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https://www.readbyqxmd.com/read/28220235/successful-discontinuation-of-eculizumab-under-immunosuppressive-therapy-in-deap-hus
#1
Agnes Hackl, Rasmus Ehren, Michael Kirschfink, Peter F Zipfel, Bodo B Beck, Lutz T Weber, Sandra Habbig
BACKGROUND: Deficiency of complement factor H-related plasma proteins and complement factor H autoantibody-positive hemolytic uremic syndrome (DEAP-HUS), which is characterized by the deficiency of complement-factor H-related (CFHR) plasma proteins and the subsequent formation of autoantibodies against complement factor H (CFH), has been reported to have an adverse outcome in one third of patients. Therapy options include prompt removal of antibodies by plasma exchange and immunosuppressive therapy...
February 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28215945/mycophenolate-mofetil-combined-with-prednisone-versus-full-dose-prednisone-in-iga-nephropathy-with-active-proliferative-lesions-a-randomized-controlled-trial
#2
Jin-Hua Hou, Wei-Bo Le, Nan Chen, Wei-Ming Wang, Zhang-Suo Liu, Dong Liu, Jiang-Hua Chen, Jiong Tian, Ping Fu, Zhang-Xue Hu, Cai-Hong Zeng, Shao-Shan Liang, Min-Lin Zhou, Hai-Tao Zhang, Zhi-Hong Liu
BACKGROUND: Observational studies suggest that patients with immunoglobulin A nephropathy (IgAN) with active proliferative lesions show a good response to immunosuppressive treatment. STUDY DESIGN: Multicenter, prospective, randomized, controlled trial. SETTING & PARTICIPANTS: 176 patients with IgAN with active proliferative lesions (cellular and fibrocellular crescents, endocapillary hypercellularity, or necrosis), proteinuria with protein excretion ≥ 1...
February 15, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#3
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28207973/risk-factors-of-human-herpesvirus-6-encephalitis-myelitis-after-allogeneic-hematopoietic-stem-cell-transplantation
#4
Naohiro Miyashita, Tomoyuki Endo, Masahiro Onozawa, Daigo Hashimoto, Takeshi Kondo, Katsuya Fujimoto, Kaoru Kahata, Junichi Sugita, Hideki Goto, Toshihiro Matsukawa, Satoshi Hashino, Takanori Teshima
BACKGROUND: Human herpesvirus 6 (HHV-6) encephalitis/myelitis is now a well-known complication after allogeneic stem cell transplantation (allo-HSCT), particularly after cord blood transplantation (CBT). In this study, we evaluated the risk factors of HHV-6 encephalitis/myelitis. METHODS: We evaluated 253 patients who received allo-HSCT from 2007 to 2015 at our institute. HHV-6 encephalitis/myelitis was defined as HHV-6 DNA detection in the cerebrospinal fluid or peripheral blood by polymerase chain reaction in the presence of typical manifestations without other concurrent condition which led to the manifestations...
February 16, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28206810/safety-of-oral-and-intravenous-mycophenolate-mofetil-in-healthy-cats
#5
Jennifer E Slovak, Nicolas F Villarino
Objectives The aim of this study was to evaluate the safety and clinical effects of intravenous (IV) and oral mycophenolate mofetil (MMF) in healthy cats. Methods A total of 24 healthy adult cats weighing >3.5 kg were either administered IV MMF (over a 2 h infusion) or oral MMF. The dosages used were as follows: 5 mg/kg IV once (n = 2), 10 mg/kg q12h IV for 1 day (n = 1), 20 mg/kg q12h IV for 1 day (n = 6) and 10 mg/kg q12h IV for 3 days (n = 5). Blood was collected from each cat at intervals of up to 12 h from the last dose for analysis purposes...
