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Literature DB >> 17666281

Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.

Abstract

A serious disorder with characteristic microvascular thrombosis involving the brain and other organs, thrombotic thrombocytopenic purpura (TTP) typically presents with thrombocytopenia, hemolysis with schistocytes on blood smears, and mental changes or seizures. It may progress rapidly to a fatal end if the patient is not treated immediately with plasma. Recent advances have shown that TTP is caused by deficiency of a circulating, von Willebrand factor cleaving metalloprotease, ADAMTS13. This new knowledge will provide clues to improve the diagnosis and management of this intriguing disease.

Entities: Disease Gene Species

Mesh：

Substances：

Year: 2007 PMID： 17666281 PMCID： PMC2001253 DOI： 10.1016/j.hoc.2007.06.003

Source DB: PubMed Journal: Hematol Oncol Clin North Am ISSN： 0889-8588 Impact factor: 3.722

103 in total

1. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias.

Authors: Sarah L Allford; Beverley J Hunt; Peter Rose; Samuel J Machin
Journal: Br J Haematol Date: 2003-02 Impact factor: 6.998

2. Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.

Authors: Xinglong Zheng; Kenji Nishio; Elaine M Majerus; J Evan Sadler
Journal: J Biol Chem Date: 2003-06-05 Impact factor: 5.157

3. Presence of ADAMTS13 activity in a patient with metastatic cancer and thrombotic microangiopathy.

Authors: Robin B Forman; Seth A Benkel; Yelena Novik; Han-Mou Tsai
Journal: Acta Haematol Date: 2003 Impact factor: 2.195

4. Cleavage of the ADAMTS13 propeptide is not required for protease activity.

Authors: Elaine M Majerus; Xinglong Zheng; Elodee A Tuley; J Evan Sadler
Journal: J Biol Chem Date: 2003-09-15 Impact factor: 5.157

5. ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage.

Authors: Kenji Soejima; Masanori Matsumoto; Koichi Kokame; Hideo Yagi; Hiromichi Ishizashi; Hiroaki Maeda; Chikateru Nozaki; Toshiyuki Miyata; Yoshihiro Fujimura; Tomohiro Nakagaki
Journal: Blood Date: 2003-07-17 Impact factor: 22.113

6. Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).

Authors: Pier Mannuccio Mannucci; Massimo Vanoli; Ileana Forza; Maria Teresa Canciani; Raffaella Scorza
Journal: Haematologica Date: 2003-08 Impact factor: 9.941

7. Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.

Authors: Antonella Lattuada; Edoardo Rossi; Cinzia Calzarossa; Rosaria Candolfi; Pier Mannuccio Mannucci
Journal: Haematologica Date: 2003-09 Impact factor: 9.941

8. Acquired von Willebrand syndrome in aortic stenosis.

Authors: André Vincentelli; Sophie Susen; Thierry Le Tourneau; Isabelle Six; Olivier Fabre; Francis Juthier; Anne Bauters; Christophe Decoene; Jenny Goudemand; Alain Prat; Brigitte Jude
Journal: N Engl J Med Date: 2003-07-24 Impact factor: 91.245

9. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

Authors: David P Miller; James A Kaye; Kathleen Shea; Najat Ziyadeh; Clorinda Cali; Corri Black; Alexander M Walker
Journal: Epidemiology Date: 2004-03 Impact factor: 4.822

10. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.

Authors: Sara K Vesely; James N George; Bernhard Lämmle; Jan-Dirk Studt; Lorenzo Alberio; Mayez A El-Harake; Gary E Raskob
Journal: Blood Date: 2003-03-13 Impact factor: 22.113

21 in total

Review 1. Pathogenesis, diagnosis and management of paraneoplastic glomerulonephritis.

Authors: Yeong-Hau H Lien; Li-Wen Lai
Journal: Nat Rev Nephrol Date: 2010-12-14 Impact factor: 28.314

2. Two novel heterozygote missense mutations of the ADAMTS13 gene in a child with recurrent thrombotic thrombocytopenic purpura.

Authors: Raffaella Rossio; Barbara Ferrari; Andrea Cairo; Ilaria Mancini; Giovanni Pisapia; Giulia Palazzo; Flora Peyvandi
Journal: Blood Transfus Date: 2012-09-12 Impact factor: 3.443

Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.

1. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias.

2. Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.

3. Presence of ADAMTS13 activity in a patient with metastatic cancer and thrombotic microangiopathy.

4. Cleavage of the ADAMTS13 propeptide is not required for protease activity.

5. ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage.

6. Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).

7. Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.

8. Acquired von Willebrand syndrome in aortic stenosis.

9. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

10. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.

Review 1. Pathogenesis, diagnosis and management of paraneoplastic glomerulonephritis.

2. Two novel heterozygote missense mutations of the ADAMTS13 gene in a child with recurrent thrombotic thrombocytopenic purpura.

3. Formation and Resolution of Pial Microvascular Thrombosis in a Mouse Model of Thrombotic Thrombocytopenic Purpura.

4. Life-threatening spontaneous hemothorax in a patient with thrombotic thrombocytopenic purpura.

5. Human neutrophil peptides and complement factor Bb in pathogenesis of acquired thrombotic thrombocytopenic purpura.

6. Evolution of ADAMTS13 antibodies in a fatal case of thrombotic thrombocytopenic purpura.

7. Thrombotic Thrombocytopenic Purpura Associated with Dermatomyositis.

Review 8. Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura.

9. The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation.

10. Characterization and regulation of ADAMTS-16.