Results 51 to 60 of about 69,242 (204)
Thrombotic Thrombocytopenic Purpura in a Newborn [PDF]
This report describes a newborn who presented with hyperbilirubinemia and thrombocytopenia. The patient recovered after treatment with antibiotics, phototherapy, and a platelet transfusion. Analysis of the plasma von Willebrand factor-cleaving metalloprotease, ADAMTS13, revealed low protease activity in the patient and her two siblings, and a mild ...
Paul T, Jubinsky +2 more
openaire +2 more sources
Acute pancreatitis can be encountered as a rare complication of thrombotic thrombocytopenic purpura and it has been associated with recurrent thrombotic thrombocytopenic purpura.
Mesudiye BULUT +6 more
doaj
ABSTRACT Introduction The 2024 ISTH clinical practice guideline (CPG) for treatment of congenital haemophilia, the NBDF‐McMaster Guideline on Care Models for Haemophilia Management, and ASH ISTH NBDF WFH guidelines on the diagnosis and management of VWD all utilised GRADE methodology.
Mark W. Skinner +59 more
wiley +1 more source
The relationship between ADAMTS13 genotype and phenotype in congenital thrombotic thrombocytopenic purpura and characterisation of ADAMTS13 mutants [PDF]
Congenital thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, usually involving ADAMTS13 gene defects. ADAMTS13 processes the multimeric plasma glycoprotein Von Willebrand factor making it less reactive to platelets.
Underwood, MI
core
Current management of thrombotic thrombocytopenic purpura
PURPOSE OF REVIEW: New treatment modalities have become increasingly popular for the treatment of acute thrombotic thrombocytopenic purpura. Widespread availability of ADAMTS13 assays resulted in the increased recognition of patients with hereditary ...
Kremer Hovinga, Johanna A, Meyer, Sara C
core +1 more source
Background Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are two forms of thrombotic microangiopathies. They are characterized by severe thrombocytopenia, microangiopathic hemolysis, and thrombosis, leading to a systemic inflammatory ...
Jose Perez Lara +5 more
doaj +1 more source
ABSTRACT Aim The therapeutic significance of recombinant a disintegrin‐like and metalloprotease with thrombospondin type 1 motif 13 (rADAMTS13) in acute‐on‐chronic liver failure (ACLF) remains unclear. We aimed to investigate the therapeutic effects of rADAMTS13 in a murine model of ACLF‐like liver injury.
Jun‐ichi Hanatani +9 more
wiley +1 more source
Diagnosis of thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP
G. M. Galstyan, E. E. Klebanova
openaire +4 more sources
ABSTRACT Introduction Thrombocytopenia has heterogeneous mechanisms, including peripheral destruction, consumption, and reduced marrow production. This study developed an unsupervised global complete blood count/research‐use‐only/cell population data (CBC/RUO/CPD) phenotyping model and evaluated post hoc whether thrombocytopenia severity and clinically
Mohammad A. Altememi +5 more
wiley +1 more source
Coma in thrombotic thrombocytopenic purpura [PDF]
Thrombotic thrombocytopenic purpura (TTP) is characterised by a thrombotic, haemolytic microangiopathy leading to microvascular occlusion, haemolysis and ischaemic dysfunction of various organs including the brain. TTP may present with a variety of neurological symptoms, including headache, focal deficits, seizures and coma.
De Jong, F. J. +3 more
openaire +2 more sources

