{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T21:39:57Z","timestamp":1783028397845,"version":"3.54.6"},"reference-count":42,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2022,6,21]],"date-time":"2022-06-21T00:00:00Z","timestamp":1655769600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"MEXT\/JSPS KAKENHI","award":["21K15894"],"award-info":[{"award-number":["21K15894"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Specific antibody responses to subfornical organs, including Nax antibody, have been reported in patients with adipsic hypernatremia of unknown etiology who do not have structural lesions in the hypothalamic\u2013pituitary gland. The subfornical organ, also referred to as the window of the brain, is a sensing site that monitors sodium and osmotic pressure levels. On the other hand, ROHHAD syndrome is a rare disease for which the etiology of the hypothalamic disorder is unknown, and there have been some reports in recent years describing its association with autoimmune mechanisms. In addition, abnormal Na levels, including hypernatremia, are likely to occur in this syndrome. When comparing the clinical features of adipsic hypernatremia due to autoimmune mechanisms and ROHHAD syndrome, there are similar hypothalamic\u2013pituitary dysfunction symptoms in addition to abnormal Na levels. Since clinical diagnoses of autoimmunological adipsic hypernatremia and ROHAD syndrome might overlap, we need to understand the essential etiology and carry out precise assessments to accurately diagnose patients and provide effective treatment. In this review, I review the literature on the autoimmune mechanism reported in recent years and describe the findings obtained so far and future directions.<\/jats:p>","DOI":"10.3390\/ijms23136899","type":"journal-article","created":{"date-parts":[[2022,6,21]],"date-time":"2022-06-21T21:55:19Z","timestamp":1655848519000},"page":"6899","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Autoimmunity Related to Adipsic Hypernatremia and ROHHAD Syndrome"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7417-8923","authenticated-orcid":false,"given":"Akari","family":"Nakamura-Utsunomiya","sequence":"first","affiliation":[{"name":"Department of Genetic Medicine, Hiroshima University Graduate School, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8511, Japan"},{"name":"Department of Pediatrics, Hiroshima University Graduate School, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8511, Japan"},{"name":"Division of Neonatal Screening, National Center for Child Health and Development, 2 Chome-10-1 Okura, Setagaya, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,6,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"131","DOI":"10.7326\/0003-4819-56-1-131","article-title":"Chronic and sustained hypernatremia, absence of thirst, diabetes insipidus, and adrenocorticotrophin insufficiency resulting from widespread destruction of the hypothalamus","volume":"56","author":"Avioli","year":"1962","journal-title":"Ann. 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