🧪 Section 569.1 · Proximal (Type II) Renal Tubular Acidosis (pRTA)

Nelson Textbook of Pediatrics 22nd Edition | Defect in proximal tubular bicarbonate reabsorption. Normal anion gap metabolic acidosis, hypokalemia, urine pH <5.5 (acidification intact). Fanconi syndrome (glycosuria, phosphaturia, aminoaciduria, low molecular weight proteinuria). Causes: cystinosis (most common in children), Wilson disease, Lowe syndrome, ifosfamide. Treatment: high-dose bicarbonate (10-20 mEq/kg/day), phosphate, calcitriol.