February 1, 2017: Journal of Feline Medicine and Surgery
https://www.readbyqxmd.com/read/28191596/efficacy-and-safety-of-rituximab-in-comparison-with-common-induction-therapies-in-pediatric-active-lupus-nephritis
#6
Biswanath Basu, Birendranath Roy, Binu George Babu
BACKGROUND: Childhood-onset lupus nephritis (LN) is one of the most severe manifestations of systemic lupus erythematosus (SLE). Despite treatment-related toxicities, cyclophosphamide (CYC) and glucocorticoid-based treatment protocols are still considered standard therapy in managing this multisystem disorder. An effective and safe alternative induction regimen is needed. METHODS: Forty-four pediatric patients with active LN aged 3.5-13.8 (median 8.4) years, of whom 32 entered the study at diagnosis of SLE, were followed over 36 months...
February 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28188239/treatment-outcome-in-early-diffuse-cutaneous-systemic-sclerosis-the-european-scleroderma-observational-study-esos
#7
Ariane L Herrick, Xiaoyan Pan, Sébastien Peytrignet, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuţa, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Alison C Jordan, Paresh Jobanputra, Wendy Stevens, Pia Moinzadeh, Frances C Hall, Christian Agard, Marina E Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H Buch, Lorinda Chung, Nemanja Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat İnanç, John S McLaren, Jacob M van Laar, Sanjay Pathare, Susannah Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Christopher P Denton
OBJECTIVES: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches. METHODS: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or 'no immunosuppressant'...
February 10, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28182833/comparative-effectiveness-of-mycophenolate-mofetil-for-the-treatment-of-childhood-onset-proliferative-lupus-nephritis
#8
Simon Y Tian, Earl D Silverman, Eleanor Pullenayegum, Patrick E Brown, Joseph Beyene, Brian M Feldman
OBJECTIVES: Although childhood-onset proliferative lupus nephritis (cPLN) leads to significant morbidity and mortality, there is no trial evidence to support the treatment effectiveness of any therapy for cPLN. Marginal structural models (MSMs) enable us to estimate treatment effectiveness using observational data while accounting for confounding by indication. METHODS: We used prospectively collected data to examine the effect of mycophenolate mofetil (MMF), compared to the use of other therapies, on the long-term outcome of our cPLN cohort (age at onset of PLN < 18 years)...
February 9, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28181536/the-nrf2-transcriptional-target-osgin1-contributes-to-monomethyl-fumarate-mediated-cytoprotection-in-human-astrocytes
#9
Melanie S Brennan, Maria F Matos, Karl E Richter, Bing Li, Robert H Scannevin
Dimethyl fumarate (DMF) is indicated for the treatment of relapsing multiple sclerosis and may exert therapeutic effects via activation of the nuclear factor (erythroid-derived 2)-like 2 (NRF2) pathway. Following oral DMF administration, central nervous system (CNS) tissue is predominantly exposed to monomethyl fumarate (MMF), the bioactive metabolite of DMF, which can stabilize NRF2 and induce antioxidant gene expression; however, the detailed NRF2-dependent mechanisms modulated by MMF that lead to cytoprotection are unknown...
February 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28169970/second-line-agents-in-pediatric-patients-with-autoimmune-hepatitis-a-systematic-review-and-meta-analysis
#10
Andréanne N Zizzo, Pamela L Valentino, Prakesh S Shah, Binita M Kamath
BACKGROUND AND AIMS: Ten to 20% of children with autoimmune hepatitis require second-line therapy to achieve remission. Although current guidelines exist on first-line management, evidence for second-line therapy in treatment-refractory patients is lacking. Our aim was to perform a systematic review and meta-analysis of the efficacy and safety of second-line treatments used in this population. METHODS: Electronic and manual searches were employed to identify potential studies for inclusion...
February 4, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28166490/the-manhattan-frame-model-manhattan-world-inference-in-the-space-of-surface-normals
#11
Julian Straub, Oren Freifeld, Guy Rosman, John J Leonard, John W Fisher
Objects and structures within man-made environments typically exhibit a high degree of organization in the form of orthogonal and parallel planes. Traditional approaches utilize these regularities via the restrictive, and rather local, Manhattan World (MW) assumption which posits that every plane is perpendicular to one of the axes of a single coordinate system. The aforementioned regularities are especially evident in the surface normal distribution of a scene where they manifest as orthogonally-coupled clusters...
