Periodontal disorders in a cohort of patients with Cohen syndrome

{Aims: Cohen syndrome (CS) is an uncommon autosomal recessive disorder due to mutations in vacuolar protein sorting 13B, with an intermittent presence of neutropenia. Contrary to other clinical phenotypic features, oral health has been little investigated in CS. We described oral health and dental hygiene in a cohort of CS patients. Methods and Results: Twelve CS patients with neutropenia (<1500/mm(3)) were recruited in the dental department of Dijon University Hospital (France). Patients underwent oral examination, and blood samples were collected. Oral health markers were described and compared between patients with moderate and severe neutropenia (<500/mm(3)). In 12 patients (mean age = 21.1 years