Results 31 to 40 of about 8,844 (195)

Pathogen-reduced plasma, cryoprecipitate reduced for therapeutic plasma exchange. [PDF]

Transfusion
Abstract Background Therapeutic plasma exchange (TPE) for thrombotic thrombocytopenic purpura (TTP) and auto‐immune disorders involves repeated patient exposure to allogenic plasma with the risk of transfusion‐transmitted infection (TTI).
Tupin F, Mouriaux C, Hechler B, Kaastrup K, Yegneswaran S, Corash L, Mangin PH.   +6 more
europepmc   +2 more sources

Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia. [PDF]

, 2009
The activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease is low in several conditions, including HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome.
Bõze, Tamás, Cervenak, László, Derzsy, Zoltán, Gombos, Tímea, Hársfalvi, Jolán, Makó, Veronika, Molvarec, Attila, Prohászka, Zoltán, Rigó, János, Udvardy, Miklós László   +9 more
core   +1 more source

Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13 [PDF]

Clinical Kidney Journal, 2018
A 3-month-old male infant developed an extremely severe episode of atypical hemolytic uremic syndrome (aHUS) associated with partial deficiencies of full-length complement factor H (FH; ∼15% of infant normal) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) (39% of normal) and autoantibodies reactive with
Mini Michael, Nancy Turner, Ewa Elenberg, Linda G Shaffer, Jun Teruya, Mazen Arar, Shiu-Ki Hui, Richard J Smith, Joel Moake   +8 more
openaire   +3 more sources

Von Willebrand Factor and ADAMTS13 in COVID-19 and Beyond: A Question of Balance

European Medical Journal Nephrology, 2021
von Willebrand factor (VWF) is a large, adhesive, multimeric protein involved in haemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect in VWF can lead to von Willebrand disease
Emmanuel J Favaloro, Brandon Michael Henry, Giuseppe Lippi   +2 more
doaj  

Three disintegrin-like domain mutations of ADAMTS13： functional deficiency and association with thrombosis [PDF]

Zhenduanxue lilun yu shijian
Objective To analyze the genotypic and phenotypic characteristics of four thrombotic patients carrying heterozygous mutations in the disintegrin-like domain of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13), and to ...
LIN Liya, WU Xi, MAO Yinqi, CHEN Guangming, WU Wenman, DAI Jing, WANG Xuefeng, DING Qiulan
doaj   +1 more source

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome

Haematologica, 2011
Background The assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear.Design and ...
Shangbin Yang, Ming Jin, Shili Lin, Spero Cataland, Haifeng Wu   +4 more
doaj   +1 more source

Degradation of two novel congenital TTP ADAMTS13 mutants by the cell proteasome prevents ADAMTS13 secretion [PDF]

, 2016
INTRODUCTION: Over 150 mutations have been identified in the ADAMTS13 gene in patients with congenital thrombotic thrombocytopenic purpura (TTP). The majority of these (86%), lead to reduced (
Garagiola, I, Machin, S, Mackie, I, Peyvandi, F, Underwood, M   +4 more
core   +1 more source

Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship

Haematologica, 2007
We present a patient with a history of benign monoclonal gammopathy, who developed thrombotic thrombocytopenic purpura (TTP), initially presenting as bilateral serous retinal detachment.
Niels P Riksen, Brenda M Luken, Ina S Klasen, Jan Voorberg, Niels Crama, Marcel van Deuren   +5 more
doaj   +1 more source

Thrombotic Thrombocytopenic Purpura in a Patient With Triple‐Negative Breast Cancer Treated With PD‐L1 Inhibition and Taxane Chemotherapy [PDF]

Clin Case Rep
PET imaging of a 56 year old woman with HIV and advanced triple negative breast cancer with regional lymph node and bone metastases. ABSTRACT Thrombocytopenic purpura (TTP) is a hematologic emergency that may occur with PD‐L1 immunotherapy. New or worsening anemia and thrombocytopenia in patients started on PD‐L1 inhibitors should raise suspicion for ...
Ke J, Chong A, Hsu R, Martynova A, Wald‐Dickler N, D'Souza A, Piatek C, In G.   +7 more
europepmc   +2 more sources

Perinatal Gene Transfer to the Liver [PDF]

, 2011
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM, Chan, JK, Howe, SJ, McKay, TR, Rahim, AA, Waddington, SN, Ward, NJ   +6 more
core   +1 more source