Distal renal tubular acidosis
Alerts and Notices
Synopsis
- In adults, the most common cause is autoimmune disease, eg, Sjögren syndrome or rheumatoid arthritis, or hypercalciuria. In otherwise healthy adults with unexplained distal RTA, diagnosis of Sjögren syndrome should be considered.
- In children, hereditary causes of distal RTA are most common and are due to genetic mutations in the basolateral chloride-bicarbonate exchanger and in the apical hydrogen-ATPase. The autosomal recessive form presents in infancy, whereas the autosomal dominant form typically presents in adolescence or adulthood.
- Both ifosfamide and toluene ingestion can cause distal RTA in children and adults.
Codes
ICD10CM:N25.89 – Other disorders resulting from impaired renal tubular function
SNOMEDCT:
236461000 – Distal renal tubular acidosis
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Chronic diarrhea – Urine pH may be inappropriately elevated in this setting; however, the urine anion gap should be negative.
- Ileal loop urinary diversion
- Bladder-drained pancreas transplant
- Toluene inhalation
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:03/19/2018
Last Updated:03/19/2018
Last Updated:03/19/2018
Distal renal tubular acidosis