Chediak-Higashi syndrome and premature exfoliation of primary teeth.
The Chediak-Higashi syndrome (CHS) is a rare hereditary fatal disease, if not treated. These changes are associated with various diseases and syndromes that mainly cause periodontal disease and thus the premature loss of teeth. This paper describes the monitoring of premature loss of primary teeth that began when the child was 5 years old. On presentation his teeth were mobile and there was a history of gingival bleeding. Panoramic radiography revealed generalized and severe bone loss, and the teeth showed no bony support enough for their stability. Blood test was ordered to assess the overall health of the child and giant cells with cytoplasmic granules were found, confirming the diagnosis of CHS. The management of periodontal disease focused on the control of infection and bacterial plaque by mechanical and chemical methods.
Department of Pediatric Dentistry, School of Dentistry, USP - University of São Paulo, São PauloSP, Brazil.
Karla Mayra
KM
Rezende
Department of Pediatric Dentistry, Universidad Autonoma de Asuncion, Asuncion, Paraguay.
Alfredo Hiram Carrillo
AH
Canela
Department of Pediatric Dentistry, School of Dentistry, USP - University of São Paulo, São PauloSP, Brazil.
Adriana Oliveira Lira
AO
Ortega
Department of Pediatric Dentistry, Universidad Autonoma de Asuncion, Asuncion, Paraguay.
Claudia
C
Tintel
Department of Pediatric Dentistry, School of Dentistry, USP - University of São Paulo, São PauloSP, Brazil.
Marcelo
M
Bönecker
D016301:Alveolar Bone Loss / Q000000981:diagnostic imaging; D002609:Chediak-Higashi Syndrome / Q000175:diagnosis*; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D011862:Radiography, Panoramic; D014080:Tooth Exfoliation / Q000209:etiology*