Background: Real-world data demonstrate variability in the response to elexacaftor/tezacaftor/ivacaftor (ETI) treatment among people with CF (pwCF). The aim of this study was to evaluate long-term outcomes in pwCF that had not shown early improvement in the percentage of predicted FEV (ppFEV) following ETI treatment.
Methods: A single-center prospective study in pwCF who initiated ETI.
Lung cancer is uncommon among people with cystic fibrosis (pwCF). We describe the case of a 35-year-old man with mild, stable CF disease who presented with severe respiratory distress, systemic symptoms, elevated liver enzymes and hypereosinophilia along with a lung mass and pleural effusion. The patient was subsequently diagnosed with non-small cell lung carcinoma (NSCLC), featuring anaplastic lymphoma kinase (ALK) translocation.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Background: Risk factors for progression of adenovirus (AdV)-associated bronchiolitis (AdV-B) to post-infectious bronchiolitis obliterans (PIBO) are poorly defined. We aimed to investigate this in a multicenter cohort.
Methods: A multicenter hospital-based analysis included children admitted with AdV-B in Jerusalem during 2016-2022.
Background: Although hematopoietic stem cell transplantation (HSCT) is the only curative option for some children with malignant and nonmalignant disorders, the procedure itself carries a high risk of complications. A proportion of children undergoing HSCT develop severe transplant-related complications requiring hospitalization in the pediatric intensive care unit (PICU).
Methods: A retrospective cohort study included 793 children with malignant and nonmalignant diseases that underwent 963 HSCTs in two large pediatric hospitals over 15 years.