Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Background Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. Methods Among 1482 children (

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Veröffentlicht in:	Orphanet journal of rare diseases 2020-09, Vol.15 (1), p.1-241, Article 241
Hauptverfasser:	Le Louet, Solenne, Barkaoui, Mohamed-Aziz, Miron, Jean, Galambrun, Claire, Aladjidi, Nathalie, Chastagner, Pascal, Kebaili, Kamila, Armari-Alla, Corinne, Lambilliotte, Anne, Lejeune, Julien, Moshous, Despina, Della Valle, Valeria, Sileo, Chiara, Ducou Le Pointe, Hubert, Chateil, Jean-François, Renolleau, Sylvain, Piloquet, Jean-Eudes, Portefaix, Aurelie, Epaud, Ralph, Chiron, Raphaël, Bugnet, Emmanuelle, Lorillon, Gwenaël, Tazi, Abdelatif, Emile, Jean-François, Donadieu, Jean, Héritier, Sébastien
Format:	Artikel
Sprache:	eng
Schlagworte:	Autoimmune diseases Biopsy Childhood Children Cladribine Classification Cohort analysis Corticosteroids Cysts Dyspnea Etoposide Genetics Histiocytosis Hospitalization Infiltration Intensive care Langerhans cell histiocytosis Life Sciences Lung diseases Lung transplantation Lungs Medical prognosis Medical research Mutation Pathogenesis Patients Pneumothorax Prophylaxis Pulmonary Rare diseases Respiratory failure Severe Spleen Targeted therapy Vinblastine Young adults
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Beschreibung
Zusammenfassung:	Background Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. Methods Among 1482 children (
ISSN:	1750-1172 1750-1172
DOI:	10.1186/s13023-020-01495-5