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https://www.readbyqxmd.com/read/27911804/familial-mediterranean-fever-mutations-lift-the-obligatory-requirement-for-microtubules-in-pyrin-inflammasome-activation
#1
Hanne Van Gorp, Pedro H V Saavedra, Nathalia M de Vasconcelos, Nina Van Opdenbosch, Lieselotte Vande Walle, Magdalena Matusiak, Giusi Prencipe, Antonella Insalaco, Filip Van Hauwermeiren, Dieter Demon, Delfien J Bogaert, Melissa Dullaers, Elfride De Baere, Tino Hochepied, Joke Dehoorne, Karim Y Vermaelen, Filomeen Haerynck, Fabrizio De Benedetti, Mohamed Lamkanfi
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It is caused by mutations in the inflammasome adaptor Pyrin, but how FMF mutations alter signaling in FMF patients is unknown. Herein, we establish Clostridium difficile and its enterotoxin A (TcdA) as Pyrin-activating agents and show that wild-type and FMF Pyrin are differentially controlled by microtubules. Diverse microtubule assembly inhibitors prevented Pyrin-mediated caspase-1 activation and secretion of IL-1β and IL-18 from mouse macrophages and human peripheral blood mononuclear cells (PBMCs)...
November 22, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27909774/evaluation-of-upper-abdominal-organs-with-dwi-in-patients-with-familial-mediterranean-fever
#2
Eda Albayrak, Safak Sahin
PURPOSE: To investigate the diagnostic efficiency of diffusion-weighted magnetic resonance imaging (DWI) for the evaluation of functional changes that can occur in upper abdominal organs in patients with familial Mediterranean fever (FMF). METHODS: The study included 50 controls, 45 patients with FMF, and 14 patients with FMF who had accompanying proteinuria. Measurement of apparent diffusion coefficient (ADC) was performed using DWI sections obtained from liver, spleen, kidney, and pancreas parenchyma with 1...
December 2, 2016: Abdominal Radiology
https://www.readbyqxmd.com/read/27886801/approach-to-the-patients-with-inadequate-response-to-colchicine-in-familial-mediterranean-fever
#3
REVIEW
Ahmet Gül
Familial Mediterranean fever (FMF) is the most common form of monogenic autoinflammatory conditions, and response to colchicine has been considered as one of its distinctive features among other hereditary periodic fever disorders. Prophylactic colchicine has been shown to be effective in the prevention of inflammatory attacks and development of amyloidosis. However, the highest tolerable doses of colchicine may not be adequate enough to manage these goals in approximately 5% of FMF patients. Inadequate response to colchicine in fully compliant FMF patients may be associated with genetic and/or environmental factors affecting disease severity and colchicine bioavailability...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27860460/anakinra-for-colchicine-resistant-familial-mediterranean-fever-a-randomized-double-blind-placebo-controlled-trial
#4
Ilan Ben-Zvi, Olga Kukuy, Eitan Giat, Elon Pras, Olga Feld, Shaye Kivity, Senior Lecturer, Oleg Perski, Gil Bornstein, Chagai Grossman, Gil Harari, Merav Lidar, Avi Livneh
Objective - Familial Mediterranean fever (FMF) is refractory to colchicine prophylaxis in 10-20% of patients. A patient series showed anakinra, an IL-1 blocking agent, to prevent FMF attacks in colchicine-resistant FMF (CRFMF). Here we studied the efficacy and safety of anakinra in the treatment of CRFMF, using a randomized controlled trial. Methods - CRFMF patients receiving colchicine (≥1.5 to ≤3 mg/day) were recruited and randomly assigned to receive anakinra or placebo (vehicle). Treatment duration was 4 months...
November 11, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27856657/the-challenge-of-autoinflammatory-syndromes-with-an-emphasis-on-hyper-igd-syndrome
#5
REVIEW
Jos W M van der Meer, Anna Simon
Autoinflammatory syndromes are disorders with an exaggerated inflammatory response, mostly in the absence of an appropriate trigger. Prototypic autoinflammatory syndromes are FMF, hyper-IgD syndrome (also known as mevalonate kinase deficiency), TNF receptor-associated periodic syndrome and cryopyrin-associated periodic syndrome. The clinical phenotypes partly overlap (with fever and acute phase response), but also differ between the various syndromes (e.g. regarding fever pattern, episodic vs chronic inflammation and accompanying clinical signs)...
