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1. Full Text Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura
by Page, Evaren E and Kremer Hovinga, Johanna A and Terrell, Deirdra R and Vesely, Sara K and George, James N
Blood, ISSN 0006-4971, 06/2016, Volume 127, Issue 24, pp. 3092 - 3094
EFFICACY | HEMATOLOGY | SAFETY | Recurrence | Humans | Middle Aged | ADAMTS13 Protein - analysis | Male | Plasma Exchange | Purpura, Thrombotic Thrombocytopenic - diagnosis | Adrenal Cortex Hormones - therapeutic use | Secondary Prevention - methods | Purpura, Thrombotic Thrombocytopenic - prevention & control | Young Adult | Rituximab - therapeutic use | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Aged | Purpura, Thrombotic Thrombocytopenic - therapy | Immunologic Factors - therapeutic use
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2. Full Text Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura
by Hie, Miguel and Gay, Julie and Galicier, Lionel and Provôt, François and Presne, Claire and Poullin, Pascale and Bonmarchand, Guy and Wynckel, Alain and Benhamou, Ygal and Vanhille, Philippe and Servais, Aude and Bordessoule, Dominique and Coindre, Jean-Philippe and Hamidou, Mohamed and Vernant, Jean-Paul and Veyradier, Agnès and Coppo, Paul and French Thrombotic and French Thrombotic Microangiopathies Reference Centre and the French Thrombotic Microangiopathies Reference Centre
Blood, ISSN 0006-4971, 07/2014, Volume 124, Issue 2, pp. 204 - 210
In acquired thrombotic thrombocytopenic purpura (TTP), the persistence of severe ADAMTS13 deficiency (<10%) during remission is associated with more relapse.... 
PROPHYLAXIS | MAINTENANCE | DIAGNOSIS | PLASMA-EXCHANGE | ADAMTS13 | MANAGEMENT | THERAPY | EFFICACY | SPLENECTOMY | MICROANGIOPATHIES | HEMATOLOGY | ADAM Proteins - immunology | Cross-Sectional Studies | Autoantibodies - blood | Humans | Antibodies, Monoclonal, Murine-Derived - administration & dosage | ADAM Proteins - deficiency | Rituximab | Male | Treatment Outcome | Secondary Prevention | ADAMTS13 Protein | Purpura, Thrombotic Thrombocytopenic - prevention & control | Remission Induction | ADAM Proteins - blood | Adult | Female | Retrospective Studies | Infusions, Intravenous | Purpura, Thrombotic Thrombocytopenic - blood | Chemoprevention - methods
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3. Full Text A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura
by Scully, Marie and McDonald, Vickie and Cavenagh, Jamie and Hunt, Beverley J and Longair, Ian and Cohen, Hannah and Machin, Samuel J
Blood, ISSN 0006-4971, 08/2011, Volume 118, Issue 7, pp. 1746 - 1753
The safety and efficacy of weekly rituximab 375 mg/m(2) (x4), given within 3 days of acute TTP admission, with standard therapy (PEX and steroids) was... 
RELAPSE | TTP | ADAMTS13 ACTIVITY | PREDICTORS | HEMOLYTIC UREMIC SYNDROME | EXPERIENCE | IGG ANTIBODIES | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | CLINICAL-OUTCOMES | CYCLOSPORINE | Recurrence | ADAM Proteins - immunology | Humans | Middle Aged | Male | Plasma Exchange | Young Adult | Aged, 80 and over | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Antigens, CD19 - immunology | Antibodies, Monoclonal, Murine-Derived - immunology | Immunologic Factors - immunology | Rituximab | ADAMTS13 Protein | Purpura, Thrombotic Thrombocytopenic - prevention & control | Adolescent | Purpura, Thrombotic Thrombocytopenic - immunology | Aged | Immunologic Factors - adverse effects | Purpura, Thrombotic Thrombocytopenic - therapy | Antibodies, Monoclonal, Murine-Derived - adverse effects | Antibodies, Monoclonal, Murine-Derived - therapeutic use | Immunologic Factors - therapeutic use
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4. Full Text How I treat thrombocytopenia in pregnancy
by Gernsheimer, Terry and James, Andra H and Stasi, Roberto
Blood, ISSN 0006-4971, 01/2013, Volume 121, Issue 1, pp. 38 - 47
A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. Although representing no... 
