Gastrointestinal and hepatic involvement in immunodeficiencies and systemic disease of childhood
Many of the primary disorders of immune deficiency (Table 5.1) are associated with gastrointestinal (GI) and, to a lesser extent, hepatic disease. Manifestations of immune deficiency in the GI tract and liver may be broadly divided into three categories: (1) increased susceptibility to infection, (2) idiopathic chronic inflammatory conditions, and (3) increased risk of neoplasia. Although many of the GI lesions related to immunodeficiency are infectious (Table 5.2), chronic inflammatory conditions resembling celiac disease and inflammatory bowel disease (Table 5.3) are seen in many patients with antibody deficiencies and are probably the result of the inability of dysfunctional mononuclear cells to suppress deleterious immune responses. All patients with primary immune deficiencies are at increased risk of neoplasia (Salavoura et al. 2008) (Table 5.4), most commonly non-Hodgkin's lymphoma, and the GI tract is often the primary site of involvement (Cunningham-Rundles et al. 2002). In addition to the risk of lymphoma, some of the primary immune deficiencies are associated with increased risk for GI carcinoma (Brosens et al. 2008; Filipovich et al. 1994; van der Meer et al. 1993).
