Successful liver transplantation in common variable immune deficiency with reversal of hepatopulmonary syndrome

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...

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Bibliographic Details
Published in:BMJ case reports 2019-04, Vol.12 (4), p.e226095
Main Authors: Apostolov, Ross, Sinclair, Marie, Lokan, Julie, Angus, Peter
Format: Article
Language:English
Subjects:
Bacterial infections
Biochemistry
Case reports
Common Variable Immunodeficiency - complications
Common Variable Immunodeficiency - physiopathology
Family medical history
Female
Gallbladder diseases
Hepatitis
Hepatopulmonary Syndrome - immunology
Hepatopulmonary Syndrome - surgery
Humans
Hypertension
Hypoxia
Infections
Liver - physiopathology
Liver diseases
Liver Transplantation - methods
Liver transplants
Middle Aged
Novel Treatment (New Drug/Intervention
Established Drug/Procedure in New Situation)
Patients
Transplants & implants
Treatment Outcome
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Description
Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2018-226095