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Hepatobiliary Manifestations of Chronic Granulomatous Diseases of Childhood

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract
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Abstract

The recurrent bacterial and fungal infections that complicate chronic granulomatous disease (CGD) can involve the hepatobiliary tract and induce abscesses and obstructing lesions. CGD is a heterogeneous group of primary immunodeficiency diseases, whereby phagocytes fail to kill certain bacteria and fungi after phagocytosis. CGD is a hereditary defect characterized by deficient production of reactive oxygen metabolites required to destroy intracellular microorganisms. This failure is related to several defects in NADPH oxidase. Pediatric patients with CGD frequently show hepatomegaly and bacterial liver abscesses, which are a clinical hallmark of CGD. The frequent and complex infections can also induce hepatobiliary granulomas and their secondary effects. Venopathy of portal vein branches is common in CGD and is associated with portal hypertension and splenomegaly.

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Zimmermann, A. (2017). Hepatobiliary Manifestations of Chronic Granulomatous Diseases of Childhood. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_140

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  • DOI: https://doi.org/10.1007/978-3-319-26956-6_140

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