Lung transplantation in a patient with Niemann –Pick disease

A 62-year-old, non-smoking, Caucasian male was evaluated for lung transplantation based on severe, upper lobe ‒predominant emphysema with superimposed fibrosis in the lower lung zones (Figure 1). The patient had no significant exposure history, and previous work-up for α1-anti-trypsin deficiency, autoimmune disease, and hypersensitivity pneumonitis was unremarkable. The diagnosis of Niemann‒Pick diseas e Type B was eventually established based on a compatible family history, with an almost identical disease presentation in a sibling.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: CASE ANECDOTES, COMMENTS AND OPINIONS Source Type: research