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thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29897485/severe-underestimation-of-serum-na-following-ivig-treatment
#1
Mrigender S Virk, Nathan P Dean, Edward C C Wong
Current chemistry analyzers measure ion concentration using ion- selective electrodes; however, may differ in the specific technology at the bedside versus the central laboratory. Instruments utilized for point-of-care testing (POCT) at the bedside use direct ion-selective electrodes, whereas central-laboratory analyzers use indirect ion-selective electrodes. Under most circumstances, these instruments will deliver the same result; however, various substances can cause interferences in one or the other. An 18-year-old Hispanic woman with a history of immune thrombocytopenic purpura (ITP) presented at Children's National Medical Center (CNMC) with a severe headache and required intravenous immunoglobulin (IVIG) therapy...
June 12, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29889319/pediatric-thrombotic-thrombocytopenic-purpura
#2
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
June 11, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29885460/adamts-proteins-in-human-disorders
#3
REVIEW
Timothy J Mead, Suneel S Apte
ADAMTS proteins are a superfamily of 26 secreted molecules comprising two related, but distinct families. ADAMTS proteases are zinc metalloendopeptidases, most of whose substrates are extracellular matrix (ECM) components, whereas ADAMTS-like proteins lack a metalloprotease domain, reside in the ECM and have regulatory roles vis-à-vis ECM assembly and/or ADAMTS activity. Evolutionary conservation and expansion of ADAMTS proteins in mammals is suggestive of crucial embryologic or physiological roles in humans...
June 6, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29881971/-new-aspects-of-immunotherapy-in-multiple-sclerosis
#4
REVIEW
K Pape, F Zipp, S Bittner
The spectrum of therapeutic options for immunotherapy of multiple sclerosis is continuously broadening. After the approval of cladribine and ocrelizumab in Europe, two new drugs are now available with ocrelizumab being the first approved option for treatment of primary progressive multiple sclerosis; however, the increased use of highly effective therapies is accompanied by a rise in severe side effects. During recent months, special attention was paid to the new progressive multifocal leukoencephalopathy (PML) risk assessment in natalizumab-treated patients, cardiac side effects of fingolimod, cases of idiopathic thrombocytopenic purpura and listeria meningitis associated with alemtuzumab and cases of daclizumab-treated patients with liver failure or encephalitis...
June 7, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29880389/the-diagnostic-value-of-splenectomy-in-patients-without-a-definitive-preoperative-diagnosis
#5
Patrick T Hangge, William W Sheaffer, Matthew Neville, Nabil Wasif, Richard J Gray, Barbara A Pockaj, Chee-Chee H Stucky
BACKGROUND: The purpose of this study was to describe the diagnostic value and therapeutic benefit of diagnostic splenectomy. METHODS: Retrospective review was performed of patients undergoing splenectomy with an unknown diagnosis (UD), a hematologic malignancy (HM) or idiopathic thrombocytopenic purpura. Surgical indications and postoperative outcomes were evaluated. RESULTS: 113 splenectomy patients were identified. Of the UD patients undergoing splenectomy, 46% (n = 16) received a definitive diagnosis postoperatively...
May 29, 2018: American Journal of Surgery
https://www.readbyqxmd.com/read/29877197/association-between-helicobacter-pylori-infection-and-platelet-count-in-mice
#6
Teruko Fukuda, Eri Asou, Kimiko Nogi, Masahiko Yasuda, Kazuo Goto
Strong evidence for an association between idiopathic thrombocytopenic purpura (ITP) and Helicobacter pylori (HP) infection has been reported in humans. Chronic ITP is known to be improved by the eradication of HP. The purpose of this study was to reproduce these events by the experimental infection of several strains of mice with HP. BALB/c, C57BL/6, and DBA/2 mice were untreated or orally inoculated with HP. Two months later, platelet counts were compared in samples from HP-infected and noninfected mice. Platelet counts (mean ± SD, × 104 )/μl) in blood samples from HP-infected BALB/c, C57BL/6, and DBA/2 mice were 102...
June 7, 2018: Experimental Animals
https://www.readbyqxmd.com/read/29871663/two-potentially-lethal-conditions-of-probable-immune-origin-occurring-in-a-pregnant-woman-a-case-report
#7
H M Senanayake, M Patabendige
BACKGROUND: Thrombotic thrombocytopenic purpura and peripartum cardiomyopathy are potentially lethal complications of pregnancy. We describe a case in which both of these developed in the same patient. The etiologies of both conditions remain uncertain, but they share immune hyperreactivity as a possible cause. CASE PRESENTATION: A 33-year-old Lankan primigravida gave birth at 38 weeks of gestation by cesarean section when she presented with right-sided abdominal pain and a provisional diagnosis of appendicitis...
