What are the sporadic and genetic forms of hypokalemic (classic) distal renal tubular acidosis (type I)?

Updated: Oct 18, 2018
  • Author: Sai-Ching Jim Yeung, MD, PhD, FACP; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Primary dRTA has been described in sporadic and genetically transmitted forms.

Autoimmune disorders such as hypergammaglobulinemia, cryoglobulinemia, Sjögren syndrome, thyroiditis, idiopathic pulmonary fibrosis, chronic active hepatitis (CAH), primary biliary cirrhosis (PBC), systemic lupus erythematosus (SLE), and systemic vasculitis can be associated with dRTA.

Distal RTA can be secondary to genetically transmitted systemic diseases, including Ehlers-Danlos syndrome, hereditary elliptocytosis, sickle cell disease, Marfan syndrome, CA I deficiency or alteration, medullary cystic disease, and neuroaxonal dystrophy.

Disorders associated with nephrocalcinosis that cause hypokalemic dRTA include primary or familial hyperparathyroidism, vitamin D intoxication, milk-alkali syndrome, hyperthyroidism, idiopathic hypercalciuria, hereditary fructose intolerance, Fabry disease, and Wilson disease.

Drugs or toxins that can cause dRTA include amphotericin B, toluene, nonsteroidal anti-inflammatory drugs (NSAIDs), lithium, and cyclamate.

Renal tubulointerstitial conditions associated with dRTA include chronic pyelonephritis, obstructive uropathy, renal transplantation, leprosy, and hyperoxaluria.

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