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  1. [Fabry disease].

    Science.gov (United States)

    Boggio, Paula; Luna, Paula Carolina; Abad, María Eugenia; Larralde, Margarita

    2009-01-01

    Fabry disease is an uncommon, X-linked lysosomal storage disorder, caused by partial or complete deficiency of the enzyme a-galactosidase A. The defect leads to accumulation of uncleaved globotriaosylceramide on the vascular endothelium and visceral tissues, being the skin, heart, kidneys and central nervous system the most affected organs. We performed review of the literature related to the disease and emphasized that early recognition of angiokeratomas and hypohidrosis are key diagnostic signs of this serious disease. We also addressed the need of multidisciplinary assessment of these patients.

  2. Neurological manifestations in Fabry disease

    Institute of Scientific and Technical Information of China (English)

    Joseph Bruno Bidin Brooks; Yara Dadalti Fragoso

    2016-01-01

    Fabry disease (FD) is a rare, progressive, multisystem and highly debilitating disease. FD is an X-linked lysosome storage disorder that results in α-galactosidase A deifciency. The subsequent accumulation of glycosphingolipids is more evident in vascular endothelium and smooth-muscle cells. The resulting effect of the deposition is generalized inlfammation and vasculopathy, which can also affect the central and peripheral nervous system. FD progresses with kidney dysfunction, angiokeratoma of the skin, cardiomyopathy, cerebrovascular events and neurological disorders. In the present review, the neurological manifestations of FD are summarized with emphasis on cerebral vasculopathy, cochlear nerve dysfunction, psychiatric and cognitive symptoms, autonomic dysfunction and peripheral neuropathy. Enzyme replacement therapy is also discussed in the light of its more prominent effects when administered early in life, which make it essential to diagnose FD as soon as possible.

  3. Females with Fabry disease frequently have major organ involvement: lessons from the Fabry Registry

    DEFF Research Database (Denmark)

    Wilcox, W.R.; Oliveira, J.P.; Hopkin, R.J.;

    2008-01-01

    Fabry disease (FD) is an X-linked lysosomal storage disease caused by alpha-galactosidase A deficiency. The Fabry Registry is a global clinical effort to collect longitudinal data on FD. In the past, most "carrier" females were usually thought to be clinically unaffected. A systematic effort has ...

  4. Fabry Disease

    Science.gov (United States)

    ... Foundation National Organization for Rare Disorders (NORD) National Tay-Sachs and Allied Diseases Association See all related ... Foundation National Organization for Rare Disorders (NORD) National Tay-Sachs and Allied Diseases Association See all related ...

  5. Vertebrobasilar Dolichoectasia in Fabry Disease

    Directory of Open Access Journals (Sweden)

    Juan Politei

    2014-06-01

    Full Text Available Introduction: Fabry disease (FD is a lysosomal storage disorder associated with marked cerebrovascular involvement. Conventional magnetic resonance imaging (MRI shows different abnormalities, like white matter lesions that may already be present at an early stage in the disease. Aim: To present observations from a series of brain MRIs performed among a cohort of patients with FD and the relationship of imaging abnormalities with the presence of cardiovascular risk factors (CVRFs. Methods: A total of 70 patients with FD (43 women were enrolled. The cardiac, renal, ophthalmic, and peripheral nerve functioning was assessed. The MRI evaluation included assessment for evidence of ischemia, microbleeds, pulvinar sign, Arnold-Chiari type 1 malformation, and vertebrobasilar dolichoectasia (VBD. The presence or absence of CVRFs was examined for all patients. Results: Renal involvement was found in 60%, cardiac compromise in 30%, cornea verticillata in 91.4%, and acroparesthesias in 87.1% of patients. Brain MRI analysis found evidence of cerebral ischemic injury in 25.9% of men and 30.2% of women. Vertebrobasilar dolichoectasia was observed in imaging from 55.5% of men and 34.8% of women. The logistic regression analysis adjusted for cardiovascular risks factors, using ischemia or VBD as a dependent variable, showed no statistically significant results. Discussion: Our results have demonstrated cerebrovascular involvement before the third decade in many patients with FD. This study is further evidence confirming that women are not just carriers of FD and should be followed clinically and evaluated comprehensively to monitor for disease burden and progression. Although silent brain ischemias in MRI should be included as a key feature for the diagnoses of FD, VBD is an earlier and frequent sign.

  6. Fabry Disease in Families With Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Adalsteinsdottir, Berglind; Palsson, Runolfur; Desnick, Robert J

    2017-01-01

    BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B...

