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https://www.readbyqxmd.com/read/29774464/eculizumab-in-stec-hus-need-for-a-proper-randomized-controlled-trial
#1
Sebastian Loos, Jun Oh, Markus J Kemper
Hemolytic uremic syndrome caused by Shiga toxin-producing E. coli (STEC-HUS) is often associated with a severe morbidity including neurological involvement and a mortality of 1-5%. Although STEC-HUS is often self-limited, improvement of treatment strategies is needed for cases with complications and, among others, plasma exchange/plasmapheresis and use of antibiotics have been advocated. With the availability of the complement blocker eculizumab, now a standard treatment of atypical HUS, several series have addressed its use in STEC-HUS, with variable response; randomized controlled trials are lacking...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29768707/albumin-apheresis-for-artificial-liver-support-in-vitro-testing-of-a-novel-filter
#2
Tomasz Piatek, Joanna Giebultowicz, Marieke Rüth, Horst-Dieter Lemke, Florian Bonn, Piotr Wroczynski, Piotr Malkowski, Jacek Rozga
Currently there is no direct therapy for liver failure. We have previously described selective plasma exchange therapy using a hemofilter permeable to substances that have a molecular mass of up to 100 kDa. The proof-of-concept studies and a Phase I study in patients with decompensated cirrhosis demonstrated that hemofiltration using an albumin-leaking membrane is safe and effective in removing target molecules, alleviating severe encephalopathy and improving blood chemistry. In this study a novel large-pore filter for similar clinical application is described...
May 16, 2018: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29762845/peritoneal-dialysis-effectively-removes-toxic-substances-and-improves-liver-functions-of-liver-failure-patients
#3
W-X Zhao, X-M Liu, C-M Yu, H Xu, J-R Dai, H-Y Chen, L Li, F Chen, Y-L Ou, Z-K Zhao
OBJECTIVE: Liver failure (LF) is a clinically complex disorder that characterizes with hepatic dysfunction. This study aimed at observing the therapeutic effects of peritoneal dialysis on liver function in LF patients. PATIENTS AND METHODS: This study involves 62 patients diagnosed as LF hospitalized from February 2005 to December 2016. The 62 LF patients were randomly divided into 3 groups, including artificial liver applying plasma exchange group (PE, n = 28), peritoneal dialysis group (PD, n = 22), and conservative treatment group (CT, n=12)...
April 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29761116/estimation-of-intrathecal-igg-synthesis-simulation-of-the-risk-of-underestimation
#4
Mickael Bonnan, Mariana Gianoli-Guillerme, Henri Courtade, Stéphanie Demasles, Elsa Krim, Raluca Marasescu, Hervé Dréau, Stéphane Debeugny, Bruno Barroso
Objective: The low level of passively diffused IgG through the blood-brain barrier is sufficient to blur the estimation of intrathecal IgG synthesis (ITS). Therefore, this estimation requires a mathematical calculation derived from empirical laws, but the range of normal values in healthy controls is wide enough to prevent a precise calculation. This study investigated the precision of various methods of ITS estimations and their application to two clinical situations: plasma exchange and immune suppression targeting ITS...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29760626/toxic-epidermal-necrolysis-in-an-8-year-old-girl-successfully-treated-with-cyclosporin-a-intravenous-immunoglobulin-and-plasma-exchange
#5
Marzena Zielińska, Łukasz Matusiak, Waldemar Gołębiowski, Katarzyna Swiątek, Iwona Chlebicka, Joanna Maj, Jacek Szepietowski
No abstract text is available yet for this article.
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29739267/relapsed-refractory-thrombotic-thrombocytopenic-purpura-treated-with-n-acetylcysteine-a-case-report
#6
Sinan Demircioğlu, Ömer Ekinci, Ali Doğan, Cengiz Demir
Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition. However, some cases may be non-responsive to plasma exchange, or loss of response may occur. Treatment options for refractory cases include high-dose corticosteroids, rituximab, vincristine, cyclophosphamide, splenectomy, bortezomib and N-acetylcysteine. We present a refractory case of thrombotic thrombocytopenic purpura responding to the last of these therapies...
January 1, 2018: Scottish Medical Journal
https://www.readbyqxmd.com/read/29736379/antineutrophil-cytoplasmic-antibody-associated-vasculitides-with-renal-involvement-open-challenges-in-the-remission-induction-therapy
#7
REVIEW
Maurizio Salvadori, Aris Tsalouchos
Renal involvement with rapidly progressive glomerulonephritis is a common manifestation of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides, which is characterized by end-stage renal disease and high mortality rates in untreated and/or late referral patients. The long-term renal survival has improved dramatically since the addition of cyclophosphamide (CYC) and recently of rituximab (RTX) in association with corticosteroids in the remission induction therapeutic regimens. However, renal prognosis remains unfavorable for many patients and the mortality rate is still significantly high...
