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Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis
المؤلفون المشاركون
Nakajima, Yoshiyuki
Uemura, Masahito
Matsumoto, Masanori
Ko, Saiho
Fukui, Hiroshi
Fujimura, Yoshihiro
المصدر
International Journal of Hepatology
العدد
المجلد 2011، العدد 2011 (31 ديسمبر/كانون الأول 2011)، ص ص. 1-12، 12ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2011-07-18
دولة النشر
مصر
عدد الصفحات
12
التخصصات الرئيسية
الملخص EN
The liver plays a central role in hemostasis by synthesizing clotting factors, coagulation inhibitors, and fibrinolytic proteins.
Liver cirrhosis (LC), therefore, impacts on both primary and secondary hemostatic mechanisms.
ADAMTS13 is a metalloproteinase, produced exclusively in hepatic stellate cells, and specifically cleaves unusually large von Willebrand factor multimers (UL-VWFM).
Deficiency of ADAMTS13 results in accumulation of UL-VWFM, which induces platelet clumping or thrombi under high shear stress, followed by sinusoidal microcirculatory disturbances and subsequent progression of liver injuries, eventually leading to multiorgan failure.
The marked imbalance between decreased ADAMTS13 activity (ADAMTS13 : AC) and increased production of UL-VWFM indicating a high-risk state of platelet microthrombi formation was closely related to functional liver capacity, hepatic encephalopathy, hepatorenal syndrome, and intractable ascites in advanced LC.
Some end-stage LC patients with extremely low ADAMTS13 : AC and its IgG inhibitor may reflect conditions similar to thrombotic thrombocytopenic purpura (TTP) or may reflect “subclinical TTP.” Hence, cirrhotic patients with severe to moderate deficiency of ADAMTS13 : AC may be candidates for FFP infusion as a source of ADAMTS13 or for recombinant ADAMTS13 supplementation.
Such treatments may improve the survival of patients with decompensated LC.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Uemura, Masahito& Fujimura, Yoshihiro& Ko, Saiho& Matsumoto, Masanori& Nakajima, Yoshiyuki& Fukui, Hiroshi. 2011. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology،Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-496481
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Uemura, Masahito…[et al.]. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology No. 2011 (2011), pp.1-12.
https://search.emarefa.net/detail/BIM-496481
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Uemura, Masahito& Fujimura, Yoshihiro& Ko, Saiho& Matsumoto, Masanori& Nakajima, Yoshiyuki& Fukui, Hiroshi. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology. 2011. Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-496481
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-496481
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
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