Pediatric Non-cystic Fibrosis Bronchiectasis in a Portuguese Tertiary Care Center: A Cross-Sectional Observational Study

葡萄牙一家三级医疗中心儿童非囊性纤维化支气管扩张症:一项横断面观察研究

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Abstract

Introduction Non-cystic fibrosis bronchiectasis (bronchiectasis) is an increasingly recognized but understudied disease in children. National data on this disease are scarce. This study aimed to describe the clinical, radiological, and microbiological characteristics of Portuguese children with bronchiectasis. Methods A retrospective observational study was conducted at a tertiary pediatric pulmonology center in northern Portugal. Pediatric patients diagnosed with bronchiectasis and followed between July 2020 and September 2023 were included. Results A total of 38 patients were included, of whom 19 (50.0%) were male, with a median age at diagnosis of 6.3 years (3.8-11.0 years). Recurrent wheezing (n = 30, 78.9%) and chronic wet cough (n = 18, 47.4%) were the most common symptoms. An underlying etiology was identified in 36 (94.7%) patients, primarily postinfectious bronchiectasis (n = 18, 47.4%) and primary ciliary dyskinesia (n = 10, 26.3%). Multilobar involvement was observed in 25 (65.8%) patients, most frequently affecting the middle and lower lobes. Spirometry showed a mixed obstructive-restrictive pattern in 10 (33.3%) patients and a predominantly obstructive pattern in nine (30.0%) patients. Haemophilus influenzae and Streptococcus pneumoniae were the most frequently isolated microorganisms, both in bronchoalveolar lavage and sputum cultures. Pseudomonas aeruginosa was detected in nine (7.4%) sputum samples. Conclusion This study highlights the diverse clinical presentations, etiologies, and microbiological findings in pediatric bronchiectasis. Identifiable causes were present in most cases, emphasizing the importance of clinical vigilance for early diagnosis and intervention. Further research is warranted to explore long-term outcomes and refine treatment approaches based on microbiological profiles.

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