February 1, 2017: IEEE Transactions on Pattern Analysis and Machine Intelligence
https://www.readbyqxmd.com/read/28158088/launch-light-dependency-of-step-index-multimode-fiber-connections-analyzed-by-modal-power-distribution-using-encircled-angular-flux
#12
Shigeru Kobayashi, Manabu Yasukawa, Okihiro Sugihara
The propagating modal power distribution (MPD) of step-index multimode fibers (SI-MMFs), which strongly influences the performance of systems and components composed of these fibers, has not often been discussed, because, until recently, there has been no definition to show the MPD. Encircled angular flux (EAF) is a newly developed metric for defining the MPD in step-index multimode waveguides including fibers standardized by the International Electrotechnical Commission. Using the combined analysis of EAF and insertion loss, we studied the launch light dependency of SI-MMF connections...
February 1, 2017: Applied Optics
https://www.readbyqxmd.com/read/28158084/modified-twin-spot-launching-an-improved-launching-technique-for-enhancing-data-rates-in-multimode-fiber
#13
Thomas Joseph, Joseph John
Several launching techniques, such as center launching, offset launching, mode-field diameter-matched center launching, twin-spot launching, etc., have been employed in the past to reduce modal dispersion in multimode fibers (MMFs). Unfortunately, all these methods require stringent alignment accuracy of an input beam with the fiber axis. In this paper, we propose a new optical launching method, viz., the mode-field diameter-matched twin-spot launching technique, which combines the desirable features of two earlier techniques so as to excite only the fundamental mode in an MMF with much higher misalignment tolerance compared to existing launching techniques...
February 1, 2017: Applied Optics
https://www.readbyqxmd.com/read/28156185/evidence-of-activation-of-the-nrf2-pathway-in-multiple-sclerosis-patients-treated-with-delayed-release-dimethyl-fumarate-in-the-phase-3-define-and-confirm-studies
#14
Sreeja Gopal, Alvydas Mikulskis, Ralf Gold, Robert J Fox, Katherine T Dawson, Lakshmi Amaravadi
BACKGROUND: Delayed-release dimethyl fumarate (DMF) is an approved oral treatment for relapsing forms of multiple sclerosis (MS). Preclinical studies demonstrated that DMF activated the nuclear factor E2-related factor 2 (Nrf2) pathway. DMF and its primary metabolite monomethyl fumarate (MMF) were also shown to promote cytoprotection of cultured central nervous system (CNS) cells via the Nrf2 pathway. OBJECTIVE: To investigate the activation of Nrf2 pathway following ex vivo stimulation of human peripheral blood mononuclear cells (PBMCs) with DMF or MMF, and in DMF-treated patients from two Phase 3 relapsing MS studies DEFINE and CONFIRM...
January 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28153235/fabrication-of-a-polymeric-ionic-liquid-based-adsorbent-for-multiple-monolithic-fiber-solid-phase-microextraction-of-endocrine-disrupting-chemicals-in-complicated-samples
#15
Miao Pei, Zirui Zhang, Xiaojia Huang, Yuanfei Wu
A multiple monolithic fiber solid-phase microextraction (MMF-SPME) utilizing polymeric ionic liquid-based adsorbent was prepared. The adsorbent was obtained by in situ copolymerization of an ionic liquid, 1-trimethyl-(4-vinylbenzyl) aminium chloride and dual cross-linkers (divinylbenzene and ethylenedimethacrylate). The effect of preparation conditions including the content of ionic liquid and porogen in the polymerization mixture on extraction performance was studied in detail. Infrared spectroscopy, elemental analysis, scanning electron microscopy and mercury intrusion porosimetry were used to inspect the physicochemical properties of the new adsorbent...
