Results 31 to 40 of about 5,761 (161)

Hereditary Thrombotic Thrombocytopenic Purpura in a Chinese Boy With a Novel Compound Heterozygous Mutation of the ADAMTS13 Gene

Frontiers in Pediatrics, 2020
Hereditary thrombotic thrombocytopenic purpura (TTP) is caused by ADAMTS13 mutations with autosomal recessive inheritance. It typically presents during childhood and is frequently misdiagnosed as immune thrombocytopenia.
Yi-ling Dai, Yi-ling Dai, Xue Tang, Xue Tang, Hong-bo Chen, Hong-bo Chen, Qiu-yu Peng, Qiu-yu Peng, Xia Guo, Xia Guo, Ju Gao, Ju Gao   +11 more
doaj   +1 more source

The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease

Journal of Cardiovascular Development and Disease, 2021
von Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (
Benjamin Reardon, Leonardo Pasalic, Emmanuel J. Favaloro   +2 more
doaj   +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

Perinatal outcomes in normotensive versus hypertensive HELLP syndrome

International Journal of Gynecology &Obstetrics, EarlyView.
Abstract Objective To compare maternal and neonatal outcomes between women with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome with and without associated hypertension. Methods This was a retrospective cohort study at a tertiary university‐affiliated medical center including all singleton HELLP cases >24 weeks gestation (January ...
Matan Anteby, Keren‐Or Wertheimer, Omri Dominsky, Liran Hiersch, Yariv Yogev   +4 more
wiley   +1 more source

Shedding Light on the Possible Link between ADAMTS13 and Vaccine—Induced Thrombotic Thrombocytopenia

Cells, 2021
Several recent reports have highlighted the onset of vaccine-induced thrombotic thrombocytopaenia (VITT) in some recipients (approximately 1 case out of 100k exposures) of the ChAdOx1 nCoV-19 vaccine (AstraZeneca).
Anna Szóstek-Mioduchowska, Paweł Kordowitzki   +1 more
doaj   +1 more source

Thirty five novel nsSNPs may effect on ADAMTS13 protein leading to Thrombotic thrombocytopenic purpura (TTP) using bioinformatics approach [PDF]

, 2019
ABSTRACT Background Genetic polymorphisms in the ADAMTS13 gene are associated with thrombotic thrombocytopenic purpura or TTP, a life-threatening microangiopathic disorder.
Abdelhameed, Tebyan A., Ahmed, Arwa I., Mustafa, Mujahed I., Eltayeb, Amel N., Abdelrhman, Fatima A., Ahmed, Amal B., Ahmed, Najla B., Khadir, Hiba Y., Hassan, Adla M., Mohamed, Huda K., Osman, Soada A., Elhag, Mustafa, Hassan, Mohamed A.   +12 more
openaire   +1 more source

A Comprehensive Study of Bidirectional Interactions Between the Human Microbiome and Blood Malignancies and Hematologic Conditions: Focus on Novel Therapeutic Strategies

Journal of Clinical Laboratory Analysis, EarlyView.
Microbiota‐targeted therapeutic strategies in hematological disorders. aHSCT: Transplanted immune cells from the donor may attack the recipient's tissues, especially when damage to the intestinal epithelium disrupts the gut microbiota, contributing to GVHD. Maintaining a balanced gut microbiota that supports immune regulation helps reduce GVHD risk and
Alireza Molajafari, Hadi Sedigh Ebrahim‐Saraie, Majid Taati Moghadam, Meysam Hasannejad‐Bibalan   +3 more
wiley   +1 more source

Role of defensins in immunothrombosis

Rheumatology &Autoimmunity, EarlyView.
Defensins are important proteins that link the immune system and blood clotting, playing a key role in immunothrombosis. α‐defensins mainly come from neutrophils, while β‐defensins are produced by epithelial cells and platelets. These proteins help form blood clots in several ways: they slow down clot breakdown (fibrinolysis), activate platelets, and ...
Yuebing Wang, Guijuan Guo, Xiangjun Liu, Xiaoyu Chen, Wenqiong Wang, Patapong Towiwat, Ru Li, Chun Li   +7 more
wiley   +1 more source

Preserved Expression of mRNA Coding von Willebrand Factor-Cleaving Protease ADAMTS13 by Selenite and Activated Protein C

Molecular Medicine, 2015
In sepsis, the severity-dependent decrease of von Willebrand factor (VWF)-inactivating protease, a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), results in platelet aggregation and consumption, leading to sepsis-associated thrombotic microangiopathy (TMA) and organ failure.
Michael L, Ekaney, Clemens L, Bockmeyer, Maik, Sossdorf, Philipp A, Reuken, Florian, Conradi, Tobias, Schuerholz, Markus F, Blaess, Scott L, Friedman, Wolfgang, Lösche, Michael, Bauer, Ralf A, Claus   +10 more
openaire   +2 more sources

Thrombotic Microangiopathy‐Like Phenotype in Patients With Infection‐Associated Disseminated Intravascular Coagulation Treated With Thrombomodulin Alfa

European Journal of Haematology, EarlyView.
ABSTRACT Despite disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) sharing features of thrombocytopenia, organ dysfunction, and bleeding, the relationship between these two conditions remains unclear. We therefore conducted a post hoc analysis of post‐marketing surveillance data from Japan to evaluate the clinical ...
Naoki Takezako, Fumiyo Komatsu, Goichi Honda, Noriaki Kawano, Toshimasa Uchiyama, Kazuo Kawasugi, Seiji Madoiwa, Kei Suzuki, Yoshinobu Seki, Takayuki Ikezoe, Kohji Okamoto, Hideo Wada   +11 more
wiley   +1 more source