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https://www.readbyqxmd.com/read/28801984/low-dose-bortezomib-and-dexamethasone-as-primary-therapy-in-elderly-patients-with-waldenstr%C3%B3-m-macroglobulinemia
#1
Ya-Ping Zhang, Xi Yang, Zeng-Hua Lin, Xin-Feng Wang, Xin Cao, Xue-Fen You, Hong-Ming Huang, Wen-Yu Shi, Hong Liu
OBJECTIVE: This retrospective study was designed to determine the efficacy and safety of low-dose bortezomib and dexamethasone (lBD) in elderly Chinese patients with Waldenstrӧm macroglobulinemia (WM). METHODS: Ten WM patients aged over 60 years received first-line treatment with lBD. RESULTS: The median age was 70 years (range, 61-77 years). The overall response rate was 80%, including 1 patient who achieved a complete response, 1 patient with very good partial response, and 6 patients with a partial response...
August 12, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28798719/fulminant-acute-ascending-hemorrhagic-myelitis-treated-with-eculizumab
#2
Nang Boe Ohnmar Hsam, Klemens Angstwurm, Sebastian Peters, Kornelius Fuchs, Gerhard Schuierer, Ulrich Bogdahn, Robert Weissert
We describe an 18-year-old patient who developed back pain, rapidly ascending sensomotory deficits, bladder dysfunction, Lhermitte's sign, absent abdominal reflexes of all three levels, brisk tendon reflexes, and positive Babinski's sign. Magnetic resonance imaging of the spinal cord showed a long segment of cervical and thoracic intramedullary signal hyperintensity suggesting a longitudinally extensive transverse myelitis possibly within the course of a fast progressing ascending immune-mediated hemorrhagic myelopathy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28796305/electron-microscopic-abnormality-and-therapeutic-efficacy-in-chronic-inflammatory-demyelinating-polyneuropathy-with-anti-neurofascin155-igg4-antibody
#3
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/28794296/recurrent-aa-amyloidosis-combined-with-chronic-active-antibody-mediated-rejection-after-kidney-transplantation
#4
Min-Kyung Yeo, Young Rok Ham, Song-Yi Choi, Yong-Moon Lee, Moon Hyang Park, Kwang-Sun Suh
Kidney transplantation for amyloidosis remains a contentious issue. Recurrence of amyloidosis is one of the risks of transplantation. Chronic active antibody-mediated rejection is an important cause of chronic allograft dysfunction. A 47-year-old woman underwent kidney transplantation due to renal AA amyloidosis with unknown etiology. Six years posttransplantation, a kidney biopsy showed AA amyloidosis with chronic active antibody-mediated rejection. Donor-specific antibody class II was positive. The patient underwent intravenous plasmapheresis and treatment with rituximab and colchicine...
July 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28792176/chikungunya-fever-presenting-as-life-threatening-thrombotic-thrombocytopenic-purpura
#5
Vimal Kumar, Rujul Jain, Arvind Kumar, Neeraj Nischal, Pankaj Jorwal, Manish Soneja, Sudheer Arava, Naveet Wig
It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura...
July 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28774827/therapeutic-apheresis-in-kidney-ab0-incompatible-transplantation
#6
REVIEW
Anna Parolo, Cristina Silvestre, Flavia Neri, Paolo Rigotti, Lucrezia Furian
Desensitization strategies to safely perform ABO incompatible living donor kidney transplantations are various and still evolving. Given the successful outcome of the majority of the approaches, the current trend is focused on a minimization of treatments. Based on this consideration, the evolution at a single Center of the desensitization protocol is herein described. Starting from 2010, 58 AB0 incompatible living donor kidney transplantations were performed at the University-Hospital of Padua. Over the years, the initial desensitization strategy with rituximab single-dose induction, pre-and post-transplant plasmapheresis and CMV-specific immunoglobulin administration has been shifted to a minimized approach, omitting post-transplant antibody removal in 25 cases...
July 10, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28774729/-thrombotic-microangiopathy-and-cancer
#7
Rania Kheder El-Fekih, Clément Deltombe, Hassan Izzedine
Thrombotic microangiopathy (TMA) is a group of disorders characterized by mechanical hemolytic anemia with thrombocytopenia and an ischemic organic lesion of variable and potentially fatal importance affecting mostly the kidneys and the brain with histologically a disseminated and occlusive microvasculopathy. The incidence of TMA represents 15% of acute kidney failure in oncological setting, largely due to the introduction of anti-angiogenic agents over the past decade. It may be more rarely related to cancer itself...
