Management of severe pulmonary Langerhans cell histiocytosis in children.
Langerhans cell histiocytosis
chemotherapy
cystic lung disease
pleurodesis
pneumothorax
Journal
Pediatric pulmonology
ISSN: 1099-0496
Titre abrégé: Pediatr Pulmonol
Pays: United States
ID NLM: 8510590
Informations de publication
Date de publication:
08 2020
08 2020
Historique:
received:
12
02
2020
revised:
21
04
2020
accepted:
06
05
2020
pubmed:
9
6
2020
medline:
20
11
2020
entrez:
9
6
2020
Statut:
ppublish
Résumé
Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.
Identifiants
pubmed: 32511892
doi: 10.1002/ppul.24822
pmc: PMC7771630
mid: NIHMS1602673
doi:
Types de publication
Case Reports
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2074-2081Subventions
Organisme : NCI NIH HHS
ID : R01CA154947
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA154489
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA126752
Pays : United States
Organisme : NCI NIH HHS
ID : K12 CA090433
Pays : United States
Organisme : NCI NIH HHS
ID : P50CA126752
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA154947
Pays : United States
Organisme : NCI NIH HHS
ID : R01CA154489
Pays : United States
Organisme : NCATS NIH HHS
ID : L40 TR001681
Pays : United States
Organisme : NCI NIH HHS
ID : K12CA090433
Pays : United States
Informations de copyright
© 2020 Wiley Periodicals LLC.
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