrossRef
3.
go back to reference Savin VJ, McCarthy ET, Sharma R, Charba D, Sharma M (2008) Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity. Transl Res 151:288–292PubMedCrossRef Savin VJ, McCarthy ET, Sharma R, Charba D, Sharma M (2008) Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity. Transl Res 151:288–292PubMedCrossRef
4.
go back to reference De Smet E, Rioux JP, Ammann H, Deziel C, Querin S (2009) FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapy. Nephrol Dial Transplant 24:2938–2940PubMedCrossRef De Smet E, Rioux JP, Ammann H, Deziel C, Querin S (2009) FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapy. Nephrol Dial Transplant 24:2938–2940PubMedCrossRef
5.
go back to reference Kopac M, Meglic A, Rus RR (2011) Partial remission of resistant nephrotic syndrome after oral galactose therapy. Ther Apher Dial 15:269–272PubMedCrossRef Kopac M, Meglic A, Rus RR (2011) Partial remission of resistant nephrotic syndrome after oral galactose therapy. Ther Apher Dial 15:269–272PubMedCrossRef
6.
go back to reference Savin VJ, Sharma R, Sharma M, McCarthy ET, Swan SK, Ellis E, Lovell H, Warady B, Gunwar S, Chonko AM, Artero M, Vincenti F (1996) Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med 334:878–883PubMedCrossRef Savin VJ, Sharma R, Sharma M, McCarthy ET, Swan SK, Ellis E, Lovell H, Warady B, Gunwar S, Chonko AM, Artero M, Vincenti F (1996) Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med 334:878–883PubMedCrossRef
7.
go back to reference Savin VJ, Sharma R, Lovell HB, Welling DJ (1992) Measurement of albumin reflection coefficient with isolated rat glomeruli. J Am Soc Nephrol 3:1260–1269PubMed Savin VJ, Sharma R, Lovell HB, Welling DJ (1992) Measurement of albumin reflection coefficient with isolated rat glomeruli. J Am Soc Nephrol 3:1260–1269PubMed
8.
go back to reference McCarthy ET, Sharma M, Savin V (2010) Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 5:2115–2121PubMedCrossRef McCarthy ET, Sharma M, Savin V (2010) Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 5:2115–2121PubMedCrossRef
Metadata
Title
Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome
Authors
Kristen Sgambat
Mindy Banks
Asha Moudgil
Publication date
01-11-2013
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 11/2013
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-013-2539-z

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