July 31, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#8
Ingrid Pl Ting, Sanihah Abdul Halim, Azreen Adnan, Hasnan Jaafar
Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28762062/sequential-rituximab-and-omalizumab-for-the-treatment-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#9
REVIEW
David Aguirre-Valencia, Iván Posso-Osorio, Juan-Carlos Bravo, Fabio Bonilla-Abadía, Gabriel J Tobón, Carlos A Cañas
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms...
July 31, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28761240/circulating-monoclonal-igg1-kappa-antibodies-causing-anti-glomerular-basement-membrane-nephritis
#10
M Vankalakunti, R Nada, A Kumar, K Patro, S Ramakrishnan, D Rangarajan
Anti-glomerular basement membrane (GBM) antibody disease is a rare but well-characterized cause of glomerulonephritis. Patients present with rapidly progressive renal failure with hemoptysis. Early diagnosis is crucial in salvaging the renal damage and life-threatening pulmonary hemorrhage. Plasmapheresis and immunosuppression is the mode of therapy. Anti-GBM antibodies are polyclonal in nature. However, rare monoclonal antibodies can cause similar destruction of glomerular capillary walls. We describe distinct combination of circulating monoclonal and anti-GBM nephritis...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28751632/extracorporeal-membrane-oxygenation-support-for-rituximab-induced-acute-respiratory-distress-syndrome-in-an-abo-incompatible-living-donor-liver-transplant-recipient-successful-management-of-a-rare-complication-and-a-review-of-the-literature
#11
Ping-Chun Li, Ashok Thorat, Shih-Chao Hsu, Kin-Shing Poon, Horng-Ren Yang, Te-Hung Chen, Chun-Chieh Yeh, Long-Bin Jeng
BACKGROUND Rituximab is commonly used to reduce the agglutinin titer in ABO-incompatible liver transplant recipients. Although well-tolerated, rituximab infusion therapy may result in severe pulmonary adverse effects such as drug-induced pneumonitis, leading to acute respiratory distress syndrome (ARDS), which has a high mortality rate. Management of such rare cases in an ABO-incompatible patient has never been described before. Herein, we present successful use of extracorporeal membrane oxygenation (ECMO) support for rituximab-induced ARDS in an ABO-incompatible living donor liver transplantation (LDLT) recipient...
July 28, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28737146/autoimmune-hypoglycemia-in-type-1-diabetes-mellitus
#12
Jayakumar Ambigapathy, Jayaprakash Sahoo, Sadishkumar Kamalanathan
BACKGROUND: Antibodies against exogenous insulin are common in type 1 diabetes mellitus patients. They can cause hypoglycemia, albeit uncommonly. CASE CHARACTERISTICS: A 14-year-old girl with type 1 diabetes mellitus presented with recurrent hypoglycemia. OUTCOME: High insulin, low C-peptide and raised insulin antibody levels documented during hypoglycemia. Plasmapheresis led to remission of hypoglycemia. MESSAGE: Antibodies to exogenous insulin should be considered as a cause of recurrent refractory hypoglycemia in type 1 diabetes mellitus patients...
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28734583/kidney-transplantation-from-hla-incompatible-live-donors-efficiency-and-outcome-of-32-patients-after-desensitisation
#13
Constantino Fernández, María Calvo, Natacha Leite, Andrés López, Tamara Ferreiro, Roi Ribera, Rocío Seijo, Ángel Alonso
Desensitisation is a procedure undergone by the recipient of a kidney transplant from a donor who is cross-match positive. The aim of this study was to present the outcomes from our hospital of kidney transplant recipients from HLA-incompatible live donors after desensitisation. We studied 32 patients aged 46±14 years with a mean fluorescence intensity (MFI) versus class I HLA of 7979±4089 and 6825±4182 MFI versus class II and relative intensity scale (RIS) of 8.9±7.6. The complement-dependent cytotoxicity (CDC) cross-matching test was positive in 18 patients, flow cytometry was positive in 7 patients and donor-specific antibodies (DEA) were detected in 7...
July 19, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28725563/atypical-hemolytic-uremic-syndrome-triggered-by-varicella-infection
#14
Pauline Condom, Jean-Michel Mansuy, Stéphane Decramer, Jacques Izopet, Catherine Mengelle
Varicella Zoster Virus (VZV) is a well-known virus that belongs to the Herpesviridae family which induces a self-limited disease except in specific cases in particular among stem cell transplant patients. This virus is not known however to trigger atypical Hemolytic Uremic Syndrome (aHUS). Here we report the case of a six-year-old boy who was hospitalized with fever and abdominal pains associated to pruritic and vesicular rash, thrombocytopenia and acute renal failure. He was diagnosed with aHUS precipitated by varicella virus...
