Distal Renal Tubular Acidosis: ERKNet/ESPN Clinical Practice Points

Pietro Manuel Ferraro, Francesco Trepiccione, Steven B Walsh, Gema Ariceta, Olivia Boyer, Francesco Emma, Roberta Camilla, Dieter Haffner, Martin Konrad, Elena Levtchenko, Sergio Camilo Lopez-Garcia, Fernando Santos, Stella Stabouli, Maria Szczepanska, Velibor Tasic, Rezan Topaloglu, Rosa Vargas-Poussou, Tanja Wlodkowski, Detlef Bockenhauer

Research output: Contribution to journalArticle


Distal renal tubular acidosis (dRTA) is characterised by an impaired ability of the distal tubule to excrete acid, leading to metabolic acidosis. Associated complications include bone disease, growth failure, urolithiasis and hypokalaemia. Due to its rarity, there is a limited evidence to guide diagnosis and management, however, available data strongly suggest that metabolic control of the acidosis by alkali supplementation can halt or revert almost all complications. Despite this, cohort studies show that adequate metabolic control is present in only about half of patients, highlighting problems with treatment provision or adherence. With these clinical practice points the authors, part of the working groups tubulopathies in the European Rare Kidney Disease Reference network (ERKnet) and inherited kidney diseases of the European Society for Paediatric Nephrology (ESPN) aim to provide guidance for the management of patients with dRTA to facilitate adequate treatment and establish an initial best practice standard against which treatment of patients can be audited.
Original languageEnglish
Pages (from-to)N/A-N/A
JournalNephrology Dialysis Transplantation
Publication statusPublished - 2021


  • ATP6V0A4
  • ATP6V1B1
  • FOXI1
  • SLC4A1
  • urolithiasis
  • acidosis
  • distal renal tubular acidosis
  • nephrocalcinosis
  • WDR72


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