May 6, 2018: World Journal of Nephrology
https://www.readbyqxmd.com/read/29734473/treatment-for-hepatitis-c-virus-associated-mixed-cryoglobulinaemia
#8
REVIEW
Nuria Montero, Alexandre Favà, Eva Rodriguez, Clara Barrios, Josep M Cruzado, Julio Pascual, Maria Jose Soler
BACKGROUND: Hepatitis C virus (HCV)-associated mixed cryoglobulinaemia is the manifestation of an inflammation of small and medium-sized vessels produced by a pathogenic IgM with rheumatoid factor activity generated by an expansion of B-cells. The immune complexes formed precipitate mainly in the skin, joints, kidneys or peripheral nerve fibres. Current therapeutic approaches are aimed at elimination of HCV infection, removal of cryoglobulins and also of the B-cell clonal expansions. The optimal treatment for it has not been established...
May 7, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29732228/de-novo-atypical-haemolytic-uremic-syndrome-after-kidney-transplantation
#9
Arnaud Devresse, Martine de Meyer, Selda Aydin, Karin Dahan, Nada Kanaan
De novo thrombotic microangiopathy (TMA) can occur after kidney transplantation. An abnormality of the alternative pathway of complement must be suspected and searched for, even in presence of a secondary cause. We report the case of a 23-year-old female patient who was transplanted with a kidney from her mother for end-stage renal disease secondary to Hinman syndrome. Early after transplantation, she presented with 2 episodes of severe pyelonephritis, associated with acute kidney dysfunction and biological and histological features of TMA...
2018: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29731498/a-case-of-anti-glomerular-basement-membrane-antibody-positive-systemic-lupus-erythematosus-with-pulmonary-hemorrhage-successfully-treated-at-an-early-stage-of-the-disease
#10
Takehisa Yamada, Koji Mugishima, Seiichiro Higo, Yukie Yoshida, Fumiaki Itagaki, Shizuka Yui, Tetsuya Kashiwagi, Yoko Endo, Akira Shimizu, Shuichi Tsuruoka
We report here a case of systemic lupus erythematosus (SLE) with pulmonary hemorrhage and anti-glomerular basement membrane (anti-GBM) antibodies. A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis. Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage. Laboratory findings on admission revealed proteinuria, microscopic hematuria, anemia, leukopenia, hypoalbuminemia, hypocomplementemia, and slightly elevated levels of serum creatinine...
2018: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#11
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29725643/combination-therapy-with-rituximab-and-cyclophosphamide-for-remission-induction-in-anca-vasculitis
#12
Frank B Cortazar, Saif A Muhsin, William F Pendergraft, Zachary S Wallace, Colleen Dunbar, Karen Laliberte, John L Niles
Introduction: Remission induction in antineutrophil cytoplasmic autoantibody (ANCA) vasculitis may be complicated by slow response to treatment and toxicity from glucocorticoids. We describe outcomes with a novel remission induction regimen combining rituximab with a short course of low-dose, oral cyclophosphamide and an accelerated prednisone taper. Methods: Patients were included in this retrospective study if they had newly diagnosed or relapsing ANCA vasculitis with a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS-WG) ≥3 and received a standardized remission induction regimen...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29703920/prevalent-metabolic-derangement-and-severe-thrombocytopenia-in-abo-incompatible-liver-recipients-with-pre-transplant-plasma-exchange
#13
Hye-Mee Kwon, In-Gu Jun, JungBok Lee, Young-Jin Moon, Kyeo-Woon Jung, Hye-Won Jeong, Yong-Seok Park, Jun-Gol Song, Gyu-Sam Hwang
Desensitisation with therapeutic plasma exchange (TPE) is essential for ABO-incompatible (ABO-I) liver transplants (LTs). However, excessive citrate load and coagulation disturbances after TPE have been poorly studied, in particular in cirrhotic patients with hypocapnic alkalosis, metabolic compensation and electrolyte imbalances. We retrospectively evaluated 1123 consecutive LT recipients (923 ABO-compatible [ABO-C], 200 ABO-I) from November 2008 to May 2015. TPE was generally performed a day before LT and blood sampling was performed before anaesthesia induction...
April 27, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29703032/acetylcholine-receptor-antibody-positive-myasthenia-gravis-associated-with-small-cell-lung-cancer-a-case-report
#14
Masahiro Yamasaki, Kunihiko Funaishi, Naomi Saito, Tomomi Yonekawa, Takemori Yamawaki, Daisuke Ihara, Wakako Daido, Sayaka Ishiyama, Naoko Deguchi, Masaya Taniwaki, Noboru Hattori
RATIONALE: Only few cases of myasthenia gravis (MG) associated with small-cell lung cancer (SCLC) have been reported, and cases positive for acetylcholine receptor antibody (AChR-ab) are even rarer. The efficacy of standard MG treatment, such as cholinesterase inhibitor therapy, immunosuppressive therapy using steroids and immunosuppressive drugs, plasma exchange, and intravenous immune globulin (IVIg), for these cases is unclear. PATIENT CONCERNS AND DIAGNOSES: A 71-year-old man complained of bilateral eyelid ptosis...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29702545/steroid-responsive-atypical-hemolytic-uremic-syndrome-triggered-by-influenza-b-infection
#15
Nupur Mittal, Robert Hartemayer, Sara Jandeska, Lisa Giordano
Atypical hemolytic uremic syndrome (aHUS) is characterized by uncontrolled complement activation leading to thrombotic microangiopathy and severe end-organ damage. The most common trigger for an episode of aHUS in the background of genetic deregulation of the alternative complement pathway is systemic infection. There are only 4 reported cases of aHUS triggered by influenza B thus far. Current accepted therapies for aHUS include plasma exchange and eculizumab. We describe a unique patient with aHUS with a rare membrane cofactor protein mutation triggered by influenza B infection, who achieved complete remission with treatment with high-dose corticosteroids after failure of plasmapheresis...
