Bimaxillary fixed implant-supported zirconium oxide prosthesis therapy of an adolescent patient with non-syndromic oligodontia and two WNT10 variants: case report

Pascal Grün, F. Pfaffeneder-Mantai, Nikolai Leunig, Ditjon Bytyqi, Cornelia Maier, Martin Gencik, Patrick Bandura, D. Turhani
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Abstract

Oligodontia is a rare genetic condition characterised by more than six congenitally missing teeth, either as an isolated non-syndromic condition or in association with other genetic syndromes. The impact of WNT10A variants on dental development increases with the presence of the c.321C>A variant and the number of missing teeth. A 21-year-old man with non-syndromic oligodontia was diagnosed at 15 years of age with misaligned teeth, speech problems, and the absence of 24 permanent teeth. Interdisciplinary collaboration between specialists was initiated to enable comprehensive treatment. DNA analysis confirmed that the patient was a carrier of the known pathogenic WNT10A variant c321C>A and WNT10A variant c.113G>T of unknown clinical significance. Dental implants are a common treatment; however, bone development challenges in adolescent patients with non-syndromic oligodontia necessitate careful planning to ensure implant success. Many WNT variants play crucial roles in tooth development and are directly involved in non-syndromic oligodontia, especially the WNT10 variant c.321C>A. A full-arch implant-supported monolithic zirconia screw-retained fixed prosthesis is a viable treatment option for young adults with non-syndromic oligodontia. Further studies are needed to clarify the possible amplifying effect of the WNT10A variants c321C>A and c.113G>T on the pathogenic phenotype of non-syndromic oligodontia.
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一名患有非综合征性少齿畸形和两种 WNT10 变异的青少年患者的双颌固定种植体支撑氧化锆假体治疗:病例报告
牙齿缺失症是一种罕见的遗传病,其特征是有六颗以上的先天性牙齿缺失,既可能是一种孤立的非综合症,也可能与其他遗传综合症伴发。WNT10A 变异对牙齿发育的影响随着 c.321C>A 变异的存在和缺失牙齿数量的增加而增加。 一名 21 岁男子在 15 岁时被诊断出患有非综合征性少齿畸形,并伴有牙齿排列不齐、语言障碍和 24 颗恒牙缺失。为了进行综合治疗,专家们开始了跨学科合作。DNA 分析证实,患者是已知致病 WNT10A 变异 c321C>A 和临床意义不明的 WNT10A 变异 c.113G>T 的携带者。 牙科植入物是一种常见的治疗方法;然而,非综合征性少齿症青少年患者的骨骼发育面临挑战,因此有必要进行仔细规划,以确保植入物的成功。许多 WNT 变体在牙齿发育中起着至关重要的作用,并直接参与非综合征性少齿畸形,尤其是 WNT10 变体 c.321C>A。 对于患有非综合征性少牙症的年轻成年人来说,全拱种植体支持的整体氧化锆螺钉固位固定修复体是一种可行的治疗方案。还需要进一步研究,以明确 WNT10A 变异 c321C>A 和 c.113G>T 对非综合征性少齿畸形的致病表型可能产生的放大效应。
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