A Case of Atypical Maxillary Sinusitis Accompanied with Papillon-Lefevre Syndrome / 대한이비인후과학회지
Korean Journal of Otolaryngology - Head and Neck Surgery
;
: 456-459, 2013.
Artículo
en Coreano
| WPRIM
| ID: wpr-645884
ABSTRACT
Papillon-Lefevre syndrome (PLS) is an extremely rare autosomal recessive disorder characterized by palmoplantar keratoderma and periodontitis and occuring with an estimated incidence of 1-4 cases per million. Patients with PLS are highly susceptible to infection. The etiology of an infective susceptibility is unknown; however, an association with defects in neurophil dysfunction, insufficient lymphocyte response to pathogens, defects in monocyte functions and impairment of NK cell cytotoxic function has been suggested. To our knowledge, this is the first case report of atypical maxillary sinusitis accompanied by PLS, and we represent the case with a review of the related literatures.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Enfermedad de Papillon-Lefevre
/
Periodontitis
/
Células Asesinas Naturales
/
Linfocitos
/
Monocitos
/
Sinusitis Maxilar
/
Incidencia
/
Queratodermia Palmoplantar
/
Seno Maxilar
Tipo de estudio:
Estudio de incidencia
/
Estudio pronóstico
Límite:
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Otolaryngology - Head and Neck Surgery
Año:
2013
Tipo del documento:
Artículo
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