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Immunodeficiency Disorders Resulting in Malabsorption

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Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Abstract

Gastrointestinal (GI) disorders are present in 5–50 % of patients with primary immunodeficiencies. These manifestations mimic classic forms of disease (in the absence of immunodeficiency) such as celiac disease, food allergy, or inflammatory bowel disease, but they are often unresponsive to conventional therapies. Because GI disease may be the first presentation of an underlying immunodeficiency, it is crucial to consider immunodeficiency in any child with recurrent or chronic severe diarrhea, malabsorption, and failure to thrive resistant to conventional treatments. At the same time, routine evaluation of GI tract is useful for children with immunodeficiency, given the high incidence of GI disorders in these patients and considering that early evaluation and diagnosis can prevent potentially irreversible tissue damage. Primary immunodeficiencies are relatively common but likely underdiagnosed. A clinical history of recurrent, opportunistic, or unusual infections; histological features that do not fit the usual pattern of disease; and a poor response to conventional therapy should prompt the pediatrician to pursue further immunologic evaluation in collaboration with the immunologist.

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Author information

Authors and Affiliations

  1. Department of Translational Medical Science—Section of Pediatrics, University of Naples “Federico II”, Naples, Italy

    Margherita Di Costanzo, Marinita Morelli, Monica Malamisura, Maria Giovanna Puoti & Roberto Berni Canani

  2. European Laboratory for the Investigation of Food Induced Diseases and CEINGE Advanced Biotechnologies, University of Naples “Federico II”, Via S. Pansini 5, 80131, Naples, Italy

    Roberto Berni Canani

Authors
  1. Margherita Di Costanzo
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  2. Marinita Morelli
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  3. Monica Malamisura
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  4. Maria Giovanna Puoti
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  5. Roberto Berni Canani
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Corresponding author

Correspondence to Roberto Berni Canani .

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Editors and Affiliations

  1. Department of Pediatrics, University of Chicago, Chicago, Illinois, USA

    Stefano Guandalini

  2. Clinical Director Child Health King’s College Hospital, Professor and Director Pediatric Liver, GI and Nutrition Center, London, United Kingdom

    Anil Dhawan

  3. Department of Pediatrics, Hebrew University Hadassah Hospital, Jerusalem, Israel

    David Branski

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Di Costanzo, M., Morelli, M., Malamisura, M., Puoti, M., Berni Canani, R. (2016). Immunodeficiency Disorders Resulting in Malabsorption. In: Guandalini, S., Dhawan, A., Branski, D. (eds) Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. Springer, Cham. https://doi.org/10.1007/978-3-319-17169-2_37

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  • DOI: https://doi.org/10.1007/978-3-319-17169-2_37

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17168-5

  • Online ISBN: 978-3-319-17169-2

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