POSTPARTUM PLASMA-EXCHANGE | PLATELET COUNTS | VON-WILLEBRAND-DISEASE | IMMUNE THROMBOCYTOPENIA | OBSTETRIC PATIENTS | PURPURA | ACUTE FATTY LIVER | HELLP HEMOLYSIS | ELEVATED LIVER-ENZYMES | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | HELLP Syndrome - drug therapy | Recurrence | Humans | Immunosuppressive Agents - therapeutic use | Hemolytic-Uremic Syndrome - blood | Plasma Exchange | Pregnancy Complications, Hematologic - drug therapy | Contraindications | Purpura, Thrombocytopenic, Idiopathic - blood | Purpura, Thrombocytopenic, Idiopathic - drug therapy | Young Adult | Purpura, Thrombocytopenic, Idiopathic - therapy | HELLP Syndrome - diagnosis | Thrombocytopenia - drug therapy | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Hemolytic-Uremic Syndrome - therapy | Hemolytic-Uremic Syndrome - diagnosis | Infant, Newborn | Diagnosis, Differential | Antibodies, Monoclonal, Murine-Derived | Thrombocytopenia - etiology | HELLP Syndrome - blood | Purpura, Thrombotic Thrombocytopenic - congenital | Thrombocytopenia - congenital | Rituximab | Anticoagulants - therapeutic use | Combined Modality Therapy | Purpura, Thrombotic Thrombocytopenic - diagnosis | Anticoagulants - adverse effects | Purpura, Thrombotic Thrombocytopenic - prevention & control | Danazol | Algorithms | Purpura, Thrombocytopenic, Idiopathic - diagnosis | Pre-Eclampsia - blood | Immunosuppressive Agents - adverse effects | Thrombocytopenia - diagnosis | Disease Management | Pregnancy - blood | Purpura, Thrombotic Thrombocytopenic - blood | Fetal Monitoring
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5. Full Text N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura
by Tersteeg, Claudia and Roodt, Jan and Van Rensburg, Walter J and Dekimpe, Charlotte and Vandeputte, Nele and Pareyn, Inge and Vandenbulcke, Aline and Plaimauer, Barbara and Lamprecht, Seb and Deckmyn, Hans and Lopez, José A and De Meyer, Simon F and Vanhoorelbeke, Karen
Blood, ISSN 0006-4971, 02/2017, Volume 129, Issue 8, pp. 1030 - 1038
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder diagnosed by thrombocytopenia and hemolytic anemia, associated with a deficiency in... 
FRAGMENTS | ADAMTS13 | INHIBITION | THERAPY | VWF | IN-VIVO | INJURY | SYMPTOMS | HEMATOLOGY | VON-WILLEBRAND-FACTOR | Mice, Inbred C57BL | Male | Purpura, Thrombotic Thrombocytopenic - prevention & control | ADAMTS13 Protein - metabolism | Papio | Animals | Gene Deletion | von Willebrand Factor - chemistry | ADAMTS13 Protein - genetics | Purpura, Thrombotic Thrombocytopenic - drug therapy | Female | Mice | Acetylcysteine - therapeutic use | Drug Evaluation, Preclinical | Purpura, Thrombotic Thrombocytopenic - genetics | Protein Multimerization - drug effects | Disease Models, Animal | Purpura, Thrombotic Thrombocytopenic - metabolism | von Willebrand Factor - metabolism
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6. Full Text Long-Term Prevention of Congenital Thrombotic Thrombocytopenic Purpura in ADAMTS13 Knockout Mice by Sleeping Beauty Transposon-Mediated Gene Therapy
by Verhenne, Sebastien and Vandeputte, Nele and Pareyn, Inge and Izsvák, Zsuzsanna and Rottensteiner, Hanspeter and Deckmyn, Hans and De Meyer, Simon F and Vanhoorelbeke, Karen
Arteriosclerosis, Thrombosis, and Vascular Biology, ISSN 1079-5642, 03/2017, Volume 37, Issue 5, pp. 836 - 844
OBJECTIVE—Severe deficiency in the von Willebrand factor–cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif,... 
