Treating to Reduce Albuminuria and Normalize Hemodynamic Function in Focal ScLerosis With dApagliflozin...
Focal Segmental GlomerulosclerosisPatients with Focal Segmental Glomerulosclerosis (FSGS) constitute an increasing proportion of the total glomerulonephritis (GN) patient cohort in North America while FSGS is a risk factor for end stage renal failure. Current non-immunological FSGS therapies include the use of angiotensin converting enzyme inhibitors (ACEi) or angiotensin II receptor blockers (ARB), to reduce intraglomerular hypertension. Unfortunately, these agents lead to incomplete renal protection. The aim of the current study is to determine whether the addition of novel sodium glucose cotransport-2 inhibitors (SGLT2i) to standard of care leads to reduced intraglomerular pressure and suppression of proteinuria. We hypothesize that combination therapy of SGLT2i drugs and conventional RAASi results in additive renal protective effects in FSGS patients. A further goal is to examine mechanisms of SGLT2 inhibition by measuring renal hemodynamic function and sodium handling. Kidney function will be assessed in FSGS patients before and after an 8 week treatment with SGLT2i dapagliflozin.
Rituximab in Multirelapsing Minimal Change Disease (MCD) or Focal Segmental Glomerulosclerosis (FSGS)...
Nephrotic SyndromeBackground. Patients, especially children, with steroid-dependent or multirelapsing nephrotic syndrome (NS) secondary to minimal change disease (MCD) or idiopathic focal and segmental glomerulosclerosis (FSGS) on continuous treatment with steroids and/or other immunosuppressive agents to limit or prevent recurrences are at increased risk of severe drug-related adverse events. Case reports suggest that Rituximab, a B cell depleting monoclonal antibody, could be a safe and effective alternative to steroid or immunosuppressants to achieve and maintain remission in this population. Objectives. The study is primarily aimed at evaluating whether Rituximab may maintain stable NS remission after tapering and withdrawal of steroid and immunosuppressive therapy in patients with MCD or FSGS and steroid-dependent or multirelapsing NS. Secondarily, the study will assess whether Rituximab allows reducing maintenance doses of steroids and other immunosuppressants (in those who relapse), thus limiting treatment related side effects and costs. Methods. This prospective, sequential, open, study will include 20 patients with histology evidence of MCD or FSGS and steroid-dependant or multirelapsing NS, who are on stable complete or partial remission since at least 1 month and, based on their previous history, are expected to invariably relapse after steroid/immunosuppression withdrawal. After baseline evaluation of clinical, laboratory and kidney function parameters [including glomerular filtration rate (GFR), renal plasma flow (RPF), albumin and sodium fractional clearance and the glomerular albumin permeability assay (Palb)], patients will receive one Rituximab infusion that will be repeated 1 week later if CD20 cells are not fully depleted from the circulation. Then ongoing immunosuppression will be progressively tapered up to complete withdrawal over 6 to 9 months. 24h proteinuria will be monitored monthly and spot urine will be tested daily by albustix to early detect disease relapses. Baseline evaluations will be repeated at study end (1 year). Relapses will be treated with high-dose steroids as per center practice and the last immunosuppressive therapy effective in preventing disease reactivation will be reintroduced. Expected results. Rituximab is expected to prevent NS recurrence following tapering and discontinuation of steroid and other immunosuppressants. Maintaining remission without chronic immunosuppression is expected to minimize risks and costs of therapy and to remarkably improve patient outcomes.
Safety Study of GC1008 in Patients With Focal Segmental Glomerulosclerosis (FSGS) of Single Doses...
Focal Segmental GlomerulosclerosisThis study will investigate whether GC1008, an antibody which neutralizes TGF-beta, is safe in treating patients with the disease called focal segmental glomerulosclerosis (FSGS). The highest dose without excessive side effects will be investigated. Tests will determine how long GC1008 is in the body and how it is excreted.