April 26, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29687787/drug-induced-thrombotic-microangiopathy-with-certolizumab-pegol
#16
Mehmet Baysal, Elif Gülsüm Ümit, Fatih Sarıtaş, Nil Su Kodal, Ahmet Muzaffer Demir
BACKGROUND: Certolizumab pegol is used to treat ankylosing spondylitis (AS), chron disease, psoriatic arthritis and rheumatoid arthritis. In contrast to other monoclonal antibodies such as infliximab and adalimumab, certolizumab does not contain an Fc fraction and hence, does not induce complement activation. In this case, we are presenting a case with thrombotic microangiopathy (TMA) due to certolizumab pegol, with a touch of pathophysiological approach to TMA. CASE REPORT: A-39-year-old man with AS of ten years presented with fatigue...
April 24, 2018: Balkan Medical Journal
https://www.readbyqxmd.com/read/29686710/total-plasma-exchange-in-hypertriglyceridemia-induced-pancreatitis-case-report-and-literature-review
#17
Harleen Dehal, Michael Adashek
Objective: To emphasize the role of apheresis in management of pancreatitis. Methods: The clinical course of a patient admitted for hypertriglyceridemia-induced pancreatitis (HTGP) complicated by multiorgan dysfunction is described, who demonstrated dramatic improvement in his clinical status after total plasma exchange (TPE). In addition, the current guidelines for TPE and the alternative treatment options for HTGP are also presented. Results: A patient presenting with pancreatitis associated with severe systemic inflammatory response was admitted to our hospital with an initial triglyceride level of 1181 mg/dL...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29684566/eltrombopag-for-the-treatment-of-of-refractory-pure-red-cell-aplasia-after-major-abo-incompatible-hematopoietic-stem-cell-transplantation
#18
Alessandro Busca, Chiara Dellacasa, Luisa Giaccone, Sara Manetta, Lucia Biale, Laura Godio, Semra Aydin, Moreno Festuccia, Lucia Brunello, Benedetto Bruno
Pure red cell aplasia (PRCS) is a well-recognized complication after allogeneic hematopoietic stem cell transplantation (HSCT). Many therapeutic options are available to treat this condition, including erythropoietin, rituximab, bortezomib, plasma exchange, immunoadsorption, donor lymphocyte infusion, mesenchymal stem cells, anti-thymocyte globulin and high-dose steroids; however treatment outcomes are often variable and can sometimes lead to disappointing results. In this brief article we report our experience with two patients with PRCA after major AB0-incompatible HSCT who were resistant to multiple therapeutic interventions and who eventually benefited from treatment with eltrombopag, a thrombopoietin mimetic approved by the Food and Drug Administration for the treatment of patients with immune thrombocytopenic purpura or severe aplastic anemia (SAA), refractory to immunosuppressive agents or not eligible to HSCT...
April 20, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29684034/effect-of-plasma-exchange-on-in-hospital-mortality-in-patients-with-pulmonary-hemorrhage-secondary-to-antineutrophil-cytoplasmic-antibody-associated-vasculitis-a-propensity-matched-analysis-using-a-nationwide-administrative-database
#19
Eishi Uechi, Masato Okada, Kiyohide Fushimi
BACKGROUND: Secondary pulmonary hemorrhage increases the risk of mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV); plasma exchange therapy may improve outcomes in these patients. We conducted a retrospective cohort study to investigate the effect of plasma exchange therapy on short-term prognoses in patients with pulmonary hemorrhage secondary to AAV. METHODS: This study utilized the Diagnosis Procedure Combination database, which is a nationwide inpatient database in Japan...
2018: PloS One
https://www.readbyqxmd.com/read/29681203/the-immunotherapy-of-guillain-barr%C3%A3-syndrome
#20
Paula Restrepo-Jiménez, Yhojan Rodríguez, Paulina González, Christopher Chang, M Eric Gershwin, Juan-Manuel Anaya
Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Microorganisms such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Haemophilus influenzae and Zika virus have been linked to the disease. The most common clinical variants are acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Plasma exchange and intravenous immunoglobulins are the standard therapy for the disease. Areas covered: research to elucidate the pathophysiology of Guillain-Barré syndrome has led to the development of drugs directed towards new potential therapeutic targets...
May 8, 2018: Expert Opinion on Biological Therapy
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