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

Sgambat, Kristen; Banks, Mindy; Moudgil, Asha

doi:10.1007/s00467-013-2539-z

Cited by 58 publications

(43 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We are in agreement with many of the points noted in the letter and have discussed them in our manuscript. The in vitro studies of Savin et al [1], as well as the case reports by Kopac [2] and De Smet [3], were the catalyst for undertaking this investigation, and are reviewed in our manuscript [4]. The authors state that the lack of response to galactose in our study may be due to preexisting permanent irreversible glomerular damage; we agree with this, and have delineated this as a possible explanation in our discussion section.…”

supporting

confidence: 70%

Response: Galactose treatment in focal segmental glomerulosclerosis

Moudgil

Sgambat²

2014

Pediatr Nephrol

Self Cite

View full text Add to dashboard Cite

supporting

confidence: 70%

Response: Galactose treatment in focal segmental glomerulosclerosis

Moudgil

Sgambat²

2014

Pediatr Nephrol

Self Cite

View full text Add to dashboard Cite

“…Small case series suggest limited efficacy of Acthar in some individuals with FSGS (117,118) as has been reviewed (119); a randomized, controlled trial is in progress. Galactose, proposed as therapy for recurrent FSGS, has been shown to lack efficacy in steroid-resistant primary FSGS in children (120). Sirolimus may accelerate progression of FSGS and should be avoided (93).…”

Section: Therapeutic Approachesmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis

Rosenberg

Kopp

2017

CJASN

436

366

View full text Add to dashboard Cite

Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease worldwide. The presumed etiology of primary FSGS is a plasma factor with responsiveness to immunosuppressive therapy and a risk of recurrence after kidney transplant-important disease characteristics. In contrast, adaptive FSGS is associated with excessive nephron workload due to increased body size, reduced nephron capacity, or single glomerular hyperfiltration associated with certain diseases. Additional etiologies arenow recognized asdrivers of FSGS: high-penetrance geneticFSGSdue to mutations in one of nearly 40 genes, virus-associated FSGS, and medication-associated FSGS. Emerging data support the identification of a sixth category: APOL1 risk allele-associated FSGS in individuals with sub-Saharan ancestry. The classification of a particular patient with FSGS relies on integration of findings from clinical history, laboratory testing, kidney biopsy, and in some patients, genetic testing. The kidney biopsy can be helpful, with clues provided by features on light microscopy (e.g., glomerular size, histologic variant of FSGS, microcystic tubular changes, and tubular hypertrophy), immunofluorescence (e.g., to rule out other primary glomerulopathies), and electron microscopy (e.g., extent of podocyte foot process effacement, podocyte microvillous transformation, and tubuloreticular inclusions). A complete assessment of renal histology is important for establishing the parenchymal setting of segmental glomerulosclerosis, distinguishing FSGS associated with one of many other glomerular diseases from the clinical-pathologic syndrome of FSGS. Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. Much progress has been made in our understanding of FSGS, but important outstanding issues remain, including the identity of the plasma factor believed to be responsible for primary FSGS, the value of routine implementation of genetic testing, and the identification of more effective and less toxic therapeutic interventions for FSGS.

show abstract

“…While some case reports have described remission after galactose treatment, a prospective study did not demonstrate any change in proteinuria after 16 weeks of therapy in seven children with steroid-resistant NS [74,75]. A recently completed phase II study (Novel Therapies for Resistant FSGS-FONT II) randomized seven adult and pediatric patients to galactose and found that three achieved a reduction in proteinuria by 50 % at 26 weeks of therapy [76].…”

Section: Emerging Therapiesmentioning

confidence: 97%

Nephrotic Syndrome: Updates and Approaches to Treatment

Rheault

2016

Curr Treat Options Peds

View full text Add to dashboard Cite

Opinion statementNephrotic syndrome (NS) is among the most common pediatric kidney diseases with a high risk of morbidity and mortality due to infection and thrombosis. Goals of treatment are to reduce proteinuria to normal levels thereby reducing symptoms and risk of complications. Children with NS should initially be treated with prednisone or prednisolone at a dose of 60 mg/m 2 /day daily for 6 weeks followed by 40 mg/m 2 /day given every other day for an additional 6 weeks. While most children are steroid responsive, approximately 20 % of children with NS do not go into remission with steroids and should be treated with a calcineurin inhibitor such as cyclosporine or tacrolimus. Some children with NS who respond to steroids eventually have a frequently relapsing or steroid-dependent course and may have significant side effects from cumulative corticosteroid therapy. For these children, steroid-sparing medications are required. Treatment with mycophenolate mofetil is recommended as first-line therapy for treatment of frequently relapsing or steroiddependent NS with steroid toxicity due to its favorable side effect profile compared to alternatives. If this is not effective, alternate agents such as cyclophosphamide, calcineurin inhibitors, or rituximab could be considered after careful review of the pros and cons of each medication with the child's family. Further randomized controlled trials are necessary to determine which agents are most effective and to determine methods to predict medication response in individual children.

show abstract

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

Cited by 58 publications

References 8 publications

Response: Galactose treatment in focal segmental glomerulosclerosis

Response: Galactose treatment in focal segmental glomerulosclerosis

Focal Segmental Glomerulosclerosis

Nephrotic Syndrome: Updates and Approaches to Treatment

Contact Info

Product

Resources

About