Errores innatos de la inmunidad: ¿Qué debe saber y cuándo debe sospechar el otorrinolaringólogo? / Inborn errors of immunity: What should the otolaryngologist know, and when to suspect them?

Introducción: Los errores innatos de la inmunidad, previamente conocidos como inmunodeficiencias primarias, son un grupo heterogéneo de patologías cuya presentación clínica incluye infecciones recurrentes, persistentes o refractarias al tratamiento en el campo de la otorrinolaringología. Materiales y métodos: Se realizó una revisión narrativa de la literatura a partir de la búsqueda de documentos en PUBMED y EMBASE. Discusión y conclusiones: Los pacientes con sospecha de error innato de la inmunidad requieren un diagnóstico temprano con el fin de disminuir las complicaciones a largo plazo, por lo que la valoración y el abordaje inicial desempeñan un papel fundamental en el reconocimiento de estas enfermedades.

Introduction: Inborn errors of immunity, previously known as primary immunodeficiencies, are a heterogeneous group of pathologies whose clinical presentation includes recurrent, persistent and/or refractory infections to treatment in otorhinolaryngology. Materials and methods: Narrative review of the literature was carried out from the search for articles in PUBMED and EMBASE. Discussion and conclusions: Patients with suspected inborn error of immunity require an early diagnosis to reduce long-term complications; the initial assessment and approach play a fundamental role in the recognition of these diseases

Infections of the ears, nose, and throat in children with primary immunodeficiencies.

Children who suffer from primary immunodeficiencies have long been thought to be subject to infections of the ears, nose, and throat due to unusual or resistant organisms. A retrospective chart review was undertaken at Children's Hospital of Pittsburgh from 1979 to 1989 to determine the types and frequency of infections of the ears, nose, and throat, and the bacteriologic findings from cultures of the sinuses, ears, and head and neck abscesses, when obtained. Seventy-five patients were identified with primary immunodeficiencies, and 80% suffered from infections of the ears, nose, and throat. Cultures obtained from 33% of the group showed the majority of the organisms commonly seen in ear and sinus infections. We conclude from this study that children with primary immunodeficiencies who require hospitalization frequently have an infection of the ears, nose, and throat, and that the infection is usually caused by community acquired bacteria. Empiric treatment may therefore be directed to common organisms causing these infections.