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https://www.readbyqxmd.com/read/28413100/colchicine-resistance-and-intolerance-in-familial-mediterranean-fever-definition-causes-and-alternative-treatments
#1
REVIEW
Seza Ozen, Isabelle Kone-Paut, Ahmet Gül
BACKGROUND: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory syndrome characterized by recurrent serositis or arthritis attacks and, in some patients, chronic subclinical inflammation that predisposes to secondary amyloidosis. Colchicine is the gold standard of treatment, which reduces attack frequency and amyloidosis risk. However, up to 5% of patients are considered resistant or inadequately respond to colchicine, and some others cannot tolerate the side effects of effective doses of colchicine (colchicine intolerant)...
March 20, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28394112/effect-of-familial-mediterranean-fever-on-ivf-outcome-a-retrospective-case-series
#2
Nafiye Karakas Yilmaz, Mustafa Kara, Metin Kaba, Bugra Coskun, Selcuk Erkilinc, Salim Erkaya
Although the in vitro fertilization-intra-cytoplasmic sperm injection (IVF-ICSI) has been utilized widely, the management in patients with an autoimmune disease is still a challenge. The aim of this study was to demonstrate IVF-ICSI outcomes in infertile women with familial Mediterranean fever (FMF). Patient data were collected from the cases registered from January 2006 until January 2014. A total of 6152 assisted reproductive technology (ART) cycles were analyzed retrospectively in the Ankara Zekai Tahir Burak Women’s Health Education and Research Hospital...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28387826/clinical-update-on-inflammasomopathies
#3
Hafize Emine Sönmez, Seza Özen
Inflammasomes are important elements of the innate immune defense. The most common autoinflammatory syndromes, as well a number of rare ones, are due to hereditary defects in the inflammasomes, hence are called inflammasomopathies. The recent clinical advances in these diseases will be reviewed, with special emphasis on reflecting the international collaborative work in the field. Recent recommendations for familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS) and hyper-IgD syndrome (HIDS) / mevalonate kinase deficiency (MKD) will be presented and diagnostics tests, treatment alternatives, and follow-up recommendations will be summarized...
April 6, 2017: International Immunology
https://www.readbyqxmd.com/read/28386255/familial-mediterranean-fever-recent-developments-in-pathogenesis-and-new-recommendations-for-management
#4
REVIEW
Seza Özen, Ezgi Deniz Batu, Selcan Demir
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease (AID) affecting mainly the ethnic groups originating from Mediterranean basin. The disease is characterized by self-limited inflammatory attacks of fever and polyserositis along with elevated acute phase reactants. FMF is inherited autosomal recessively; however, a significant proportion of heterozygotes also express the phenotype. FMF is caused by mutations in the MEFV gene coding for pyrin, which is a component of inflammasome functioning in inflammatory response and production of interleukin-1β (IL-1β)...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28380758/few-mode-fiber-based-raman-distributed-temperature-sensing
#5
Meng Wang, Hao Wu, Ming Tang, Zhiyong Zhao, Yunli Dang, Can Zhao, Ruolin Liao, Wen Chen, Songnian Fu, Chen Yang, Weijun Tong, Perry Ping Shum, Deming Liu
We proposed and experimentally demonstrated a few mode fiber (FMF) based Raman distributed temperature sensor (RDTS) to extend the sensing distance with enhanced signal-to-noise ratio (SNR) of backscattered anti-Stokes spontaneous Raman scattering. Operating in the quasi-single mode (QSM) with efficient fundamental mode excitement, the FMF allows much larger input pump power before the onset of stimulated Raman scattering compared with the standard single mode fiber (SSMF) and mitigates the detrimental differential mode group delay (DMGD) existing in the conventional multimode fiber (MMF) based RDTS system...
