Primary Distal Renal Tubular Acidosis

Treatment

What are the first steps after an initial diagnosis of primary distal renal tubular acidosis?

The first step after diagnosis with primary distal renal tubular acidosis (primary dRTA) is to work with a doctor familiar with treating primary dRTA and request referrals for key team specialists in genetics, kidney care (nephrologists), audiology, endocrinology, gastroenterology, and nutrition. Specifically, a pediatric nephrologist, a doctor pediatrician that specializes in the kidney, is a key member of your care team.

Early diagnosis and treatment of primary distal renal tubular acidosis with alkali supplementation (often sodium bicarbonate) to lower the pH of the blood and potassium supplement (often potassium citrate or sodium citrate) can often enable catch-up growth and in many cases preserve kidney function. It is also important to have regular hearing (audiologic) assessments to monitor hearing and allow for early opportunities for hearing support such as hearing aids and cochlear implants.

Park E, Cho MH, Hyun HS, Shin JI, Lee JH, Park YS, Choi HJ, Kang HG, Cheong HI. Genotype-Phenotype Analysis in Pediatric Patients with Distal Renal Tubular Acidosis. Kidney Blood Press Res. 2018;43(2):513-521. doi: 10.1159/000488698. Epub 2018 Mar 29. PMID: 29627839

Swayamprakasam, A. P., Stover, E., Norgett, E., Blake-Palmer, K. G., Cunningham, M. J., & Karet, F. E. (2010). Importance of early audiologic assessment in distal renal tubular acidosis. International medical case reports journal, 4, 7-11. doi:10.2147/IMCRJ.S13667

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