title: Dental findings and special care in patients with Angelman syndrome: a report of three cases.
abstract: Angelman syndrome (AS) is a neurogenetic disorder, characterized by intellectual disability, movement or balance disorders, specific abnormal behaviors, and severe speech and language limitations. Due to its low incidence and the nonspecifity of developmental problems in newborns and young children, AS is not easily identified by clinical pediatricians. The aim of this paper is to present three cases of AS, reporting the orofacial characteristics and requisite dental care in these patients. Interestingly, this investigation found that certain typical features of mouth breathing syndrome, such as long and narrow faces, open mouth, shortened upper lip, lowered mandible position, shadows under the eyes (infraorbital cyanosis), muscular hypotonia, and enlarged and anteriorized tongue, were present in the three studied AS patients.
authors.affiliation: Department of Pediatric Clinics, Preventive and Community Dentistry, School of Dentistry of Riberão Preto, University of São Paulo, Ribeirão Preto, SP, Brazil.
authors.forename: Alexandra Mussolino
authors.initials: AM
authors.lastname: de Queiroz
authors.forename: Talitha
authors.initials: T
authors.lastname: de Siqueira Melara
authors.forename: Paula Dariana
authors.initials: PD
authors.lastname: Fernandes Ferreira
authors.forename: Marília Pacífico
authors.initials: MP
authors.lastname: Lucisano
authors.forename: Andiara
authors.initials: A
authors.lastname: De Rossi
authors.forename: Paulo
authors.initials: P
authors.lastname: Nelson-Filho
authors.forename: Raquel Assed
authors.initials: RA
authors.lastname: Bezerra Silva
mesh_terms: D017204:Angelman Syndrome / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D003730:Dental Care for Persons with Disabilities; D005145:Face / Q000002:abnormalities; D005260:Female; D006801:Humans; D008607:Intellectual Disability; D008046:Lip / Q000002:abnormalities; D008297:Male; D008310:Malocclusion / Q000209:etiology; D009048:Motor Skills / Q000502:physiology; D009056:Mouth Abnormalities / Q000209:etiology; D009058:Mouth Breathing / Q000209:etiology; D009123:Muscle Hypotonia / Q000209:etiology; D009909:Oral Health; D011378:Prognathism / Q000209:etiology; D014059:Tongue / Q000002:abnormalities; D055815:Young Adult