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https://www.readbyqxmd.com/read/28811766/prognostic-factors-determining-morbidity-and-mortality-in-organophosphate-poisoning
#1
Ayca Acikalin, Nezihat Rana Dişel, Selcuk Matyar, Ahmet Sebe, Zeynep Kekec, Yuksel Gokel, Emre Karakoc
OBJECTIVE: Our aim in this retrospective study was to determine the factors affecting poor prognosis and mortality of organophosphate (OP) poisoning by reviewing patient data. We also reviewed present knowledge to make conclusions on certain longstanding debates in light of the literature. METHODS: In this retrospective descriptive study, patients who were admitted to and hospitalized in the emergency department (ED) or intensive care unit (ICU) of a university hospital with the diagnosis of OP poisoning between December 2010 and December 2015 were evaluated...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28800066/treatment-of-the-first-acute-relapse-following-therapeutic-plasma-exchange-in-formerly-glucocorticosteroid-unresponsive-multiple-sclerosis-patients-a-multicenter-study-to-evaluate-glucocorticosteroid-responsiveness
#2
Johannes Ehler, Stephan Blechinger, Paulus S Rommer, Sebastian Koball, Steffen Mitzner, Hans-Peter Hartung, Fritz Leutmezer, Martin Sauer, Uwe K Zettl
Therapeutic options to treat multiple sclerosis (MS) relapses comprise glucocorticosteroids (GCS) as first-line and therapeutic plasma exchange (TPE) as second-line treatments in GCS-unresponsive patients. No guidelines exist for the treatment of another relapse following TPE. We retrospectively analyzed the responsiveness to GCS in a subsequent relapse following TPE in previously GCS-unresponsive MS patients. Thirty-seven patients with GCS-unresponsive MS relapses received TPE (relapse A). All patients developed another relapse after the completion of TPE and received GCS again (relapse B)...
August 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28798244/atypical-haemolytic-uremic-syndrome-ahus-and-membranoproliferative-glomerulonephritis-mpgn-different-diseases-or-a-spectrum-of-complement-mediated-glomerular-diseases
#3
Ghada A Ankawi, William F Clark
Historically, patients with kidney diseases caused by genetic or acquired dysregulation of the complement alternative pathway have been grouped into clinical syndromes, C3 glomerulopathy (C3GN/DDD) and thrombotic microangiopathy (TMA), specifically atypical haemolytic uremic syndrome (aHUS). Recent data suggested that these diseases share a common pathophysiology and that patients can transition between glomerulopathies in this spectrum. Histopathologically, the main difference cited is the immunofluorescence (IF) findings, with C3 predominance in C3 glomerulopathy (compared with immunoglobulins and complements in immune complex-mediated membranoproliferative glomerulonephritis (MPGN)) and negative IF in TMA...
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28796431/acute-kidney-injury-in-a-postpartum-woman-with-paroxysmal-nocturnal-hemoglobinuria-a-case-report-and-literature-review
#4
Masatoshi Nishimoto, Masaru Matsui, Hideo Tsushima, Kaori Tanabe, Miho Tagawa, Ken-Ich Samejima, Yasuhiro Akai, Yoshihiko Saito
Paroxysmal nocturnal hemoglobinuria is a rare clonal hematopoietic stem cell disorder characterized by intravascular hemolysis, hemoglobinuria, and inflammatory thrombotic state. Intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) can lead to acute and chronic renal injury through hemoglobin-mediated toxicity. A 32-year-old pregnant woman with myelodysplastic syndrome was admitted to our hospital with severe preeclampsia. Shortly after an urgent caesarean section, she became obtunded and showed signs of acute kidney injury (AKI) with anuria, severe intravascular hemolysis, and hypermagnesemia...
August 10, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28796305/electron-microscopic-abnormality-and-therapeutic-efficacy-in-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin155-igg4-antibody
#5
Motoi Kuwahara, Hidekazu Suzuki, Nobuyuki Oka, Hidenori Ogata, Satoshi Yanagimoto, Shuji Sadakane, Yuta Fukumoto, Masaki Yamana, Yoshiko Yuhara, Keisuke Yoshikawa, Miyuki Morikawa, Shigeru Kawai, Masahiro Okazaki, Toru Tsujimoto, Jun-Ichi Kira, Susumu Kusunoki
INTRODUCTION: Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: We report the cases of 4 patients with anti-NF155 IgG4 antibody-positive CIDP who underwent sural nerve biopsies. RESULTS: All patients were relatively young at onset. Three experienced tremors and 2 had severe ataxia. Although the response to intravenous immunoglobulin (IVIg) was poor in all patients, plasma exchange (PE) and corticosteroids were at least partially effective...
