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MINERVA STOMATOLOGICA

A Journal on Dentistry and Maxillofacial Surgery


Official Journal of the Italian Society of Odontostomatology and Maxillofacial Surgery
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Minerva Stomatologica 2011 July-August;60(7/8):391-402

language: English, Italian

Oral features in five adult patients with Wolf-Hirschhorn syndrome

Dellavia C. 1, Raiteri S. 2, Ottolina P. 2, Pregliasco F. 3

1 Department of Human Morphology and Biomedical Sciences “Città Studi”, Faculty of Medicine and Surgery, Università degli Studi di Milano, Milan, Italy;
2 Foundation Institute Sacra Famiglia Onlus, Cesano Boscone, Milan, Italy;
3 Department of Public Health, Microbiology and Virology, Università degli Studi di Milano, Foundation Institute Sacra Famiglia Onlus, Cesano Boscone, Milan, Italy


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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.

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claudia.dellavia@unimi.it