congenital TTP | gene therapy | sleeping beauty transposon system | ADAMTS13 | von Willebrand factor | FACTOR-VIII | INFUSION | SUFFICIENT | DEFICIENCY | RECOVERY | PLASMA | INTEGRATION | LIVER | PERIPHERAL VASCULAR DISEASE | HEMATOLOGY | HEMOPHILIC MICE | EXPRESSION | Genetic Predisposition to Disease | Mice, Inbred C57BL | Mice, 129 Strain | Purpura, Thrombotic Thrombocytopenic - prevention & control | DNA Transposable Elements | Feasibility Studies | Mice, Knockout | Phenotype | Animals | Purpura, Thrombotic Thrombocytopenic - enzymology | Time Factors | Transposases - genetics | ADAMTS13 Protein - genetics | ADAMTS13 Protein - deficiency | von Willebrand Factor | Purpura, Thrombotic Thrombocytopenic - genetics | Disease Models, Animal | Genetic Therapy - methods
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7. Full Text Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura
by Jiang, Yang and McIntosh, Jennifer J and Reese, Jessica A and Deford, Cassandra C and Kremer Hovinga, Johanna A and Lämmle, Bernhard and Terrell, Deirdra R and Vesely, Sara K and Knudtson, Eric J and George, James N
Blood, ISSN 0006-4971, 03/2014, Volume 123, Issue 11, pp. 1674 - 1680
Pregnancy may precipitate acute episodes of thrombotic thrombocytopenic purpura (TTP), but pregnancy outcomes in women who have recovered from acquired TTP are... 
WOMEN | HYPERTENSIVE DISORDERS | SUCCESSFUL MANAGEMENT | MORBIDITY | ADAMTS13 ACTIVITY | RISK | PREECLAMPSIA | HEMATOLOGY | UPSHAW-SCHULMAN SYNDROME | Recurrence | Prospective Studies | Follow-Up Studies | Pregnancy Complications, Neoplastic - etiology | Humans | Risk Factors | ADAM Proteins - deficiency | Pregnancy Complications, Neoplastic - epidemiology | Purpura, Thrombotic Thrombocytopenic - complications | ADAMTS13 Protein | Pre-Eclampsia - physiopathology | Purpura, Thrombotic Thrombocytopenic - prevention & control | Purpura, Thrombotic Thrombocytopenic - epidemiology | ADAM Proteins - blood | Pregnancy | Young Adult | Oklahoma - epidemiology | Review Literature as Topic | Adolescent | Adult | Female | Registries | Pregnancy Outcome | Clinical Trials and Observations | 101
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8. Full Text Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: A study of 11 cases
by Fakhouri, Fadi and Vernant, Jean-Paul and Veyradier, Agnès and Wolf, Martine and Kaplanski, Gilles and Binaut, Raynald and Rieger, Manfred and Scheiflinger, Friedrich and Poullin, Pascale and Deroure, Benjamin and Delarue, Richard and Lesavre, Philippe and Vanhille, Philippe and Hermine, Olivier and Remuzzi, Giuseppe and Grünfeld, Jean-Pierre
Blood, ISSN 0006-4971, 09/2005, Volume 106, Issue 6, pp. 1932 - 1937
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that occurs mainly in young adults. Acquired cases are usually a result of antibodies... 
