{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T02:55:57Z","timestamp":1789181757240,"version":"build-2803163510"},"reference-count":41,"publisher":"The Endocrine Society","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,2,1]]},"abstract":"<jats:sec><jats:title>Context:<\/jats:title><jats:p>Neonatal central diabetes insipidus (CDI) with or without adipsia is a very rare complication of various complex hypothalamic disorders. It is associated with greater morbidity and a high risk of developing both hypernatremia and hyponatremia, due to the condition itself or secondary to treatment with vasopressin analogs or fluid administration. Its outcomes have yet to be evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective:<\/jats:title><jats:p>To investigate the clinical outcomes of patients with neonatal-onset CDI or adipsic CDI with hypernatremia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design, Setting, and Participants:<\/jats:title><jats:p>All patients diagnosed with neonatal CDI in a university hospital-based observational study and followed between 2005 and 2015 were included and analyzed retrospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Main Outcome Measures:<\/jats:title><jats:p>The various causes of CDI were grouped. Clinical outcome and comorbidities were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Ten of the 12 patients had an underlying condition with brain malformations: optic nerve hypoplasia (n = 3), septo-optic dysplasia (n = 2), semilobar holoprosencephaly (n = 1), ectopic neurohypophysis (n = 3), and unilateral absence of the internal carotid artery (n = 1). The other two were idiopathic cases. During the median follow-up period of 7.8 (4.9\u201316.8) years, all but one patient displayed anterior pituitary deficiency. Transient CDI was found in three (25%) patients for whom a posterior pituitary hyperintense signal was observed with (n = 2) and without (n = 1) structural hypothalamic pituitary abnormalities, and with no other underlying cerebral malformations. Patients with permanent CDI with persistent adipsia (n = 4) and without adipsia (n = 5) required adequate fluid intake and various doses of desamino-D-arginine-8-vasopressin. Those with adipsia were more likely to develop hypernatremia (45 vs 33%), hyponatremia (16 vs 4%) (P &amp;lt; .0001), and severe neurodevelopmental delay (P &amp;lt; .05) than those without adipsia. Comorbidities were common. The underlying cause remains unknown at the age of 23 years for one patient with CDI and normal thirst.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion:<\/jats:title><jats:p>Neonatal CDI may be transient or permanent. These vulnerable patients have high rates of comorbidity and require careful monitoring.<\/jats:p><\/jats:sec>","DOI":"10.1210\/jc.2015-3108","type":"journal-article","created":{"date-parts":[[2015,11,20]],"date-time":"2015-11-20T19:54:28Z","timestamp":1448049268000},"page":"635-643","source":"Crossref","is-referenced-by-count":38,"title":["Central Diabetes Insipidus in Infancy With or Without Hypothalamic Adipsic Hypernatremia Syndrome: Early Identification and Outcome"],"prefix":"10.1210","volume":"101","author":[{"given":"Adel","family":"Djermane","sequence":"first","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris (A.D., D.S., A.P., J.-C.C., J.L.), H\u00f4pital Robert Debr\u00e9, Service d'Endocrinologie Diab\u00e9tologie P\u00e9diatrique, Centre de R\u00e9f\u00e9rence des Maladies Endocriniennes Rares de la Croissance, F-75019 Paris, France"},{"name":"Universit\u00e9 Paris Diderot (A.P., J.-C.C., J.L.), Sorbonne Paris Cit\u00e9, F-75019 Paris, 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