  7. Dermatologic Aspects of Fabry Disease

    Directory of Open Access Journals (Sweden)

    Paula C. Luna MD

    2016-09-01

    Full Text Available Isolated angiokeratomas (AKs are common cutaneous lesions, generally deemed unworthy of further investigation. In contrast, diffuse AKs should alert the physician to a possible diagnosis of Fabry disease (FD. Angiokeratomas often do not appear until adolescence or young adulthood. The number of lesions and the extension over the body increase progressively with time, so that generalization and mucosal involvement are frequent. Although rare, FD remains an important diagnosis to consider in patients with AKs, with or without familial history. Dermatologists must have a high index of suspicion, especially when skin features are associated with other earlier symptoms such as acroparesthesia, hypohidrosis, or heat intolerance. Once the diagnosis is established, prompt screening of family members should be performed. In all cases, a multidisciplinary team is necessary for the long-term follow-up and treatment.

  8. Genetics Home Reference: Fabry disease

    Science.gov (United States)

    ... AJ, Germain DP, Goldman M, Grabowski G, Packman S, Wilcox WR. Fabry disease, an under-recognized multisystemic disorder: ... Sims K, Brodie SE, Pastores GM, Strotmann JM, Wilcox WR. Fabry disease: guidelines for the evaluation and ...

  9. Fabry disease in children

    DEFF Research Database (Denmark)

    Borgwardt, Line Gutte; Feldt-Rasmussen, U; Rasmussen, AK

    2013-01-01

    Fabry disease is a rare, multiorgan disease. The most serious complications involve the kidney, brain and heart. This study aims to assess the effect of enzyme replacement therapy (ERT) using agalsidase-beta in children with Fabry disease. We carried out a nationwide, descriptive and observational...... retrospective cohort study of 10 children (9-16 years at baseline), who underwent regular systematic investigations for 1-8 years after initiation of ERT with agalsidase-beta (Fabryzyme®, Genzyme). Ophthalmological, echocardiographic abnormalities and hypohidrosis were found at baseline and during the follow......-up period. Serious kidney, heart or brain involvement had not developed at the last follow-up examination. For the majority of the patients improvements were found concerning headache, acroparaesthesias and gastrointestinal pain during the follow-up period. The level of energy and physical activity also...

  10. Fabry's disease and psychosis: causality or coincidence?

    Science.gov (United States)

    Gairing, S; Wiest, R; Metzler, S; Theodoridou, A; Hoff, P

    2011-01-01

    A 21-year-old female with Fabry's disease (FD) presented acute psychotic symptoms such as delusions, auditory hallucinations and formal thought disorders. Since the age of 14, she had suffered from various psychiatric symptoms increasing in frequency and intensity. We considered the differential diagnoses of prodromal symptoms of schizophrenia and organic schizophrenia-like disorder. Routine examinations including cognitive testing, electroencephalography and structural magnetic resonance imaging revealed no pathological findings. Additional structural and functional imaging demonstrated a minor CNS involvement of FD, yet without functional limitations. In summary our examination results support the thesis that in the case of our patient a mere coincidence of FD and psychotic symptoms is more likely than a causal connection.

  11. Screening, diagnosis, and management of patients with Fabry disease

    DEFF Research Database (Denmark)

    Schiffmann, Raphael; Hughes, Derralynn A; Linthorst, Gabor E

    2017-01-01

    Patients with Fabry disease (FD) are at a high risk for developing chronic kidney disease and cardiovascular disease. The availability of specific but costly therapy has elevated the profile of this rare condition. This KDIGO conference addressed controversial areas in the diagnosis, screening, a...

  12. Upregulation of inward rectifying currents and Fabry disease neuropathy.

    Science.gov (United States)

    Geevasinga, Nimeshan; Tchan, Michel; Sillence, David; Vucic, Steve

    2012-12-01

    Peripheral neuropathy and neuropathic pain are common clinical manifestations of Fabry disease (FD). Although the mechanisms underlying the development of sensory neuropathy remain to be fully elucidated, a chronic ischemic process was proposed. Consequently, this study utilized axonal excitability techniques to gain further insights into the pathophysiological mechanisms underlying the development of FD neuropathy. Median motor and sensory axonal excitability studies were undertaken in 13 FD patients and results were compared to 19 healthy subjects. A "fanning-in" of threshold electrotonus, suggestive of membrane depolarization, was evident only in motor axons in FD patients. In contrast, the sensory axons exhibited a lower threshold in FD (p < 0.05) and a significantly increased hyperpolarizing current/threshold (I/V) gradient (FD 0.48 ± 0.03; controls, 0.31 ± 0.02, p < 0.001), which correlated with clinical scores of disease severity (Rho = 0.65, p < 0.05), neuropathy (Rho = 0.54, p < 0.05) and neuropathic pain (Rho = 0.56, p < 0.05). These findings indicate that upregulation of I(h) , rather than ischemia, may underlie the sensory symptoms and possibly development of neuropathy in FD. Modulation of sensory I(h) may prove therapeutically useful in Fabry disease.