Recurrence | Acute Disease | Antibodies, Monoclonal, Murine-Derived | Follow-Up Studies | Autoantibodies - blood | Humans | Metalloendopeptidases - deficiency | Middle Aged | Rituximab | Male | Treatment Outcome | ADAM Proteins | Metalloendopeptidases - immunology | ADAMTS13 Protein | Purpura, Thrombotic Thrombocytopenic - prevention & control | Remission Induction | Purpura, Thrombotic Thrombocytopenic - etiology | Antibodies, Monoclonal - administration & dosage | Autoantibodies - drug effects | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Premedication
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9. Full Text Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models
by Pickens, Brandy and Mao, Yingying and Li, Dengju and Siegel, Don L and Poncz, Mortimer and Cines, Douglas B and Zheng, X. Long
Blood, ISSN 0006-4971, 2015, Volume 125, Issue 21, pp. 3326 - 3334
ADAMTS13 metalloprotease cleaves von Willebrand factor (VWF), thereby inhibiting platelet aggregation and arterial thrombosis. An inability to cleave... 
Platelet-delivered adamts13 also prevents thrombotic thrombocytopenic purpura | Platelet-delivered adamts13 inhibits arterial thrombosis after vascular injury | AUTOANTIBODIES | ANTIBODIES | SPACER DOMAIN | PLASMA-EXCHANGE | HEMOLYTIC-UREMIC SYNDROME | SUBSTRATE | ENDOTHELIAL-CELLS | BINDING-SITE | CLEAVAGE | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | Thrombosis - complications | Humans | Mice, Transgenic | Reverse Transcriptase Polymerase Chain Reaction | ADAMTS13 Protein | Purpura, Thrombotic Thrombocytopenic - prevention & control | ADAM Proteins - administration & dosage | Blotting, Western | Purpura, Thrombotic Thrombocytopenic - etiology | ADAM Proteins - metabolism | Animals | Transfection | Blood Platelets - metabolism | Mice | Microscopy, Fluorescence | Disease Models, Animal | Genetic Therapy - methods | Thrombosis and Hemostasis | 101
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10. Full Text Platelet rescue by macrophage depletion in obese ADAMTS‐13‐deficient mice at risk of thrombotic thrombocytopenic purpura
by Geys, L and Roose, E and Scroyen, I and Rottensteiner, H and Tersteeg, C and Hoylaerts, M. F and Vanhoorelbeke, K and Lijnen, H. R
Journal of Thrombosis and Haemostasis, ISSN 1538-7933, 01/2018, Volume 16, Issue 1, pp. 150 - 163
Essentials Obesity is a potential risk factor for development of thrombotic thrombocytopenic purpura (TTP). Obese ADAMTS‐13‐deficient mice were triggered with... 
