{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:07:39Z","timestamp":1792786059837,"version":"4.5.0"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"European Society for Pediatric Nephrology"},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["101085068"],"award-info":[{"award-number":["101085068"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Advicenne Pharma SA","id":[{"id":"https:\/\/ror.org\/04gvvdd93","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,30]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Distal renal tubular acidosis (dRTA) is characterized by metabolic acidosis, growth failure, nephrocalcinosis, nephrolithiasis and chronic kidney disease (CKD). Previous retrospective data suggested improved outcome with adequate metabolic control, but confirmation from prospective data is lacking. Since 2019, the European dRTA registry has collected prospective data on patients with dRTA. Herein we present results of the first data analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The registry is hosted as a subregistry of the European Rare Kidney Disease Network (https:\/\/www.erknet.org\/). In addition to the standard items of growth and plasma creatinine, additional data on plasma and urine biochemistries, genetics, treatment and clinical manifestations were collected from February 2019 through May 2024. Logistic regression analysis was used to identify predictors of CKD and impaired growth.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of the 214 patients enrolled in the registry, only 22% were aged &amp;gt;18 years at the last visit. A genetic diagnosis was documented in 69% of patients. On average, low blood bicarbonate levels (&amp;lt;22 mmol\/L) and hypercalciuria were observed in 42% and 25% of patients, respectively, independently of age. Multivariable analysis showed that height standard deviation score (SDS) was positively associated with serum bicarbonate levels (P\u00a0=\u00a0.008) and with early diagnosis (P\u00a0= .005). Further modelling based on odds ratios indicated substantial odds of impaired growth for low serum bicarbonate levels. Patients aged &amp;gt;30 years had significantly lower estimated glomerular filtration rate (P\u00a0&amp;lt;\u00a0.001) and the overall prevalence of CKD stage \u22652 was 36%. Patients with SLC4A1 variants were at higher risk of CKD stage &amp;gt;1 (P\u00a0=\u00a0.013).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our results in this primarily paediatric cohort highlight the importance of metabolic control and support increasing the blood bicarbonate level for therapy to 24\u00a0mmol\/L to improve growth. Compared with the overall population, patients with dRTA are at higher risk of CKD from childhood, particularly if they have underlying SLC4A1 variants.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ndt\/gfag051","type":"journal-article","created":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T12:36:53Z","timestamp":1772800613000},"page":"1839-1848","source":"Crossref","is-referenced-by-count":3,"title":["The European distal renal tubular acidosis registry: a 5-year analysis"],"prefix":"10.1093","volume":"41","author":[{"given":"Marta","family":"Giaccari","sequence":"first","affiliation":[{"name":"Universit\u00e1 Cattolica del Sacro Cuore , Rome ,","place":["Italy"]},{"name":"Division of Nephrology, Bambino Ges\u00f9 Children\u2019s Hospital \u2013 IRCCS , Rome ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faidra","family":"Veligratli","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS 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