  13. Cardiac device implantation in Fabry disease

    Science.gov (United States)

    Sené, Thomas; Lidove, Olivier; Sebbah, Joel; Darondel, Jean-Marc; Picard, Hervé; Aaron, Laurent; Fain, Olivier; Zenone, Thierry; Joly, Dominique; Charron, Philippe; Ziza, Jean-Marc

    2016-01-01

    Abstract The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD. Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy. Six patients (4M/2F) required a pacemaker (PM) for sinus node dysfunction (n = 4) or atrioventricular disease (n = 2). One female patient required an internal cardioverter-defibrillator (ICD) to prevent sudden cardiac death because of nonsustained ventricular tachycardia (nSVT). One female patient required PM-ICD for sinus node dysfunction and nSVT. One patient underwent CD implantation before the diagnosis of FD. The annual rate of CD implantation was estimated at 1.90 per 100 person years. On univariate analysis at the end of the follow-up period, the factors associated with ACAs requiring CD implantation were as follows: delayed diagnosis of FD, delayed initiation of enzyme replacement therapy, age at the last follow-up visit, and severe multiorgan phenotype (hypertrophic cardiomyopathy, chronic kidney disease, and/or sensorineural hearing loss). On multivariate analysis, age at diagnosis of FD and age at the last follow-up visit were independently associated with an increased risk of ACAs requiring CD (P sudden death in patients with FD, regular monitoring is mandatory, especially in patients with a late diagnosis of FD and/or with a severe phenotype. Regular Holter ECGs, therapeutic education of patients, and deliverance of an emergency card including a phenotype summary are crucial in the care of FD patients. Available guidelines for device therapy and the

  14. Neurological manifestations in Fabry's disease

    DEFF Research Database (Denmark)

    Møller, Anette Torvin; Jensen, Troels Staehelin

    2007-01-01

    . Neurological symptoms, such as burning sensations (occasionally accompanied by acroparesthesia) and stroke, are among the first to appear, and occur in both male and female patients. A delay in establishing the diagnosis of Fabry's disease can cause unnecessary problems, especially now that enzyme replacement...... treatment is available to prevent irreversible organ damage. Females with Fabry's disease who present with pain have often been ignored and misdiagnosed because of the disorder's X-linked inheritance. This Review will stress the importance of recognizing neurological symptoms for the diagnosis of Fabry...

  15. Fabry disease simulating Crohn's ileitis.

    Science.gov (United States)

    Rubio, Carlos A; Villnow, Elizabeth; Sundelin, Birgitta; Eriksson, Elina; Dolapcsiev, Karoli; Björk, Jan; Befrits, Ragnar; Tengvar, Magnus; Iversen, Henrik

    2014-05-01

    Fabry disease is an inherited (X-linked) lysosomal storage disorder caused by deficiency of α-galactosidase A, leading to accumulation of globotriaosylceramide in various tissues. A 57-year-old male with a family history and laboratory findings of Fabry disease, was consulted for severe abdominal pain, undulating pyrexia, weight loss and diarrhea. The tentative clinical diagnosis of Crohn's ileitis was supported at computed tomographic examination, at laparotomy and at inspection of the resected ileal segment. Histology revealed chronic and acute inflammation, thick-walled occluded vessels, fibrosis and characteristic bi-refringent lamellar deposits of globotriaosylceramide and calcifications. Multi-nucleated giant cells contained phagocytized bi-refringent material. Transmission electron microscopy showed cells with irregular cytoplasmic bodies displaying distinctive zebra-like lamellar structures. It is submitted that the gastrointestinal phenotype of Fabry disease may concur with symptoms resembling abdominal Crohn's disease.

  16. Neurological manifestations in Fabry's disease

    DEFF Research Database (Denmark)

    Møller, Anette Torvin; Jensen, Troels Staehelin

    2007-01-01

    Fabry's disease is an X-linked lysosomal storage disorder caused by a defect in the gene that encodes the lysosomal enzyme alpha-galactosidase A. Symptoms arise because of accumulation of globotriaosylceramide in multiple organs, resulting in severely reduced quality of life and premature death....... Neurological symptoms, such as burning sensations (occasionally accompanied by acroparesthesia) and stroke, are among the first to appear, and occur in both male and female patients. A delay in establishing the diagnosis of Fabry's disease can cause unnecessary problems, especially now that enzyme replacement...

  17. Fabry disease and early stroke

    DEFF Research Database (Denmark)

    Feldt-Rasmussen, U

    2011-01-01

    Fabry disease, an X-linked lysosomal storage disorder, results from deficient activity of the enzyme a-galactosidase A. Affected males with the classic phoenotype have acroparaesthesias, hypohidrosis, and corneal opacities in childhood and develop renal failure, cardiac hypertrophy or strokes...... in the third to fifth decade of life. Some female heterozygotes are asymptomatic, some as severely affected as males. The natural history of Fabry patients includes transitory cerebral ischaemia and strokes, even in very young persons of both genders. The mechanism is partly due to vascular endothelial...... accumulation of GL-3. White matter lesions on MRI occur. Both males and females can be safely treated with enzyme replacement; and thus screening for Fabry disease of young stroke populations should be considered. There are, however, no hard data of treatment effect on mortality and morbidity. The analyses...

