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DOI 10.3390/genes16080910

Медицинская библиотека · Genes · 2025

Clinical and Genetic Management of a Patient with Rubinstein-Taybi Syndrome Type 1: A Case Report.

Santos Victor, Souza Pedro Paulo Chaves de, Campos Talyta, Winterly Hiane, Vieira Thaís, Gigonzac Marc, Honda Alex, Pinto Irene, Zatarin Raffael, Azevedo Fernando, Nascimento Anna, da Silva Cláudio, da Cruz Aparecido

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Аннотация

Rubinstein-Taybi Syndrome type 1 (RSTS1) is an uncommon autosomal dominant genetic disorder associated with neurodevelopmental impairments and multiple congenital anomalies, with an incidence of 1:100,000-125,000 live births. The syndrome, caused by de novo mutations in the CREBBP gene, is characterized by phenotypic variability, including intellectual disability, facial dysmorphisms, and systemic abnormalities. The current case report describes a 15-year-old Brazilian female diagnosed with RSTS1 through whole-exome sequencing, which identified a de novo heterozygous missense mutation in the CREBBP gene (NM_004380.3; c.4393G > C; p.Gly1465Arg), classified as pathogenic. The patient's clinical presentation included facial dysmorphisms, skeletal abnormalities, neurodevelopmental delay, psychiatric conditions, and other systemic manifestations. A comprehensive genetic counseling process facilitated the differential diagnosis and management strategies, emphasizing the importance of early and precise diagnosis for improving clinical outcomes. This report contributes to the growing knowledge of the genotype-phenotype correlations in RSTS1, aiding in the understanding and management of this uncommon condition.

Для цитирования: Santos Victor, Souza Pedro Paulo Chaves de, Campos Talyta, Winterly Hiane, Vieira Thaís, Gigonzac Marc, Honda Alex, Pinto Irene, Zatarin Raffael, Azevedo Fernando, Nascimento Anna, da Silva Cláudio, da Cruz Aparecido. Clinical and Genetic Management of a Patient with Rubinstein-Taybi Syndrome Type 1: A Case Report.. Genes. 2025. DOI: 10.3390/genes16080910.

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