Literature DB >> 35174959

KBG syndrome in a Chinese population: A case series.

Stephanie Ho1, Ho-Ming Luk1, Ivan F M Lo1.   

Abstract

KBG syndrome (OMIM #148050) is an autosomal dominant neurodevelopmental disorder characterized by the presence of macrodontia of the permanent central upper incisors, characteristic facial features, delay in development, intellectual disability, short stature, and various skeletal abnormalities. Over 200 affected individuals have been described worldwide, though underdiagnosis is suspected because the characteristic features are variably present and affected individuals can have a mild phenotype. This case series provides a summary of the clinical and molecular characteristics of 10 Chinese KBG syndrome patients recruited from a single center. To our knowledge, this is the first case series for Chinese KBG patients. This case series aimed at exploring potential ethnicity-related variability in KBG syndrome.
© 2022 Wiley Periodicals LLC.

Entities:  

Keywords:  ANKRD11; Chinese; KBG syndrome; macrodontia

Mesh:

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Year:  2022        PMID: 35174959     DOI: 10.1002/ajmg.a.62688

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  2 in total

1.  Expanding the Molecular Spectrum of ANKRD11 Gene Defects in 33 Patients with a Clinical Presentation of KBG Syndrome.

Authors:  Ilaria Bestetti; Milena Crippa; Alessandra Sironi; Francesca Tumiatti; Maura Masciadri; Marie Falkenberg Smeland; Swati Naik; Oliver Murch; Maria Teresa Bonati; Alice Spano; Elisa Cattaneo; Milena Mariani; Fabio Gotta; Francesca Crosti; Pietro Cavalli; Chiara Pantaleoni; Federica Natacci; Maria Francesca Bedeschi; Donatella Milani; Silvia Maitz; Angelo Selicorni; Luigina Spaccini; Angela Peron; Silvia Russo; Lidia Larizza; Karen Low; Palma Finelli
Journal:  Int J Mol Sci       Date:  2022-05-25       Impact factor: 6.208

2.  Obsessive Compulsive "Paper Handling": A Potential Distinctive Behavior in Children and Adolescents with KBG Syndrome.

Authors:  Francesco Demaria; Paolo Alfieri; Maria Cristina Digilio; Maria Pontillo; Cristina Di Vincenzo; Federica Alice Maria Montanaro; Valentina Ciullo; Giuseppe Zampino; Stefano Vicari
Journal:  J Clin Med       Date:  2022-08-11       Impact factor: 4.964

  2 in total

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