Renal tubular acidosis is characterized by
non-gap metabolic acidosis caused by defects in acid excretion or bicarbonate reabsorption.
Distal Renal Tubular Acidosis (Type 1)
Associated with decreased hydrogen secretion in the
distal nephron.
As a result of decreased hydrogen secretion, no new bicarbonate can be generated, which leads to acidosis.
Causes Include:
- Amphotericin B toxicity
- Analgesic nephropathy
- Autoimmune diseases (SLE and Sjogren's)
- Urinary tract obstructions
Signs and Symptoms:
- Polydipsia and polyuria
- Muscle weakness
- Nephrolithiasis (due to hypercalciuria and hypocitraturia)
- In children: growth retardation and rickets.
Urine and Serum Values:
- Urine pH is relatively basic > 5.5
- Serum bicarbonate is reduced, between 10-15 mmol/L
- Serum potassium is reduced (hypokalemia).
Diagnosis:
Perform an ammonium load test and look for a positive urinary anion gap.
Treatment:
Administrate alkaline substances, such as sodium bicarbonate or potassium citrate, and thiazide diuretics.
Proximal renal tubular acidosis (Type 2 RTA)
Decreased bicarbonate reabsorption in the proximal tubule.
Causes Include:
- Fanconi syndrome, in which proximal tubule malfunction leads to excess excretion of several solutes (glucose, bicarbonate, phosphate, uric acid, potassium, and some amino acids).
- Carbonic anhydrase inhibitors, which are used to treat glaucoma, epilepsy, congestive heart failure, seizures, and other disorders.
- Complication of multiple myelom, in which the excreted light chains are toxic to the proximal nephron cells.
Signs and Symptoms:
- Muscle weakness
- Growth retardation
- Rickets
Urine and Serum Values:
- Urine pH is variable; it is > 7 as long as plasma bicarbonate remains normal, but falls to < 5.5 when plasma bicarbonate is depleted.
- Serum bicarbonate is somewhat low, 16-20 mmol/L.
- Potassium is reduced.
Diagnosis:
Proximal RTA is indicated when a bicarbonate loading test produces Fractional Excretion of bicarbonate of greater than 15% and a urine pH of > 7.5.
Treatment:
Alkaline solutions and thiazide diuretics.
Hyperkalemic renal tubular acidosis (Type 4)
Characterized by
aldosterone deficiencies or resistance in the distal and collecting tubules; this leads to hyperkalemia and reduced acid excretion in the form of NH4+ (ammonium).
Causes Include:
- Diabetic and obstructive nephropathies, chronic interstitial nephritis, adrenal insufficiency
- Several medications, including ACE inhibitors, angiotensin II receptor blockers (ARBs), potassium-sparing diuretics, and trimethoprim-sulfamethoxazole.
Signs and Symptoms:
- Most patients are asymptomatic, but if hyperkalemia is severe, they may experience muscle weakness or cardiac arrythmias.
Urine and Serum Values:
- Urine pH is low, < 5.5
- Acidosis is milder with serum bicarbonate levels > 17 mmol/L.
- Elevated serum potassium levels are key to differentiating this type of renal tubular acidosis from the proximal and distal forms.
Diagnosis:
Urinary potassium is less than 40 mmol/L and Fractional Excretion of potassium is less than 20%, both the result of potassium retention in this disorder.
Treatments:
Volume expansion and dietary potassium restriction with potassium-wasting diuretics.