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Definition and EtiologyTop
Distal renal tubular acidosis (dRTA), also known as type 1 renal tubular acidosis, results from reduction in net acid secretion in the distal nephron, leading to maximal urinary bicarbonate losses and preventing urinary acidification, thereby minimizing urinary ammonium loss. A constant feature of dRTA is the inability to acidify urine to pH <5.5 in the presence of nonrespiratory acidosis. Impaired H+ secretion results in urinary sodium loss, secondary activation of the renin-angiotensin-aldosterone system, and increased urinary potassium loss. Hypercalciuria and hypocitraturia (causative factors for nephrocalcinosis and urolithiasis) frequently occur.
dRTA can be caused by a number of illnesses (Table 1). Rare autosomal dominant and autosomal recessive channelopathies will not be discussed, as they are beyond the scope of this chapter.
DiagnosisTop
In patients with dRTA urinary pH is persistently high, independently of alkali challenge testing. To make a diagnosis of dRTA, urinary pH should be ≥5.5, regardless of serum bicarbonate levels, with a urine sodium concentration >25 mmol/L.
TreatmentTop
If renal tubular acidosis (RTA) persists following treatment of the underlying cause, it can be managed with supplemental doses of alkali therapy.
Treatment of patients with dRTA requires relatively lower doses of supplemental bicarbonate therapy (1-2 mEq/kg/day) compared with proximal RTA. Children have higher requirements than adults because of their overall net acid production being higher on a daily basis. Sodium bicarbonate or sodium citrate is usually adequate to raise serum bicarbonate concentration back to the normal range. Potassium-based citrate, alone or with sodium citrate, can be used if hypokalemia persists.
TablesTop
Autoimmune diseases |
Sjögren syndrome,a systemic lupus erythematosus, rheumatoid arthritis, primary biliary cirrhosis |
Drugs/toxins |
Lithium,a antibiotics (amphotericin Ba), chemotherapeutics (ifosfamide) |
Tubulointerstitial disorders |
Status post renal transplantation, obstructive uropathy |
Other |
Nephrocalcinosis (hypercalciuria, vitamin D toxicity, hyperparathyroidism), sickle cell disease |
a The more common causes. |