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Los errores innatos de la inmunidad se asocian frecuentemente con bronquiectasias. Actualmente, el diagn\u00f3stico de los errores innatos de la inmunidad ha mejorado porque se conoce con certeza la asociaci\u00f3n de estas entidades con el da\u00f1o progresivo de las v\u00edas respiratorias. Esto ha permitido el reconocimiento y la intervenci\u00f3n adecuada, lo cual reduce el deterioro de la funci\u00f3n pulmonar y mejora la calidad de vida.Objetivo. Describir un grupo de pacientes con bronquiectasias no relacionadas con la fibrosis qu\u00edstica y con diagn\u00f3stico de errores innatos de la inmunidad, estudiados en un centro de referencia de inmunolog\u00eda en Cali, Colombia.Materiales y m\u00e9todos. Se desarroll\u00f3 un estudio observacional, descriptivo y retrospectivo de pacientes menores de 18 a\u00f1os con diagn\u00f3stico de errores innatos de la inmunidad y bronquiectasias no relacionadas con fibrosis qu\u00edstica, entre diciembre de 2013 y diciembre de 2023 en la Fundaci\u00f3n Valle del Lili, en Cali (Colombia).Resultados. Se incluyeron 17 pacientes con diagn\u00f3stico de bronquiectasias no relacionadas con fibrosis qu\u00edstica y errores innatos de la inmunidad, cuya edad media fue de nueve a\u00f1os. El inferior fue el l\u00f3bulo pulmonar m\u00e1s frecuentemente afectado y su compromiso fue unilateral en la mayor\u00eda de los casos. La inmunodeficiencia con predominio de defectos de los anticuerpos fue la m\u00e1s com\u00fan, seguida de las inmunodeficiencias combinadas asociadas con s\u00edndromes. Trece pacientes presentaron compromiso de la inmunidad humoral y 4 pacientes, alteraciones en la inmunidad humoral y celular. En 12 pacientes se identificaron modificaciones gen\u00e9ticas relacionadas con su fenotipo. Trece pacientes recibieron suplemento de inmunoglobulina intravenosa y 3 fallecieron.Conclusi\u00f3n. La inmunodeficiencia con predominio de defectos de los anticuerpos, seguida de las inmunodeficiencias combinadas asociadas con caracter\u00edsticas sindr\u00f3micas, fueron los errores innatos de la inmunidad que con mayor frecuencia se acompa\u00f1aron de bronquiectasias no relacionadas con la fibrosis qu\u00edstica.<\/jats:p>","DOI":"10.7705\/biomedica.7558","type":"journal-article","created":{"date-parts":[[2025,1,9]],"date-time":"2025-01-09T15:50:52Z","timestamp":1736437852000},"page":"131-139","update-policy":"https:\/\/doi.org\/10.7705\/crossmark","source":"Crossref","is-referenced-by-count":0,"title":["Bronquiectasias no relacionadas con fibrosis qu\u00edstica en pediatr\u00eda: perfil de una cohorte de pacientes con errores innatos de la inmunidad en un centro de referencia de Cali, Colombia","Non-cystic fibrosis bronchiectasis in pediatrics: A cohort profile of patients with inborn errors of immunity at a referral center in Cali, Colombia"],"prefix":"10.7705","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3905-0104","authenticated-orcid":false,"given":"Andrea","family":"Murillo","sequence":"first","affiliation":[{"name":"Facultad de Ciencias de la Salud, Departamento de Medicina, Universidad ICESI, Cali, Colombia; 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