  18. Fabry disease and early stroke

    DEFF Research Database (Denmark)

    Feldt-Rasmussen, U

    2011-01-01

    Fabry disease, an X-linked lysosomal storage disorder, results from deficient activity of the enzyme α-galactosidase A. Affected males with the classic phoenotype have acroparaesthesias, hypohidrosis, and corneal opacities in childhood and develop renal failure, cardiac hypertrophy or strokes...... in the third to fifth decade of life. Some female heterozygotes are asymptomatic, some as severely affected as males. The natural history of Fabry patients includes transitory cerebral ischaemia and strokes, even in very young persons of both genders. The mechanism is partly due to vascular endothelial...... accumulation of GL-3. White matter lesions on MRI occur. Both males and females can be safely treated with enzyme replacement; and thus screening for Fabry disease of young stroke populations should be considered. There are, however, no hard data of treatment effect on mortality and morbidity. The analyses...

  19. Fabry disease in Spain. Description of Spanish patients and a comparison with other European countries using data from the Fabry Outcome Survey (FOS)

    OpenAIRE

    Barba Romero, Miguel Angel; Rivera Gallego, Alberto; Pintos Morell, Guillem

    2011-01-01

    Abstract Aims: Fabry disease (FD) is an X chromosome-linked transmitted lysosomal storage disorder due to the deficient activity of enzyme ?-galactosidase A. This leads to accumulation of neutral glycosphingolipids associated with organ involvement and premature death. We report the clinical characteristics of Spanish patients enrolled on the Fabry Outcome Survey (FOS; an international multicentre registry for the disease) and also compare these data with those from the rest of Eur...

  20. Cochleovestibular Manifestations in Fabry Disease

    Directory of Open Access Journals (Sweden)

    Alberto Ciceran MD

    2016-09-01

    Full Text Available Fabry disease is a rare, X-linked lysosomal storage disorder resulting from deficient α-galactosidase A activity and globotriaosylceramide accumulation throughout the body. This accumulation leads to various clinical disorders, including inner ear lesions, with sensorineural hearing loss and dizziness. Although hearing loss is recognized in these patients, its incidence and natural history have not been characterized. Hearing disorders develop mainly in adulthood, and tinnitus may be an earlier symptom in Fabry disease. A significant incidence of mid- and high-frequency sensorineural hearing loss in affected males is commonly reported, whereas in female carriers, it is much less frequent. In addition, a high incidence of vestibular disorders with dizziness and chronic instability is also observed in these patients. The few studies about the effects of enzyme replacement therapy (ERT on cochleovestibular symptoms show controversial results. Based on the model of densely stained material accumulation in the inner ear, stria vascularis cell, and organ damage, an early indication of ERT may prevent hearing loss due to the reduction in substrate accumulation.

  1. Cochleovestibular Manifestations in Fabry Disease

    Directory of Open Access Journals (Sweden)

    Alberto Ciceran MD

    2016-09-01

    Full Text Available Fabry disease is a rare, X-linked lysosomal storage disorder resulting from deficient α-galactosidase A activity and globotriaosylceramide accumulation throughout the body. This accumulation leads to various clinical disorders, including inner ear lesions, with sensorineural hearing loss and dizziness. Although hearing loss is recognized in these patients, its incidence and natural history have not been characterized. Hearing disorders develop mainly in adulthood, and tinnitus may be an earlier symptom in Fabry disease. A significant incidence of mid- and high-frequency sensorineural hearing loss in affected males is commonly reported, whereas in female carriers, it is much less frequent. In addition, a high incidence of vestibular disorders with dizziness and chronic instability is also observed in these patients. The few studies about the effects of enzyme replacement therapy (ERT on cochleovestibular symptoms show controversial results. Based on the model of densely stained material accumulation in the inner ear, stria vascularis cell, and organ damage, an early indication of ERT may prevent hearing loss due to the reduction in substrate accumulation.

  2. Screening, diagnosis, and management of patients with Fabry disease: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

    Science.gov (United States)

    Schiffmann, Raphael; Hughes, Derralynn A; Linthorst, Gabor E; Ortiz, Alberto; Svarstad, Einar; Warnock, David G; West, Michael L; Wanner, Christoph

    2017-02-01

    Patients with Fabry disease (FD) are at a high risk for developing chronic kidney disease and cardiovascular disease. The availability of specific but costly therapy has elevated the profile of this rare condition. This KDIGO conference addressed controversial areas in the diagnosis, screening, and management of FD, and included enzyme replacement therapy and nonspecific standard-of-care therapy for the various manifestations of FD. Despite marked advances in patient care and improved overall outlook, there is a need to better understand the pathogenesis of this glycosphingolipidosis and to determine the appropriate age to initiate therapy in all types of patients. The need to develop more effective specific therapies was also emphasized.

