II. Pathophysiology

  1. Variant of Type I RTA (Distal RTA) affecting children
  2. Results in severe bicarbonate wasting

III. Signs

  1. See Type I Renal Tubular Acidosis
  2. Acute hypokalemic paralysis
  3. Coma
  4. Shock

IV. Labs

  1. See Type I Renal Tubular Acidosis
  2. Serum Bicarbonate
    1. May precipitously fall to lethal levels (<3 meq/L)

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