{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T01:27:04Z","timestamp":1762306024457,"version":"build-2065373602"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Primary nephrogenic diabetes insipidus (NDI) is a rare inherited disorder with limited data on long-term outcomes. This study assessed longitudinal outcomes with primary NDI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This multicenter retrospective study included 63 patients with primary NDI. Growth rates and estimated glomerular filtration (eGFR) were analyzed via a piecewise linear mixed-effects model during the pediatric period.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>AVPR2 and AQP2 mutations were identified in 74.6% and 9.5% of patients, respectively. The median ages at diagnosis and last follow up were 0.41 and 12.37 years, respectively. Height Z-scores declined from birth to 1.5 years and improved thereafter, with slopes of \u22122.626, \u22120.563, 0.102, and 0.031 per year across breakpoints at 0.5, 1.5, and 7 years. Although none of the patients met the criteria for short stature by age 18, the mean final height Z-score remained below average and was significantly lower than that at birth. Weight and weight-for-height Z-scores initially declined and then improved, with slope changes at 0.5 and 0.7 years. By the age of 18, 38.9% were overweight or obese. eGFR increased rapidly before 1.4 years (slope 25.61\u00a0ml\/min\/1.73 m2\/year), and slowly thereafter (slope 0.58), reaching eGFR \u226590\u00a0ml\/min\/1.73 m2 at a median age of 5.1 years. The proportion of patients with eGFR &amp;lt;90 ml\/min\/1.73 m2 decreased to 34.6% at 14 years, then rose to 43.8% by the age of 18. Treatment-associated complications included hypokalemia (64.5%), alkalosis (61.1%), and hyperuricemia (60.7%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Patients with primary NDI exhibit a dynamic growth trajectory, delayed achievement of normal GFR with subsequent risk of chronic kidney disease, and frequent treatment-associated complications. These findings underscore the need for timely and individualized long-term management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ckj\/sfaf303","type":"journal-article","created":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T12:08:27Z","timestamp":1759320507000},"source":"Crossref","is-referenced-by-count":0,"title":["Clinical outcomes among children with primary nephrogenic diabetes insipidus"],"prefix":"10.1093","volume":"18","author":[{"given":"Seon Hee","family":"Lim","sequence":"first","affiliation":[{"name":"Pusan National University Yangsan Children\u2019s Hospital and School of Medicine Department of Pediatrics, , Yangsan ,","place":["South Korea"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jin-Soon","family":"Suh","sequence":"additional","affiliation":[{"name":"Bucheon St. Mary\u2019s Hospital, College of Medicine, The Catholic University of Korea Department of Pediatrics, , Seoul ,","place":["South 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