August 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28792089/plasma-exchange-for-the-management-of-refractory-pruritus-of-cholestasis-a-report-of-three-cases-and-review-of-literature
#6
Rati Chkheidze, Ranjit Joseph, James Burner, Karen Matevosyan
BACKGROUND: Intractable pruritus of cholestasis leads to significant morbidity. Therapeutic plasma exchange (TPE) has been shown to be an effective alternative in the setting of refractory pruritus associated with cholestatic liver disease based on several individual reports. Due to rarity of this approach to intractable pruritus, the literature is sparse and therefore TPE, as a treatment for refractory pruritus is currently not in the apheresis guidelines. We present three additional patients with severe intractable pruritus of cholestasis successfully treated with plasma exchange to add to the mounting literature showing this as an effective and safe adjunctive therapy...
August 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#7
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28781825/hepatotoxicity-after-high-dose-intravenous-methylprednisolone-in-multiple-sclerosis-patients
#8
Milagros Hidalgo de la Cruz, Jahir Andrés Miranda Acuña, Alberto Lozano Ros, María Vega Catalina, Emilio Salinero Paniagua, Gerardo Clemente Ricote, Clara Dionisia De Andrés Frutos, María Luisa Martínez Ginés
Hepatotoxicity is a rare adverse event of methylprednisolone that should be considered in clinical practice. In patients at risk, we propose liver function surveillance, by measuring hepatic enzymes concentration 15-30 days after methylprednisolone administration. Additionally, we propose ACTH, dexamethasone, or plasma exchange as alternate treatment options for these patients.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28780041/quinine-induced-thrombotic-microangiopathy-a-report-of%C3%A2-19%C3%A2-patients
#9
Evaren E Page, Dustin J Little, Sara K Vesely, James N George
BACKGROUND: Quinine can cause diverse and severe immune-mediated adverse reactions, including thrombotic microangiopathy (TMA). Our objective was to describe the presenting features and long-term outcomes of patients with quinine-induced TMA. STUDY DESIGN: A case series of 19 patients with quinine-induced TMA treated with plasma exchange. SETTING & PARTICIPANTS: Patients with quinine-induced TMA initially suspected of having thrombotic thrombocytopenic purpura (TTP) were identified among patients enrolled in the Oklahoma TTP-Hemolytic Uremic Syndrome Registry...
August 3, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28777260/persistence-of-parenchymal-and-perivascular-t-cells-in-treatment-refractory-anti-n-methyl-d-aspartate-receptor-encephalitis
#10
Alexander Filatenkov, Timothy E Richardson, Elena Daoud, Sarah F Johnson-Welch, Denise M Ramirez, Jose Torrealba, Benjamin Greenberg, Nancy L Monson, Veena Rajaram
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease mediated by IgG1 or IgG3 antibodies to the GluN1 subunit of the NMDAR, resulting in downregulation of NMDARs. Early diagnosis, prompt reduction of anti-NMDAR antibodies, and removal of associated ovarian tumors when identified are important drivers of prognosis. Immunohistochemical studies were carried out to evaluate B cell, plasma cell, and T-cell infiltrates in the brain of a 3-year-old patient with anti-NMDAR encephalitis who failed to show improvement after plasma exchange and Rituximab treatment...
August 2, 2017: Neuroreport
https://www.readbyqxmd.com/read/28775083/rare-cause-of-respiratory-failure-in-a-young-woman-isolated-diffuse-alveolar-haemorrhage-requiring-extracorporeal-membrane-oxygenation
#11
David Kelly, Damodar Makkuni, Dhiraj Ail
A previously healthy 21-year-old young woman presented with worsening dyspnoea and haemoptysis. Imaging was suggestive of widespread pulmonary haemorrhage. There was no other organ system involvement in particular no evidence of renal involvement. Raised antimyeloperoxidase titres allowed diagnosis of isolated diffuse alveolar haemorrhage (DAH) secondary to microscopic polyangiitis (MPA). The patient rapidly deteriorated with worsening respiratory failure despite invasive mechanical ventilation and required extracorporeal membrane oxygenation (ECMO)...
August 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28768626/pathophysiology-of-thrombotic-thrombocytopenic-purpura
#12
J Evan Sadler
The discovery of ADAMTS13 revolutionized our approach to thrombotic thrombocytopenic purpura (TTP). Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor (VWF) and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes and tissue injury that characterize TTP. Most patients with acquired TTP respond to a combination of plasma exchange and rituximab, but some die or acquire irreversible neurological deficits before they can respond, and relapses can occur unpredictably...
August 2, 2017: Blood
https://www.readbyqxmd.com/read/28766066/outcome-of-kidney-transplantation-in-pediatric-patients-with-anca-associated-glomerulonephritis-a-single-center-experience
#13
Damien Noone, Rae S M Yeung, Diane Hebert
OBJECTIVES: Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients. METHODS: We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014...