macrophages | thrombotic thrombocytopenic purpura | mice | ADAMTS‐13 protein | obesity | ADAMTS-13 protein | SEVERE HEMOPHILIA-A | UNITED-STATES | FACTOR-VIII | CROSS-LINKING | FIBRIN-STABILIZING FACTOR | FACTOR-XIII DEFICIENCY | RECOMBINANT | COAGULATION | PERIPHERAL VASCULAR DISEASE | INHIBITORS | HEMATOLOGY | BLOOD | Clodronic Acid - pharmacology | Obesity - drug therapy | Male | Spleen - drug effects | Mice, 129 Strain | Obesity - blood | Purpura, Thrombotic Thrombocytopenic - etiology | Time Factors | Kupffer Cells - metabolism | von Willebrand Factor | Blood Platelets - drug effects | Disease Models, Animal | Phagocytosis - drug effects | Obesity - complications | Mice, Inbred C57BL | Cells, Cultured | Purpura, Thrombotic Thrombocytopenic - prevention & control | Kupffer Cells - drug effects | Mice, Knockout | Macrophages - metabolism | Animals | Spleen - metabolism | Blood Platelets - metabolism | ADAMTS13 Protein - genetics | ADAMTS13 Protein - deficiency | Macrophages - drug effects | Purpura, Thrombotic Thrombocytopenic - blood | Thrombocytopenic purpura | Obesity | Macrophages | Risk factors | Von Willebrand factor | Spleen | Thrombocytopenia | Liver | Injection | Bisphosphonates | L-Lactate dehydrogenase | Clodronic acid | Thrombotic thrombocytopenic purpura | Purpura | Liposomes | Lactic acid | Health risk assessment | Platelets | Phagocytosis
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11. Full Text Blood and Marrow Transplant Clinical Trials Network Toxicity Committee Consensus Summary: Thrombotic Microangiopathy after Hematopoietic Stem Cell Transplantation
by Ho, Vincent T and Cutler, Corey and Carter, Shelly and Martin, Paul and Adams, Roberta and Horowitz, Mary and Ferrara, James and Soiffer, Robert and Giralt, Sergio
Biology of Blood and Marrow Transplantation, ISSN 1083-8791, 2005, Volume 11, Issue 8, pp. 571 - 575
The syndrome of microangiopathic hemolysis associated with renal failure, neurologic impairment, or both is a recognized complication of hematopoietic stem... 
Thrombocytopenia | Stem cell transport | Thrombotic thrombocytopenic purpura | Hemolytic uremic syndrome | Microangiopathy | Anemia | MANAGEMENT | stem cell transport | thrombocytopenia | thrombotic thrombocytopenic purpura | IMMUNOLOGY | hemolytic uremic syndrome | CHILDREN | TRANSPLANTATION | PLASMA-EXCHANGE | THROMBOCYTOPENIC PURPURA | CASE SERIES | INFECTION | ANEMIA | HEMATOLOGY | microangiopathy anemia | Purpura, Thrombotic Thrombocytopenic - etiology | Purpura, Thrombotic Thrombocytopenic - pathology | Plasma Exchange - methods | Humans | Hematopoietic Stem Cell Transplantation | Clinical Trials as Topic | Professional Staff Committees | Purpura, Thrombotic Thrombocytopenic - prevention & control | Consensus | Transplantation, Homologous | Complications and side effects | Medical colleges | Oncology, Experimental | Stem cells | Bone marrow | Clinical trials | Transplantation | Research | Hematopoietic stem cells | Cancer
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12. Full Text AAV-mediated expression of an ADAMTS13 variant prevents shigatoxin-induced thrombotic thrombocytopenic purpura
by Jin, Sheng-Yu and Jin, Sheng-Yu and Xiao, Juan and Xiao, Juan and Bao, Jialing and Bao, Jialing and Zhou, Shangzhen and Zhou, Shangzhen and Wright, J Fraser and Wright, J. Fraser and Zheng, X. Long and Zheng, X Long
Blood, ISSN 0006-4971, 05/2013, Volume 121, Issue 19, pp. 3825 - 3829
Severe deficiency of plasma ADAMTS13 activity causes thrombotic thrombocytopenic purpura (TTP), a life-threatening syndrome for which plasma is the only... 
PATHOGENESIS | PLASMA-EXCHANGE | THERAPY | FACTOR-IX | IN-VIVO | HEMOLYTIC-UREMIC-SYNDROME | GENE-TRANSFER | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | DEFICIENCY | HEMOPHILIA-B DOGS | Metalloendopeptidases - genetics | Transformation, Genetic | Dependovirus | ADAMTS13 Protein | Codon, Nonsense - physiology | Purpura, Thrombotic Thrombocytopenic - chemically induced | Purpura, Thrombotic Thrombocytopenic - prevention & control | Genetic Vectors - genetics | Mice, Knockout | Animals | Cytoprotection - genetics | Mice | Shiga Toxin 2 - toxicity | Genetic Therapy - methods | Gene Therapy | Brief Report
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13. Full Text Cumulative Review of Thrombotic Microangiopathy, Thrombotic Thrombocytopenic Purpura, and Hemolytic Uremic Syndrome Reports with Subcutaneous Interferon β-1a
by Ben-Amor, Ali-Frédéric and Trochanov, Anton and Fischer, Tanya Z
Advances in Therapy, ISSN 0741-238X, 5/2015, Volume 32, Issue 5, pp. 445 - 454
Rare cases of thrombotic microangiopathy (TMA), manifested as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS), have been reported... 
