الفهرس 
ADAMTS13Only 14 pages are availabe for public view
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Abstract
Microangiopathy: a disorder involving the small blood vessels. thrombotic microangiopathy formation of thrombi in the arterioles and capillaries; proposed name for a syndrome that would include both thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (Mosby’s Medical Dictionary, 9th edition. © 2009, Elsevier) In our study we measured adamts13 in microangiopathy in thrombotic thrombocytopenic purpura, microangiopathic hemolytic anemia , hemolytic uremic syndrome , DIC .we made comparison between sever deficiency of adamts13 level <10% and moderate deficiency of adamts13 level >10%. ADAMTS13 (a disintegrin and metallo-proteinase with a thrombospondin type 1 motif, member 13)-also known as von Willebrand factor-cleaving protease (VWFCP)-is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. It is secreted in blood and degrades large vWf multimers, decreasing their activity (Levy et al. 2005). The ADAMTS13 gene maps to the ninth chromosome (9q34). Levy GG, Motto DG, Ginsburg D (2005).
Aim of the study: The aim of such study is to assess the role of measurement of ADAMTS13 in adult patients with suspected microangiopathy.
Subjects and methods: The study will be conducted on 90 subjects including: 60 patients suspected microangiopathy compared to 30 healthy control subjects in Ain Shams University Hospitals, inpatient departments & out-patient clinics.
The subjects will be divided into two groups:
• group 1 (60 subjects): patients with suspected microangiopathy.
• group 2 (30 subjects): healthy control subjects.
Including criteria:
• Adult at or larger than 18 years old.
• Suspected microangiopathy (such as progressive microangiopathy hemolytic anaemia with or without thrombocytopenia, edema, effusion, organ failure.
All patients will be subjected to the following:
Detailed history taking and physical examination.
Laboratory investigations including:
• Complete blood picture (CBC) and peripheral blood film (with schistocytes), reticulocytic count, serum ferritin and erythrocyte sedimentation rate (ESR).
• Coagulation profile: prothrombin time (PT), and activated partial thromboplastin time (aPTT).
• Metabolic profile including renal function tests (s.creat , urea ,Na & K) ,Liver function tests (SGOT , SGPT, total & direct Bilirubin, Total protein & albumin).
• Serum LDH.
Other investigation as needed for diagnosis.
Measurement of ADAMTS13 level in prepheral blood (ELIZA)
Ethical aspects:
The study will be initiated after obtaining approval of Ethical Committee of faculty of Medicine Ain Shams University.
Statistical analysis of results
CONCLUSION:
TTP and other TMAs remain diagnostically difficult and emergency condition and need to rapid investigation. Measurement of ADAMTS13 is more important in diagnosis but till now is not used as routine laboratory investigation in these cases because it is very expensive and we hope using widely in the futures.
Key words: thrombotic microangiopathy, ADAMTS13, thrombotic thrombocytopenic purpura