What are the causes of hyperkalemic distal renal tubular acidosis (type IV)

Updated: Sep 03, 2020
  • Author: Sai-Ching Jim Yeung, MD, PhD, FACP; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
  • Print

Deficiency of or resistance to aldosterone is the most common cause of hyperkalemic dRTA. Deficiency of aldosterone with glucocorticoid deficiency is associated with Addison disease, bilateral adrenalectomy, and certain enzymatic defects in the steroidogenetic biochemical pathways (eg, 21-hydroxylase deficiency, 3 beta-hydroxysteroid-dehydrogenase deficiency, desmolase deficiency). Isolated aldosterone deficiency can be secondary to states of deficient renin secretion, including diabetic nephropathy, tubulointerstitial renal disease, nonsteroidal anti-inflammatory drug (NSAID) use, beta-adrenergic blocker use, AIDS/HIV disease, and renal transplantation.

Isolated aldosterone deficiency can also be observed secondary to heparin use; in corticosterone methyl oxidase (CMO) deficiency, a genetically transmitted disorder; and in a transient infantile form.

Angiotensin1-converting enzyme (ACE) inhibition, either endogenously or through ACE inhibitors such as captopril, and the newer angiotensin AT1 receptor blockers can cause hyperkalemic dRTA.

Resistance to aldosterone secretion is observed in pseudohypoaldosteronism, childhood forms of obstructive uropathy, cyclosporine nephrotoxicity, renal transplantation, and the use of spironolactone.

Voltage-mediated defects that cause hyperkalemic dRTA can be observed in obstructive uropathy; sickle cell disease; and the use of lithium, triamterene, amiloride, trimethoprim, or pentamidine.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!