Articles: amyloidosis.
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Case Reports
Syndrome of acquired factor X deficiency and systemic amyloidosis; in vivo studies of the metabolic fate of factor X.
To determine the metabolic fate of factor X in primary amyloidosis associated with factor X deficiency, we examined the pathways of its catabolism in a man with this syndrome. Intravenous infusion of human or bovine 131I-labeled factor X established a triphasic plasma clearance pattern for factor X. About 85 per cent of the factor X disappeared, with a disappearance half-time of less than 30 seconds. ⋯ Relatively minor quantities of 131I were cleared into the urine. We observed a diffuse distribution of radioactivity over the body surface, with a concentration in the hepatic and splenic regions. These studies demonstrate than factor X deficiency associated with systemic amyloidosis is due to binding of factor X to body tissue, probably within the circulatory system.
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Case Reports
Poikiloderma-like cutaneous amyloidosis. Report of a case and review of the literature.
Cases of cutaneous amyloidosis which exhibit poikiloderma-like changes are extremely rare. There are at least two clinical forms of poikiloderma-like cutaneous amyloidosis (PCA): (1) the ordinary type, and (2) PCA syndrome. The PCA syndrome includes poikilodermatous skin manifestations whicm may appear early in life and lichenoid papules, both with cutaneous amyloid deposits, frequently associated with light sensitivity and short stature, occasionally with palmoplantar keratosis and blister formation. We carried out an examination of a 5-year-old girl who was compatible with the syndrome.