Childhood nephrotic syndrome does not always end at puberty: about a quarter relapse as adults

The edition in brief

A Kidney International review of long-term outcomes in childhood idiopathic nephrotic syndrome reports that about half of steroid-sensitive children have frequent relapses or steroid dependence, and around a quarter relapse at least once in adulthood, with childhood frequent relapses and second-line immunosuppression the main predictors. Steroid-resistant disease carries far more complications, and kidney failure is three to four times more likely with monogenic or multidrug-resistant forms. A second review describes congenital anomalies of the kidney and urinary tract as a life-course condition: kidney replacement therapy starts at an average age of about 31, and patients carry raised cardiovascular, metabolic and neurocognitive risks, with genetic testing advised in severe, syndromic, familial or bilateral disease. A commentary explains how an African ancestry-specific GATM variant raises serum creatinine without changing measured kidney function, a source of bias in creatinine-based eGFR. A small open-label study in five men with AVPR2-related congenital vasopressin resistance found fluconazole did not reduce urine output or improve concentrating ability.