- Variant of Type I RTA (Distal RTA) affecting children
- Results in severe bicarbonate wasting
- See Type I Renal Tubular Acidosis
- Acute hypokalemic paralysis
- Coma
- Shock
- See Type I Renal Tubular Acidosis
- Serum Bicarbonate
- May precipitously fall to lethal levels (<3 meq/L)