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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
#1
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/28210092/first-successful-perinatal-management-of-pregnancy-after-abo-incompatible-liver-transplantation
#2
Hisanobu Higashi, Hideaki Obara, Kei Miyakoshi, Masahiro Shinoda, Minoru Kitago, Naoki Shimojima, Yuta Abe, Taizo Hibi, Hiroshi Yagi, Kentaro Matsubara, Yohei Yamada, Osamu Itano, Ken Hoshino, Tatsuo Kuroda, Yuko Kitagawa
Many papers have reported on pregnancy and delivery after liver transplantation, but there have been no reports on pregnancy after ABO-incompatible liver transplantation. This paper reports the first successful pregnancy and delivery of a newborn after ABO-incompatible liver transplantation for fulminant hepatic failure. The patient was a 39-year-old female. She had an ABO-incompatible liver transplantation, donated from her husband, due to subacute fulminant hepatitis of unknown etiology. She was taking tacrolimus, methylprednisolone, and mizoribine orally for the maintenance of immunosuppression at the time of discharge...
January 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28209661/non-topical-management-of-recalcitrant-paediatric-atopic-dermatitis
#3
REVIEW
Jin Ho Chong, Mark Jean Aan Koh
Atopic dermatitis (AD) is a common chronic, pruritic skin disease in children. As the incidence of AD increases, especially in high-income countries, paediatricians may see an increasing number of recalcitrant AD cases in their practice. Although these cases are principally managed by paediatric dermatologists, it is important for paediatricians to be aware of the use and side effects of non-topical treatment like phototherapy and systemic agents as well as the evidence for alternative treatment, which caregivers may ask about...
February 16, 2017: Archives of Disease in Childhood
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/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#6
REVIEW
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
February 16, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28205247/a-novel-freeze-dried-storage-and-preparation-method-for-the-determination-of-mycophenolic-acid-in-plasma-by-high-performance-liquid-chromatography
#7
Lei Wang, Wei Qiang, Ying Li, Zeneng Cheng, Mengmeng Xie
Plasma samples were conventionally stored at freezing conditions until the time of detection. Such a technique, when carried out over an extended period, is energy-consuming, in addition, preparation and transportation of stored samples is inconvenient. In this study, a freeze-dried storage and preparation method was proposed to determine the presence of mycophenolic acid (MPA) in plasma. Fresh plasma samples were freeze-dried using a device, and then stored at ambient temperature. After the stored samples were soaked with methanol spiked with the internal standard, high-performance liquid chromatography was conducted to detect MPA...
February 15, 2017: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/28191596/efficacy-and-safety-of-rituximab-in-comparison-with-common-induction-therapies-in-pediatric-active-lupus-nephritis
#8
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
#9
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
#10
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/28176617/progressive-multifocal-leukoencephalopathy-in-a-kidney-transplant-recipient
#11
Federico Cicora, Javier Roberti
Progressive multifocal leukoencephalopathy (PML) is a debilitating and often fatal viral disease of the central nervous system. A 39-year-old male kidney transplant recipient presented to our hospital with weakness of the left arm and leg, his immunosuppressive regimen consisted of tacrolimus, mycophenolate sodium, and meprednisone. Magnetic resonance imaging of the brain revealed hypercellularity in neural tissue and a lesion affecting the right ventral and lateral areas of the thalamus and the posterior limb of the internal capsule...
February 7, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28176473/durable-remission-of-c3-glomerulonephritis-with-mycophenolate-mofetil
#12
REVIEW
Nicole Lioufas, Moira Finlay, Thomas Barbour
In C3 glomerulopathy, uncontrolled complement C3 activation via the alternative pathway results in glomerular C3 deposition and, in many cases, progressive renal failure. Despite advances in understanding of C3G pathogenesis over the last few years, there are no proven treatments. We describe a patient in whom C3 glomerulopathy was associated with renal impairment and elevated serum free kappa light chains. An initial response to corticosteroids was followed by relapse once steroids were weaned, prompting use of mycophenolate mofetil to maintain remission...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28174010/a-case-of-t-cell-lymphoproliferative-disorder-associated-with-hypereosinophilia-with-excellent-response-to-mycophenolate-mofetil
#13
Amr Hanbali, Marwan Shaheen, Feras Alfraih, Wafa Al-Otaibi, Riad El Fakih, Tarek Owaidah, Syed Ahmed
Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×10(9)/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level...
