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Updated: Dec 13 2017

Renal Tubular Acidosis (RTA)

  • Snapshot
    • A 36-year-old woman presents to the emergency department with left-sided back pain that radiates to her left groin. Medical history is significant for Sjogren syndrome. On physical exam there is left-sided costovertebral angle tenderness. Laboratory testing is notable for hyperchloremic and normal anion gap metabolic acidosis and hypokalemia. Non-contrast computerized tomography (CT) of the abdomen demonstrates urolithiasis. She is started on ibuprofen and intravenous fluids. Once the calculi passed it was found to be a calcium phosphate stone. (Type I renal tubular acidosis)
  • Introduction
    • Clinical definition
      • renal tubular dysfunction that results in a hyperchloremic and normal anion gap metabolic acidosis
        • there is a relatively normal glomerular filtration rate
      • Renal Tubular Acidosis
      • Type
      • Etiology
      • Pathogenesis
      • Treatment
      • Comments
      • Type II (proximal) 
      • Fanconi syndrome
      • Carbonic anhydrase II deficiency
      • Medications
        • acetazolamide
        • ifosfamide
        • topiramate
        • expired tetracyclines
      • Heavy metals
        • e.g., mercury, cadmium, copper, and lead
      • Multiple myeloma
      • Wilson disease
      • ↓ bicarbonate reabsorption in the proximal tubule
      • Potassium citrate
      • Thiazide diuretics
      • Associated with hypokalemia
      • At risk of developing hypophosphatemic rickets
      • Urine pH is initially > 5.5 and then later becomes < 5.5
      • Type I (distal)
      • Sjogren syndrome
      • Rheumatoid arthritis
      • Anion exchanger gene mutations
      • Medications
        • lithium
        • ifosfamide
        • analgesics
        • amphotericin B
      • Defective hydrogen secretion
      • Sodium bicarbonate or sodium citrate
      • Associated with hypokalemia
      • At risk of developing calcium phosphate stones
      • Urine pH > 5.5
      • Type IV (hyporeninemic hypoaldosteronism)
      • Diabetic nephropathy
      • Mineralocorticoid deficiency
      • Sickle cell disease
      • Systemic lupus erythematosus
      • Medications
        • heparin
        • angiotensin converting enzyme (ACE) inhibitors
        • angiogensin receptor blockers (ARBs)
        • cyclosporine
        • TMP-SMX
        • potassium sparing diuretics
      • Hyperkalemia results in ↓ ammonia (NH3) synthesis
        • ↓ the capacity to carry acid
      • Treatment depends on the underlying cause
      • Associated with hyperkalemia
      • Urine pH < 5.5
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