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Gernot Kriegshäuser, Dietmar Enko, Hasmik Hayrapetyan, Stepan Atoyan, Christian Oberkanins, Tamara Sarkisian
PurposeThis work aimed at investigating demographic, clinical, and genetic characteristics of individuals experiencing their first familial Mediterranean fever (FMF) attack at age ≥40 years in a very large cohort of Armenian FMF patients.MethodsIn total, 10,370 Armenian patients diagnosed with FMF based on the Tel Hashomer criteria and carrying at least one MEFV mutant allele were included in this study.ResultsA total of 354 (3.40%) patients had late-onset FMF. Of these, 194 (54.80%) were female and 160 (45...
March 15, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Karin P S Langenberg-Ververgaert, Ronald M Laxer, Angela S Punnett, L Lee Dupuis, Yaron Finkelstein, Oussama Abla
Familial Mediterranean fever (FMF) has been associated with hematological malignancies but has not been reported in association with Hodgkin lymphoma (HL). We hereby describe the first pediatric patient with FMF and stage IIA nodular sclerosis HL. She was treated with prednisone, doxorubicin, vincristine and etoposide (OEPA) being on therapy with colchicine. However, she suffered more than expected treatment-related toxicity attributed either to chemotherapy (severe neutropenia) or colchicine (Abdominal pains and diarrhoea)...
2018: Mediterranean Journal of Hematology and Infectious Diseases
Shams Kholoussi, Naglaa Kholoussi, Moushira Erfan Zaki, Hala T El-Bassyouni, Hala Elnady, Botros Morcos, Asem Abo-Shanab
OBJECTIVE: This study aimed to investigate T & B lymphocyte subsets and Natural Killer (NK) cells patterns in children with FMF versus normal control subjects, to estimate the immunoglobulins IgG, IgM, and IgA levels, and to scrutinize the possible use of Neutrophil / Lymphocyte ratio (NLR) as a marker for subclinical inflammation in FMF patients. PATIENTS AND METHODS: A group of 42 patients with FMF attending the Genetics Clinic at National Research Centre were included in this study...
February 15, 2018: Open Access Macedonian Journal of Medical Sciences
Shoulin Jiang, Lin Ma, Zuyuan He
We propose orientation-insensitive azimuthally asymmetric mode rotators (OI-AAMR) using chirally-coupled-core fiber. The proposed mode rotator can convert azimuthally asymmetric mode to the orthogonal degenerate mode without the requirement for angle alignment. An LP11 mode rotator with a rotation efficiency and an extinction ratio as high as 97% and 17 dB over C-band respectively, has been successfully demonstrated for any incident lobe orientation for the first time to the best of our knowledge. Owing to its circular center core structure, low insertion loss and crosstalk at the connection with few-mode fibers (FMF) can be expected...
March 5, 2018: Optics Express
Kazuki Kimura, Masafumi Mizooka, Kiyoshi Migita, Ryoko Ishida, Masatoshi Matsumoto, Satoshi Yamasaki, Nobusuke Kishikawa, Akihiro Kawahara, Yuka Kikuchi, Yuichiro Otani, Tomoki Kobayashi, Daisuke Miyamori, Takuya Ikuta, Hiroshi Nakamura, Kenichi Yokobayashi, Shuichi Iwamoto, Keishi Kanno, Hiromasa Ohira, Susumu Tazuma
Familial Mediterranean fever (FMF) is the most common genetic autoinflammatory disease, but it has been considered a rare disease in Japan. We herein describe five patients with FMF who were diagnosed both clinically and genetically at a single Japanese institute. A genetic investigation of MEFV detected heterozygosity for the compound mutations L110P/E148Q (n=2) and L110P/148Q/P369S/R406Q (n=1), and heterozygosity for M694I (n=1) and S503C (n=1). Colchicine prevented febrile attacks and accompanying symptoms in four patients...
March 9, 2018: Internal Medicine
S Georgin-Lavialle, V Hentgen, K Stankovic Stojanovic, C Bachmeyer, F Rodrigues, L Savey, S Abbara, P-L Conan, T Fraisse, M Delplanque, A Rouet, N Sbeih, I Koné-Paut, G Grateau
Familial Mediterranean Fever (FMF) is the most frequent monogenic auto-inflammatory disease. FMF is an autosomal recessive disease, which affects populations from Mediterranean origin and is associated with MEFV gene mutations encoding for the protein pyrin. Pyrin activation enhances the secretion of interleukin 1 by myelo-monocytic cells. Main features of the disease are acute attacks of serositis mainly located on the abdomen, less frequently on chest and joints, accompanied by fever and biological inflammatory markers elevation...
