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primary amyloidosis


Definition: several forms of amyloidosis are known, following autosomal dominant [MIM *104750, *105120, *105150, *105200, *105210, *105250] recessive [MIM 204850 and *204900], and X-linked [MIM 301220] inheritance and not associated with other recognized disease. Tends to involve diffusely the arterial walls and mesenchymal tissues in the tongue, lungs, intestinal tract, skin, skeletal muscle, and myocardium, interfering with vital functions; the amyloid frequently does not manifest the usual affinity for Congo red stain, and sometimes provokes a foreign-body type of inflammatory reaction in the adjacent tissue.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.