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https://www.readbyqxmd.com/read/29740210/emerging-treatment-options-for-the-management-of-pemphigus-vulgaris
#1
REVIEW
Khalaf Kridin
Pemphigus vulgaris (PV) is a life-threatening disease belonging to the pemphigus group of autoimmune intra-epidermal bullous diseases of the skin and mucosae. The therapeutic management of PV remains challenging and, in some cases, conventional therapy is not adequate to induce clinical remission. The cornerstone of PV treatment remains systemic corticosteroids. Although very effective, long-term corticosteroid administration is characterized by substantial adverse effects. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids...
2018: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/29669532/pulmonary-lymphangioleimyomatosis-and-systemic-lupus-erythematosus-in-a-menopausal-woman
#2
Hong Hong, Ruiheng Yang, Xiuzhen Li, Mengjun Wang, Zhongchao Ma
BACKGROUND: Pulmonary lymphangioleimyomatosis (PLAM) is a rare disease involving lung. PLAM primarily affects young women, a characteristic it shares with systemic lupus erythematosus (SLE). Estrogen has long been assumed to play an important role both in PLAM and SLE. We report a menopausal woman, who was found to have PLAM 1 year after she was diagnosed with SLE. Her chest radiograph was normal in the early phase of SLE. CASE PRESENTATION: A 52-year-old Chinese woman was referred to our hospital in August 2014 because of swelling in both legs...
April 18, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29667720/mycophenolate-mofetil-administered-every-8-hours-in-combination-with-tacrolimus-is-efficacious-in-the-prophylaxis-of-acute-graft-versus-host-disease-in-childhood-adolescent-and-young-adult-allogeneic-stem-cell-transplantation-recipients
#3
Olga Militano, Mehmet F Ozkaynak, Brinda Mehta, Carmella van deVen, Carl Hamby, Mitchell S Cairo
BACKGROUND: The optimal dose and schedule of mycophenolate mofetil (MMF) in pediatric allogeneic stem cell transplant recipients remains to be determined. We previously reported safety and pharmacokinetics of MMF at 900 mg/m2 q6h dosing. This study was conducted to investigate the efficacy of tacrolimus plus q8h MMF dosing for acute graft versus host disease (GVHD) prophylaxis in a heterogeneous population of children, adolescent, young adult allogeneic stem cell transplant recipients, utilizing multiple allogeneic donor sources...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29610699/hemorrhagic-tamponade-as-initial-manifestation-of-systemic-lupus-with-subsequent-refractory-and-progressive-lupus-myocarditis-resulting-in-cardiomyopathy-and-mitral-regurgitation
#4
Nicole Marijanovich, Alexandra Halalau
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a wide range of clinical and serological manifestations. Cardiac disease among patients with SLE is common and can involve the pericardium, myocardium, valves, conduction system, and coronary arteries. We are reporting a case of SLE in a young woman that is unique is unique in that initial symptoms consisted of pericarditis and hemorrhagic tamponade which remained progressive and resistant to aggressive immunosuppressive treatment and led to severe cardiomyopathy (ejection fraction of 25%) and severe (+4) mitral regurgitation...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29558355/-cutaneous-polyarteritis-nodosa
#5
Monika Turska, Jolanta Parada-Turska
Cutaneous polyarteritis nodosa is a rare disease that affects vessels of the deep skin and the subcutaneous tissue. Its etiopathology remains unknown. It predominantly affects skin, and the main cutaneous symptoms are subcutaneous nodules, livedo reticularis, and ulcerations that are mainly located on legs. Cutaneous polyarteritis nodosa can also cause extracutaneous symptoms (fever, malaise, myalgias, arthralgias, neuropathy). It is a chronic benign disease with a relapsing course. Diagnostic criteria for this disease were recently proposed and both clinical and typical histological features must be present to confirm the diagnosis...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29511796/pharmacokinetics-of-intravenous-mycophenolate-mofetil-in-allogeneic-hematopoietic-stem-cell-transplanted-japanese-patients
#6
Keiji Kurata, Kimikazu Yakushijin, Atsuo Okamura, Motohiro Yamamori, Hiroya Ichikawa, Rina Sakai, Yu Mizutani, Seiji Kakiuchi, Yoshiharu Miyata, Akihito Kitao, Shinichiro Kawamoto, Hiroshi Matsuoka, Tohru Murayama, Hironobu Minami
PURPOSE: Mycophenolate mofetil (MMF) is increasingly used among Japanese patients undergoing allogeneic hematopoietic stem cell transplantation (allo-SCT). Because pharmacokinetic data for MMF in the Asian population are limited, we conducted this investigation. METHODS: Intravenous MMF (1000 mg/dose) was administered to 10 patients along with cyclosporine or tacrolimus for 10 days after allo-SCT; it was administered every 8 h in peripheral blood stem cell- and bone marrow-transplanted patients, and every 12 h in cord blood-transplanted patients...
