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Type 1 Renal Tubular Acidosis
Aka: Type 1 Renal Tubular Acidosis, Type I Renal Tubular Acidosis, Type I RTA, Distal RTA, Distal Renal Tubular Acidosis, Classic RTA
- See Also
- Renal Tubular Acidosis
- Pathophysiology
- Distal Tubule fails to excrete ammonium
- Causes
- Autosomal Dominant inherited disorder
- Acquired causes
- Systemic Lupus Erythematosus
- Sickle Cell Anemia
- Nephrocalcinosis related disorders
- Hyperparathyroidism
- Medullary sponge Kidney
- Medications and Toxins
- Amphotericin B
- Lithium
- Toluene
- Signs
- Musculoskeletal weakness
- Recurrent Nephrolithiasis
- Labs
- Arterial Blood Gas (ABG)
- Non-Anion Gap Metabolic Acidosis
- Urine pH
- Elevated >5.5 despite Metabolic Acidosis
- Serum Potassium
- Low or normal
- Fractional Excretion of Bicarbonate
- FE-HCO3 <5%: Distal RTA
- FE-HCO3 >15%: Proximal RTA
- Assumes serum bicarbonate >20 meq/L
- Management: Bicarbonate Supplementation
- Dose: 1-2 meq/kg/day
- Goal: Serum bicarbonate > 22 meq/L
- Corrects Hypokalemia