Abstract:
:Patients with common variable immunodeficiency disorders are monitored for liver function test abnormalities. A proportion of patients develop deranged liver function and some also develop hepatomegaly. We investigated the prevalence of abnormalities and types of liver disease, aiming to identify those at risk and determine outcomes. The local primary immunodeficiency database was searched for patients with a common variable immunodeficiency disorder and abnormal liver function and/or a liver biopsy. Patterns of liver dysfunction were determined and biopsies reviewed. A total of 47 of 108 patients had deranged liver function, most commonly raised alkaline phosphatase levels. Twenty-three patients had liver biopsies. Nodular regenerative hyperplasia was found in 13 of 16 with unexplained pathology. These patients were more likely to have other disease-related complications of common variable immunodeficiency disorders, in particular non-coeliac (gluten insensitive) lymphocytic enteropathy. However, five had no symptoms of liver disease and only one died of liver complications. Nodular regenerative hyperplasia is a common complication of common variable immunodeficiency disorders but was rarely complicated by portal hypertension.
journal_name
Clin Exp Immunoljournal_title
Clinical and experimental immunologyauthors
Ward C,Lucas M,Piris J,Collier J,Chapel Hdoi
10.1111/j.1365-2249.2008.03711.xsubject
Has Abstractpub_date
2008-09-01 00:00:00pages
331-7issue
3eissn
0009-9104issn
1365-2249pii
CEI3711journal_volume
153pub_type
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journal_title:Clinical and experimental immunology
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pub_type: 杂志文章
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