作者: Kyle Rowe , Jon Pankow , Fredy Nehme , William Salyers
DOI: 10.7759/CUREUS.1228
关键词:
摘要: Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents complex common pathway that encompasses multiple etiologies and presentations. It significant diagnostic treatment challenge. The disease results from the of insoluble extracellular fragments have been rendered resistant to digestion. GIA can be acquired or genetic, most commonly chronic inflammatory disorders (AA amyloidosis), hematologic malignancy (AL end-stage renal (Beta-2 amyloidosis). these abnormal proteins interferes with gastrointestinal tract (GI) organ structure function, notably in liver small bowel. Presentation GI involvement includes cirrhotic sequelae, abdominal pain, malabsorption, bleeding. Diagnosis hinges on pathologic examination affected tissue, classic green birefringence under polarized light. Abdominal fat pad rectal mucosal biopsy described as sites higher sensitivity for diagnosis. Serum amyloid P scintigraphy is near 90% sensitive diagnosis AA amyloidosis. Patients should considered further evaluation rule out additional involvement, cardiac renal. Treatment an adequate suppression predisposing malignancy, followed by supportive therapy. Prognosis varies depending etiology disease, AL subtype showing worse outcomes, well those hepatic involvement.