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Open AccessArticle

Knowledge of Rare Respiratory Diseases among Paediatricians and Medical School Students

1
Paediatrics Unit, Hellín Hospital, 02400 Albacete, Spain
2
Fundación Investigación Hospital Clínico Universitario de Valencia/Instituto de Investigación Sanitaria INCLIVA, 46010 Valencia, Spain
3
Department of Physiology, School of Medicine, University of Valencia, 46010 Valencia, Spain
4
Paediatrics Pulmonology Unit, Hospital Clínico Universitario Valencia, 46010 Valencia, Spain
5
Department of Paediatrics, Obstetrics and Gynaecology, School of Medicine, University of Valencia, 46010 Valencia, Spain
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(3), 869; https://doi.org/10.3390/jcm9030869
Received: 10 March 2020 / Revised: 15 March 2020 / Accepted: 18 March 2020 / Published: 22 March 2020
(This article belongs to the Special Issue Rare Respiratory Diseases: A Personal and a Public Health Problem)
Alpha-1-antitrypsin deficiency (AATD) and primary ciliary dyskinesia (PCD) are underdiagnosed rare diseases showing a median diagnostic delay of five to ten years, which has negative effects on patient prognosis. Lack of awareness and education among healthcare professionals involved in the management of these patients have been suggested as possible causes. Our aim was to assess knowledge of these diseases among paediatricians and medical school students to determine which knowledge areas are most deficient. A survey was designed with questions testing fundamental aspects of the diagnosis and treatment of AATD and PCD. A score equal to or greater than 50% of the maximum score was set as the level necessary to ensure a good knowledge of both diseases. Our results indicate a profound lack of knowledge of rare respiratory diseases among paediatric professionals and medical students, suggesting that it is necessary to increase rare respiratory diseases training among all physicians responsible for suspecting and diagnosing them; this will allow early diagnosis and the setup of preventive measures and appropriate early-stage treatment. The first step in closing this knowledge gap could be to include relevant material in the medical syllabus. View Full-Text
Keywords: alpha-1 antitrypsin deficiency; primary ciliary dyskinesia; rare respiratory diseases alpha-1 antitrypsin deficiency; primary ciliary dyskinesia; rare respiratory diseases
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Requena-Fernández, M.Á.; Dasí, F.; Castillo, S.; Barajas-Cenobi, R.; Navarro-García, M.M.; Escribano, A. Knowledge of Rare Respiratory Diseases among Paediatricians and Medical School Students. J. Clin. Med. 2020, 9, 869.

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