  3. [Fabry's disease: an example of cardiorenal syndrome type 5].

    Science.gov (United States)

    Villa, Gianluca; Romagnoli, Stefano; Sharma, Aashish; Ronco, Claudio

    2017-03-01

    Fabry's disease (FD) is a severe congenital metabolic disorder characterized by the deficient activity of lysosomal exoglycohydrolase alpha-galactosidase, characterized by glycosphingolipid deposition in several cells, such as capillary endothelial cells, renal, cardiac, and nerve cells. As a systemic disease leading to a contemporaneous myocardial and renal dysfunction, FD might be an example of cardiorenal syndrome type 5 (CRS-5). Kidney damage is commonly characterized by proteinuria, isosthenuria and altered tubular function when occurs at the second-third decade, azotemia and end-stage renal disease in third-fifth decade. Beyond the irreversible glomerular, tubular and vascular damages, the podocytes foot process effacement is the major cause of kidney dysfunction. Myocardial damage is usually observed with right and left ventricular hypertrophy, arrhythmias (due to sinus node and conduction system impairment), diastolic dysfunction, congestive heart failure, myocardial ischemia, fibrosis and cardiac death. The enzymatic replacement therapy is essential for the management of FD, as well as the control of renal (with anti-proteinuric agents such as angiotensin-converting enzyme inhibitors- and/or angiotensin II receptor blockers), brain (coated aspirin, clopidogrel and statins to prevent strokes) and heart complications (calcium channel blockers for ischemic cardiomyopathy, warfarin and amiodarone or cardioverter device for arrhythmias). Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.

  4. Gaucher disease and Fabry disease: new markers and insights in pathophysiology for two distinct glycosphingolipidoses.

    Science.gov (United States)

    Ferraz, Maria J; Kallemeijn, Wouter W; Mirzaian, Mina; Herrera Moro, Daniela; Marques, Andre; Wisse, Patrick; Boot, Rolf G; Willems, Lianne I; Overkleeft, H S; Aerts, J M

    2014-05-01

    Gaucher disease (GD) and Fabry disease (FD) are two relatively common inherited glycosphingolipidoses caused by deficiencies in the lysosomal glycosidases glucocerebrosidase and alpha-galactosidase A, respectively. For both diseases enzyme supplementation is presently used as therapy. Cells and tissues of GD and FD patients are uniformly deficient in enzyme activity, but the two diseases markedly differ in cell types showing lysosomal accumulation of the glycosphingolipid substrates glucosylceramide and globotriaosylceramide, respectively. The clinical manifestation of Gaucher disease and Fabry disease is consequently entirely different and the response to enzyme therapy is only impressive in the case of GD patients. This review compares both glycosphingolipid storage disorders with respect to similarities and differences. Presented is an update on insights regarding pathophysiological mechanisms as well as recently available biochemical markers and diagnostic tools for both disorders. Special attention is paid to sphingoid bases of the primary storage lipids in both diseases. The value of elevated glucosylsphingosine in Gaucher disease and globotriaosylsphingosine in Fabry disease for diagnosis and monitoring of disease is discussed as well as the possible contribution of the sphingoid bases to (patho)physiology. This article is part of a Special Issue entitled New Frontiers in Sphingolipid Biology.

  5. Corpus callosum involvement: a useful clue for differentiating Fabry disease from multiple sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Cocozza, Sirio; Olivo, Gaia; Pontillo, Giuseppe; Ugga, Lorenzo; De Rosa, Dario; Imbriaco, Massimo; Brunetti, Arturo; Tedeschi, Enrico [University ' ' Federico II' ' , Department of Advanced Biomedical Sciences, Naples (Italy); Riccio, Eleonora; Migliaccio, Silvia; Pisani, Antonio [University ' ' Federico II' ' , Department of Public Health, Nephrology Unit, Naples (Italy); Russo, Camilla [University ' ' Federico II' ' , Department of Advanced Biomedical Sciences, Naples (Italy); Feriozzi, Sandro [Belcolle Hospital, Nephrology and Dialysis Department, Viterbo (Italy); Veroux, Massimiliano [University Hospital of Catania, Department of Medical and Surgical Sciences and Advanced Technologies, Catania (Italy); Battaglia, Yuri [St. Anna Hospital-University, Department of Specialized Medicine, Division of Nephrology and Dialysis, Ferrara (Italy); Concolino, Daniela [University Magna Graecia, Department of Pediatrics, Catanzaro (Italy); Pieruzzi, Federico [University of Milano-Bicocca, Nephrology Unit, Milan (Italy); Tuttolomondo, Antonino [University of Palermo, Internal Medicine, DiBiMIS, Palermo (Italy); Caronia, Aurelio [Triolo Zancia Care Home, Palermo (Italy); Russo, Cinzia Valeria; Lanzillo, Roberta; Brescia Morra, Vincenzo [University ' ' Federico II' ' , Department of Neurosciences and Reproductive and Odontostomatological Sciences, Naples (Italy)