August 1, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28764998/validation-of-the-plasmic-score-at-a-university-medical-center
#14
Ryan Jajosky, Mark Floyd, Thomas Thompson, James Shikle
BACKGROUND: The PLASMIC score was recently described as a convenient tool for predicting ADAMTS13 activity ≤10% in patients with possible thrombotic thrombocytopenic purpura (TTP), while awaiting the results of this send-out test. The purpose of this study was to validate the PLASMIC score at our University Medical Center. METHODS: Apheresis records were reviewed from 2008 to 2017 to identify patients who received plasma exchange (PLEX) for suspected TTP. The ADAMTS13 activity and PLASMIC scoring criteria were recorded, and the PLASMIC score was calculated...
July 8, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28761227/acute-kidney-injury-in-pregnancy-specific-disorders
#15
REVIEW
J Prakash, V C Ganiger
The incidence of acute kidney injury in pregnancy (P-AKI) has declined significantly over the last three decades in developing countries. However, it is still associated with significant fetomaternal mortality and morbidity. The diagnosis of P-AKI is based on the serum creatinine increase. The usual formulas for estimating glomerular filtration rate (GFR) are not validated in this population. The incidence of P-AKI with respect to total AKI cases has decreased in the last three decades from 25% in 1980s to 9% in 2000s at our centre...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28760487/complications-of-therapeutic-apheresis-in-pediatric-kidney-transplantation
#16
REVIEW
Mattia Parolin, Enrico Vidal
In the setting of kidney transplantation, therapeutic apheresis (TA) is employed both for pre-intervention procedures and during the post-transplant period. In pediatric nephrology units, TA is usually performed as a therapeutic plasma exchange (TPE) with dialysis equipment, and using non-plasma replacement fluids. In children undergoing kidney transplantation, complications of TPE are mainly related to its depletive properties combined with the iatrogenic immunodeficiency status of the patient. Moreover, the use of small central venous catheters and the equipment standardized for adults can increase the risk of adverse events...
July 10, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28759473/novel-therapeutic-approaches-for-thrombotic-thrombocytopenic-purpura
#17
Yvette C Tanhehco, Gowthami Arepally, Ara Metjian
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with therapeutic plasma exchange and immunosuppression with steroids results in high remission and low mortality rates. However, a number of patients remain refractory to frontline therapy and/or experience multiple relapses. This study reviews emerging therapies for thrombotic thrombocytopenic purpura...
July 28, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28752492/pulmonary-involvement-in-systemic-vasculitis
#18
REVIEW
Luis Felipe Flores-Suárez, Marco A Alba, Heidegger Mateos-Toledo, Natllely Ruiz
PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease...
September 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28750931/factor-h-autoantibody-is-associated-with-atypical-hemolytic-uremic-syndrome-in-children-in-the-united-kingdom-and-ireland
#19
Vicky Brocklebank, Sally Johnson, Thomas P Sheerin, Stephen D Marks, Rodney D Gilbert, Kay Tyerman, Meredith Kinoshita, Atif Awan, Amrit Kaur, Nicholas Webb, Shivaram Hegde, Eric Finlay, Maggie Fitzpatrick, Patrick R Walsh, Edwin K S Wong, Caroline Booth, Larissa Kerecuk, Alan D Salama, Mike Almond, Carol Inward, Timothy H Goodship, Neil S Sheerin, Kevin J Marchbank, David Kavanagh
Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort analysis. Here we examined 175 children presenting with atypical hemolytic uremic syndrome in the United Kingdom and Ireland for factor H autoantibodies that included 17 children with titers above the international standard. Of the 17, seven had a concomitant rare genetic variant in a gene encoding a complement pathway component or regulator...
July 24, 2017: Kidney International
https://www.readbyqxmd.com/read/28747261/enterovirus-d68-infection-in-a-cluster-of-children-with-acute-flaccid-myelitis-buenos-aires-argentina-2016
#20
Victor Ruggieri, Maria Ivana Paz, María Gabriela Peretti, Carlos Rugilo, Rosa Bologna, Cecilia Freire, Silvana Vergel, Andrea Savransky
OBJECTIVE: To report a outbreak of 11 cases of acute asymmetric flaccid myelopathy due to spinal motor neuron injury. MATERIAL AND METHODS: Eleven children, six male, with a mean age of 3 years presented with acute flaccid myelitis. We analyzed clinical features, etiology, neuroradiological images, treatment, and outcome. RESULTS: Nine children had bilateral and asymmetric flaccid myelitis of the upper limbs, 1 had upper limb monoplegia, and 1 presented with hemiparesis...
July 19, 2017: European Journal of Paediatric Neurology: EJPN
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