title: Kostmann syndrome: oral aspects and 10-year follow-up case report.
abstract: Kostmann syndrome (KS) is an autosomal recessive disorder characterized by a low neutrophil count and recurrent bacterial infections, including periodontal disease. This report describes the case of a 5-year-old female KS patient treated with regular infusions of granulocyte stimulating factor (granulokine), with primary complaint of gingival bleeding and tooth mobility, and followed up for 10 years. Conventional periodontal treatment and some extractions were performed, after neutrophil function tests using flow cytometry. The invasive procedures were carried out at an outpatient clinic under antibiotic prophylaxis due to a low neutrophil count and an impaired neutrophil function presented. During the 10-year follow-up period, despite episodes of recurrent gingivitis and periodontitis the present report describes the positive outcome of dental treatment of a patient with KS.
authors.affiliation: Postdoctoral Researcher, Special Care Dentistry Center, Dental School, University of São Paulo, São Paulo, Brazil. karinsf@gmail.com.
authors.forename: Karin Sá
authors.initials: KS
authors.lastname: Fernandes
authors.affiliation: Assistant Professor, Department of Stomatology, Bauru School of Dentistry, University of São Paulo, São Paulo, Brazil.
authors.forename: Paulo Sérgio
authors.initials: PS
authors.lastname: da Silva Santos
authors.affiliation: Assistant Professor, Centro Universitário Hermínio Ometto - UNIARARAS.
authors.forename: Nathalie Pepe Medeiros
authors.initials: NP
authors.lastname: de Rezende
authors.affiliation: Head and Full Professor, Special Care Dentistry Center, Dental School, University of São Paulo, São Paulo, Brazil.
authors.forename: Marina
authors.initials: M
authors.lastname: Gallottini
mesh_terms: D002675:Child, Preschool; D000080984:Congenital Bone Marrow Failure Syndromes; D019140:Dental Care for Children; D017742:Dental Care for Chronically Ill; D005260:Female; D006801:Humans; D009059:Mouth Diseases / Q000209:etiology* / Q000628:therapy*; D009503:Neutropenia / Q000150:complications / Q000151:congenital*
keywords: Kostmann syndrome; bacterial infections; neutrophil; periodontal disease