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Systemic amyloidosis and the gastrointestinal tract

Sattianayagam, PT; Hawkins, PN; Gillmore, JD; (2009) Systemic amyloidosis and the gastrointestinal tract. NAT REV GASTRO HEPAT , 6 (10) 608 - 617. 10.1038/nrgastro.2009.147.

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Abstract

Systemic amyloidosis is characterized by the extracellular deposition of protein in an abnormal fibrillar form. Several different types of amyloidosis exist, each defined by the identity of their respective fibril precursor protein. Among patients with systemic amyloidosis, histological involvement of the gastrointestinal tract is very common but is often subclinical. Conversely, primary diseases of the gastrointestinal tract can cause systemic amyloidosis; for example, AA amyloidosis can occur secondary to IBD. The presence and pattern of gastrointestinal symptoms varies substantially, not only between the different types of amyloidosis but also within them. Typical clinical presentations, most of which are nonspecific, include macroglossia, hemorrhage, motility disorders, disturbance of bowel habit and malabsorption. Endoscopic and radiological features are also nonspecific, with the small intestine most commonly affected. Currently, the aim of therapy for amyloidosis is to slow amyloid formation by reducing the abundance of the fibril precursor protein. No specific treatments for the gastrointestinal symptoms of systemic amyloidosis are available; however, case reports and small published series encourage nutritional support for patients with motility disorders and pharmacological agents for treatment of diarrhea. Surgical procedures should be contemplated only in an emergency setting because of the risk of decompensation of organs affected by amyloid deposition.

Type: Article
Title: Systemic amyloidosis and the gastrointestinal tract
DOI: 10.1038/nrgastro.2009.147
Keywords: INFLAMMATORY-BOWEL-DISEASE, PROTEIN-LOSING ENTEROPATHY, LIVER-TRANSPLANTATION, SECONDARY AMYLOIDOSIS, AL-AMYLOIDOSIS, FAMILIAL AMYLOIDOSIS, AA AMYLOIDOSIS, CHEMICAL TYPES, P COMPONENT, BLEEDING MANIFESTATIONS
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: http://discovery.ucl.ac.uk/id/eprint/141091
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