{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T16:14:15Z","timestamp":1788970455478,"version":"build-2803163510"},"reference-count":50,"publisher":"The Endocrine Society","license":[{"start":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T00:00:00Z","timestamp":1787788800000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Arginine vasopressin deficiency (AVP-D) is an uncommon but clinically important cause of the polyuria\u2013polydipsia syndrome. Establishing the diagnosis extends beyond confirming hypotonic polyuria and requires differentiation from primary polydipsia and arginine vasopressin resistance, together with identification of the underlying etiology. Unlike adults, children and adolescents with AVP-D frequently present with the earliest manifestation of an evolving neoplastic, infiltrative, inflammatory, congenital, autoimmune, or genetic disorder, making longitudinal clinical, endocrine, and magnetic resonance imaging (MRI) surveillance integral to the diagnostic process. Using three illustrative clinical cases, this review presents a practical approach to the evaluation of AVP-D, integrating clinical assessment, biochemical investigation, dedicated hypothalamic\u2013pituitary MRI, and risk-adapted longitudinal surveillance. We discuss the strengths and limitations of the water deprivation test, the emerging role of copeptin-based diagnostics, and current evidence supporting arginine-, urea-, and glucagon-stimulated copeptin testing. Emphasis is placed on the longitudinal interpretation of MRI, consensus recommendations for PST, and the concept that idiopathic AVP-D should be regarded as a provisional diagnosis requiring continued etiological reassessment. Emerging biomarkers, including neurophysin I and oxytocin, may further refine the assessment of hypothalamic\u2013neurohypophyseal dysfunction but remain investigational in children. Ultimately, AVP-D should be viewed not as the end of the diagnostic process but as its beginning, with clinical assessment, neuroimaging, endocrine evaluation, and structured surveillance integrated to achieve the earliest possible etiological diagnosis.<\/jats:p>","DOI":"10.1210\/clinem\/dgag347","type":"journal-article","created":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T11:41:11Z","timestamp":1787830871000},"source":"Crossref","is-referenced-by-count":0,"title":["Arginine vasopressin deficiency in children\u2014a practical guide to etiological diagnosis and long-term surveillance"],"prefix":"10.1210","author":[{"given":"Chiara","family":"Morreale","sequence":"first","affiliation":[{"name":"Department of Woman and Child, ASST-Settelaghi, University of Insubria , Varese 21100 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelica","family":"Pisati","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa , Genoa 16132 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Panciroli","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa , Genoa 16132 ,","place":["Italy"]},{"name":"Department of Pediatrics, Pediatric Endocrinology Unit, IRCCS Istituto Giannina Gaslini , Genoa 16147 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3567-6118","authenticated-orcid":false,"given":"Flavia","family":"Napoli","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit, IRCCS Istituto Giannina Gaslini , Genoa 16147 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppa","family":"Patti","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa , Genoa 16132 ,","place":["Italy"]},{"name":"Department of Pediatrics, Pediatric Endocrinology Unit, IRCCS Istituto Giannina Gaslini , Genoa 16147 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8575-700X","authenticated-orcid":false,"given":"Andrea","family":"Rossi","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit, IRCCS Istituto Giannina Gaslini , Genoa 16147 ,","place":["Italy"]},{"name":"Department of Health Sciences (DISSAL), University of Genoa , Genoa 16132 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