Primary ciliary dyskinesia (PCD) is an autosomal recessive disease in which ciliary dysfunction leads to chronic lung, sinus, and middle ear disease. PCD is often not diagnosed until late childhood due to its presumed rarity and the technical expertise necessary for diagnosis; as such, little is known about lung disease in young children with PCD. We report on 3 young children with PCD who had evidence of lung disease on infant pulmonary function testing, bronchoscopy, and/or computed tomography (CT) of the chest before 3 years of age.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/ppul.20792 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Clinical remission in patients with severe eosinophilic asthma treated with mepolizumab: A analysis of RELIght study.
Allergy Asthma Proc
January 2025
2nd Respiratory Department, Attikon University Hospital, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Remission of asthma can occur as part of the natural history of the disease; however, the use of biologics can result in disease remission in some patients. In this post hoc analysis of the RELIght study, we aimed to evaluate clinical remission in real life among patients treated with mepolizumab, to detect possible differences between "remitters" and "nonremitters," and to evaluate possible predictors of remission. Clinical remission was defined as the absence of asthma exacerbations, discontinuation of oral corticosteroids (OCS), achievement of asthma control (Asthma Control Test [ACT] ≥ 20), and stable or improved lung function.
View Article and Find Full Text PDFExosomes loaded with the anti-cancer molecule mir-1-3p inhibit intrapulmonary colonization and growth of human esophageal squamous carcinoma cells.
J Transl Med
December 2024
Gastroenterology Department, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No.324 JingwuWeiqi Road, Jinan, Shandong, 250021, China.
Background: The overall prognosis of patients with esophageal cancer (EC) is extremely poor. There is an urgent need to develop innovative therapeutic strategies. This study will investigate the anti-cancer effects of exosomes loaded with specific anti-cancer microRNAs in vivo and in vitro.
View Article and Find Full Text PDFIdentification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study.
Respir Res
December 2024
National Jewish Health, Denver, USA.
Background: We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process.
Methods: Following a literature review, statements on the prediction, identification and monitoring of progression of ILD were developed by a panel of physicians with specialist expertise. Practising respiratory physicians were sent a survey asking them to indicate their level of agreement with these statements on a binary scale or 7-point Likert scale (- 3 to 3), or to select answers from a list.
Efficacy of transbronchial biopsy and frequency of pneumothorax in lung transplant recipients: single center experience.
BMC Pulm Med
December 2024
Department of Thoracic Surgery, Kartal Kosuyolu High Specialization Education & Research Hospital, Istanbul, Turkey.
Objective: Bronchoscopy plays a critical role in the diagnosis and management of lung transplant recipients. We retrospectively evaluated the safety, complications, and efficacy of transbronchial biopsy (TBB) in detecting and grading early rejection.
Materials And Methods: We retrospectively assessed the complications associated with TBB and the adequacy of pathological diagnoses in patients who underwent lung transplantation at Koşuyolu Yüksek İhtisas Training and Research Hospital from December 1, 2016, to April 30, 2023.
A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma.
BMC Cancer
December 2024
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China.
Objective: The aim of this study is to explore the clinicopathological features, radiographic manifestations, treatment options, and prognosis of primary pulmonary angiosarcoma (PPAS).
Method: We summarized and analyzed the clinical data of 11 patients with primary pulmonary angiosarcoma treated at the First Affiliated Hospital of Guangzhou Medical University between January 2018 and January 2024. A retrospective analysis was conducted in conjunction with a review of the relevant literature.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!