Thrombotic microangiopathy | Thrombotic thrombocytopenic purpura | Medicine & Public Health | Hemolytic uremic syndrome | Rheumatology | Interferon β-1a | Internal Medicine | Oncology | Cardiology | Pharmacology/Toxicology | Endocrinology | Adjuvants, Immunologic - administration & dosage | Hemolytic-Uremic Syndrome - chemically induced | Multiple Sclerosis, Relapsing-Remitting - drug therapy | Humans | Middle Aged | Male | Interferon beta-1a - adverse effects | Purpura, Thrombotic Thrombocytopenic - chemically induced | Purpura, Thrombotic Thrombocytopenic - prevention & control | Switzerland | Adjuvants, Immunologic - adverse effects | Interferon beta-1a - administration & dosage | Injections, Subcutaneous | Adult | Female | Aged | Hemolytic-Uremic Syndrome - prevention & control | Original Research
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14. Full Text Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab
by Chemnitz, Jens Marcus and Uener, Jens and Hallek, Michael and Scheid, Christof
Annals of Hematology, ISSN 0939-5555, 10/2010, Volume 89, Issue 10, pp. 1029 - 1033
Rituximab may be used to treat patients with thrombotic thrombocytopenic purpura (TTP) refractory to plasma exchange or recurrent disease. While initial... 
TTP | Treatment | Medicine & Public Health | Hematology | Rituximab | Oncology | Follow-up | ANTIBODIES | SURVIVAL | HEMOLYTIC-UREMIC SYNDROME | DISORDERS | VON-WILLEBRAND-FACTOR | FACTOR-CLEAVING PROTEASE | ADAMTS13 | THERAPY | HEMATOLOGY | Recurrence | ADAM Proteins - antagonists & inhibitors | Antibodies, Monoclonal, Murine-Derived | Follow-Up Studies | Purpura, Thrombocytopenic, Idiopathic - prevention & control | Humans | Antibodies, Monoclonal - therapeutic use | Treatment Outcome | Plasma Exchange | ADAMTS13 Protein | Purpura, Thrombotic Thrombocytopenic - prevention & control | Remission Induction | Purpura, Thrombocytopenic, Idiopathic - drug therapy | ADAM Proteins - metabolism | Purpura, Thrombotic Thrombocytopenic - pathology | Purpura, Thrombocytopenic, Idiopathic - pathology | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Purpura, Thrombotic Thrombocytopenic - physiopathology | Immunologic Factors - therapeutic use | Purpura, Thrombocytopenic, Idiopathic - physiopathology | Thrombocytopenic purpura
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15. Full Text Special delivery for ADAMTS13
by Desch, Karl
Blood, ISSN 0006-4971, 05/2015, Volume 125, Issue 21, pp. 3218 - 3219
In this issue of Blood, using a transgenic mouse model, Pickens et al describe that ectopic expression of ADAMTS13, exclusively in platelets, is able to... 
ARTERIAL THROMBOSIS | THROMBOTIC THROMBOCYTOPENIC PURPURA | HEMATOLOGY | PLATELETS | ADAM Proteins - metabolism | Animals | Thrombosis - complications | Purpura, Thrombotic Thrombocytopenic - prevention & control | Humans | Genetic Therapy - methods
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