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https://www.readbyqxmd.com/read/29790849/-n-methyl-d-aspartate-receptor-antibody-encephalitis-the-janus-faced-disorder
#1
Ildiko Sipos
The recognition of the antibody-mediated encephalitis as a separate entity among the immune disorders of the central nervous system was one of the greatest breakthroughs of the last two decades in neurology. Unlike viral or tumor-related encephalitis, the antibody-mediated form has a good response to immunotherapy, which gives a special clinical importance to the discovery. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is one of the first fully characterized antibody-mediated encephalitises. This article attempts to summarize the clinical features of this complex neuropsychiatric disorder with the aim to help its early recognition and to report the clinical course and the outcome of our six seropositive anti-NMDAR cases...
March 2018: Neuropsychopharmacologia Hungarica
https://www.readbyqxmd.com/read/29790193/myasthenic-ophthalmoparesis-time-to-resolution-after-initiating-immune-therapies
#2
T A Europa, M Nel, J M Heckmann
INTRODUCTION: Although immunotherapies such as prednisone are effective in treating myasthenic muscle weakness, their effect on resolution of myasthenic-induced persistent ophthalmoparesis is unknown. METHODS: We observed myasthenia gravis patients during their first year of immunotherapy, documenting ophthalmoplegia scores and drug doses. RESULTS: Seventy-six of 87 cases had persistent ophthalmoparesis. On immunotherapy, the median time to resolution of ophthalmoparesis was 7 months and 37% resolved within 3 months...
May 23, 2018: Muscle & Nerve
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
#3
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/29781127/removal-dynamics-of-autoantibodies-immunoglobulins-and-coagulation-factors-by-selective-plasma-exchange-on-three-consecutive-days
#4
Satoko Miyamoto, Atsushi Ohkubo, Hiroshi Seshima, Hiroko Yamamoto, Ayako Itagaki, Takuma Maeda, Naoki Kurashima, Takayasu Mori, Soichiro Iimori, Shotaro Naito, Eisei Sohara, Tatemitsu Rai, Shinichi Uchida, Tomokazu Okado
Selective plasma exchange has been shown to be effective in various diseases, but no studies have assessed the benefits of daily treatment. We aimed to investigate the removal dynamics of immunoglobulins, fibrinogen, and factor XIII on three consecutive days in three patients. For mean processed plasma volumes of 1.06 × plasma volume, reductions of 79.6%, 49.3%, and 8.6% were seen for immunoglobulins G, A, and M, respectively. The reductions for fibrinogen and factor XIII were 18.4% and 13.0%, respectively...
May 20, 2018: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29780690/an-update-on-the-treatment-of-pediatric-autoimmune-encephalitis
#5
Cory Stingl, Kathleen Cardinale, Heather Van Mater
Purpose of review: Autoimmune encephalitis (AE) is an increasingly recognized etiology for neuropsychiatric deficits that are highly responsive to immunotherapy. As a result, rheumatologists are often called upon to help with the diagnosis and treatment of these conditions. The purpose of this review is to provide an update on the pharmacologic treatment of AE. Recent findings: To date, there are no prospective randomized placebo-controlled trials to guide treatment recommendations for AE...
March 2018: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#6
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/29774464/eculizumab-in-stec-hus-need-for-a-proper-randomized-controlled-trial
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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)
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