Diagnosis and management in Rubinstein-Taybi syndrome: first international consensus statement.

Didier Lacombe, Agnès Bloch-Zupan,Cecilie Bredrup, Edward B Cooper,Sofia Douzgou Houge,Sixto García-Miñaúr,Hülya Kayserili,Lidia Larizza,Vanesa Lopez Gonzalez,Leonie A Menke,Donatella Milani,Francesco Saettini, Cathy A Stevens,Lloyd Tooke, Jill A Van der Zee,Maria M Van Genderen,Julien Van-Gils,Jane Waite,Jean-Louis Adrien,Oliver Bartsch,Pierre Bitoun, Antonia H M Bouts,Anna M Cueto-González, Elena Dominguez-Garrido, Floor A Duijkers, Patricia Fergelot, Elisabeth Halstead,Sylvia A Huisman, Camilla Meossi, Jo Mullins, Sarah M Nikkel,Chris Oliver,Elisabetta Prada, Alessandra Rei,Ilka Riddle, Cristina Rodriguez-Fonseca, Rebecca Rodríguez Pena, Janet Russell, Alicia Saba,Fernando Santos-Simarro,Brittany N Simpson, David F Smith, Markus F Stevens,Katalin Szakszon,Emmanuelle Taupiac, Nadia Totaro, Irene Valenzuena Palafoll, Daniëlle C M Van Der Kaay, Michiel P Van Wijk, Klea Vyshka, Susan Wiley,Raoul C Hennekam

Journal of medical genetics(2024)

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摘要
Rubinstein-Taybi syndrome (RTS) is an archetypical genetic syndrome that is characterised by intellectual disability, well-defined facial features, distal limb anomalies and atypical growth, among numerous other signs and symptoms. It is caused by variants in either of two genes (CREBBP, EP300) which encode for the proteins CBP and p300, which both have a function in transcription regulation and histone acetylation. As a group of international experts and national support groups dedicated to the syndrome, we realised that marked heterogeneity currently exists in clinical and molecular diagnostic approaches and care practices in various parts of the world. Here, we outline a series of recommendations that document the consensus of a group of international experts on clinical diagnostic criteria for types of RTS (RTS1: CREBBP; RTS2: EP300), molecular investigations, long-term management of various particular physical and behavioural issues and care planning. The recommendations as presented here will need to be evaluated for improvements to allow for continued optimisation of diagnostics and care.
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