December 2016: Rheumatology
https://www.readbyqxmd.com/read/27837188/capsular-stroke-modeling-based-on-somatotopic-mapping-of-motor-fibers
#6
Hanlim Song, Wonbin Jung, Eulgi Lee, Ji-Young Park, Min Sun Kim, Min-Cheol Lee, Hyoung-Ihl Kim
Recently, several capsular stroke models have been reported with different targets of destruction. This study was performed to establish an accurate internal capsule (IC) target for capsular stroke modeling in rats. We injected adeno-associated virus serotype 5 (AAV)-CaMKII-EYFP into forelimb motor cortex and AAV-CaMKII-mCherry into hindlimb motor cortex (n = 9) to anterogradely trace the pyramidal fibers and map their somatotopic distribution in the IC. On the basis of the neural tracing results, we created photothrombotic infarct lesions in rat forelimb and hindlimb motor fiber (FMF and HMF) areas of the IC (n = 29) and assessed motor behavior using a forelimb-use asymmetry test, a foot-fault test, and a single-pellet reaching test...
November 11, 2016: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/27828627/inflammasomes-and-dermatology
#7
Daniel Coelho de Sá, Cyro Festa
Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies. Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA)...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27828313/cost-effective-wavelength-reused-mdm-system-for-bidirectional-mobile-fronthaul
#8
Yuanxiang Chen, Juhao Li, Paikun Zhu, Huanfa Peng, Jinglong Zhu, Yu Tian, Zhongying Wu, Dawei Ge, Peng Zhou, Ke Liu, Yongchi Xu, Jingbiao Chen, Yongqi He, Zhangyuan Chen
In this paper, we propose a cost-effective wavelength-reused mode-division-multiplexing (MDM) system for high speed symmetrical bidirectional mobile fronthaul application. At the base band unit (BBU) pool, one of the spatial modes is used to transmit signal carrier while the others are used for downstream (DS) signal channels. At the remote radio unit (RRU) side, the signal carrier is split and reused as modulation carrier for all the upstream (US) signal channels after mode demultiplexing. Thanks to the low mode crosstalk characteristic of the mode multiplexer/demultiplexer (MUX/DEMUX) and few-mode fiber (FMF), the signal carrier and each signal channel can be effectively separated...
October 3, 2016: Optics Express
https://www.readbyqxmd.com/read/27822927/decreased-chitotriosidase-activity-and-levels-in-familial-mediterranean-fever
#9
Halef Okan Doğan, Ahmet Omma, Turan Turhan, Nihal Boğdaycıoğlu, Yaşar Karaaslan, Hayrettin Yavuz, Özlem Demirpençe, Hüseyin Aydın, Sevtap Bakır
Different studies have demonstrated changes in chitotriosidase (ChT) activity and concentrations in multiple diseases. However, changes in ChT activity and concentrations have not been concurrently evaluated in patients with Familial Mediterranean Fever (FMF). In this study, we analyzed the changes in serum ChT activity and concentrations in patients with FMF. The study included a total of 80 patients with FMF and 80 healthy controls. ChT enzyme activity and concentrations were measured and then compared between the groups...
