title: Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis.
abstract: Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed.
authors: Pediatric Pulmonology and Allergy Unit and Cystic Fibrosis Center, Department of Pediatrics, Istituto Giannina Gaslini, Genoa.
authors: Oliviero
authors: O
authors: Sacco
authors: Department of Critical Care and Perinatal Medicine, Neonatal and Pediatric Intensive Care Unit, Istituto Giannina Gaslini, Genoa.
authors: Andrea
authors: A
authors: Moscatelli
authors: Department of Oncology, Istituto Giannina Gaslini, Genoa.
authors: Massimo
authors: M
authors: Conte
authors: Department of Critical Care and Perinatal Medicine, Neonatal and Pediatric Intensive Care Unit, Istituto Giannina Gaslini, Genoa.
authors: Chiara
authors: C
authors: Grasso
authors: Department of Radiology, Istituto Giannina Gaslini, Genoa.
authors: Gian Michele
authors: GM
authors: Magnano
authors: Department of Pathology, Istituto Giannina Gaslini, Genoa.
authors: Angela Rita
authors: AR
authors: Sementa
authors: Department of Pediatrics, G. Salvini Hospital, Garbagnate Milanese, (Milan), Italy.
authors: Alberto
authors: A
authors: Martelli
authors: Pediatric Pulmonology and Allergy Unit and Cystic Fibrosis Center, Department of Pediatrics, Istituto Giannina Gaslini, Genoa.
authors: Giovanni A
authors: GA
authors: Rossi
mesh_terms: D002648:Child; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods*; D006646:Histiocytosis, Langerhans-Cell / Q000628:therapy*; D006801:Humans; D008171:Lung Diseases / Q000628:therapy*; D016040:Lung Transplantation; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage; D013997:Time Factors; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D011239:Prednisolone
keywords: critical care medicine; histiocytosis; transplantation