Systemic and oral abnormalities in Kabuki syndrome: a case series.

Autor: Pinto LC; Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Brazil., Kokitsu-Nakata NM; Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Brazil., da Silva Dalben G; Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Brazil., de Azevedo Silva LJ; Bauru School of Dentistry, University of São Paulo, Brazil., de Almeida ALPF; Department of Prosthodontics and Periodontics, Bauru School of Dentistry and Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Brazil. Electronic address: analmeida@usp.br.
Jazyk: angličtina
Zdroj: Oral surgery, oral medicine, oral pathology and oral radiology [Oral Surg Oral Med Oral Pathol Oral Radiol] 2024 May; Vol. 137 (5), pp. e91-e98. Date of Electronic Publication: 2023 Dec 29.
DOI: 10.1016/j.oooo.2023.12.788
Abstrakt: Objective: This study analyzed the systemic and oral abnormalities in individuals with Kabuki syndrome (KS) that might be investigated to enhance the early diagnosis and treatment by a multidisciplinary team, minimizing the consequences to the individual's health.
Study Design: Clinical examination was conducted on 15 individuals to investigate orodental alterations such as tooth abnormalities and cleft lip and/or palate, and the patient records were also reviewed to investigate systemic diseases such as cardiopathies, infectious and immunologic diseases, nephropathies, and delayed neuropsychomotor development.
Results: All individuals with KS presented cleft lip and/or palate, 11 (73.34%) tooth abnormalities, 5 (33.34%) congenital cardiopathies, 12 (80%) infectious or immunologic diseases, 1 (6.67%) nephropathy, and 14 (93.34%) had an intellectual disability.
Conclusion: Individuals with KS often have dental anomalies such as hypodontia, cleft or palate, and systemic disorders such as congenital heart disease and infectious diseases. Intellectual disability is present in most cases. These alterations should be investigated as early as possible to prevent the increase in morbidity in these individuals.
Competing Interests: Declaration of interest None.
(Copyright © 2023 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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