April 1, 2017: Talanta
https://www.readbyqxmd.com/read/28127978/surgical-treatment-of-condylar-head-fractures-the-way-to-minimize-the-postraumatic-tmj-ankylosis
#16
D Hirjak, V Machon, M Beno, B Galis, I Kupcova
Management of condylar head fractures (CHF) of the temporomandibular joint (TMJ) remains a source of controversy. Based on established literature, group of CHFs and conservative treatment connected with period of maxillomandibular fixation (MMF) increases the risk for TMJ ankylosis. This paper presents anatomical and functional results of surgical treatment of condylar head fractures in a group of 24 patients (29 joints). Fractures were diagnosed based on conventional radiographs and computed tomography (CT) scans...
2017: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/28121342/clinical-applications-of-haploidentical-hematopoietic-stem-cell-transplantation-in-severe-aplastic-anemia
#17
P Zhang, K Feng, Y Xue, C-X Zhang, Y Wang, X-L Li
OBJECTIVE: The purpose of this study was to investigate the efficacy and safety of haploidentical allogeneic hemopoietic stem cell transplantation (allo-HSCT) in severe aplastic anemia (SAA) and prophylaxis of complications involved. PATIENTS AND METHODS: 8 patients with clinically diagnosed SAA (5 cases of SAA-I and 3 cases of SAA-II) were recruited, with the parents as the donors of hemopoietic stem cells. The conditioning regimen before HSCT included cyclophosphamide, fludarabine, pig anti-human lymphocyte immune globulin (p-ALG) and/or total body irradiation (TBI)...
January 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28104150/comparison-of-mizoribine-and-mycophenolate-mofetil-with-a-tacrolimus-based-immunosuppressive-regimen-in-living-donor-kidney-transplantation-recipients-a-retrospective-study-in-china
#18
Y Shi, H Liu, X-G Chen, Z-Y Shen
BACKGROUND: A retrospective study was conducted to investigate the prevalence of gastrointestinal (GI) symptoms as well as the efficacy and safety of mizoribine (MZR) and mycophenolate mofetil (MMF) in Chinese living-donor kidney transplantation (LDKT). METHODS: Forty-two recipients enrolled between January 2012 and March 2014 were treated with either MZR (n = 22) or MMF (n = 20). All patients were treated in combination with a tacrolimus-based immunosuppressive regimen, besides the study drugs...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28104146/successful-rescue-of-late-onset-antibody-mediated-rejection-12-years-after-living-donor-intestinal-transplantation-a-case-report
#19
G S Wu, Q C Zhao, Z S Li, Y Wu, W Z Wang, M B Li, H Shi, M Wang, D L Chen, J Y Zheng, X Wang, D M Fan
BACKGROUND: Antibody-mediated rejection (ABMR) has recently surfaced as a potential form of graft dysfunction after intestinal transplantation. METHODS: We present a case of an intestinal transplant recipient who developed late-onset ABMR 12 years after living-donor transplantation. An 18-year-old male recipient with a history of extensive intestinal resection secondary to acute bowel volvulus exhibited an excellent baseline immune profile for transplantation, including ABO-identical and HLA-haploidentical to the donor; a negative cross-match with a panel reactive antibody of 3...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28104127/calcineurin-inhibitor-induced-pain-syndrome-in-abo-incompatible-living-kidney-transplantation-a-case-report
#20
S Ishida, M Kato, T Fujita, Y Funahashi, N Sassa, Y Matsukawa, Y Yoshino, T Yamamoto, T Katsuno, S Maruyama, M Gotoh
BACKGROUND: Calcineurin-inhibitor-induced pain syndrome (CIPS) was used as a reference in the literature as reflex sympathetic dystrophy syndrome related to calcineurin inhibitors. Much of the literature describes CIPS that occurred after kidney and bone marrow transplantation. We describe a rare case of CIPS in induction immunosuppression before kidney transplantation, under administration of an anti-rheumatoid drug. METHODS: A 53-year-old woman had pre-status of ABO-incompatible living kidney transplantation...
January 2017: Transplantation Proceedings
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