Distal Renal Tubular Acidosis Distal RTA is the true Nephrogenic RTA and can be truly divided into two variants- the hypokalemic and the hyperkalemic types. Type A intercalated cells - A Low K problem • H secretion defect - Mutations in the luminal H ATPase - AEI mutations (basolateral side) - Carbonic anhydrase II defects - Autoimmune diseases(Sjogren's Syndrome) - Analgesic Nephropathy - Sickle cell disease - Nephrocalcinosis - Drugs( Ifosfamide, topiramate, CA inhibitors) • H gradient defect - Back leak via luminal side via holes (amphotericin B induced) Generalized distal RTA - A high K problem • Voltage defect - ENaC mutation - SCNN mutation - Familial Hypokalemic HTN (WNKI and 4 mutations) - CNI agents - Amiloride and Triamterene use - Pentamidine, Trimethoprim • Low Mineralocorticoid State - Hyporenin Hypoaldosteronism (classic Type IV) - NH3 secretion defects - Drugs (heparin) - DMII - Obstructive Uropathy - ACEI/ARB Dr. Kenar Jhaveri @kdjhaveri #Distal #Renal #Tubular #Acidosis #RTA #diagnosis #differential