Proximal renal tubular acidosis
Alerts and Notices
Synopsis

Major causes of proximal RTA in adults include increased excretion of immunoglobulins in the setting of monoclonal gammopathies and use of carbonic anhydrase inhibitors (eg, acetazolamide, and topiramate or ifosfamide). In children, proximal RTA may be caused by an autosomal recessive mutation in the gene for the sodium bicarbonate transporter (SLC4A4) or ifosfamide use, although in many cases, no cause is identified.
Related topic: Distal renal tubular acidosis
Codes
ICD10CM:N25.89 – Other disorders resulting from impaired renal tubular function
SNOMEDCT:
24790002 – Proximal renal tubular acidosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Distal renal tubular acidosis
- Chronic diarrhea – In this case, the urine anion pH should be appropriately low, and urine anion gap should be negative.
- Diarrhea
- Ureterosigmoid fistula
- Addison disease
- Small bowel fistula
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:03/06/2018
Last Updated:01/23/2022
Last Updated:01/23/2022