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Biochemistry · Clinical biochemistry: distal renal tubular acidosis
Sample itemA 25-year-old woman with Sjogren syndrome has a normal anion gap metabolic acidosis, hypokalaemia, nephrocalcinosis and a urine pH persistently above 5.5. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Distal (type 1) renal tubular acidosis
In distal RTA the collecting duct cannot secrete hydrogen ions, so urine pH stays above 5.5 despite systemic acidosis. Potassium wasting, hypercalciuria with hypocitraturia, stones and nephrocalcinosis are typical, and autoimmune diseases such as Sjogren syndrome are associated. Type 4 causes hyperkalaemia.
Option by option
A. Hyporeninaemic hypoaldosteronism (type 4) renal tubular acidosis
Type 4 RTA causes hyperkalaemia, not hypokalaemia.
B. Diarrhoea-related bicarbonate loss
Diarrhoea causes acidosis with an acidic urine and a positive urine ammonium response.
C. Proximal (type 2) renal tubular acidosis
Proximal RTA is characterised by bicarbonate wasting and a urine pH that falls below 5.5 once serum bicarbonate is low.
D. Distal (type 1) renal tubular acidosis (correct)
Correct. High urine pH with acidosis, hypokalaemia and nephrocalcinosis is distal RTA.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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