{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T07:49:57Z","timestamp":1753861797242,"version":"3.41.2"},"reference-count":17,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,4,16]],"date-time":"2025-04-16T00:00:00Z","timestamp":1744761600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Bainbridge\u2010Ropers Syndrome(BPRS) is a rare autosomal dominant genetic disorder resulting from heterozygous mutations in the <jats:italic>ASXL3<\/jats:italic>(Additional Sex Comb\u2010Like 3) gene located on chromosome 18q12. To date, only 45 cases have been documented in the literature. BPRS is characterized by a range of clinical features, including feeding difficulties, hypotonia, distinctive dysmorphic facial features, high\u2010arched palate, and intellectual disability. This case report aims to present two pediatric patients diagnosed with BPRS, emphasize newly identified oro\u2010dental manifestations, and propose a comprehensive dental management plan. In Case #1, a 10\u2010year\u2010old female patient presented to the clinic with concerns of developmental delay and spinal deformity. Physical examination revealed trigonocephaly, thoracic kyphosis, strabismus, hirsutism, bitemporal narrowing, and bilateral coxa valga. Additionally, the patient exhibited failure to thrive, language difficulties, and mild intellectual disability. Oral examination identified a high\u2010arched palate, fibrotic frenulum, narrow maxilla, and posterior crossbite. In Case #2, a 6\u2010year\u2010old female patient presented with developmental delay, language difficulties, and mouth breathing. Physical findings included trigonocephaly, bitemporal narrowing, strabismus, hirsutism, and arched eyebrows. Oral examination revealed a high\u2010arched palate, narrow maxilla, and open bite. For both cases, preventive dental interventions were implemented, including the application of fissure sealants, fluoride varnishes, dietary regulation, and the use of interceptive orthodontic appliances. Children diagnosed with BPRS require ongoing medical and dental management through a multidisciplinary approach to address the complex and varied manifestations of the disorder effectively.<\/jats:p>","DOI":"10.1002\/ajmg.a.64090","type":"journal-article","created":{"date-parts":[[2025,4,16]],"date-time":"2025-04-16T10:26:40Z","timestamp":1744799200000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Dentofacial Findings and Management of two Pediatric Patients With Bainbridge\u2010Ropers Syndrome: A Case Report"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4865-3249","authenticated-orcid":false,"given":"Asl\u0131","family":"A\u015f\u0131k","sequence":"first","affiliation":[{"name":"Department of Pediatric Dentistry Faculty of Dentistry, Izmir T\u0131naztepe University  \u0130zmir Turkey"},{"name":"Department of Pediatric Dentistry Faculty of Dentistry, Ege University  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