March 6, 2017: Optics Express
https://www.readbyqxmd.com/read/28371574/tofacitinib-suppresses-disease-activity-and-febrile-attacks-in-a-patient-with-coexisting-rheumatoid-arthritis-and-familial-mediterranean-fever
#6
Kevser Gök, Gizem Cengiz, Kemal Erol, Salih Ozgocmen
Familial Mediterranean fever (FMF) is the most common hereditary auto-inflammatory (periodic fever) syndrome, and usually successfully treated with colchicine. However, nearly 5-10% of FMF cases are resistant or intolerant to colchicine and treatment options are highly restricted in these cases. Biologics including anakinra, canakinumab, rilonacept, etanercept, infliximab, interferon-alpha, and tocilizumab are shown to have efficacy to control FMF attacks. Tofacitinib, a Janus kinase (JAK) inhibitor, is an orally administered non-biologic disease modifying anti-rheumatic drug for the treatment of rheumatoid arthritis (RA)...
January 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28367474/genetic-analysis-of-southwestern-iranian-patients-with-familial-mediterranean-fever
#7
Mahmoud Haghighat, Mozhgan Moghtaderi, Shirin Farjadian
BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive genetic disorder characterized by recurrent episodes of self-limited fever and serosal tissues inflammation. METHODS: To evaluate clinical symptoms and common genetic mutations in southwestern Iranian patients with FMF, 20 unrelated patients were enrolled in this study based on clinical criteria. A panel of 12 common MEFV gene mutations was tested. RESULTS: The most frequent clinical presentations of the patients were fever, colicky abdominal pain and arthritis...
April 2017: Reports of Biochemistry & Molecular Biology
https://www.readbyqxmd.com/read/28362189/canakinumab-for-the-treatment-of-familial-mediterranean-fever
#8
Huri Ozdogan, Serdal Ugurlu
Familial Mediterranean fever (FMF) is the most frequent of all hereditary autoinflammatory syndromes. It is characterized by recurrent attacks of fever and serositis. If not treated it may be complicated with AA amyloidosis. It is caused by mutations in the MEFV gene that encodes pyrin which is involved in the regulation of IL-1β. The mainstay of treatment is colchicine, however a subset of patients requires an alternative treatment either due to inadequate response or intolerance. The accumulating data indicates that anti IL-1 drugs are effective in treating colchicine resistant FMF cases and improving their quality of life...
April 10, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28342915/redd1-links-stress-with-il-1%C3%AE-mediated-familial-mediterranean-fever-attack-through-autophagy-driven-neutrophil-extracellular-traps
#9
Panagiotis Skendros, Akrivi Chrysanthopoulou, François Rousset, Konstantinos Kambas, Athanasios Arampatzioglou, Alexandros Mitsios, Veronique Bocly, Theocharis Konstantinidis, Philippe Pellet, Iliana Angelidou, Eirini Apostolidou, Dimitrios Ritis, Victoria Tsironidou, Sotiris Galtsidis, Charalampos Papagoras, Dimitrios Stakos, Georgios Kouklakis, Vasiliki Dalla, Maria Koffa, Ioannis Mitroulis, Ioannis Theodorou, Konstantinos Ritis
BACKGROUND: Familial Mediterranean fever (FMF) is an IL-1β-dependent autoinflammatory disease caused by mutations of MEFV encoding pyrin, and characterised by inflammatory attacks, induced by physical or psychological stress. OBJECTIVE: We investigate the underlying mechanism that links stress-induced inflammatory attacks with neutrophil activation and release of IL-1β-bearing neutrophil extracellular traps (NETs) in FMF. METHODS: RNA-seq was performed in peripheral neutrophils from 3 FMF patients, isolated both during attack and remission, 8 patients in remission and 8 healthy individuals...
March 22, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28341008/serum-vitamin-d-level-in-egyptian-children-with-familial-mediterranean-fever
#10
Hala M Lotfy, Huda Marzouk, Yomna Farag, Ahmed Salah, Heba Taher, Mohamed Nabih, Laila Rashed, Kamal El-Garf
BACKGROUND: The aim of the study is to measure plasma vitamin D levels in a group of Egyptian children with familial Mediterranean fever (FMF) compared to healthy children. METHODS: The study enrolled 52 children with FMF and 40 apparently healthy controls. Serum vitamin D level was measured by enzyme-linked immunosorbent assay. RESULTS: The mean serum vitamin D level was significantly lower in children with FMF than control group (12.3±3...
