What causes proximal renal tubular acidosis (type II)?

Updated: Sep 03, 2020
  • Author: Sai-Ching Jim Yeung, MD, PhD, FACP; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Causes of proximal tubular bicarbonate wasting are numerous. A selective defect (eg, isolated bicarbonate wasting) can occur as a primary disorder (with no obvious associated disease) that can be genetically transmitted or occur in transient form in infants.

Alterations in CA activity through drugs such as acetazolamide, sulfanilamide, and mafenide acetate produce bicarbonate wasting. Osteopetrosis with CA II deficiency and genetically transmitted and idiopathic CA deficiency also fall into the selective defect category.

A generalized PCT defect associated with multiple dysfunctions of the PCT can also occur as a primary disorder in sporadic and genetically transmitted forms. It also occurs in association with genetically transmitted systemic diseases, including Wilson disease, cystinosis and tyrosinemia, Lowe syndrome, hereditary fructose intolerance, pyruvate carboxylase deficiency, metachromatic leukodystrophy, and methylmalonic acidemia.

Proximal RTA is also observed in conditions associated with chronic hypocalcemia and secondary hyperparathyroidism, such as vitamin D deficiency or vitamin D resistance. Dysproteinemic states, such as multiple myeloma and monoclonal gammopathy, are also associated with pRTA.

Drugs or toxins that can induce pRTA include streptozotocin, lead, mercury, L-arginine, valproic acid, gentamicin, ifosfamide, and outdated tetracycline.

Renal tubulointerstitial conditions that are associated with pRTA include renal transplantation, Sjögren syndrome, and medullary cystic disease. Other renal causes include nephrotic syndrome and amyloidosis.

Paroxysmal nocturnal hemoglobinuria (PNH) and hyperparathyroidism can also cause pRTA.

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