Congenital kidney anomalies need a life-course plan, with genetic testing for the right patients

The edition in brief

A review of congenital anomalies of the kidney and urinary tract (CAKUT) notes a birth prevalence of about 3.8 per 1000, a genetic cause in about a fifth, and kidney replacement starting at an average age of about 31, with raised cardiovascular, metabolic and neurocognitive risk; it recommends genetic testing for severe, syndromic, familial or bilateral dysplasia and for unexplained CKD. A review of childhood idiopathic nephrotic syndrome reports that about a quarter of steroid-sensitive patients relapse at least once as adults, predicted by frequent relapses or steroid dependence in childhood, while steroid-resistant and monogenic disease carry three- to fourfold higher kidney failure risk. In a post hoc VANISH analysis of septic shock, high proenkephalin patients given hydrocortisone had the fewest kidney-failure-free days (median 11 vs 26 with placebo), an exploratory finding. In five men with AVPR2-related vasopressin resistance, fluconazole 400 mg daily for 14 days did not reduce urine output or improve concentration.