March 21, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28340799/is-there-a-long-term-risk-for-donors-with-heterozygous-mefv-mutation-after-kidney-donation
#11
S Karakose, S Erdogmus, S Akturk, A Tuzuner, S Sengul, K Keven
BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disorder manifested severely by systemic amyloidosis. It has been hypothesized that heterozygous carriers may also have susceptibility to certain symptoms or even diseases. Because the living kidney donors of patients with FMF are generally relatives of the kidney recipients, there is a high possibility that the donors will have a heterozygous mutation of the FMF gene. The goal of this study was to investigate the long-term kidney function of donors who are carriers of the Mediterranean fever (MEFV) gene...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28319450/retinal-and-choroidal-thickness-in-adult-patients-with-familial-mediterranean-fever
#12
Tolga Bicer, Osman Celikay, Mustafa Kosker, Muhammed Yunus Alp, Cem Ozisler, Ahmet Yesilyurt, Burcu Kucuk Bicer, Canan Gurdal
PURPOSE: We aimed to evaluate changes in retinal, choroidal, ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) thicknesses in genetically diagnosed adult patients with familial Mediterranean fever (FMF). METHODS: A total of 50 eyes of 50 genetically diagnosed patients with FMF and 50 eyes of controls were analyzed. Patients were recruited from the Genetic Diagnostic Center of Dışkapı Yıldırım Beyazıt Research and Training Hospital, Turkey. Retinal and choroidal thicknesses were obtained using spectral-domain optical coherence tomography from choroid, retina, GCC, and RNFL...
March 20, 2017: Ophthalmic Epidemiology
https://www.readbyqxmd.com/read/28302131/a-survey-of-resistance-to-colchicine-treatment-for-french-patients-with-familial-mediterranean-fever
#13
REVIEW
Alice Corsia, Sophie Georgin-Lavialle, Véronique Hentgen, Eric Hachulla, Gilles Grateau, Albert Faye, Pierre Quartier, Linda Rossi-Semerano, Isabelle Koné-Paut
BACKGROUND: Colchicine is the standard treatment for familial Mediterranean fever (FMF), preventing attacks and inflammatory complications. True resistance is rare and yet not clearly defined. We evaluated physicians' definition of colchicine resistance and report how they manage it. PATIENTS AND METHODS: We recruited patients with a clinical diagnosis of FMF, one exon-10 Mediterranean fever (MEFV) gene mutation and considered resistant to colchicine, via networks of expert physicians...
March 16, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28295782/multicenter-screening-for-preeclampsia-by-maternal-factors-and-biomarkers-at-11-13-weeks-gestation-comparison-to-nice-guidelines-and-acog-recommendations
#14
Neil O' Gorman, David Wright, Liona C Poon, Daniel L Rolnik, Argyro Syngelaki, Mercedes de Alvarado, Ilma F Carbone, Vivien Dutemeyer, Madgalena Fiolna, Alex Frick, Natalia Karagiotis, Sofia Mastrodima, Catalina de Paco Matallana, George Papaioannou, Andrea Pazos, Walter Plasencia, Kypros H Nicolaides
OBJECTIVE: To compare the performance of screening for preeclampsia (PE) based on risk factors from the medical history, as recommended by NICE and ACOG, with the method proposed by the Fetal Medicine Foundation (FMF), which uses Bayes theorem to combine the a priori risk from maternal factors, derived by a multivariable logistic model, with the results of various combinations of biophysical and biochemical measurements. METHODS: This was a prospective multicenter study of screening for PE in 8,775 singleton pregnancies at 11-13 weeks' gestation...