March 8, 2018: La Revue de Médecine Interne
Özlem Fentoğlu, Gözde Dinç, Atalay Doğru, Nermin Karahan, İlter İlhan, F Yeşim Kırzıoğlu, Mehmet Fatih Şentürk, Hikmet Orhan
BACKGROUND: There are no published studies regarding the role of the plasminogen (PLG) system in familial Mediterranean fever (FMF), FMF-associated secondary amyloidosis, or chronic periodontitis (CP), although recent limited data have focused on the association between FMF and chronic periodontitis. Therefore, the aim of this study was to evaluate the serum, salivary, and gingival tissue levels of PLG in patients with CP, FMF, and amyloidosis. METHODS: The study population included 122 patients with FMF (only FMF, and FMF and amyloidosis and 128 individuals who were systemically healthy controls...
February 21, 2018: Journal of Periodontology
Kensuke Asakura, Shunichi Yanai, Shotaro Nakamura, Keisuke Kawaski, Makoto Eizuka, Kazuyuki Ishida, Masaki Endo, Tamotsu Sugai, Kiyoshi Migita, Takayuki Matsumoto
RATIONALE: Familial Mediterranean fever (FMF) is the most common form of autoinflammatory disease. We report a rare case of FMF with gastrointestinal lesions mimicking Crohn disease. PATIENT CONCERNS: A 21-year-old Japanese man was referred to our institution, complaining of refractory diarrhea and weight loss of 14 kg during the past two years. He had presented with recurrent fever, abdominal pain, anal fistula and stomatitis. His father and one of his brothers had ulcerative colitis...
January 2018: Medicine (Baltimore)
Fatma Yazılıtaş, Özlem Aydoğ, Sare Gülfem Özlü, Evrim Kargın Çakıcı, Tülin Güngör, Fehime Kara Eroğlu, Gökçe Gür, Mehmet Bülbül
Familial Mediterranean fever (FMF), the most common hereditary autoinflammatory disorder is characterized by recurrent episodes of fever, serositis, arthritis. The major long-term result is amyloidosis. Colchicine remains the principle of the treatment; it not only prevents the acute attacks but also prevents the long-term complications such as amyloidosis; 5-10% of the patients are unresponsive to treatment. Recently new therapeutic options as anti-interleukin 1 agents are successfully used for the patients who do not respond to colchicine treatment...
February 15, 2018: Rheumatology International
Tolga Yildirim, Rahmi Yilmaz, Muge Uzerk Kibar, Yunus Erdem
Colchicine is the first-line treatment for familial Mediterranean fever (FMF), preventing both inflammatory attacks as well as the development of amyloidosis in the majority of the patients. However approximately 5-10% of patients are colchicine resistant/intolerant. Side effects of colchicine are more prominent in renal transplant recipients due to interaction with immunosuppressive drugs. Anti-interleukin (IL)-1 drugs (anakinra, canakinumab and rilonacept) have emerged as the most promising drugs in the treatment of colchicine-resistant and/or intolerant FMF...
February 14, 2018: Journal of Nephrology
Hacer Orsdemir Hortu, Emin Karaca, Betul Sozeri, Nesrin Gulez, Balahan Makay, Cumhur Gunduz, Tahir Atik, Ismihan Merve Tekin, Sevket Erbil Unsal, Cogulu Ozgur
Familial Mediterranean fever (FMF) is an inherited autoinflammatory disorder that can result in attacks with accompanying recurrent episodes of fever, serositis, and skin rash. MiRNAs are demonstrated to be associated with a number of other diseases; however, no comprehensive study has revealed its association with FMF disease. The aim is to investigate the role of microRNAs in FMF. We included 51 patients with genetically diagnosed FMF who had clinical symptoms and 49 healthy volunteers. Fifteen miRNAs that were found to be associated with autoinflammatory diseases and have a part in immune response were evaluated...
February 13, 2018: Clinical Rheumatology
Burak Tanyıldız, Mehmet Engin Tezcan, Baran Kandemir, Nesrin Tutaş Günaydın, Eren Göktaş, Aise Tangılntız, Aysu Karatay Arsan
BACKGROUND: The purpose of this study is to investigate whether oral colchicine has an effect on peripapillary retinal nerve fiber layer (pRNFL) thickness of familial Mediterranean fever (FMF) patients. METHODS: We conducted a cross sectional study by comparing pRNFL thickness of FMF patients on colchicine (treated group), newly diagnosed colchicine naïve FMF patients (untreated group) and healthy controls. The study included 66 FMF patients and 32 healthy control subjects...
February 5, 2018: BMC Ophthalmology
Yaping Liu, Zhiqun Yang, Jian Zhao, Lin Zhang, Zhihong Li, Guifang Li
We summarize theoretical formulae for determining the intrinsic loss of few-mode fibers and compare intrinsic losses among step-index and parabolic graded-index few-mode fibers (FMF) with different dopants, and with single-mode fibers (SMF). The low-loss characteristics of pure-silica-core FMFs, in comparison with SMFs, give rise to additional motivations for pursuing mode-division multiplexed transmission over parallel-SMF transmission, and quasi-single-mode (QSM) transmission over ultra-large-area SMF transmission...
