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Children with primary or acquired immunodeficiency are at increased risk for infectious and inflammatory gastrointestinal disorders.1 The risk and severity of infection depends on the type of immunodeficiency. Individuals with deficiencies of antibody response are predisposed to extracellular bacterial infections and intestinal pathogens. Patients with deficiencies of T cells are predisposed to both intracellular and extracellular infections. In addition, patients with primary immunodeficiencies are more prone to develop autoimmune disorders because of their decreased ability to distinguish self-organisms from foreign organisms. Autoimmune diseases and celiac disease are more common in the IgA-deficient patients.

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Clinical Features and Differential Diagnosis

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Gastrointestinal disorders in children with immunodeficiencies can be associated with infectious (viruses, bacteria, mycobacteria, fungi, or protozoa) and noninfectious disorders (autoimmune and alloimmune). Dysmotility, malabsorption, and malnutrition can be associated with any of these disorders. In addition, medical treatments prescribed for children with immunodeficiencies may have important gastrointestinal complications.

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Gastrointestinal Infections

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The pandemic of human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) has heightened our awareness of opportunistic infections, most of which have been described either in patients with primary immunodeficiencies or in immunosuppressed patients with malignancies. These infections are listed in Table 391-1 according to the sites of gastrointestinal involvement and are discussed in more detail in Section 17. In addition to those listed, immunodeficient patients also are at increased risk for common bacterial and viral pathogens or infections with multiple organisms.

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Table 391-1. Opportunistic Gastrointestinal Infections
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Cytomegalovirus, rotavirus, adenovirus, and herpes simplex virus are the most common viral agents. Cytomegalovirus is commonly identified in children with immunodeficiency and can cause inflammation or ulceration throughout the gastrointestinal (GI) tract, including the pancreatobiliary system. Symptoms may include diarrhea, dysphagia, vomiting, abdominal pain, and GI bleeding. Histologic identification of cytomegalovirus within the intestinal tissue is required to establish a pathogenic role because cytomegalovirus commonly is excreted in the urine or stool of asymptomatic individuals. Rotavirus is a cause of vomiting and diarrhea in immunocompromised children and may disseminate into the liver parenchyma. In contrast, rotavirus rarely is a pathogen in normal or immunocompromised adults, most of whom have previously established immunity. Adenoviruses is reported to cause colitis in adults with AIDS and fulminant hepatitis in immunocompromised children. Diagnosis of adenovirus infection depends on histologic identification, which should be confirmed by culture.2 Herpes simplex virus usually causes oral and esophageal lesions and produces symptoms of dysphagia and odynophagia. Other viruses, such as astrovirus, picornavirus, and calicivirus, have been identified in the stool of HIV-infected adults with diarrhea, and they also may have a role in pediatric ...

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