Protein S deficiency: a clinical perspective.
نویسندگان
چکیده
Protein S (PS) is an extensively studied protein with an important function in the down-regulation of thrombin generation. Because of the presence of a pseudogene and two different forms of PS in plasma, a bound and a free form, it is one of the most difficult thrombophilias to study. A deficiency of PS predisposes subjects to (recurrent) venous thromboembolism (VTE) and foetal loss. However, the conundrum of diagnosing PS deficiency has led to conflicting reports of PS as a risk factor for VTE. In this review, we aim to present a clinical perspective of PS deficiency.
منابع مشابه
Lacunar infarction in child with Protein S deficiency: a case report
Abstract Arterial ischemic stroke defines as a new focal neurologic deficit that lasted 24 hours or longer. Stroke is relatively rare in children and incidence of cerebrovascular disease is 1 per 4000 in neonates and 1 per 7000 to 1 per 70000 in older children (1 month to 18 years). Protein S deficiency is one of the causes of the stroke in children. Major manifestations of protein S deficienc...
متن کاملبررسی میزان فراوانی مقاومت به پروتئین C فعال و نقص کیفی پروتئین‌های S، C و آنتی ترومبین در مبتلایان به ترومبوز وریدی عمقی مراجعه کننده به سازمان انتقال خون ایران
Background and Objective: Deep vein thrombosis (DVT) is one of the most common forms of venous thromboembolism (VTE). Deficiency in natural anticoagulant proteins is considered to be the major cause of thrombosis. In the present study, we investigated the incidence of defects in protein S, protein C, antithrombin and resistance to activated protein C (APC-R) in patients with DVT referred to the...
متن کاملگزارش موردی نادر از وقوع ترومبوز شریانی با تظاهر ایسکمی اندام تحتانی در دو بیمار مبتلا به گرانولوماتوز وگنر
Introduction: Wegener’s Granolomatosis (WG) is a systemic, necrotizing, small-vessel vasculitis. Vascular inflammation and occlusion leading to tissue ischemia is a hallmark of WG. WG has a clinical predilection for the upper airways, lungs, and kidneys. Thromboembolic events do not usually occur and arterial thrombosis is extremely rare. Case Report: Here we reported 2 rare cases of arteria...
متن کاملبررسی سطح مهارکننده‌های طبیعی انعقاد و آنتی‌فسفولیپید آنتی بادی در بیماران با سابقه ترمبوز عروقی در شهر تهران
ABSTRACT Thrombophilia is a clinical problem throughout the world and it is particularly a major problem in western countries. Factors causing thromboembolic diseases are numerous and they may be inherited or aquired. In order to investigate causes of venous thrombosis. The level of antithrombin (AT), protein C, protein S and the presence of antiphospholipid antibodies (APA) in 300 p...
متن کاملFactor VII Gene Defects: Review of Functional Studies and Their Clinical Implications
Coagulation factors belong to a family of plasma glycosylated proteins that should be activated for appropriate blood coagulation. Congenital deficiencies of these factors cause inheritable hemorrhagic diseases. Factor VII (FVII) deficiency is a rare bleeding disorder with variable clinical symptoms. Various mutations have been identified throughout the F7 gene and can affect all the protein do...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haemophilia : the official journal of the World Federation of Hemophilia
دوره 14 6 شماره
صفحات -
تاریخ انتشار 2008