    2017-06-15

    Multiple sclerosis (MS) has been proposed as a possible differential diagnosis for Fabry disease (FD). The aim of this work was to evaluate the involvement of corpus callosum (CC) on MR images and its possible role as a radiological sign to differentiate between FD and MS. In this multicentric study, we retrospectively evaluated the presence of white matter lesions (WMLs) on the FLAIR images of 104 patients with FD and 117 patients with MS. The incidence of CC-WML was assessed in the two groups and also in a subgroup of 37 FD patients showing neurological symptoms. WMLs were detected in 50 of 104 FD patients (48.1%) and in all MS patients. However, a lesion in the CC was detected in only 3 FD patients (2.9%) and in 106 MS patients (90.6%). In the FD subgroup with neurological symptoms, WMLs were present in 26 of 37 patients (70.3%), with two subjects (5.4%) showing a definite callosal lesion. FD patients have a very low incidence of CC involvement on conventional MR images compared to MS, independently from the clinical presentation and the overall degree of WM involvement. Evaluating the presence of CC lesions on brain MR scans can be used as a radiological sign for a differential diagnosis between MS and FD, rapidly addressing the physician toward a correct diagnosis and subsequent treatment options. (orig.)

  6. Doença de Fabry Fabry disease

    Directory of Open Access Journals (Sweden)

    Paula Boggio

    2009-08-01

    Full Text Available A doença de Fabry é enfermidade de armazenamento lisossômico rara, ligada ao cromossomo-X, causada pela deficiência parcial ou completa da enzima alfagalactosidase A. O defeito resulta no acúmulo de globotriaosilceramida no endotélio vascular e tecidos viscerais, sendo a pele, o coração, os rins e o sistema nervoso central os mais afetados. As autoras realizam revisão da literatura relacionada a essa afecção e ressaltam que o reconhecimento precoce dos angioqueratomas e da hipoidrose constitui sinal-chave no diagnóstico dessa doença grave. Destacam também a necessidade de esses doentes serem avaliados por equipe multidisciplinar.Fabry disease is an uncommon, X-linked lysosomal storage disorder, caused by partial or complete deficiency of the enzyme a-galactosidase A. The defect leads to accumulation of uncleaved globotriaosylceramide on the vascular endothelium and visceral tissues, being the skin, heart, kidneys and central nervous system the most affected organs. We performed review of the literature related to the disease and emphasized that early recognition of angiokeratomas and hypohidrosis are key diagnostic signs of this serious disease. We also addressed the need of multidisciplinary assessment of these patients.

  7. Social-adaptive and psychological functioning of patients affected by Fabry disease.

    Science.gov (United States)

    Laney, Dawn Alyssia; Gruskin, Daniel J; Fernhoff, Paul M; Cubells, Joseph F; Ousley, Opal Y; Hipp, Heather; Mehta, Ami J

    2010-12-01

    Fabry disease (FD) is an X-linked lysosomal storage disorder caused by the deficiency of alpha-galactosidase A. In addition to the debilitating physical symptoms of FD, there are also under-recognized and poorly characterized psychiatric features. As a first step toward characterizing psychiatric features of FD, we administered the Achenbach adult self report questionnaire to 30 FD patients and the Achenbach adult behavior checklist questionnaire to 28 partners/parents/friends of FD patients. Data from at least one of the questionnaires were available on 33 subjects. Analysis focused on social-adaptive functioning in various aspects of daily life and on criteria related to the Diagnostic and statistical manual of mental disorders IV (DSM-IV). Adaptive functioning scale values, which primarily measure social and relationship functioning and occupational success, showed that eight FD patients (six female and two male) had mean adaptive functioning deficits as compared to population norms. Greater rates of depression (P personality (P Individuals affected by Fabry disease exhibited social-adaptive functioning deficits that were significantly correlated with anxiety, depression, antisocial behavior, and AD/H problems in a sampling of our male and female patients aged between 18 years and 59 years.

  8. Recommendations for initiation and cessation of enzyme replacement therapy in patients with Fabry disease

    DEFF Research Database (Denmark)

    Biegstraaten, Marieke; Arngrímsson, Reynir; Barbey, Frederic;

    2015-01-01

    INTRODUCTION: Fabry disease (FD) is a lysosomal storage disorder resulting in progressive nervous system, kidney and heart disease. Enzyme replacement therapy (ERT) may halt or attenuate disease progression. Since administration is burdensome and expensive, appropriate use is mandatory. We aimed...... to define European consensus recommendations for the initiation and cessation of ERT in patients with FD. METHODS: A Delphi procedure was conducted with an online survey (n = 28) and a meeting (n = 15). Patient organization representatives were present at the meeting to give their views. Recommendations...