January 22, 2018: Optics Express
Pengcheng Chen, Xuewen Shu, Kate Sugden
A novel low-cost, compact, assembly-free and sensitive optical fiber curvature sensor is presented. This device consists of an off-axis positive refractive index modified zone (PRIMZ), induced by a direct femtosecond laser, written in a single-mode fiber (SMF) core. The PRIMZ transforms the original SMF section into a few-mode fiber (FMF). As a result, the whole fiber forms an assembly-free "SMF-FMF-SMF" sandwich Mach-Zehnder interferometer. When the device is bent, a direction-dependent spectral shift of the interference pattern is produced...
February 1, 2018: Optics Letters
Amr M Mahros, Islam Ashry
We theoretically design and analyze the performance of a fiber-based linearly polarized (LP) mode demultiplexer using a Fabry-Perot interferometer. The all-fiber geometry of the reported demultiplexer is obtained by writing fiber Bragg gratings (FBGs) in a few-mode fiber (FMF), such that the FBGs act as partial reflecting mirrors for the LP modes. We also demonstrate the ability to decompose a received optical signal into its individual LP components in wavelength-division multiplexing networks.
February 1, 2018: Applied Optics
S Gangemi, S Manti, V Procopio, M Casciaro, E Di Salvo, M Cutrupi, G Ganci, C Salpietro, R Chimenz, C Cuppari
Familial Mediterranean fever (FMF) is the most common autosomal recessive autoinflammatory disease. To date, following the isolation of more than 280 MEFV sequence variants, the genotype-phenotype correlation in FMF patients has been intensively investigated; however, an univocal and clear consensus has not been yet reached. Thus, the aim of this systematic review was to analyze the available literature findings in order to provide to scientific community an indirect estimation of the impact of genetic factors on the phenotypic variability of FMF...
February 2, 2018: Clinical Genetics
Orr Levy, Isak Nethanël Gath
We investigate the spread from ancestors to descendants, under a model of sexual reproduction, of hereditary elements distinguishing individuals from their fellow human beings. These hereditary elements, termed labels, are either symbolic, implying a socio-cultural or ethnic self-determined category, or biological, i.e. a DNA sequence (for example founder mutations). The impact of various modes of preferential (assortative) mating on the dissemination of a known ancestral label was studied for both kinds of labels, the symbolic and the biological...
January 27, 2018: Journal of Theoretical Biology
Anca-Daniela Stanescu, Roxana Banica, Romina-Marina Sima, Liana Ples
The purpose of this study was to investigate when in pregnancy to stop the administration of low dose aspirin (150 mg/daily) so as to prevent fetal growth restriction (FGR) A randomised, placebo-controlled study was designed. The patients were all screened positive using the Fetal Medicine Foundation (FMF) early pregnancy screening test for preeclampsia (PE) and FGR prediction. One hundred and fifty patients were enrolled and divided equally into three groups: A - the controls who received placebo treatment; B - those who received aspirin till 32 weeks of gestation and C - those who received aspirin till 36 weeks of gestation...
January 30, 2018: Journal of Perinatal Medicine
Liona C Poon, Daniel L Rolnik, Min Yi Tan, Juan Luis Delgado, Theodora Tsokaki, Ranjit Akolekar, Mandeep Singh, Walkyria Andrade, Tunay Efeturk, Jacques C Jani, Walter Plasencia, George Papaioannou, Ana Rosa Blazquez, Ilma Floriana Carbone, David Wright, Kypros H Nicolaides
OBJECTIVE: To report the incidence of preterm preeclampsia (PE) in women that fulfil the screening criteria of the National Institute for Health and Clinical Excellence (NICE) and American Congress of Obstetricians and Gynecologists (ACOG) and compare the incidence in those that are screen positive and screen negative by the Fetal Medicine Foundation (FMF) algorithm. STUDY DESIGN: This was a secondary analysis of data from the ASPRE study. The study population consisted of women with singleton pregnancies who had prospective screening for preterm-PE by means of the FMF algorithm that combines maternal factors and biomarkers at 11-13 weeks' gestation...
January 30, 2018: Ultrasound in Obstetrics & Gynecology
C Wang, C Lin, W Su, Y Zhang, F Wang, Y Wang, C Shi, Z Lu
In the present study, 2 experiments were performed to study the effects of feeding fermented corn and soybean meal mixed feed (FMF) with Bacillus subtilis and Enterococcus faecium to lactating sows on the performance of the sows and their progeny. In Exp. 1, 60 sows were allocated to the following 3 dietary treatments: 1) sows fed a corn and soybean meal basal diet (control) from d 3 before parturition to weaning, 2) sows fed a diet with 7.5% FMF, and 3) sows fed a diet with 15% FMF. Results indicated that feeding 15% FMF significantly improved (P < 005) the sows' ADFI, the individual piglet weaning weights, and piglet weight gain and reduced (P < 005) the backfat loss of sows compared with the control group...
January 25, 2018: Journal of Animal Science
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