Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Amyloidosis, Gastrointestinal

  • Susana Rodrigues
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1560

Synonyms

Gastrointestinal manifestations of amyloidosis; Systemic amyloidosis

Definition

Amyloidoses are a diverse group of diseases characterized by abnormal folding of proteins and the extracellular tissue deposition of fibrils of low molecular weight subunits of normal serum proteins which disrupt tissue structure and function. The conversion of peptides or proteins from their insoluble functional states into highly organized fibrillar aggregates showing a cross-beta super-secondary structure is termed as “amyloid.” At least 28 different human proteins have been identified as amyloidogenic. Amyloidogenic deposition can be localized or systemic. Acquired causes of systemic amyloidoses include: (1) primary or AL amyloidosis in which the fibrils are composed of fragments of monoclonal light chains and affected patients may have amyloidosis alone or in association with other plasma cell dyscrasias, and (2) reactive (secondary) or AA amyloidosis in which the fibrils are composed of...

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References and Further Reading

  1. Cowan, A., Skinner, M., & Seldin, D. et al. (2012) Amyloidosis of the gastrointestinal tract: A 13-year single center referral experience. Haematologica, 97. (epub ahead of print).Google Scholar
  2. Madsen, L. G., Gimsing, P., & Schiødt, F. V. (2009). Primary (AL) amyloidosis with gastrointestinal involvement. Scandinavian Journal of Gastroenterology, 44, 708.PubMedCrossRefGoogle Scholar
  3. Merlini, G., Seldin, D., & Gertz, M. (2011). Amyloidosis: Pathogenesis and new therapeutic options. Journal of Clinical Oncology, 14, 1924–1933.CrossRefGoogle Scholar
  4. Sattianayagam, P. T., Hawkins, P. N., & Gillmore, J. D. (2009). Systemic amyloidosis and the gastrointestinal tract. Nature Reviews. Gastroenterology & Hepatology, 6, 608–617.CrossRefGoogle Scholar
  5. Tada, S., Iida, M., Yao, T., et al. (1994). Endoscopic features in amyloidosis of the small intestine: Clinical and morphologic differences between chemical types of amyloid protein. Gastrointestinal Endoscopy, 40, 45.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Serviço de Gastrenterologia, Centro Hospitalar de São JoãoAlameda Professor Hernani MonteiroPortoPortugal