May 2018: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/29509140/-efficacy-of-the-combination-of-glucocorticoids-mycophenolate-mofetil-and-human-immunoglobulin-for-the-therapy-of-immune-mediated-haemolytic-anaemia-in-dogs
#7
D Oggier, K Tomsa, M Mevissen, T Glaus
Idiopathic immune-mediated haemolytic anaemia (IMHA) is one of the most common immune-mediated diseases in dogs with a high mortality rate. In this retrospective study, we examined the effect of mycophenolate- mofetil (MMF) and human intravenous immunoglobulin (hIVIG) in combination with glucocorticoids on canine IMHA patients. Six dogs were treated with prednisolone and MMF (hIVIG-) and in 15 patients hIVIG was added (hIVIG+). There was no significant difference between the groups regarding age, weight, number of blood transfusions or hematocrit on the day of diagnosis...
March 2018: Schweizer Archiv Für Tierheilkunde
https://www.readbyqxmd.com/read/29476421/immunosuppression-in-pregnant-women-with-renal-disease-review-of-the-latest-evidence-in-the-biologics-era
#8
REVIEW
Loredana Colla, Davide Diena, Maura Rossetti, Ana Maria Manzione, Luca Marozio, Chiara Benedetto, Luigi Biancone
Care of pregnant woman, including fertility and procreation counseling, has become a significant part of the nephrological practice in the last years. In this context, the management of immunosuppression assumes a primary role both for autoimmune diseases and for post-transplant follow up. The present review analyzes the latest evidence on immunosuppressive drugs of current use in nephrology and kidney transplantation. Although the placenta inactivates prednisone and prednisolone, it is advisable to limit the dose to the minimal effective one, to prevent side effects...
June 2018: Journal of Nephrology
https://www.readbyqxmd.com/read/29406904/comparison-of-efficacy-and-tolerability-of-azathioprine-mycophenolate-mofetil-and-lower-dosages-of-rituximab-among-patients-with-neuromyelitis-optica-spectrum-disorder
#9
Yang Yang, Chun-Juan Wang, Bao-Jie Wang, Zi-Ling Zeng, Shou-Gang Guo
OBJECTIVE: To observe and compare the efficacy and tolerability of azathioprine (AZA), mycophenolate mofetil (MMF) and lower dosages of rituximab (RTX) among patients with neuromyelitis optica spectrum disorder. METHODS: In this prospective cohort, AQP4-IgG-seropositive patients with neuromyelitis optica spectrum disorder (NMOSD) were enrolled and randomly divided into three groups, using AZA, MMF or lower dosages of RTX (defined as 100mg RTX intravenous injection, once per week for 4 consecutive weeks) respectively...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29406049/hypocomplementemic-urticarial-vasculitis-syndrome-with-crescentic-glomerulonephritis
#10
Sohail Abdul Salim, Tauqeer Yousuf, Asha Patel, Tibor Fülöp, Mohit Agarwal
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation...
February 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29396246/mycophenolate-plus-methylprednisolone-versus-methylprednisolone-alone-in-active-moderate-to-severe-graves-orbitopathy-mingo-a-randomised-observer-masked-multicentre-trial
#11
George J Kahaly, Michaela Riedl, Jochem König, Susanne Pitz, Katharina Ponto, Tanja Diana, Elena Kampmann, Elisa Kolbe, Anja Eckstein, Lars C Moeller, Dagmar Führer, Mario Salvi, Nicola Curro, Irene Campi, Danila Covelli, Marenza Leo, Michele Marinò, Francesca Menconi, Claudio Marcocci, Luigi Bartalena, Petros Perros, Wilmar M Wiersinga
BACKGROUND: European guidelines recommend intravenous methylprednisolone as first-line treatment for active and severe Graves' orbitopathy; however, it is common for patients to have no response or have relapse after discontinuation of treatment. We aimed to compare the efficacy and safety of add-on mycophenolate to methylprednisolone in comparison with methylprednisolone alone in patients with moderate-to-severe Graves' orbitopathy. METHODS: MINGO was an observer-masked, multicentre, block-randomised, centre-stratified trial done in two centres in Germany and two in Italy...
April 2018: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/29325970/the-role-of-adjuvant-immunomodulatory-agents-for-treatment-of-severe-influenza
#12
REVIEW
David S Hui, Nelson Lee, Paul K Chan, John H Beigel
A severe inflammatory immune response with hypercytokinemia occurs in patients hospitalized with severe influenza, such as avian influenza A(H5N1), A(H7N9), and seasonal A(H1N1)pdm09 virus infections. The role of immunomodulatory therapy is unclear as there have been limited published data based on randomized controlled trials (RCTs). Passive immunotherapy such as convalescent plasma and hyperimmune globulin have some studies demonstrating benefit when administered as an adjunctive therapy for severe influenza...
