Major causes of type 1 (distal) renal tubular acidosis (RTA)
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Primary |
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Idiopathic (sporadic) |
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Familial |
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Autosomal dominant (mainly due to mutations causing defects in the kidney anion exchanger [kAE1] in distal tubule/collecting duct intercalated cells) |
|
Autosomal recessive (due to a variety of mutations causing defects in one of the multiple subunits of V-ATPase in distal tubule/collecting duct intercalated cells) |
|
Secondary |
|
Autoimmune disorders |
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Sjögren's syndrome |
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Autoimmune hepatitis/primary biliary cirrhosis |
|
Systemic lupus erythematosus (also can generate hyperkalemic RTA) |
|
Rheumatoid arthritis |
|
Drugs |
|
Ifosfamide (also can cause proximal RTA with Fanconi syndrome) |
|
Amphotericin B |
|
Lithium carbonate |
|
Ibuprofen |
|
Hypercalciuric conditions |
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Hyperparathyroidism |
|
Vitamin D intoxication |
|
Sarcoidosis |
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Idiopathic hypercalciuria |
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Other |
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Medullary sponge kidney |
|
Obstructive uropathy |
|
Renal transplant rejection |
|
Wilson disease |
REF. UpToDate 2020.08.11