Title
Lung transplantation in gaucher disease a learning lesson in trying to avoid both scylla and charybdis Lung transplantation in gaucher disease a learning lesson in trying to avoid both scylla and charybdis
Author
Faculty/Department
Faculty of Medicine and Health Sciences
Publication type
article
Publication
Chicago, Ill. ,
Subject
Human medicine
Source (journal)
Chest. - Chicago, Ill.
Volume/pages
149(2016) :1 , p. E1-E5
ISSN
0012-3692
ISI
000369655600001
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
Gaucher disease (GD), a lysosomal storage disorder, may result in end-stage lung disease. We report successful bilateral lung transplantation in a 49-year-old woman with GD complicated by severe pulmonary hypertension and fibrotic changes in the lungs. Before receiving the lung transplant, the patient was undergoing both enzyme replacement therapy (imiglucerase) and triple pulmonary hypertension treatment (epoprostenol, bosentan, and sildenafil). She had a history of splenectomy, severe bone disease, and renal involvement, all of which were related to GD and considered as relative contraindications for a lung transplantation. In the literature, lung transplantation has been suggested for severe pulmonary involvement in GD but has been reported only once in a child. To our knowledge, until now, no successful procedure has been reported in adults, and no reports deal with the severe potential posttransplantation complications specifically related to GD.
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https://repository.uantwerpen.be/docman/iruaauth/cb00d3/132258.pdf
Handle