Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran

Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran


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دانشگاه علوم پزشکی تبریز
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نویسندگان: محمد رضا اردلان , پریسا رضائی فر

کلمات کلیدی: Purpura; Syndrome; Thrombotic Microangiopathy

نشریه: 25023 , 6 , 6 , 2014

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نویسنده ثبت کننده مقاله محمد رضا اردلان
مرحله جاری مقاله تایید نهایی
دانشکده/مرکز مربوطه مرکز تحقیقات کلیه
کد مقاله 66448
عنوان فارسی مقاله Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran
عنوان لاتین مقاله Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran
ناشر 2
آیا مقاله از طرح تحقیقاتی و یا منتورشیپ استخراج شده است؟ بلی
عنوان نشریه (خارج از لیست فوق)
نوع مقاله Original Article
نحوه ایندکس شدن مقاله ایندکس شده سطح سه – Scopus
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Background: Thrombotic microangiopathy (TMA) is a rare but devastating small vessels disorder that is characterized by intravascular platelet thrombi, thrombocytopenia, and various degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. Objectives: The Aim of this study was to determine the von Willebrand factor-cleaving protease (ADAMTS13) activity during the acute phase of TMA. We also investigated inhibiting antibodies against ADAMTS13 in these patients. Patients and Methods: In a collaborative work with Mario-Negro institute of pharmacological research in Bergamo-Italy, we registered the clinical and laboratory data, collected the serum samples, and transferred the samples to the laboratories. Serum samples were taken before the start of plasmapheresis or at least 15 days after the fnal exchange. Results: We recruited 40 patients (14 males and 26 females) with the mean age of 46.12 ± 17.26 years. The mean activity of ADAMTS13 was 34.58% ± 21.83%. Two patients had inhibitory antibodies against ADAMTS13 with profound defciency of ADAMTS13 activity (< 6%). Infectious diseases were the most common underlying condition, followed by systemic lupus erythematous. Conclusions: Majority of patients had an underlying condition and had various ADAMTS13 activity. The presence of inhibiting antibodies and accompanied complete defciency of ADAMTS13 activity is an indicator of severity.

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نویسنده نفر چندم مقاله
محمد رضا اردلاناول
پریسا رضائی فردوم

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