Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys.

Background: Apheresis is the gold standard for idiopathic nephrotic syndrome (INS) relapse after transplantation, but it remains unknown whether such treatment is useful for adults with refractory INS on native kidneys.

Methods: This retrospective study included patients older than 16 years with biopsy-proven refractory (persistent nephrotic syndrome on corticosteroids plus at least 1 immunosuppressive drug) INS treated by apheresis and followed for at least 3 months.

Results: Between September 1997 and January 2020, 21 patients (focal segmental glomerulosclerosis: 12, minimal change nephrotic syndrome: 9, men: 67%, median age: 34 years) were identified.

Advances and unmet needs in genetic, basic and clinical science in Alport syndrome: report from the 2015 International Workshop on Alport Syndrome.

Alport syndrome (AS) is a genetic disease characterized by haematuric glomerulopathy variably associated with hearing loss and anterior lenticonus. It is caused by mutations in the COL4A3, COL4A4 or COL4A5 genes encoding the α3α4α5(IV) collagen heterotrimer. AS is rare, but it accounts for >1% of patients receiving renal replacement therapy.

Endoplasmic reticulum stress in UMOD-related kidney disease: a human pathologic study.

Mutations of the UMOD gene, which encodes the uromodulin protein, are associated with tubulointerstitial nephritis and hyperuricemia. UMOD mutations impair uromodulin folding, resulting in its retention within the endoplasmic reticulum (ER) of renal tubular cells. The aim of this study was to investigate whether mutant uromodulin accumulation in epithelial tubular cells is associated with ER stress.

Peritoneal dialysis in polycystic kidney disease patients. Report from the French peritoneal dialysis registry (RDPLF).

Background: It is commonly believed that polycystic kidney disease (PKD) patients on peritoneal dialysis (PD) are over-exposed to technique failure and peritonitis compared with other patients. This study was carried out to assess whether PKD is associated with technique failure and to evaluate the outcome of PKD patients on PD.

Methods: This was a retrospective cohort study based on the data of the French Language Peritoneal Dialysis Registry.