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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
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
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
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: Eur J Rheumatol
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
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
Hakan Güneş, Tarık Kıvrak, Mustafa Tatlısu, Hakkı Kaya, Mehmet Birhan Yılmaz
OBJECTIVE: The aim of our study is to investigate the relationship between microalbuminuria and flow-mediated dilatation in familial Mediterranean fever (FMF) patients. MATERIAL AND METHODS: In our study, there were two groups consisting of 54 patients who were out of the attack period (43 of whom had no microalbuminuria and 11 of whom had microalbuminuria) and 40 healthy controls (M/F: 12/28). RESULTS: There was no statistically difference between patient and control groups'age (25...
June 2016: Eur J Rheumatol
E Merz, C Thode, B Eiben, S Wellek
AIM: Both previous versions of the German PRC algorithm developed by our group for routine first-trimester screening relied on the assumption that maternal blood sampling and fetal ultrasonography are performed at the same visit of a pregnant women. In this paper we present an extension of our method allowing also for constellations where this synchronization is abandoned through preponing blood sampling to dates before 11 weeks of gestation. METHODS: In contrast to the directly measured concentrations of the serum parameters PAPP-A and free ß-hCG, the logarithmically transformed values could be shown to admit the construction of reference bands covering the whole range from 16 to 84 mm CRL [corresponding to 63 to 98 days of gestation]...
March 2016: Ultrasound Int Open
Nele Schnipper, Hans H Stassen, Tilmann Kallinich, Karl Sperling, Katrin Hoffmann
The nuclear morphology of neutrophils depends on different endogenous and exogenous factors, which can lead to hypo- or hypersegmentation of the normally 2-4 segmented nucleus. Hyposegmentation can be due to mutations in the LBR-gene (Pelger-Huët-Anomaly) or can be induced, for example, by colchicine treatment. The range of this phenotypic variation is known as "norm of reaction", which can be of major relevance for clinical diagnosis and therapeutic intervention. In this project, we studied the "norm of reaction" in 26 subjects with 0-3 wild type LBR alleles...
September 29, 2016: Cytometry. Part B, Clinical Cytometry
Mette Christiansen, Sandra Ammann, Carsten Speckmann, Trine Hyrup Mogensen
A 16-year-old boy of Caucasian ethnicity was evaluated for recurrent febrile episodes occurring during most of his life without establishment of any microbial aetiology. During febrile episodes he developed extensive splenomegaly, lymphadenopathy, anaemia, severe abdominal pain and general malaise. Lymph node biopsies demonstrated inflammation and sinus histiocytosis but no malignancy or granuloma. The patient underwent seroconversion for Epstein-Barr virus (EBV) infection during the hospitalisation. Genetic testing identified a hemizygous frameshift mutation in the X linked inhibitor of apoptosis (XIAP)-gene as well as variants in the MEFV gene indicating Familial Mediterranean Fever (FMF)...
2016: BMJ Case Reports
Hafize Emine Sönmez, Ezgi Deniz Batu, Yelda Bilginer, Seza Özen
Familial Mediterranean fever (FMF) is inherited autosomal recessively; however, heterozygotes may express FMF phenotype. We aimed to define the characteristics of FMF patients heterozygous for MEFV (MEditerranean FeVer) mutations in whom colchicine was stopped after a period of treatment, with close follow-up. We reviewed the charts of 182 children who were heterozygous for MEFV variants. We excluded the patients (n = 34) heterozygous for MEFV variants of unknown significance and patients with typical periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (n = 2)...
September 27, 2016: Clinical Rheumatology
Zhongying Wu, Juhao Li, Dawei Ge, Fang Ren, Paikun Zhu, Qi Mo, Zhengbin Li, Zhangyuan Chen, Yongqi He
Mode division multiplexing (MDM) has been widely investigated in optical transmission systems and networks to improve network capacity. However, the MDM receiver is always expensive and complex because coherent detection and multiplex-input-and-multiplex-output (MIMO) digital signal processing (DSP) are required to demultiplex each spatial mode. In this paper, we investigate the application of MDM in short-reach scenarios such as datacenter networking. Two-dimensional MDM and wavelength division multiplexing node structure based on low modal-crosstalk few-mode fiber (FMF) and components is proposed, in which signal in each mode or wavelength can be independently switched...
September 19, 2016: Optics Express
Nikolaos K Gatselis, Panagiotis Skendros, Konstantinos Ritis, George N Dalekos
Familial Mediterranean fever (FMF) is characterised by recurrent, self-limited fever attacks and serositis. Severe liver involvement has rarely been reported. We present two FMF cases of a 55-year-old man and a 20-year-old woman in whom the prevailing manifestations were recurrent unexplained episodes of anicteric hepatitis (man) and recurrent severe jaundice (woman). A long-term history of recurrent self-limited episodes of fever was also claimed in both. After exclusion of infectious, malignant, autoimmune, and liver and biliary diseases, a diagnosis of FMF as confirmed by molecular analysis was established...
