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https://www.readbyqxmd.com/read/28445600/caplacizumab-reduces-the-frequency-of-major-thromboembolic-events-exacerbations-and-death-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#1
F Peyvandi, M Scully, J A Kremer Hovinga, P Knöbl, S Cataland, K De Beuf, F Callewaert, H De Winter, R K Zeldin
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations and death. In the Phase II TITAN study, treatment with caplacizumab, an anti-vWF Nanobody(®) , was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. OBJECTIVE: The clinical benefit of caplacizumab was further investigated in a post-hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and TTP-related death during the study...
April 26, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28444411/-acute-liver-failure
#2
A Koch, C Trautwein, F Tacke
Acute liver failure (ALF) is a rare, but life-threatening disease that is characterized by the acute onset of jaundice, coagulopathy, and hepatic encephalopathy (HE) in patients without pre-existing liver disease. Main causes in Germany are drug toxicity, acetaminophen overdose, and viral hepatitis (A, B, E). For the initial assessment of patients with ALF and the diagnostic algorithm, the early detection of HE, exclusion of liver cirrhosis, immediate diagnosis of the underlying etiology, and evaluation for the necessity of liver transplantation (LT) are critical...
April 25, 2017: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/28439919/motor-polyradiculopathy-during-pembrolizumab-treatment-of-metastatic-melanoma
#3
Maria Sepúlveda, Eugenia Martinez-Hernandez, Lydia Gaba, Ivan Victoria, Nuria Sola-Valls, Neus Falgàs, Jordi Casanova-Molla, Francesc Graus
INTRODUCTION: Pembrolizumab, a monoclonal antibody directed against the immune checkpoint PD-1 (programmed cell death-1 receptor), has improved survival in patients with advanced melanoma. Neuromuscular immune-mediated side effects have been rarely reported. METHODS: We describe a 44-year-old man with metastatic melanoma who presented with progressive muscle weakness after 23 doses of pembrolizumab. RESULTS: The patient developed asymmetric, proximal muscle weakness and atrophy in all four limbs...
April 25, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28438080/guillain-barr%C3%A3-syndrome-in-pregnancy-a-case-report
#4
Misai Hukuimwe, Tawanda T Matsa, Muchabayiwa F Gidiri
Guillain-Barré syndrome is a rare condition in pregnancy which is characterised by symmetrical progressive ascending polyneuropathy. A case of a 16-year-old nulliparous woman who presented with rapidly progressive limb paralysis following an upper respiratory tract infection a week prior to presentation is discussed. She was intubated as she had developed respiratory failure and managed in the intensive care unit by a multidisciplinary team. Plasma exchange and intravenous immunoglobulin were not readily available so she was managed conservatively...
April 1, 2017: Women's Health
https://www.readbyqxmd.com/read/28436106/therapeutic-plasma-exchange-as-part-of-multimodal-treatment-of-acquired-hemophilia-in-a-patient-with-concurrent-acute-intracerebral-bleed-and-pulmonary-embolism
#5
Geoffrey D Wool, David Chapel, Angela Treml, Jonathan L Miller
BACKGROUND: Autoantibodies against Factor VIII (FVIII) define the rare but life-threatening bleeding disorder acquired hemophilia A (AHA). Correction of FVIII deficiency and eradication of the factor inhibitor are the ultimate therapeutic goals in this disorder. Bypassing agents such as recombinant factor VIIa (rFVIIa) or FVIII inhibitor bypassing agent are often used to control coagulopathy before the inhibitor is eradicated. Bypassing agents carry a risk of thrombosis, however. CASE REPORT: We report a patient with newly diagnosed AHA and thalamic bleed who additionally had active atrial fibrillation and developed a segmental pulmonary embolism, limiting tolerable rFVIIa dosage...
April 24, 2017: Transfusion
https://www.readbyqxmd.com/read/28427710/clinical-outcomes-and-predictive-factors-related-to-good-outcomes-in-plasma-exchange-in-severe-attack-of-nmosd-and-long-extensive-transverse-myelitis-case-series-and-review-of-the-literature
#6
Saharat Aungsumart, Metha Apiwattanakul
OBJECTIVE: To investigate the predictive factors associated with good outcomes of plasma exchange in severe attacks through neuromyelitis optica spectrum disorder (NMOSD) and long extensive transverse myelitis (LETM). In addition, to review the literature of predictive factors associated with the good outcomes of plasma exchange in central nervous system inflammatory demyelinating diseases (CNS IDDs). METHODS: Retrospective study in 27 episodes of severe acute attacks myelitis and optic neuritis in 24 patients, including 20 patients with NMOSD seropositive, 1 patient with NMOSD seronegative and 3 patients with LETM...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28427692/treatment-resistant-neuromyelitis-optica-spectrum-disorders-associated-with-toxocara-canis-infection-a-case-report
#7
Daisuke Kambe, Kayo Takeoka, Kenta Ogawa, Kosuke Doi, Haruhiko Maruyama, Ayako Yoshida, Toshihiko Suenaga, Takashi Kageyama
A 53-year-old woman was admitted to the department of neurology in Tenri Hospital because of progressive thoracic myelitis a month after she had eaten uncooked bovine liver. A previous episode of right optic neuritis and a positive test for serum anti-aquaporin-4 antibodies indicated a diagnosis of neuromyelitis optica spectrum disorders. Although the patient initially recovered with the reduction of anti-aquaporin-4 antibodies during treatment with intravenous methylprednisolone infusion and plasma exchange, her neurological symptoms deteriorated soon after the completion of plasma exchange...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28417344/biotherapy-in-inflammatory-diseases-of-the-cns-current-knowledge-and-applications
#8
REVIEW
Nicolas Collongues, Laure Michel, Jérôme de Seze
Biotherapy represents an innovative therapeutic approach that includes immunotherapy (vaccines, apheresis, and antibodies); gene therapy; and stem cell transplants. Their development helps to cross the bridge from bench to bedside and brings new hope of a cure for severe diseases in different fields of medicine. In neurology, a growing range of applications is being developed for these medications. Valuable results are now available in the field of autoimmunity, neuro-oncology, paraneoplastic manifestations, and neurodegenerative disorders...
