Rubinstein-Taybi syndrome, medical and dental care for special needs patients: Clinical case report

Jorge Alejandro Reyes Saberbein, Roberto Paúl Paz Soldan Medina, Raúl Claver Palian Pucumucha, Úrsula Margarita Gutiérrez Samaniego

Resumen


Medical and dental care for special needs patients oftentimes
require effective and rapid clinical approach so as to improve
patients’ quality of life. Rubinstein-Taybi syndrome is a rare disorder where anomalies in genes CREBBP and EP300 are observed. This syndrome was fi rst described in 1963 by two North American physicians. Among the main characteristics and manifestations of this syndrome we can count mental retardation ( moderate or severe) microcephaly, thickened first finger or toe, growth retardation, characteristic face with hypoplastic lower jaw, downward-sloping arched palate, palpebral fissure, irregularly implanted teeth, crowded teeth, strabismus, low-set, malformed ears, beak-shaped nose, bushy eyebrows and long eyelids. The concept of patient adaptation to the dental office entails achieving and preserving his collaboration during treatment by means of a show-and-tell process. Behavior was modeled with gradual exposition, positive reinforcement and active physical restraint with the mother´s help.
Results: The patient’s oral health was satisfactorily reestablished


Palabras clave


Dental clinical approach, Rubinstein-Taybi syndrome.

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DOI: http://dx.doi.org/10.1016/j.rodmex.2016.08.017


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ISSN: 1870-199X