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The lung in inborn errors of immunity.
Restrepo-Gualteros, Sonia; Nino, Gustavo; Gutierrez, Maria J.
Afiliação
  • Restrepo-Gualteros S; School of Medicine, Universidad Nacional de Colombia, Bogotá, Colombia.
  • Nino G; Department of Pediatrics, Fundación Santa Fé de Bogotá, Bogotá, Colombia.
  • Gutierrez MJ; Division of Pulmonary and Sleep Medicine, Children's National Hospital, George Washington University, Washington, District of Columbia, USA.
Pediatr Pulmonol ; 2024 Sep 19.
Article em En | MEDLINE | ID: mdl-39295479
ABSTRACT
The lungs are integral to immune defense, and inborn errors of immunity (IEI) often manifest as lung disease. Lung complications of IEI can involve the airways, alveolar spaces, interstitium, vasculature, and pleura. Accurate identification of these lung disease patterns requires a thorough clinical history, physical examination, and high-resolution computed tomography (HRCT), as lung imaging patterns guide further respiratory and immunological evaluations. Respiratory assessment may also include pulmonary function tests, bronchoscopy with bronchoalveolar lavage, and, in some cases, lung biopsy. Additionally, molecular diagnosis of underlying immune defects, typically through comprehensive clinical phenotyping, functional immune studies, and genetic testing, is crucial for informing patient management and guiding targeted therapies. Importantly, given the complexity of IEI, a multidisciplinary approach is necessary. Furthermore, ongoing research is required to refine therapies and improve outcomes for lung complications.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Pediatr Pulmonol Assunto da revista: PEDIATRIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Colômbia País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Pediatr Pulmonol Assunto da revista: PEDIATRIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Colômbia País de publicação: Estados Unidos