June 6, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29868970/does-this-patient-with-thrombotic-thrombocytopenic-purpura-have-a-cardiac-involvement
#8
Lara Zafrani, Lene Russell, Elie Azoulay
No abstract text is available yet for this article.
June 4, 2018: Intensive Care Medicine
https://www.readbyqxmd.com/read/29849587/increased-frequency-of-immune-thrombocytopenic-purpura-in-coeliac-disease-and-vice-versa-a-prospective-observational-study
#9
Stefano Bibbò, Claudio Fozza, Giovanni Mario Pes, Rodrigo Rojas, Roberto Manetti, Maria Pina Dore
Introduction: Coeliac disease (CD) and immune thrombocytopenic purpura (ITP) are immune conditions, often associated with other immune disorders. In recent years, increasing attention has been directed towards the association between ITP and CD. Aim: To investigate the frequency of ITP in CD patients and vice versa and to assess the risk of their association. Patients and Methods: This was a prospective observational study. All consecutive patients with CD or ITP attending our department were enrolled between January 2016 and December 2017...
2018: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/29847840/targeting-von-willebrand-factor-in-ischaemic-stroke-focus-on-clinical-evidence
#10
Nina Buchtele, Michael Schwameis, James C Gilbert, Christian Schörgenhofer, Bernd Jilma
Despite great efforts in stroke research, disability and recurrence rates in ischaemic stroke remain unacceptably high. To address this issue, one potential target for novel therapeutics is the glycoprotein von Willebrand factor (vWF), which increases in thrombogenicity especially under high shear rates as it bridges between vascular sub-endothelial collagen and platelets. The rationale for vWF as a potential target in stroke comes from four bodies of evidence. (1) Animal models which recapitulate the pathogenesis of stroke and validate the concept of targeting vWF for stroke prevention and the use of the vWF cleavage enzyme ADAMTS13 in acute stroke treatment...
June 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29846661/therapeutic-plasma-exchange-performed-via-2-apheresis-devices-and-its-impact-on-the-blood-profile-of-hematological-and-neurological-patients
#11
Eiman Hussein
Background: An efficient apheresis device performs therapeutic plasma exchange (TPE) sessions in short times, while avoiding significant potential cell losses in the removed plasma. Methods: To evaluate TPE performed via Optia (hereinafter, Optia) and COBE Spectra (hereinafter, COBE) apheresis devices, a paired randomized comparison study was conducted. We evaluated the device performance and its impact on cell loss in the removed plasma, as well as blood counts of patients...
May 26, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29786866/characterization-of-efficacy-and-safety-of-pathogen-inactivated-and-quarantine-plasma-in-routine-use-for-treatment-of-acquired-immune-thrombotic-thrombocytopenic-purpura
#12
R Herbrecht, M Ojeda-Uribe, D Kientz, C Fohrer, A Bohbot, O Hinschberger, K-L Liu, E Remy, C Ernst, J-S Lin, L Corash, J P Cazenave
BACKGROUND: Auto-immune thrombotic thrombocytopenic purpura (TTP) is a morbid multi-organ disorder. Cardiac involvement not recognized in initial disease descriptions is a major cause of morbidity. Therapeutic plasma exchange (TPE) requires exposure to multiple plasma donors with risk of transfusion-transmitted infection (TTI). Pathogen inactivation (PI) with amotosalen-UVA, the INTERCEPT Blood System for Plasma (IBSP) is licensed to reduce TTI risk. METHODS: An open-label, retrospective study evaluated the efficacy of quarantine plasma (QP) and IBSP in TTP and defined treatment emergent cardiac abnormalities...
May 21, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29780064/systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome-complicated-by-conversion-disorder-a-case-report
#13
Misa Nakamura, Seiji Tanaka, Tadashi Inoue, Yasuto Maeda, Kiyohito Okumiya, Takuya Esaki, G O Shimomura, Kenji Masunaga, Shinichiro Nagamitsu, Yushiro Yamashita
Conversion disorder (CD) is sometimes accompanied by motor and sensory impairments, such as muscle weakness, paralysis, sensory hypersensitivity, and sensory loss. Sjögren's syndrome (SS) complicates 5-10% of cases of systemic lupus erythematosus (SLE). Patients with SS or SLE present with various neurological symptoms and psychiatric manifestations. When neurological symptoms are present, it is important to distinguish whether the symptoms are caused by a neurological or a mental disorder because the former requires early intensive intervention, such as methylprednisolone pulse therapy (MPT), whereas psychotherapy or antidepressant drugs are recommended for mental disorders...