2016: BMJ Case Reports
Maryam Beheshtian, Nasim Izadi, Gernot Kriegshauser, Kimia Kahrizi, Elham Parsi Mehr, Maryam Rostami, Masoumeh Hosseini, Maryam Azad, Mona Montajabiniat, Ariana Kariminejad, Stefan Nemeth, Christian Oberkanins, Hossein Najmabadi
Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder caused by mutations in the MEFV gene. The disease is especially common among Armenian, Turkish, Jewish and Middle East Arab populations. To identify the frequency and the spectrum of common MEFV mutations in different Iranian populations, we investigated a cohort of 208 unselected asymptomatic individuals and 743 FMF patients. Nine hundred and fifty-one samples were analysed for the presence of 12 MEFV mutations by PCR and reverse-hybridization (FMF StripAssay, ViennaLab, Vienna, Austria)...
September 2016: Journal of Genetics
H Latsoudis, M-F Mashreghi, J R Grün, H-D Chang, B Stuhlmueller, A Repa, I Gergiannaki, E Kabouraki, T Haeupl, A Radbruch, P Sidiropoulos, D Kardassis, D T Boumpas, G N Goulielmos
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent, acute and self-limiting attacks of fever. Mutations in MEFV gene encoding pyrin account for FMF but the high number of heterozygote patients with typical symptoms of the disease has driven a number of alternative aetiopathogenic hypotheses. The MEFV gene was knocked down in human myelomonocytic cells that express endogenous pyrin to identify deregulated microRNAs (miRNAs). Microarray analyses revealed 29 significantly differentially expressed miRNAs implicated in pathways associated with cellular integrity and survival...
September 16, 2016: Journal of Cellular Physiology
A Kirbas, K Daglar, O Kirbas, C Koseoglu, O Kara, E Biberoglu, O Kurmus, C Yayla, N Danisman
OBJECTIVE: Familial mediterranean fever (FMF) is the most common auto-inflammatory disease that is characterized by recurrent, self-limited attacks of fever and serous membrane inflammation. Patients with inflammatory rheumatic diseases are considered to have a raised cardiovascular diseases risk. The aim of this study was to investigate; by means of P wave dispersion (Pd) and QT dispersion (QTd) parameters detected by simple standard electrocardiogram (ECG), atrial and ventricular repolarization changes in pregnant women with and without FMF...
August 2016: European Review for Medical and Pharmacological Sciences
Ruby Haviv, Philip J Hashkes
INTRODUCTION: Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. The treatment of choice is colchicine. However, ~40% of patients are only partial responders and 5-10% are non-responders. Advances in the understanding of the role of pyrin in the regulation of interleukin (IL)-1β activation has led to use of anti-IL-1 agents for colchicine-resistant FMF. AREAS COVERED: The authors performed a literature search of anti-IL-1 treatment for FMF, particularly canakinumab, a humanized IL-1β antibody, by searching PubMed/Medline/Scopus since 2001 and proceedings of major rheumatologic conferences since 2011 for unpublished studies...
November 2016: Expert Opinion on Biological Therapy
Betul Sozeri, Nesrin Gulez, Malik Ergin, Erkin Serdaroglu
INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. Patients may develop renal amyloidosis. Colchicine prevents attacks and renal amyloidosis. Five to 10 % of the patients with FMF are resistant or intolerant to colchicine. CASE DESCRIPTION: Herein, we reported our experience with clinical-laboratory features and treatment responses of a pediatric FMF patient with amyloidosis treated with canakinumab...
December 2016: Molecular and Cellular Pediatrics
Yi Weng, Ezra Ip, Zhongqi Pan, Ting Wang
The concepts of spatial-division multiplexing (SDM) technology were first proposed in the telecommunications industry as an indispensable solution to reduce the cost-per-bit of optical fiber transmission. Recently, such spatial channels and modes have been applied in optical sensing applications where the returned echo is analyzed for the collection of essential environmental information. The key advantages of implementing SDM techniques in optical measurement systems include the multi-parameter discriminative capability and accuracy improvement...
2016: Sensors
Per Wekell, Anna Karlsson, Anders Fasth, Stefan Berg
Familial Mediterranean fever - an important disease in a globalised world Familial Mediterranean fever (FMF) is characterized by recurrent febrile attacks during 1/2-3 days associated with peritonitis, pleuritis and arthritis. FMF is the most common monogenic autoinflammatory disease in the world, with over 100 000 affected individuals. It is particularly common in individuals with an origin in the eastern Mediterranean Basin, where the disease has a prevalence of 100-200 per 100 000. The gene for FMF (MEFV) was identified in 1997 with an autosomal recessive inheritance; however, a significant proportion (≈25%) of clinical patients lack two mutations...
2016: Läkartidningen
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