February 1, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28172692/p613-mycophenolate-mofetil-is-a-valid-option-in-patients-with-inflammatory-bowel-disease-resistant-to-tnf-alpha-inhibitors-and-conventional-immunosuppressants
#14
F S Macaluso, M Maida, S Renna, E Orlando, M Affronti, C Sapienza, M Dimarco, R Orlando, G Rizzuto, M Cottone, A Orlando
No abstract text is available yet for this article.
February 1, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28169970/second-line-agents-in-pediatric-patients-with-autoimmune-hepatitis-a-systematic-review-and-meta-analysis
#15
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/28160394/cardiovascular-morbidity-and-mortality-after-liver-transplantation-the-protective-role-of-mycophenolate-mofetil
#16
D D'Avola, V Cuervas-Mons, J Martí, J Ortiz de Urbina, L Lladó, C Jimenez, E Otero, F Suarez, J M Rodrigo, M A Gómez, E Fraga, P Lopez, T Serrano, A Rios, E Fábrega, J I Herrero
: Cardiovascular (CV) diseases are recognized long-term causes of death after liver transplantation (LT). The objective of this multicenter study was to analyse the prevalence and the evolution of CV risk factors and CV morbidity and mortality in 1819 liver transplant recipients along 5 years after LT. The influence of baseline variables on survival, morbidity and mortality was studied. There was a continuous and significant increase of the prevalence of all the CV risk factors (except smoking) after LT...
February 3, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28160307/treatment-of-presumptive-primary-immune-mediated-thrombocytopenia-with-mycophenolate-mofetil-versus-cyclosporine-in-dogs
#17
F O Cummings, S A Rizzo
OBJECTIVES: The objective of this study was to compare hospitalisation duration, survival times, adverse events and cost of therapy in dogs with presumptive primary immune-mediated thrombocytopenia undergoing therapy with mycophenolate mofetil and corticosteroids versus cyclosporine and corticosteroids. METHODS: A retrospective study of medical case records of dogs with presumed primary immune-mediated thrombocytopenia was conducted. Data collected included signalment, presenting complaints, haematologic and biochemical profiles, vector-borne disease testing, thoracic and abdominal radiographs, abdominal ultrasound, medications administered, duration of hospitalisation, 30- and 60-day survival, adverse events and cost of therapy...
February 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28152659/development-of-primary-central-nervous-system-lymphoma-in-a-systemic-lupus-erythematosus-patient-after-treatment-with-mycophenolate-mofetil-and-review-of-the-literature
#18
M A Balci, G E Pamuk, E Unlu, U Usta, O N Pamuk
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28152123/association-of-therapy-for-autoimmune-disease-with-myelodysplastic-syndromes-and-acute-myeloid-leukemia
#19
Natalie Ertz-Archambault, Heidi Kosiorek, Gretchen E Taylor, Katalin Kelemen, Amylou Dueck, Janna Castro, Robert Marino, Susanne Gauthier, Laura Finn, Lisa Z Sproat, Jeanne Palmer, Ruben A Mesa, Aref Al-Kali, James Foran, Raoul Tibes
Importance: Therapy-related myeloid neoplasms are a potentially life-threatening consequence of treatment for autoimmune disease (AID) and an emerging clinical phenomenon. Objective: To query the association of cytotoxic, anti-inflammatory, and immunomodulating agents to treat patients with AID with the risk for developing myeloid neoplasm. Design, Setting, and Participants: This retrospective case-control study and medical record review included 40 011 patients with an International Classification of Diseases, Ninth Revision, coded diagnosis of primary AID who were seen at 2 centers from January 1, 2004, to December 31, 2014; of these, 311 patients had a concomitant coded diagnosis of myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML)...
February 2, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28148558/risk-factors-for-renal-survival-in-chinese-patients-with-myeloperoxidase-anca-associated-gn
#20
Yinghua Chen, Hao Bao, Zhengzhao Liu, Xia Liu, Erzhi Gao, Caihong Zeng, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND AND OBJECTIVES: Our study explored the association of histopathologic classification of ANCA-associated GN with renal survival in Chinese patients with myeloperoxidase-ANCA-associated GN. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Two hundred fifteen patients with biopsy-proven myeloperoxidase-ANCA-associated GN were included from January of 1996 to December of 2014. The biopsies included focal (n=27), mixed (n=82), crescentic (n=47), and sclerotic (n=59) classes...
February 1, 2017: Clinical Journal of the American Society of Nephrology: CJASN
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