December 2016: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27791951/mefv-and-saa1-genotype-associations-with-clinical-features-of-familial-mediterranean-fever-and-amyloidosis-in-armenia
#10
Stepan Atoyan, Hasmik Hayrapetyan, Tamara Sarkisian, Eldad Ben-Chetrit
OBJECTIVES: Familial Mediterranean fever (FMF) is a hereditary periodic disease characterised by recurrent attacks of fever and serositis. The most devastating complication of FMF is amyloidosis (AA) affecting mainly the kidneys. Aim of the study is to search for correlations between the MEFV genotype and the SAA polymorphisms with the clinical manifestations of FMF and the occurrence of amyloidosis in a large cohort of Armenian patients. METHODS: Information about the MEFV mutations, SAA polymorphisms and FMF clinical features, were obtained for 1017 FMF patients, from the database of the Center of Medical Genetics in Yerevan...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27791950/a-novel-assessment-tool-for-clinical-care-of-patients-with-autoinflammatory-disease-juvenile-autoinflammatory-disease-multidimensional-assessment-report
#11
Dilek Konukbay, Marco Gattorno, Dilek Yildiz, Joost Frenkel, Cengizhan Acikel, Betul Sozeri, Balahan Makay, Nuray Aktay Ayaz, Kenan Barut, Aysenur Kisaarslan, Yelda Bilginer, Dursun Karaman, Harun Peru, Dogan Simsek, Ozlem Aydog, Erbil Unsal, Zubeyde Gunduz, Berna Eren Fidanci, Isabelle Kone-Paut, Ozgur Kasapcopur, Angelo Ravelli, Seza Ozen, Erkan Demirkaya
OBJECTIVES: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27790798/higher-pentraxin-3-levels-are-associated-with-inflammation-in-familial-mediterranean-fever
#12
Mesudiye Bulut, Mevlut Ceri, Selman Unverdi, Mustafa Altay, Mehmet Senes, Zafer Aydın Ecemis, Murat Duranay
BACKGROUND: Circulating levels of Pentraxin-3 (PTX3) have been shown to increase in several inflammatory conditions. However, there is no information about the levels of PTX3 in patients with familial Mediterranean fever (FMF). This study was designed to evaluate the serum PTX3 levels in patients with FMF during attack and free-attack periods. METHODS: Twenty FMF patients in attack and free-attack period, and 20 age-, sex-, and body mass index-matched healthy controls were included in the study...
November 2016: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/27776949/beh%C3%A3-et-s-disease%C3%A2-and-fmf-two-sides-of-the-same-coin-or-just-an-association-a-cross-sectional-study%C3%A2
#13
Abdulla Watad, Shmuel Tiosano, Dana Yahav, Doron Comaneshter, Yehuda Shoenfeld, Arnon D Cohen, Howard Amital
BACKGROUND: Familial Mediterranean fever (FMF) is a genetic disease, characterized by attacks of fever, arthritis, serositis and pain. Behçet's disease (BD) is an inflammatory disorder with a genetic basis, characterized by oral and genital ulcers, uveitis, pustular erythematous cutaneous lesions, arthritis, central nervous system involvement and possible vascular manifestations such as venous thrombosis, arteritis and aneurysms. OBJECTIVES: To investigate the association and actual differentiation between these two entities in a large-scale population-based study...
October 21, 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27776765/evaluation-of-the-effectiveness-of-acupuncture-therapy-by-verbal-pain-scale-in-patients-with-abdominal-pain-of-familial-mediterranean-fever
#14
Sinan Becel, Yılmaz Sezgin, Fatih Akçay
In this study, we evaluated the effectiveness of acupuncture therapy based on Verbal Pain Scale (VPS) scores in familial Mediterranean fever (FMF) patients admitted to the emergency department with attacks of abdominal pain. This observational study was conducted in Erzurum Regional Training and Research Hospital between August 2014 and December 2014. Twenty patients admitted to the emergency department with FMF attacks were included in the study. Acupuncture therapy was applied to three points including LI4 (Hegu), ST25 (Tianshu), and Ren12 (Zhongwan)...
October 2016: Journal of Acupuncture and Meridian Studies
https://www.readbyqxmd.com/read/27775003/rayleigh-scattering-in-few-mode-optical-fibers
#15
Zhen Wang, Hao Wu, Xiaolong Hu, Ningbo Zhao, Qi Mo, Guifang Li
The extremely low loss of silica fibers has enabled the telecommunication revolution, but single-mode fiber-optic communication systems have been driven to their capacity limits. As a means to overcome this capacity crunch, space-division multiplexing (SDM) using few-mode fibers (FMF) has been proposed and demonstrated. In single-mode optical fibers, Rayleigh scattering serves as the dominant mechanism for optical loss. However, to date, the role of Rayleigh scattering in FMFs remains elusive. Here we establish and experimentally validate a general model for Rayleigh scattering in FMFs...