Acthar for Treatment-Resistant or Treatment-Intolerant Proteinuria
Idiopathic Focal Segmental GlomerulosclerosisFocal segmental glomerulosclerosis (FSGS) is a condition that harms the kidney "filters" that remove waste from the blood. Proteins are supposed to stay in the blood. Damaged "filters" let protein get into the kidney. FSGS is a serious condition that can lead to kidney failure. The only treatment for kidney failure is dialysis or kidney transplant. Proteinuria means too much protein came through the kidneys into the urine. If the doctor cannot figure out what is causing the problem, it is primary (idiopathic) FSGS. This kind of FSGS is very hard to treat. This study will test Acthar in patients with this condition who have not responded to other treatments. It primarily investigates how well the therapy is tolerated by the patients and how well they respond to this treatment.
Stem Cell Therapy for Patients With Focal Segmental Glomerulosclerosis
Focal Segmental GlomerulosclerosisChronic Kidney DiseasesThe purpose of this study is to analyze the safety, renal function, metabolic disorders and quality of life data in patients with focal segmental glomerulosclerosis treated with endovascular infusion of bone marrow derived mononuclear cells.
Novel Therapies for Resistant FSGS (FONTII): Phase II Clinical Trial
Focal Segmental GlomerulosclerosisThis project will test whether adalimumab,and/or galactose can safely reduce proteinuria (abnormal amounts of protein in the urine) and protect kidney function better than standard treatment for patients with focal segmental glomerulosclerosis (FSGS).
Oral Galactose in Children With Steroid Resistant Nephrotic Syndrome
Focal Segmental GlomerulosclerosisSteroid Resistant Nephrotic SyndromeFocal Segmental Glomerulosclerosis (FSGS) is a devastating kidney disease which is difficult to treat and carries a poor prognosis, with 50% of affected children progressing to end stage renal disease (ESRD). The purpose of this study is to investigate oral galactose as a benign treatment for FSGS in children. The investigators hypothesize that galactose, a simple milk sugar thought to bind to the protein factor (FSPF) that causes FSGS thereby inactivating it and stopping it from damaging the kidney, resulting in a reduction in glomerular permeability to albumin and decrease in proteinuria in children with nephrotic syndrome secondary to FSGS.
Regional Citrate Anticoagulation in Plasma Exchange Treatment
Focal Segmental GlomerulosclerosisGuillain-Barre SyndromeThe investigators want to compare the efficacy of plasma exchange treatment with using two different citrates ( 4% and 15% ) as anticoagulants in plasma exchange treatment. The efficacy of plasma exchange treatment is better with using 15% trisodium citrate as anticoagulant during the plasma exchange procedure.
Mycophenolate Mofetil in Membranous Nephropathy and Focal Segmental
GlomerulonephritisMembranous2 moreThis is a prospective randomized open-label pilot study to investigate the effect of mycophenolate mofetil treatment in patients with abnormal urine protein excretion due to membranous nephropathy (MN) or focal segmental glomerulosclerosis (FSGS). The change in urine protein excretion will be the primary outcome studied. The treatment regimen comprising prednisolone and mycophenolate mofetil will be compared with prednisolone and chlorambucil in MN, and compared with prednisolone in FSGS. The study duration will be 12 months for each patient.
Podocyte Retinoids
Collapsing GlomerulopathyGlomerulosclerosis1 moreThis is a pilot study of retinoids for patients with unsatisfactory response to conventional treatment of nephrotic syndrome due to focal segmental glomerulosclerosis or minimal change disease, two renal disorders associated with putatively pathogenic malfunctioning of glomerular podocytes. The hypothesis that retinoids may have reparative effects on these cells is based on previous research showing that retinoids promote the differentiation or redifferentiation of aberrant epithelial cells. Results obtained by 6 months of treatment with retinoids (that have been approved for non-renal indications) will be used as preliminary information upon which to base further testing of these agents in formal clinical trials in refractory cases of these nephrotic syndromes.