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Lung Transplantation in Gaucher Disease: A Learning Lesson in Trying to Avoid Both Scylla and Charybdis

Geertje M. de Boer, BSc; Laura van Dussen, MD, PhD; Leon M. van den Toorn, MD, PhD; Michael A. den Bakker, MD, PhD; Rogier A.S. Hoek, MD; Dennis A. Hesselink, MD, PhD; Carla E.M. Hollak, MD, PhD; Peter Th. W. van Hal, MD, PhD
Author and Funding Information

Dr van Hal is presently with the Department of Respiratory Medicine, Antwerp University Hospital, Edegem, Belgium.

CORRESPONDENCE TO: Peter Th. W. van Hal, Department of Respiratory Medicine, Antwerp University Hospital, Wilrijkstraat 10, 2650 Edegem, Belgium


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(1):e1-e5. doi:10.1016/j.chest.2015.09.011
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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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