October 24, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27766107/case-report-recurrent-abdominal-symptoms-in-a-child-with-panhypopituitarism-there-is-always-a-differential
#16
Laura Olbrich, Eva Schmidt, Ertan Mayatepek, Markus Vogel
BACKGROUND: We report the case of a 6 year old boy suffering from adenohypophysis aplasia as well as ectopic neurohypophysis and delayed diagnosis of familial Mediterranean fever (FMF). CASE PRESENTATION: The boy was diagnosed with panhypopituitarism during the neonatal period and suffered from recurrent episodes during the following years suggesting infections. He also showed signs of adrenal insufficiency. Finally, at the age of 6 years, an additional diagnosis of familial Mediterranean fever (FMF) was clinically suspected and later confirmed by molecular analysis...
2016: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/27760547/secondary-bladder-amyloidosis-with-familial-mediterranean-fever-in-a-living-donor-kidney-transplant-recipient-a-case-report
#17
Sentaro Imamura, Shintaro Narita, Ryuta Nishikomori, Hiroshi Tsuruta, Kazuyuki Numakura, Atsushi Maeno, Mitsuru Saito, Takamitsu Inoue, Norihiko Tsuchiya, Hiroshi Nanjo, Toshio Heike, Shigeru Satoh, Tomonori Habuchi
BACKGROUND: Secondary bladder amyloidosis is an extremely rare disease, resulting from a chronic systematic inflammatory disorder associated with amyloid deposits. Although uncommon in Japan, familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever of short duration and serositis and is frequently associated with systemic amyloidosis. Here, we present a case of a Japanese patient complaining of fever and macroscopic hematuria after a living donor renal transplantation...
October 19, 2016: BMC Research Notes
https://www.readbyqxmd.com/read/27733942/the-role-of-mefv-mutations-in-the-concurrent-disorders-observed-in-patients-with-familial-mediterranean-fever
#18
Sabri Güncan, N Şule Y Bilge, Döndü Üsküdar Cansu, Timuçin Kaşifoğlu, Cengiz Korkmaz
OBJECTIVE: This study aimed to investigate the frequency in which familial Mediterranean fever (FMF) coexists with other diseases and determine whether Mediterranean fever (MEFV) gene mutations are involved in such coexistence. MATERIAL AND METHODS: In total, 142 consecutive patients with FMF investigated for MEFV mutation were enrolled in this study [Female: 87; Male: 55, mean age 32±12 years (11-62)]. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively...
September 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27725555/familial-mediterranean-fever-with-rheumatoid-arthritis-complicated-by-pulmonary-paragonimiasis
#19
Shin-Ichi Nureki, Koji Ishii, Hideaki Fujisaki, Masataka Torigoe, Keisuke Maeshima, Hirotaka Shibata, Eishi Miyazaki, Jun-Ichi Kadota
A 42-year-old woman presented with an intermittent fever and chest and back pain, and an abnormal chest shadow was detected. She was diagnosed with paragonimiasis caused by Paragonimus westermani. Praziquantel therapy improved the abnormal chest shadow, but did not relieve her symptoms. She was also diagnosed with familial Mediterranean fever (FMF), and colchicine therapy resolved her symptoms. She subsequently developed arthralgia and morning stiffness in her hands. We also diagnosed the patient with rheumatoid arthritis (RA), and corticosteroid and salazosulfapyridine therapy improved her symptoms...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27723279/international-retrospective-chart-review-of-treatment-patterns-in-severe-fmf-traps-and-mkd-hids
#20
Seza Ozen, Jasmin B Kuemmerle-Deschner, Rolando Cimaz, Avi Livneh, Pierre Quartier, Isabelle Kone-Paut, Andrew Zeft, Steve Spalding, Ahmet Gul, Veronique Hentgen, Sinisa Savic, Ivan Foeldvari, Joost Frenkel, Luca Cantarini, Dony Patel, Jeffrey Weiss, Nina Marinsek, Ravi Degun, Kathleen G Lomax, Helen J Lachmann
OBJECTIVES: Periodic fever syndromes (PFS) are characterised by recurrent attacks of fever and localised inflammation. This study examined the diagnostic pathway and treatments at tertiary centres for FMF, TRAPS and MKD/HIDS. METHODS: PFS specialists at medical centres in the USA, European Union and Eastern Mediterranean participated in a retrospective chart review, providing de-identified data in an electronic case report form. Patients were treated between 2008-2012 with at least 1 year of follow-up, all had clinical and/or genetically proven disease, and were on/eligible for biologic treatment...
October 9, 2016: Arthritis Care & Research
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