title: Partial remission of resistant nephrotic syndrome after oral galactose therapy.
abstract: Focal segmental glomerulosclerosis is sometimes associated with a circulating permeability factor. It was proposed that this factor interacts with the sugars of the glycocalyx, and its high affinity for galactose was shown on the basis of chromatographic studies. Galactose inactivates it and seems to lead to its clearance from plasma. A toddler with a nephrotic syndrome resistant to corticosteroids was admitted. A renal biopsy revealed minimal change disease with deposition of immunoglobulin M. Immunosuppressive therapy with pulses of cyclophosphamide, low-dose combination immunosuppressive therapy, and later with mycophenolate mofetil failed to induce remission. A renal biopsy six years later showed transformation to FSGS. After unsuccessful treatment with monthly pulses of cyclophosphamide, we began therapy with tacrolimus, which showed no effect. After two months, we added oral galactose to tacrolimus for one month, after which proteinuria decreased by 50%. Seven months later, galactose was again added for six months, after which proteinuria remained below 2 g/24 h and the plasma albumin and cholesterol concentrations normalized. An adolescent girl with a nephrotic syndrome resistant to corticosteroids was admitted. A renal biopsy revealed mesangioproliferative glomerulonephritis with C1q nephropathy. Therapy with tacrolimus failed to induce remission. After six months, we added galactose for three months, which reduced proteinuria to 0.76 g/24 h. After the discontinuation of galactose therapy, proteinuria increased to 2.48 g/24 h, despite further treatment with tacrolimus. It seems that oral galactose at a dose of 0.2 g/kg twice a day could be a promising new and nontoxic therapy for the treatment of resistant nephrotic syndrome.
authors.affiliation: Department of Nephrology, Division of Pediatrics, University Medical Centre Ljubljana, Ljubljana, Slovenia. matjaz.kopac@kclj.si
authors.forename: Matjaž
authors.initials: M
authors.lastname: Kopač
authors.forename: Anamarija
authors.initials: A
authors.lastname: Meglič
authors.forename: Rina R
authors.initials: RR
authors.lastname: Rus
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D001706:Biopsy; D002675:Child, Preschool; D004351:Drug Resistance; D005260:Female; D005690:Galactose / Q000008:administration & dosage / Q000627:therapeutic use*; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy* / Q000503:physiopathology; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy* / Q000503:physiopathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D012074:Remission Induction / Q000379:methods
chemical_list: D005938:Glucocorticoids; D005690:Galactose