Successful liver transplantation in common variable immune deficiency with reversal of hepatopulmonary syndrome. Issue 4 (3rd April 2019)

• Record Type: Journal Article
• Authors: Apostolov, Ross; Sinclair, Marie; Lokan, Julie; Angus, Peter
• Is Part Of: BMJ case reports. Volume 12:Issue 4(2019)
• Publication Date: 2019-04-03
• Subjects: portal hypertension -- immunology -- transplantation; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2018-226095
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Common variable immune deficiency (CVID) is a primary immunodeficiency disorder that is associated with abnormal liver function tests, however advanced liver disease is uncommon. Hepatopulmonary syndrome (HPS) is a rare but debilitating complication of CVID-associated liver disease. We report a case of CVID complicated by HPS that was successfully treated with orthotopic liver transplant, with the patient recovering to normal hepatic function and successfully weaning off domiciliary oxygen post-transplantation.