March 10, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28293457/anakinra-use-during-pregnancy-report-of-a-case-with-familial-mediterranean-fever-and-infertility
#15
Ufuk İlgen, Orhan Küçükşahin
Familial Mediterranean fever (FMF), affecting people of Mediterranean origin, is an endemic and sometimes problematic disease because of colchicine resistance/intolerance, with relative lack of treatment alternatives, and disease- or treatment-related issues, such as subfertility. Anakinra, being a rational and effective treatment alternative, has no conclusive human pregnancy data. Here we report a case of FMF with infertility who became pregnant with in vitro fertilization (IVF) under treatment with anakinra, along with the pregnancy outcome...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28289585/the-mefv-gene-pathogenic-variants-and-phenotype-genotype-correlation-in-children-with-familial-mediterranean-fever-in-the-%C3%A3-anakkale-population
#16
F Battal, F Silan, N Topaloğlu, H Aylanç, Ş Yıldırım, F Köksal Binnetoğlu, M Tekin, N Kaymaz, O Ozdemir
The aim of the current study was to determine the frequency of the Mediterranean fever (MEFV) gene pathogenic variants in 60 children diagnosed with familial Mediterranean fever (FMF) and to compare the phenotype-genotype correlation. Genomic DNA was isolated by the spin-column method from peripheral blood samples (collected in vacutainers containing EDTA) and buccal smears. The MEFV gene profiles for the current FMF cohort were genotyped by pyrosequencing and direct Sanger sequencing techniques for the target pathogenic variants...
December 1, 2016: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28260482/late-diagnosis-of-e148q-mutation-positive-familial-mediterranean-fever-in-a-kidney-transplant-patient-with-fever-of-unknown-origin-a-case-report
#17
Erhan Tatar, Adam Uslu, Cenk Simsek, Ahmet Aykas, Giray Bozkaya, Cetin Imamoglu
Fever of unknown origin is a rare condition after solid organ transplant and is generally associated with atypical infections (eg, tuberculosis, fungal infections) and/or lymphoproliferative disorders. Here, we present a kidney transplant patient with a late diagnosis of E148Q mutation-positive familial Mediterranean fever as the cause of fever of unknown origin. A 22-year-old female patient with a previous history of 4 years of hemodialysis and unknown primary renal disease received a deceased-donor kidney transplant at our center 5 years previously...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28252867/vernachl%C3%A3-ssigte-klinische-merkmale-der-follikulotropen-mycosis-fungoides-eine-gro%C3%A3-e-klinische-fallserie
#18
Can Baykal, Tugba Atci, Sule Ozturk Sari, Algun Polat Ekinci, Nesimi Buyukbabani
HINTERGRUND UND ZIEL: Als seltene Form der Mycosis fungoides (MF), ist die follikulotrope MF (FMF) durch ein breites Spektrum klinischer Symptome gekennzeichnet. Dazu gehören, neben den vorherrschenden follikulären Läsionen, auch viele atypische Manifestationen. Das Ziel der vorliegenden Studie war eine klinische Bewertung von FMF-Patienten, unter besonderer Berücksichtigung von vernachlässigten dermatologischen Merkmalen. PATIENTEN UND METHODIK: Insgesamt wurden 27 FMF-Patienten aus dem 572 Patienten umfassenden MF-Register unserer Abteilung retrospektiv bezüglich ihrer Demographie sowie der klinischen Merkmale, Behandlungsformen, Nachsorge und Therapieergebnisse bewertet...
March 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28241574/demonstration-of-analog-links-using-spatial-modes-in-km-scale-few-mode-fiber
#19
Jing Du, Dequan Xie, Chen Yang, Jian Wang
Analog links in a 5-km few mode fiber (FMF) are experimentally investigated by exploiting fundamental and high-order linearly polarized (LP) and OAM modes (LP<sub>01</sub>, LP<sub>11a</sub>, LP<sub>11b</sub>, OAM<sub>+1</sub>, OAM<sub>-1</sub>). We use spurious free dynamic range (SFDR) of the second-order harmonic distortion (SHD) to evaluate the analog transmission performance. The dependence of analog link performance on the mode relative loss (MRL) between the fundamental and high-order LP and OAM modes is studied...
February 20, 2017: Optics Express
https://www.readbyqxmd.com/read/28240409/underrecognized-clinical-features-of-folliculotropic-mycosis-fungoides-a-large-clinical-series
#20
Can Baykal, Tugba Atci, Sule Ozturk Sari, Algun Polat Ekinci, Nesimi Buyukbabani
BACKGROUND AND OBJECTIVE: A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features. PATIENTS AND METHODS: Overall, 27 FMF patients enrolled in our department';s MF registry, which includes 572 patients, were retrospectively reevaluated with regard to demographics, clinical features, treatment modalities, follow-up, and outcomes...
February 27, 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
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