The importance of dental care for a child with severe congenital neutropenia: a case report.
Congenital neutropenia is characterized by a decrease in the absolute number of circulating neutrophils and an increased susceptibility to infections. This paper describes the case history of a child with severe congenital neutropenia who had aggressive periodontitis associated with generalized carious lesions. She had Kostmann syndrome, which is associated with recessive mutation of the HAX-1 gene. She had extensive dental caries, which is not common in patients with Kostmann syndrome. The caries caused oral pain and loss of weight and could have been avoided if the parents had received early oral hygiene instructions, and if the child had received regular, professional dental care.
Department of Pediatric Dentistry and Orthodontics, School of Dentistry, Federal University of Rio de Janeiro, Brazil.
Andréa Gonçalves
AG
Antonio
Paula Cristina da Costa
PC
Alcantara
Maria Eliza Barboza
ME
Ramos
Ivete Pomarico Ribeiro
IP
de Souza
D002675:Child, Preschool; D000080984:Congenital Bone Marrow Failure Syndromes; D019140:Dental Care for Children; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000150:complications* / Q000628:therapy; D005260:Female; D005891:Gingivitis / Q000150:complications / Q000628:therapy; D006801:Humans; D009503:Neutropenia / Q000150:complications / Q000151:congenital; D010518:Periodontitis / Q000150:complications* / Q000628:therapy; D014094:Tooth, Deciduous