May 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#9
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#10
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#11
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28414674/a-clot-possibly-due-to-loss-of-tnf-%C3%AE-supression
#12
J Broussard, M Berlinger, D Lauret
INTRODUCTION: Inflammation and venous thrombosis enjoy a close Relationship. We present a patient who had multiple DVTs following the discontinuation of anti- TNF-α therapy. CASE: A 34 year old African American female with a history of multiple DVT's, miscarriages, and reported Crohn's disease presented with shortness of breath. In the Emergency Department, CTA showed bilateral pulmonary emboli. The patient had been off adalimumab for one year and reported abdominal pain with 6-7 non-bloody bowel movements daily...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28413713/catastrophic-outcome-of-patients-with-a-rebound-after-natalizumab-treatment-discontinuation
#13
Inés González-Suarez, Luis Rodríguez de Antonio, Aida Orviz, Sara Moreno-García, María D Valle-Arcos, Jordi A Matias-Guiu, Cristina Valencia, Manuela Jorquera Moya, Celia Oreja-Guevara
INTRODUCTION: Natalizumab (NTZ) is an effective drug for the treatment of relapsing-remitting multiple sclerosis. In some patients discontinuation is mandatory due to the risk of progressive multifocal leukoencephalopathy. However, severe clinical and radiological worsening has been described after drug cessation. Our aim was to describe the clinical and radiological features of the rebound phenomenon. MATERIAL AND METHODS: Patients switched from NTZ to Fingolimod (FTY) who had presented a rebound after discontinuation were selected...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28413093/-thrombotic-thrombocytopenia-purpura-in-martinique-retrospective-study-between-2008%C3%A2-and-2015
#14
M Patient, P Fuseau, C Deligny
INTRODUCTION: Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. METHODS: We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28405075/acquired-thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-successfully-treated-with-eculizumab
#15
Appalanaidu Sasapu, Michele Cottler-Fox, Pooja Motwani
Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with a mortality of up to 90%, if not promptly recognized and treated. We report a 64-year-old woman with this condition who presented with left-sided weakness and seizure-like activity preceded by headache and easy bruising. She did not achieve optimal response to plasma exchange, corticosteroids, rituximab, and vincristine. We initiated treatment with eculizumab, following which she had durable remission that continued for 30 months after discontinuation of the drug...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28401611/pegylated-bovine-carboxyhaemoglobin-utilisation-in-a-thrombotic-thrombocytopenic-purpura-patient
#16
C Sam, P Desai, D Laber, A Patel, N Visweshwar, M Jaglal
OBJECTIVE: To determine if pegylated bovine carboxyhaemoglobin can be utilised in a thrombotic thrombocytopenic purpura (TTP) patient. BACKGROUND: TTP is a condition characterized by thrombotic microangiopathy and has a high mortality rate when left untreated. Therapeutic plasma exchange is well established as the most effective and evidence-based treatment of TTP. METHODS: The ability to administer plasma exchange therapy is limited in Jehovah's Witnesses who decline blood products due to religious beliefs...
April 12, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28383422/acquired-idiopathic-thrombotic-thrombocytopenic-purpura-successfully-treated-with-intravenous-immunoglobulin-and-glucocorticoid-a-case-report
#17
Hiro Nakao, Akira Ishiguro, Nahoko Ikoma, Kentaro Nishi, Chemin Su, Hisaya Nakadate, Mitsuru Kubota, Masaki Hayakawa, Masanori Matsumoto
RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#18
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28382612/clinical-profile-and-outcome-of-myasthenic-crisis-in-central-taiwan
#19
Yi-Sin Wong, Cheung-Ter Ong, Sheng-Feng Sung, Chi-Shun Wu, Yung-Chu Hsu, Yu-Hsiang Su, Ling-Chien Hung
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by antibodies to acetylcholine receptors of the skeletal muscle. Myasthenic crisis (MC) is a complication observed during both early and late stage MG cases. In this study, we examined current treatments and three years outcomes in patients with MG and MC. We also investigated the impact of thymectomy and systemic lupus erythematosus (SLE) in patients with MG and MC. METHODS: In this retrospective study, we reviewed the medical records of all patients admitted to one teaching hospital between January 2006 and December 2014 and identified those for whom discharge diagnosis included the International Classification of Diseases, ninth revision (ICD-9) codes corresponding to MG (358...
December 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/28382598/patients-with-anca-associated-vasculitis-admitted-to-the-intensive-care-unit-with-acute-vasculitis-manifestations-a-retrospective-and-comparative-multicentric-study
#20
Julien Demiselle, Johann Auchabie, François Beloncle, Philippe Gatault, Steven Grangé, Damien Du Cheyron, Jean Dellamonica, Sonia Boyer, Dimitri Titeca Beauport, Lise Piquilloud, Julien Letheulle, Christophe Guitton, Nicolas Chudeau, Guillaume Geri, François Fourrier, René Robert, Emmanuel Guérot, Julie Boisramé-Helms, Pierre Galichon, Pierre-François Dequin, Alexandre Lautrette, Pierre-Edouard Bollaert, Ferhat Meziani, Loïc Guillevin, Nicolas Lerolle, Jean-François Augusto
PURPOSE: Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. METHODS: We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group...
December 2017: Annals of Intensive Care
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