2017: IDCases
https://www.readbyqxmd.com/read/28725307/acute-pancreatitis-secondary-to-severe-hypertriglyceridemia-management-of-severe-hypertriglyceridemia-in-emergency-setting
#15
Ahmad Chaudhary, Umair Iqbal, Hafsa Anwar, Hafiz Umair Siddiqui, Madiha Alvi
Hypertriglyceridemia (HTG) is the third most common cause of acute pancreatitis (AP). The incidence of AP is around 10-20% with levels > 2,000 mg/dL. We present here a case of a 44-year-old male with history of uncontrolled diabetes mellitus and HTG admitted with severe abdominal pain. Labs revealed elevated lipase and amylase. CT of abdomen with contrast showed AP. He was found to have a triglyceride (TG) level of 6,672 mg/dL. Besides conventional treatment for AP with intravenous (IV) hydration, he was started on IV regular insulin along with dextrose saline...
June 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28724844/a-review-of-the-active-treatments-for-toxic-epidermal-necrolysis
#16
Yuri Kinoshita, Hidehisa Saeki
Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with the separation of skin and mucous membranes at the dermal-epidermal junction. Although it is rare, many treatments have been trialed because of its high mortality rate. Active interventions performed to date include the use of systemic corticosteroids, intravenous immunoglobulins (IVIg), cyclosporine, plasmapheresis, anti-tumor necrosis factor drugs and N-acetylcysteine, but none has been established as the most effective therapy...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/28724445/sustained-benefit-from-combined-plasmapheresis-and-allogeneic-mesenchymal-stem-cells-transplantation-therapy-in-systemic-sclerosis
#17
Huayong Zhang, Jun Liang, Xiaojun Tang, Dandan Wang, Xuebing Feng, Fan Wang, Bingzhu Hua, Hong Wang, Lingyun Sun
BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease involving the skin and several internal organs. Most therapies available for this disease are symptomatic. Given the difficulty in treating SSc, we conducted this study to investigate the effect of combined plasmapheresis (PE) and allogeneic mesenchymal stem cells transplantation (MSCT) therapy on SSc. METHODS: Fourteen patients underwent three repeated PE treatments with subsequent pulse cyclophosphamide on days 1, 3 and 5...
July 19, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28718268/oxymorphone-induced-thrombotic-microangiopathy-mimicking-atypical-haemolytic-uremic-syndrome
#18
Amibhen Gandhi, Saad Ullah, Shani Kotadia, Samer Nasser
Atypical Haemolytic Uremic Syndrome (aHUS) is a rare life threatening entity characterized by thrombocytopenia, haemolytic anaemia and renal dysfunction. It is a thrombotic microangiopathy related to genetic mutations in the alternate complement pathway and has a distinct pathophysiology which makes it harder to distinguish from other microangiopathies. We present a case of a 25-year-old male patient with history of polysubstance abuse who presented with chest pain and dyspnoea. He admitted to using injectable oxymorphone (Opana) two weeks before presentation...
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28715820/therapeutic-plasma-exchange-in-neonates-and-infants-successful-use-of-a-miniaturized-machine
#19
Enrico Vidal, Francesco Garzotto, Mattia Parolin, Chiara Manenti, Anna Zanin, Massimo Bellettato, Giuseppe Remuzzi, Stuart L Goldstein, Luisa Murer, Claudio Ronco
Therapeutic plasma exchange (TPE) in neonates and small infants is a treatment method at the forefront that may become a potentially life-saving procedure in a wide array of severe conditions. Indications for TPE in the pediatric population have been mainly derived from adult literature, with neonatal hyperbilirubinemia being the most notable exception. The only alternative to TPE in small pediatric patients is manual blood exchange transfusion, which, however, bears an unacceptably high risk of severe complications...
2017: Blood Purification
https://www.readbyqxmd.com/read/28711273/treatment-methods-following-suicidal-self-administration-of-iv-organophosphate-what-can-additional-lipid-administration-change
#20
Kenan Ahmet Turkdogan, Ali Duman, Mucahit Avcil, Ayhan Akoz, Selcuk Eren Canakcı, Burcak Kantekin
Organic phosphor compounds are highly toxic and life-threatening compounds that are widely used in agriculture, households and gardens worldwide. While oral intakes are observed frequently, toxic effects can also be seen through contract or inhalation. However, toxic cases through the parenteral route have rarely been reported. We had three main aims: first, to present a male patient who self-administered 2ml of monocrotophos intravenously to commit suicide. Our second goal was to present the current multiple treatment methods, such as fast general support, antidote treatment, lipid emulsion treatment, and plasmapheresis along with the literature...
July 8, 2017: American Journal of Emergency Medicine
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