Successful Lung Transplantation in a Patient with Chronic Granulomatous Disease

Excerpt

Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder caused by defective NADPH oxidase and impaired production of superoxide that is necessary for effective intracellular killing of pathogens. Patients with CGD are prone to severe, recurrent bacterial and invasive fungal infections of the lungs, skin, GI tract, and liver. Pulmonary complications are common and include pneumonia, abscess, granulomatous disease, and chronic inflammation that can lead to fibrosis and bronchiectasis [1]. For those patients with CGD who develop end-stage lung disease, lung transplantation is a potential life-saving treatment, but necessitates life-long therapy with immunosuppressive medications. We present the first case of a successful double-lung transplant in a CGD patient with severe bronchiectasis and end-stage lung disease. …