  9. Cognitive and Psychological Functioning in Fabry Disease

    Science.gov (United States)

    Sigmundsdottir, Linda; Tchan, Michel C.; Knopman, Alex A.; Menzies, Graham C.; Batchelor, Jennifer; Sillence, David O.

    2014-01-01

    Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity. PMID:25319043

  10. Measuring patient experiences in Fabry disease: validation of the Fabry-specific Pediatric Health and Pain Questionnaire (FPHPQ

    Directory of Open Access Journals (Sweden)

    Ramaswami Uma

    2012-09-01

    Full Text Available Abstract Introduction Common symptoms for children with Anderson-Fabry Disease (FD such as acroparaesthesia and gastrointestinal manifestations can only be objectively assessed in patients using a valid instrument. To date, no such instrument exists. Methods A preliminary 40-item measure of symptoms and experience with FD, the Fabry-specific Paediatric Health and Pain Questionnaire (FPHPQ was developed, but lacked a formal assessment of its measurement properties. The FPHPQ was used in the Fabry Outcome Survey (FOS, a registry for all patients with a confirmed diagnosis of FD who are receiving agalsidase alfa, or are treatment naïve and who are managed by physicians participating in FOS. After an item analysis to explore how items performed and combined into domains, a battery of psychometric analyses was performed to assess the measurement properties of this new instrument. Results Eighty-seven children (ages 4-18 years completed the questionnaire. Twenty-three items in three subscales of the questionnaire emerged: pain associated with heat or exertion, pain associated with cold, and abdominal pain and fatigue symptoms. Internal consistency reliability for all three subscales was good (Cronbach alpha ≥ 0.84. Reliability was equally high for all age groups (4-7, 8-12, and 13-18. Test-retest reliability was high for all three subscales (intraclass correlation coefficient ≥ 0.74. Construct validity was demonstrated by moderate correlation with brief pain inventory (BPI, KINDL, and EQ-5D. Known group validity showed all subscales were able to discriminate between Fabry disease severity groups as classified by above or below median of the FOS MSSI (Mainz Severity Score Index grade. The heat or exertion subscale was responsive to change in symptoms between responders and non-responders as defined by change in EQ-5D index scores between the first and second visit. Conclusions Preliminary results indicate that the measurement properties

  11. Neuro-Otological and Peripheral Nerve Involvement in Fabry Disease

    Science.gov (United States)

    Carmona, Sergio; Weinschelbaum, Romina; Pardal, Ana; Marchesoni, Cintia; Zuberbuhler, Paz; Acosta, Patricia; Cáceres, Guillermo; Kisinovsky, Isaac; Bayón, Luciana; Reisin, Ricardo

    2017-01-01

    Fabry disease (FD) is an X-linked lysosomal storage disease, with multisystemic glycosphingolipids deposits. Neuro-otological involvement leading to hearing loss and vestibular dysfunctions has been described, but there is limited information about the frequency, site of lesion, or the relationship with peripheral neuropathy. The aim was to evaluate the presence of auditory and vestibular symptoms, and assess neurophysiological involvement of the VIII cranial nerve, correlating these findings with clinical and neurophysiological features of peripheral neuropathy. We studied 36 patients with FD with a complete neurological and neuro-otological evaluation including nerve conduction studies, quantitative sensory testing (to evaluate small fiber by warm and cold threshold detection and cold and heat pain), vestibular evoked myogenic potentials, videonistagmography, audiometry and brainstem auditory evoked potentials. Neuro-otologic symptoms included hearing loss (22.2%), vertigo (27.8%) or both (25%). An involvement of either cochlear or vestibular function was identified in most patients (75%). In 70% of our patients the involvement of both cochlear and vestibular function could not be explained by a neural or vascular mechanism. Small fiber neuropathy was identified in 77.7%. There were no significant associations between neuro-otological and QST abnormalities. Neuro-otologic involvement is frequent and most likely under-recognized in patients with FD. It lacks a specific neural or vascular pattern, suggesting multi-systemic, end organ damage. Small fiber neuropathy is an earlier manifestation of FD, but there is no correlation between the development of neuropathy and neuro-otological abnormalities. PMID:28794847

  12. The coincidence of IgA nephropathy and Fabry disease

    Directory of Open Access Journals (Sweden)

