Open AccessSuccessful liver transplantation in common variable immune deficiency with reversal of hepatopulmonary syndrome
Author(s) -
Ross Apostolov,
Marie Sinclair,
Julie Lokan,
Peter W Angus
Publication year - 2019
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2018-226095
Subject(s) - common variable immunodeficiency , hepatopulmonary syndrome , medicine , liver transplantation , liver disease , complication , transplantation , liver function tests , gastroenterology , immune dysregulation , disease , immunology , antibody
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.