التفاصيل البيبلوغرافية
| العنوان: |
Pediatric interstitial lung disease |
| المؤلفون: |
Bush, Andrew, Gilbert, Carlee, Gregory, Jo, Nicholson, Andrew Gordon, Semple, Thomas, Zampoli, Marco, Pabary, Rishi |
| المصدر: |
Journal of the Pan African Thoracic Society; Vol. 2 No. 1 (2021); 18-32 ; 2694-4561 |
| بيانات النشر: |
Scientific Scholar LLC |
| سنة النشر: |
2022 |
| المجموعة: |
AJOL - African Journals Online |
| مصطلحات موضوعية: |
Alveolar capillary dysplasia, Congenital alveolar dysplasia, Hypersensitivity pneumonitis, Lipoid pneumonia, Neuroendocrine cell hyperplasia of infancy, Pulmonary alveolar proteinosis, Pulmonary interstitial glycogenosis, Surfactant protein gene |
| الوصف: |
Interstitial lung disease in children (chILD) is rare and encompasses more than 200 entities, with new especially genetic causes being discovered. Several classifications have been proposed, and there is considerable overlap with entities which present in adult life. Presentation may be shortly after birth with acute respiratory distress and in infancy and childhood either with a primary respiratory presentation or with systemic symptoms such as poor feeding and failure to thrive. Newborn acute presentations are usually due either to a mutation in one of the surfactant protein (Sp) genes or the alveolar capillary dysplasia (ACD)-congenital alveolar dysplasia (CAD) spectrum. The latter usually progress rapidly to extracorporeal membrane oxygenation, and early lung biopsy is advisable to prevent prolonged futile treatment being offered. Outside the newborn period, a staged protocol for investigation is proposed. This starts with a computed tomography scan, which confirms or otherwise the presence of chILD, and occasionally can lead to a specific diagnosis. In particular in settings where there is a high burden of infection, infective mimics of chILD need to be excluded. The next investigations aim to try to move from pattern recognition to specific diagnoses, both genetic and environmental. The speed of progression to lung biopsy will depend on the clinical state of the child, and the biopsy itself may suggest a hunt for a new underlying cause, such as immunodeficiency. Specific genetic causing chILD includes mutations in SpB and SpC and processing genes (thyroid transcription factor-1 [TTF-1] and adenosine triphosphate-binding cassette subfamily A) (the last three can present at any time in the life course); genes involved in Sp catabolism (granulocyte-macrophage colony factor receptor A and B genes), an increasing number implicated in the ACD-CAD spectrum, and other non-Sp related genes such as Filamin-A and integrin genes. Environmental causes are also important and vary across the world. Vaping has been ... |
| نوع الوثيقة: |
article in journal/newspaper |
| وصف الملف: |
application/pdf |
| اللغة: |
English |
| العلاقة: |
https://www.ajol.info/index.php/jpats/article/view/239144/226008Test; https://www.ajol.info/index.php/jpats/article/view/239144Test |
| الإتاحة: |
https://www.ajol.info/index.php/jpats/article/view/239144Test |
| حقوق: |
https://creativecommons.org/licenses/by-nc-sa/4.0Test |
| رقم الانضمام: |
edsbas.E7051C74 |
| قاعدة البيانات: |
BASE |
