{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T18:22:49Z","timestamp":1785435769253,"version":"3.56.0"},"reference-count":24,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100018780","name":"Andalusian Health Service","doi-asserted-by":"crossref","award":["A2-0010-2025"],"award-info":[{"award-number":["A2-0010-2025"]}],"id":[{"id":"10.13039\/501100018780","id-type":"DOI","asserted-by":"crossref"}]},{"award":["A2-0010-2025"],"award-info":[{"award-number":["A2-0010-2025"]}],"id":[{"id":"https:\/\/ror.org\/03q4c3e69","id-type":"ROR","asserted-by":"publisher"}]},{"name":"Spanish Association for Research and Support to Wolfram Syndrome"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background\/Objectives: Wolfram syndrome type 1 (WS1) is a rare, progressive, multisystem neurodegenerative disorder characterized by diabetes mellitus, optic atrophy, diabetes insipidus, and sensorineural hearing loss. As survival has improved, an increasing number of affected women are reaching reproductive age. However, evidence on pregnancy and peripartum management in WS1 remains scarce, and practical guidance is limited. This case report describes the multidisciplinary management of pregnancy and delivery in a woman with genetically confirmed WS1 and highlights key considerations for peripartum care. Case Presentation: A woman with genetically confirmed WS1 and long-standing multisystem involvement, including diabetes mellitus, diabetes insipidus, neurogenic bladder requiring frequent self-catheterization, progressive neurologic manifestations, and severe sensory impairment, achieved pregnancy through assisted reproduction with oocyte donation and was closely monitored by a multidisciplinary team. Due to persistent breech presentation, a planned external cephalic version was performed at 37 + 5 weeks\u2019 gestation with immediate availability for cesarean delivery. After unsuccessful attempts, cesarean delivery was performed under combined spinal\u2013epidural anesthesia. Peripartum management focused on strict glycemic control, careful monitoring of fluid balance and urine output, neuraxial anesthesia with proactive hemodynamic management, precautions related to the cochlear implant, and tailored communication strategies. Postpartum recovery was favorable, although anemia on postoperative day 1 required transfusion of one unit of packed red blood cells and intravenous iron therapy. Discussion and Conclusions: Pregnancy in WS1 represents a high-risk clinical scenario because of the coexistence of endocrine, urologic, and neurologic comorbidities, while published evidence on peripartum management remains limited. This case supports an individualized, multidisciplinary approach to obstetric and anesthetic planning and the use of a practical framework to optimize peripartum management and enhance maternal\u2013fetal safety in this rare condition.<\/jats:p>","DOI":"10.3390\/diagnostics16081117","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T09:49:44Z","timestamp":1775641784000},"page":"1117","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pregnancy and Peripartum Multidisciplinary Management in Wolfram Syndrome Type 1: A Case Report"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7554-9434","authenticated-orcid":false,"given":"Gema","family":"Esteban-Bueno","sequence":"first","affiliation":[{"name":"Clinical Management Unit Almer\u00eda Periferia, Almer\u00eda Health District, Andalusian Health Service (SAS), 04009 Almeria, Spain"},{"name":"Spanish Association for Research and Support to Wolfram Syndrome, 04120 Almeria, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-1799-1157","authenticated-orcid":false,"given":"Mar\u00eda Luz","family":"Serrano Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Hospital Universitario Fundaci\u00f3n Alcorc\u00f3n, 28922 Alcorcon, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,8]]},"reference":[{"key":"ref_1","unstructured":"Adam, M.P., Bick, S., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., and Amemiya, A. 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