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Liver diseases in patients with primary antibody deficiency

Author(s): M Motamed | M Soltani | A Aghamohammadi | M Mansouri | Z Pourpak | Sh Teimourian | N Parvaneh

Journal: Iranian Journal of Pediatrics
ISSN 2008-2142

Volume: 16;
Issue: 4;
Start page: 433;
Date: 2006;
Original page

Keywords: Liver disease | Primary antibody deficiency | Chronic active hepatitis | Hyper-IgM syndrome

Background: Liver disease can complicate primary antibody deficiency in several ways. This study investigates the prevalence and causes of hepatobiliary disease in 62 Iranian patients with primary antibody deficiency. Methods: Sixty-two primary antibody deficient patients were followed up and signs and symptoms of liver disease were recorded. All patients were tested for hepatitis C virus (HCV-RNA) and almost all for Cryptosporidium parvum (CSP). The patients with liver involvement were examined from clinical, laboratory and radiological aspects on a regular basis. Findings: Clinical evidences of liver disease were documented in 22 patients (35.5%). Eight patients (13%) had clinical, radiological and laboratory criteria of chronic liver disease. Only one patient was HCV-RNA positive, he had stigmata of chronic liver disease and pathologic evidence of chronic active hepatitis with cirrhosis. One patient with hyper-IgM syndrome was found to be positive for Cryptosporidium parvum. In liver biopsy of patients with liver involvement, one had histological findings related to sclerosing cholangitis, five had mild to moderate chronic active hepatitis with unknown reason. Conclusions: Hepatobiliary disease is a frequent complication in primary antibody deficiency (13%). Liver disease in HCV-RNA negative patients usually accompany other organ involvements. Chronic active hepatitis with unknown origin is the most common feature of liver injury in Iranian patients with primary antibody deficiency.
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