Search results for "Purpura"
showing 8 items of 88 documents
Efficacy and safety of open-label caplacizumab in patients with exacerbations of acquired thrombotic thrombocytopenic purpura in the HERCULES study.
2020
BACKGROUND Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Caplacizumab, an anti-von Willebrand Factor Nanobody® , is effective for treating aTTP episodes and is well tolerated. OBJECTIVES AND METHODS In the phase 3 HERCULES trial (NCT02553317), patients with aTTP received double-blind caplacizumab or placebo during daily therapeutic plasma exchange (TPE) and for ≥30 days thereafter. Patients who experienced an exacerbation while on blinded study drug treatment switched to receive open-label caplacizumab plus re-initiation of daily TPE. Exacerbations were defined as recurrence of disease occurring within 30 days after ce…
Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management
2021
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTT…
Algoritmo diagnóstico-terapéutico de las microangiopatías trombóticas. A propósito de 2 casos
2022
Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).
Thrombotic thrombocytopenic purpura (TTP) leading to pseudotumour's autoimmune pancreatitis (AIP): A case report
2012
International audience; Introduction: Autoimmune pancreatitis is an idiopathic inflammatory disease that produces pancreatic masses and ductal strictures. This benign disease can be associated with extrapancreatic manifestations including cholangitis, sialadenitis, inflammatory bowel disease or retroperitoneal fibrosis, mediastinal adenopathy, interstitial nephritis mainly due to immunoglobulin G4 (Ig G4), and occasional association with other auto-immune diseases. Observation: We report a 57-year-old woman who developed thrombotic thrombocytopenic purpura (UP) and pseudo-tumour's seronegative autoimmune pancreatitis (ATP) type 1. The patient was initially treated with pulse corticosteroids…
Patterns of changes of anti‐ADAMTS13 after plasma exchange
2006
An enzyme-linked immunosorbent assay (ELISA) has recently been developed to detect antibodies against the von Willebrand factor-cleaving protease ADAMTS13 in patients with thrombotic thrombocytopenic purpura (TTP). The ELISA is based on incubation of plasma with immobilized recombinant ADAMTS13 followed by visualization of IgG and IgM antibodies by means of secondary enzyme-labeled antibodies [1]. In a recent study, anti-ADAMTS13 IgG antibodies were detected in most patients with TTP (97%) characterized by severe ADAMTS13 deficiency (< 10% of normal) [1]. The ELISA was more sensitive than the standard inhibitor assay based upon ADAMTS13 neutralizing activity, which gave positive results in …
Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.
2010
The Developmental Transcriptome for Lytechinus variegatus Exhibits Temporally Punctuated Gene Expression Changes
2019
AbstractEmbryonic development is arguably the most complex process an organism undergoes during its lifetime, and understanding this complexity is best approached with a systems-level perspective. The sea urchin has become a highly valuable model organism for understanding developmental specification, morphogenesis, and evolution. As a non-chordate deuterostome, the sea urchin occupies an important evolutionary niche between protostomes and vertebrates.Lytechinus variegatus(Lv) is an Atlantic species that has been well studied, and which has provided important insights into signal transduction, patterning, and morphogenetic changes during embryonic and larval development. The Pacific specie…
Naratīva struktūra un kino stils Giljermo del Toro filmās “Pāna labirints”, “Purpura smaile” un “Ūdens forma”
2018
Bakalaura darba tēma ir “Naratīva struktūra un kino stils Giljermo del Toro filmās “Pāna labirints”, “Purpura smaile” un “Ūdens forma””. Pētījuma mērķis ir saprast naratīvo struktūru un režisoriskās iezīmes, jeb stila iezīmes Giljermo del Toro filmās, šim mērķim teoritiskāja daļa tiek apskatītas kino valodas, semiotikas un naratīva teoriju tēmas. Lietojot semiotisko analīzi, naratīvo analīzi, kā arī izmantojot kinozinātnieka Jurija Civjana (Yuri Tsivian) rīku “Cinemetrics” tiks analizētas augstākminētās kinofilmas. Analizējot pētījumā rezultātus secināt par režisora kino stilu un filmu naratīvo analīzi.