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.

A síndrome de Chediak-Higashi (CHS) é uma doença rara hereditária e fatal se não for tratada. Estas alterações estão associadas com várias doenças e síndromes que, principalmente, causa a doença periodontal assim como perda prematura de dentes. Este artigo descreve o monitoramento de perda prematura de dentes decíduos, que começou quando a criança tinha 05 anos. Na apresentação os dentes estavam com mobilidade e apresentava sangramento gengival. As radiografias panorâmicas revelaram perda óssea generalizada e grave, e os dentes sem suporte ósseo suficiente para a sua estabilidade. Foi realizada o exame de sangue para avaliar a saúde geral da criança no qual foi encontrado células gigantes com grânulos citoplasmáticos, confirmando o diagnóstico de CHS. O tratamento da doença periodontal por meio de métodos mecânicos e químicos são necessários para controle da infecção e da placa bacteriana.

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.