Fibrinogen amyloidosis: the clot thickens!
نویسنده
چکیده
In this issue of Blood, Stangou et al discuss a possible role for hepatorenal transplantation in the management of patients with fibrinogen amyloidosis, based on a detailed evaluation of 22 patients, the largest group to date.1 The authors also considerably expand the current phenotypic description of fibrinogen amyloidosis, including their interpretation of previously unrecognized disease manifestations and risks.
منابع مشابه
Inhibitor of the thrombin time in systemic amyloidosis: a common coagulation abnormality.
Patients with primary systemic amyloidosis (AL) often experience bleeding, and we report a newly recognized coagulation abnormality in AL. Of 103 patients with primary systemic AL studied over 2 years, 41 had prolongation of the thrombin time (range, 25 to 46 seconds; normal, less than 22 seconds) and reptilase time (range, 17 to 39 seconds; normal, 14 to 16 seconds). The fibrinogen from the pl...
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Fibrinogen γ’ is known to influence fibrin clot structure in purified experimental models but little is known regarding its influence on clot structure in plasma. Furthermore, the environmental and biological factors that affect its concentration are poorly described. We analysed fibrinogen γ’, total fibrinogen concentration and fibrin clot structure in 2010 apparently healthy black South Afric...
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A new American kindred with amyloidosis was found by single-strand conformation polymorphism analysis to have a mutation in the fibrinogen A alpha chain gene. Affected members in this kindred have autosomal dominant amyloid nephropathy. DNA sequencing showed a single nucleotide deletion at the third base of codon 524 of the fibrinogen A alpha chain genes (4904delG) that resulted in a frame shif...
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عنوان ژورنال:
- Blood
دوره 115 15 شماره
صفحات -
تاریخ انتشار 2010