Publications by authors named "A Hadchouel"
The main monogenic causes of pulmonary fibrosis in adults are mutations in telomere-related genes. These mutations may be associated with extrapulmonary signs (hepatic, haematological and dermatological) and typically present radiologically as usual interstitial pneumonia or unclassifiable fibrosis. In children, the monogenic causes of pulmonary fibrosis are dominated by mutations in surfactant-related genes.
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- Pulmonary alveolar proteinosis (PAP) is a rare lung syndrome characterized by the accumulation of proteinaceous material, leading to symptoms like progressive dyspnea and hypoxemia, with various diagnostic methods such as CT scans, bronchoalveolar lavage, and genetic testing suggested for evaluation.
- A European Respiratory Society Task Force, comprised of diverse experts, developed evidence-based guidelines for diagnosing and managing PAP using a systematic review of literature and the GRADE approach for assessing the strength of recommendations.
- The Task Force provided specific management recommendations, including whole lung lavage, GM-CSF therapy, and potential treatments like rituximab, alongside diagnostic approaches involving GM-CSF antibody
Unlabelled: Computed tomography (CT) is commonly used for paediatric thoracic diseases but involves radiation exposure and often requires intravenous contrast. We evaluated the performance of a magnetic resonance imaging (MRI) protocol including a 3D zero echo time (3D-ZTE) sequence for radiation-free and contrast-free imaging of the paediatric chest. In this prospective, single-centre study, children aged 6-16 years underwent chest CT and MRI within 48 h.
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- A study examined the prevalence and incidence of interstitial lung disease in children (chILD) in France, finding it affects 44 children per million in 2022 and has an incidence of 4.4 per million.
- The research included data from 790 patients across 42 centers, noting that the median age for diagnosis was 3 months, with a significant portion having familial forms.
- Management typically involved oxygen therapy and corticosteroids, with survival rates being 57.3% for children diagnosed before 2 years and 86% for those diagnosed between 2 and 18 years; the study highlights the need for improved international data collection and standardized practices.