title: Oral features in five adult patients with Wolf-Hirschhorn syndrome.
abstract: The Wolf-Hirschhorn syndrome (WHS) is a rare genetic condition presenting with severe mental disability, growth retardation, muscular hypotonia, seizures, craniofacial abnormalities and defects in the cardiovascular, genitourinary and digestive apparata. To date, few data about oral status of afflicted patients were reported, and this syndrome is still unfamiliar among dental and maxillofacial professionals. Aim of the present case series was to provide oral data from five patients with WHS (3 women and 2 men) aged 19-41 years. All patients entered in a long-stay Institution with an inner dental service in 1998 and underwent regular dental examination and hygiene treatment. Neither tooth agenesis or dental size and shape anomalies were found in the analyzed subjects except for one man showing multiple cone-shaped teeth. At the beginning, bad dental and periodontal conditions with gingival signs and recurrent mucosal inflammation were found in all patients. After motivation of their tutors, dental and periodontal parameters were recorded during periodic assessments and a large decrease in the gingival index was found over time. These data could aware dentists about the therapeutic modalities to improve oral health of WHS patients.
authors.affiliation: Department of Human Morphology and Biomedical Sciences "CittàStudi"Faculty of Medicine and Surgery, Universitàdegli Studi di Milano, Milan, Italy. claudia.dellavia@unimi.it
authors.forename: C
authors.initials: C
authors.lastname: Dellavia
authors.forename: S
authors.initials: S
authors.lastname: Raiteri
authors.forename: P
authors.initials: P
authors.lastname: Ottolina
authors.forename: F
authors.initials: F
authors.lastname: Pregliasco
mesh_terms: D000328:Adult; D017028:Caregivers / Q000523:psychology; D003728:Dental Calculus / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D003730:Dental Care for Persons with Disabilities; D003731:Dental Caries / Q000209:etiology / Q000517:prevention & control / Q000628:therapy; D005260:Female; D005891:Gingivitis / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D006801:Humans; D007326:Institutionalization; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D008318:Malpractice; D010512:Periodontal Index; D010518:Periodontitis / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D016388:Tooth Loss / Q000209:etiology / Q000517:prevention & control; D054877:Wolf-Hirschhorn Syndrome / Q000473:pathology* / Q000523:psychology; D055815:Young Adult