May 21, 2018: Kurume Medical Journal
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#14
REVIEW
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
May 17, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29777164/mrna-treatment-produces-sustained-expression-of-enzymatically-active-human-adamts13-in-mice
#15
Susan Liu-Chen, Brendan Connolly, Lei Cheng, Romesh R Subramanian, Zhaozhong Han
Thrombotic thrombocytopenic purpura (TTP) is primarily caused by deficiency of ADAMTS13 within the blood stream due to either genetic defects or presence of inhibitory autoantibodies. Preclinical and clinical studies suggest that enzyme replacement therapy with recombinant human ADAMTS13 protein (rhADAMTS13) is effective and safe in treatment of TTP. However, frequent dosing would be required due to the relatively short half-life of rhADAMTS13 in circulation as well as the presence of inhibitory autoantibodies that collectively result in the poor pharmacological profile of rhADAMTS13...
May 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29771863/upshaw-schulman-syndrome-with-c-2728c-t-mutation-in-adamts13-gene
#16
Shahzadi Resham, Zehra Fadoo, Bushra Moiz
Congenital thrombotic thrombocytopenic purpura is a rare autosomal recessive disorder presenting with hemolytic anemia, thrombocytopenia, micro vascular thrombosis, and end organ damage. Here, we present a case of a 7-year-old girl having recurrent neonatal hemolysis, developmental delay, frequent seizures, and thrombocytopenia. Characteristic clinical picture and gene sequencing of a disintegrin and metalloproteinase with thrombospondin motifs 13 confirmed the diagnosis of Upshaw-Schulman syndrome. She was treated successfully with plasma infusion...
May 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29763513/child-onset-thrombotic-thrombocytopenic-purpura-caused-by-p-r498c-and-p-g259pfsx133-mutations-in-adamts13
#17
An-Sofie Schelpe, Christelle Orlando, Bogaç Ercig, Chloë Geeroms, Inge Pareyn, Nele Vandeputte, Leydi Carolina Velásquez Pereira, Elien Roose, Karel Fostier, Gerry A F Nicolaes, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke, Kristin Jochmans
INTRODUCTION: Patients suffering from congenital thrombotic thrombocytopenic purpura (cTTP) have a deficiency in ADAMTS13 due to mutations in their ADAMTS13 gene. OBJECTIVE: The aim of this study was to determine ADAMTS13 parameters (activity, antigen and mutations), to investigate if the propositus suffered from child-onset cTTP and to study the in vitro effect of the ADAMTS13 mutations. METHODS: ADAMTS13 activity and antigen were determined using the FRETS VWF73 assay and ELISA and ADAMTS13 mutations via sequencing of the exons...
May 15, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29761111/myocardial-infarction-as-an-early-presentation-in-thrombotic-thrombocytopenic-purpura-a-rare-case-series
#18
Sumit Dahal, Dipesh K C Ghimire, Saroj Sapkota, Suyash Dahal, Paritosh Kafle, Manjul Bhandari
Renal and neurological involvements are frequently seen in thrombotic thrombocytopenic purpura (TTP). Cardiac involvement, however, has been rarely reported. In this article, we present 2 cases of myocardial infarction in patients with TTP. In the first case, a young man presented with non-ST-segment elevation myocardial infarction that resolved promptly with plasmapheresis. The second patient developed ST-segment elevation myocardial infarction early in the course of the disease and died before plasmapheresis could be initiated...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29758989/the-double-hazard-of-bleeding-and-thrombosis-in-hemostasis-from-a-clinical-point-of-view-a-global-assessment-by-rotational-thromboelastometry-rotem
#19
Olga Meltem Akay
Hemostasis is a complex dynamic process involving bleeding and thrombosis as two end-points. Conventional coagulation tests which are measured in plasma examine only isolated portions of the coagulation cascade, thereby giving no information on important interactions essential to the clinical evaluation of hemostatic function. Thromboelastography (TEG), originally described in 1948 has improved over the decades and become a valuable tool of coagulation testing because of the limitations of standard coagulation tests...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29755528/hypogammaglobulinemia-observed-one-year-after-rituximab-treatment-for-idiopathic-thrombocytopenic-purpura
#20
Bilal Ahmad Shoukat, Osama Ali, Dileep Kumar, Muhammad Bilal Gilani, Adeela Zahid, Shaheer Aslam Joiya, Maqsood Anwar Malik
We present the case of a 19-year-old female with severe hypogammaglobulinemia after having had treatment with rituximab for idiopathic thrombocytopenic purpura requiring intravenous immunoglobulins. She was admitted with the diagnosis of left-sided pneumonia with parapneumonic effusion. The patient was treated with piperacillin/tazobactam after having a poor response to co-amoxiclav. The patient had been tested for immunoglobulin levels, and the levels were very low. She has a history of ITP for which she received steroids...
2018: Case Reports in Medicine
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