Distal renal tubular acidosis


The distal renal tubular acidosis (dRTA) Registry

The distal renal tubular acidosis subregistry is a joint effort of ERKNet and ESPN. 

General information

Distal renal tubular acidosis (dRTA) is a rare kidney disorder, characterised by the impaired secretion of acid from the intercalated cells in the collecting duct, leading to systemic metabolic acidosis. Clinical consequences may include impaired growth, rickets/osteodystrophy, nephrocalcinosis, cysts, stones and chronic kidney disease. Several genes have been associated with dRTA and dependent on the underlying gene, additional clinical manifestations, such as sensorineural deafness or amelogenesis imperfecta can occur.


The registry aims to capture data on the presentation, treatment and long-term outcome in dRTA. These will be used to assess the impact of treatment on long-term outcome and to establish criteria for adequate treatment and monitoring.


Eligible are all patients with a clinical diagnosis of dRTA, defined as systemic non-anion gap metabolic acidosis with an inappropriately elevated urine pH (≥5.5) in the absence of alkali supplementation. Both primary (inherited) and secondary (acquired) forms of dRTA are eligible.

Patients with acidosis from other causes, such as proximal renal tubular acidosis (urine pH <5.5 in the absence of alkali supplementation) or increased metabolic acid production or acid intoxication (anion-gap acidosis and urine pH <5.5) are excluded.

Ethical aspects

Data entry in this subregistry is covered by the consent for ERKNet participation.

Data collection 

At the initial visit, data on baseline demographics, family history, establishment of the diagnosis and underlying cause are requested. For all visits, clinical information on height and weight, biochemistries of blood and urine, treatment, as well as imaging data are requested annually.

The data dictionary of ERKReg, including the collected parameters for this subregistry, can be found here.

ERKNet and ESPN experts published clinical practice points on the management of patients with dRTA. Please find the paper here.

For more information please contact: Detlef Bockenhauer (detlef.bockenhauer@uz-leuven.be)