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Issue: March 1, 2007
Case: Acid-Base Disorders
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Question:

Which of the following best describes the acid-base disorder seen in all forms of renal tubular acidosis (RTA)?
  1. Normal anion gap (hyperchloremic) metabolic acidosis
  2. Wide anion gap metabolic acidosis
  3. Compensated metabolic acidosis
     

Answer:
A

Remediation:
RTA develops as a result of the inability of renal tubules to resorb filtered bicarbonate and excrete hydrogen when necessary. There are three major subgroups of RTA, and they each have different clinical characteristics. They are:
  • Distal, or type 1 RTA
  • Proximal, or type 2 RTA
  • Hypoaldosteronism, or type 4 RTA

Although initially used to describe a transiently severe form of type 1 RTA in infants, the term type 3 RTA is now most often applied to a rare autosomal recessive syndrome (resulting from carbonic anhydrase II deficiency) with features of both type 1 and type 2 RTA. In addition to RTA, affected patients suffer from osteopetrosis, cerebral calcification, and mental retardation.

About the Image(s):
Image 1:
Nephrocalcinosis due to Renal Tubular Acidosis
These calcifications are typical of nephrocalcinosis in the medullary portion of the kidney. The location of the calcifications distinguishes this condition from nephrolithiasis, although the latter may be associated. This patient had renal tubular acidosis. Other etiologies include medullary sponge kidney, hyperparathyroidism, sarcoid, and various disorders with hypercalcemia. The pattern is characteristic.

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