Results 101 to 110 of about 8,844 (195)

Presentation of HIV‐Associated Thrombotic Microangiopathy and Response to Therapeutic Plasma Exchange: A 10‐year Retrospective Single‐Centre Cohort Study

open access: yesHealth Science Reports, Volume 9, Issue 3, March 2026.
ABSTRACT Background HIV is a significant aetiological factor in thrombotic microangiopathy (TMA) in regions of high seroprevalence, but description of HIV‐associated TMA (HIV‐TMA) remains limited to small case series. We sought to describe the presentation, complications of TPE, and mortality and renal outcomes of HIV‐TMA.
Malcolm Davies   +4 more
wiley   +1 more source

Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion

open access: yesHaematologica, 2008
Background In mammalian cells a regulatory mechanism, known as nonsense-mediated mRNA decay, degrades mRNA harboring premature termination codons. This mechanism is intron-dependent and functions as a quality control mechanism to eliminate abnormal ...
Isabella Garagiola   +5 more
doaj   +1 more source

A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13 [PDF]

open access: yes, 2017
In this case report, we describe for the first time a patient with thrombotic thrombocytopaenic purpura (TTP) accompanying highly active systemic lupus erythematosus (SLE) that was associated with non-neutralizing antibodies against the plasma ...
Ferrari, Silvia   +4 more
core  

Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS

open access: yesBlood Advances
: Allosteric regulation of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type-1 motif, member 13) activity involves an interaction between its spacer (S) and 2 complement C1r/C1s, Uegf and BMP1 (CUB; CUB1-2) domains to keep the enzyme
Quintijn Bonnez   +8 more
doaj   +1 more source

Successful Treatment of Refractory Immune Thrombotic Thrombocytopenic Purpura with Daratumumab

open access: yesКлиническая онкогематология
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening disease manifested by thrombocytopenia, microangiopathic hemolytic anemia, and impaired functions of organs and systems due to the thrombosis of small arteries.
E. E. Klebanova   +5 more
doaj   +1 more source

Moschcowitz Disease in pediatric. [PDF]

open access: yesBatna Journal of Medical Sciences
Hemichorea Moskowitz disease is a rare hematological disorder known to be an autoimmune disease in most patients. Most cases of thrombotic thrombopenic purpura (TTP) are caused by problems with an enzyme or protein in the blood called ADAMTS13.
Samira AGGOUNE, Mohamed Faouzi SEDDIKI
doaj   +1 more source

ADAMTS9, a member of the ADAMTS family, in Xenopus development [PDF]

open access: yes, 2018
Extracellular matrix (ECM) remodeling by metalloproteinases is crucial during development. The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin type I motifs) enzymes are secreted, multi-domain matrix-associated zinc metalloendopeptidases ...
Boerboom   +37 more
core   +1 more source

[Management of congenital thrombotic thrombocytopenic purpura in the era of recombinant ADAMTS13 protein: Recommendations from the Reference Center for Thrombotic Microangiopathies (CNR-MAT)].

open access: yesLa Revue de medecine interne
Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene.
Joly, Bérangère S.   +8 more
openaire   +2 more sources

Rat models of thrombotic thrombocytopenic purpura reveal crucial role of placental ADAMTS13 in perinatal survival

open access: yesBlood Advances
: Thrombotic thrombocytopenic purpura (TTP), a life-threatening thrombotic microangiopathy, is caused by severe deficiency of plasma ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, 13) activity.
Quan Zhang   +6 more
doaj   +1 more source

Biocompatibility and antibacterial properties of zirconium nitride coating on titanium abutments: An in vitro study [PDF]

open access: yes, 2018
Improving soft tissue attachment and reducing bacterial colonization on titanium abutments are key factors for the long-term maintenance of healthy soft and hard peri-implant tissues.
Bressan, Eriberto   +7 more
core   +2 more sources

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