February 2018: Antiviral Research
https://www.readbyqxmd.com/read/29314776/chronicity-index-especially-glomerular-sclerosis-is-the-most-powerful-predictor-of-renal-response-following-immunosuppressive-treatment-in-patients-with-lupus-nephritis
#13
Dong-Jin Park, Sung-Eun Choi, Haimuzi Xu, Ji-Hyoun Kang, Kyung-Eun Lee, Ji Shin Lee, Yoo-Duk Choi, Shin-Seok Lee
AIM: Renal responses to immunosuppressive agents in patients with lupus nephritis (LN) differ depending on ethnicity, follow-up duration, disease severity and treatment. Thus, we evaluated predictors of complete remission during the first year following immunosuppressive treatment in patients with LN. METHODS: We retrospectively reviewed 79 patients who underwent kidney biopsy prior to the start of induction treatment and who were subsequently treated with immunosuppressive drugs for at least 6 months and followed-up for more than a year...
February 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29305608/disease-course-and-treatment-responses-in-children-with-relapsing-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
#14
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan, Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen, Evangeline Wassmer, Chery Hemingway, Rob Forsyth, Eva Maria Hennes, M Isabel Leite, Olga Ciccarelli, Banu Anlar, Rogier Hintzen, Romain Marignier, Jacqueline Palace, Matthias Baumann, Kevin Rostásy, Rinze Neuteboom, Kumaran Deiva, Ming Lim
Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated. Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. Design, Setting, and Participants: This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016...
April 1, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29245301/copper-associated-hepatitis-in-a-patient-with-chronic-myeloid-leukemia-following-hematopoietic-stem-cell-transplantation-a-case-report
#15
Ching-Fen Lee, Chi-Hua Chen, Yu-Chuan Wen, Tsung-Yen Chang, Ming-Wei Lai, Tang-Her Jaing
RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29242962/-mucous-membrane-pemphigoid
#16
M M Holtsche, D Zillikens, E Schmidt
Mucous membrane pemphigoid (MMP) is a pemphigoid disease defined by the presence of autoantibodies against the dermal-epidermal junction and predominant involvement of mucous membranes. Diagnosis is made by the clinical presentation and linear deposits of IgG and/or IgA and/or C3 at the dermal-epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy. Circulating autoantibodies can be detected in most patients by indirect immunofluorescence microscopy on salt-split human skin as well as ELISA and immunoblotting with recombinant and cell-derived target antigens...
January 2018: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/29204394/paraneoplastic-pemphigus-with-underlying-retroperitoneal-inflammatory-myofibroblastic-tumor-a-case-report-and-review-of-the-literature
#17
Mohammad Shahidi-Dadras, Fahimeh Abdollahimajd, Nasibeh Barzkar, Zahra Asadi Kani, Mohammad Nikvar
Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. The patient had a favourable response following treatment with a low dose of systemic steroid, mycophenolate mofetil, and intravenous immunoglobulin (IVIG). He subsequently underwent surgery for resection of the tumor with nephrectomy and five courses of IVIG were administered after surgery due to a minor relapse...
November 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29142145/clinical-course-therapeutic-responses-and-outcomes-in-relapsing-mog-antibody-associated-demyelination
#18
Sudarshini Ramanathan, Shekeeb Mohammad, Esther Tantsis, Tina Kim Nguyen, Vera Merheb, Victor S C Fung, Owen Bruce White, Simon Broadley, Jeannette Lechner-Scott, Steve Vucic, Andrew P D Henderson, Michael Harry Barnett, Stephen W Reddel, Fabienne Brilot, Russell C Dale
OBJECTIVE: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. METHODS: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. RESULTS: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children...
February 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29127744/-endocrine-orbitopathy-the-present-view-of-a-clinical-endocrinologist
#19
Jan Jiskra
Graves orbitopathy (GO) occurs in 25-50 % cases of Graves disease. Only in 5 % of patients the eye threatening GO is present. About 5-10 % and 10 % cases are present in euthyroid and hypothyroid patients respectively. All patients with GO should be assessed for activity (clinical activity score - CAS) and severity of the disease. Basic preconditions of the treatment are maintenance of euthyroidism, an effort to stop smoking, and referring of patients with moderate to severe and sight threatening GO to specialized thyroid eye centers...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29107213/current-and-future-treatment-options-for-pemphigus-is-it-time-to-move-towards-more-effective-treatments
#20
REVIEW
Soheil Tavakolpour
Systemic administration of corticosteroid (CS) remains the standard gold treatment for pemphigus. However, because of several long-term adverse effects, steroid-sparing agents are usually prescribed in combination with CSs. Despite the high number of available studies, the choice of best drugs to treat pemphigus remains controversial. Therapeutic approaches for pemphigus can be divided into traditional treatment and emerging ones. Personalized medicine, which aims to increase the efficacy as well as reduce adverse effects of treatments, could be considered as the future option...
December 2017: International Immunopharmacology
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