    Maixnerová Dita

    2013-01-01

    Full Text Available Abstract Background IgA nephropathy (IgAN is the most common glomerulonephritis, which may also coexist with other diseases. We present two patients with an unusual coincidence of IgAN and Fabry disease (FD. Case presentation A 26 year-old man underwent a renal biopsy in February 2001. Histopathology showed very advanced IgAN and vascular changes as a result of hypertension. Because of his progressive renal insufficiency the patient began hemodialysis in August 2001. By means of the blood spot test screening method the diagnosis of FD was suspected. Low activity of alpha-galactosidase A in the patient’s plasma and leukocytes and DNA analysis confirmed the diagnosis of FD. Enzyme replacement therapy started in July 2004. Then the patient underwent kidney transplantation in November 2005. Currently, his actual serum creatinine level is 250 μmol/l. Other organ damages included hypertrophic cardiomyopathy, neuropathic pain and febrile crisis. After enzyme replacement therapy, myocardial hypertrophy has stabilized and other symptoms have disappeared. No further progression of the disease has been noted. The other patient, a 30 year-old woman, suffered from long-term hematuria with a good renal function. Recently, proteinuria (2.6 g/day appeared and a renal biopsy was performed. Histopathology showed IgAN with remarkably enlarged podocytes. A combination of IgAN and a high suspicion of FD was diagnosed. Electron microscopy revealed dense deposits in paramesangial areas typical for IgAN and podocytes with inclusive zebra bodies and myelin figures characteristic of FD. FD was confirmed by the decreased alpha-galactosidase A activity in plasma and leukocytes and by DNA and RNA analysis. Enzyme replacement therapy and family screening were initiated. Conclusions Our results emphasize the role of complexity in the process of diagnostic evaluation of kidney biopsy samples. Electron microscopy represents an integral part of histopathology, and genetic

  13. Enzyme replacement therapy for Fabry disease: some answers but more questions

    Directory of Open Access Journals (Sweden)

    Majid Alfadhel

    2011-02-01

    Full Text Available Majid Alfadhel1, Sandra Sirrs21Division of Biochemical Diseases, Department of Paediatrics, BC Children’s and Women’s Hospital, University of British Columbia, Vancouver, BC, Canada; 2Adult Metabolic Diseases Clinic, Division of Endocrinology, Department of Medicine, Vancouver Hospital and Health Sciences Centre, University of British Columbia, Vancouver, BC, CanadaAbstract: Fabry disease (FD is a multisystem, X-linked disorder of glycosphingolipid metabolism caused by enzyme deficiency of α-galactosidase A. Affected patients have symptoms including acroparesthesias, angiokeratomas, and hypohidrosis. More serious manifestations include debilitating pain and gastrointestinal symptoms, proteinuria and gradual deterioration of renal function leading to end-stage renal disease, hypertrophic cardiomyopathy, and stroke. Heterozygous females may have symptoms as severe as males with the classic phenotype. Before 2001, treatment of patients with FD was supportive. The successful development of enzyme replacement therapy (ERT has been a great advancement in the treatment of patients with FD and can stabilize renal function and cardiac size, as well as improve pain and quality of life of patients with FD. In this review, we have provided a critical appraisal of the literature on the effects of ERT for FD. This analysis shows that data available on the treatment of FD are often derived from studies which are not controlled, rely on surrogate markers, and are of insufficient power to detect differences on hard clinical endpoints. Further studies of higher quality are needed to answer the questions that remain concerning the efficacy of ERT for FD.Keywords: Fabry disease, agalsidase α, agalsidase β, Replagal, Fabrazyme, critical appraisal, evidence-based medicine

  14. Fabry disease, respiratory symptoms, and airway limitation

    DEFF Research Database (Denmark)

    Svensson, Camilla Kara; Feldt-Rasmussen, Ulla; Backer, Vibeke

    2015-01-01

    abnormalities in patients with Fabry disease. Electron microscopy of lung biopsy and induced sputum show lamellar inclusion bodies (Zebra bodies) in the cytoplasm of cells in the airway wall. X-ray and CT scan have shown patchy ground-glass pulmonary infiltrations, fibrosis, and air trapping. Fibrosis diagnosed...

  15. Multiple parapelvic cysts in Fabry disease.

    Science.gov (United States)

    Azancot, María A; Vila, Josefa; Domínguez, Carmen; Serres, Xavier; Espinel, Eugenia

    2016-01-01

    Fabry disease is an inherited, X-linked lysosomal storage disorder caused by deficiency of the enzyme alpha galactosidase A (alpha-GLA A), which leads to glycosphingolipid accumulation, mainly globotriaosylceramide, in tissues. Disease prevalence and the index of suspicion are both low, which tends to result in delayed diagnosis and treatment. We present the case of a male Fabry disease patient who manifested no angiokeratoma lesions but presented multiple parapelvic cysts and renal failure. The genetic study revealed an alpha-GLA A gene mutation that had not been recorded in the mutations registry. The de novo mutation was not found in his relatives and it was not transmitted to his offspring. The large number and peculiar appearance of the parapelvic cysts led to the diagnosis. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  16. Mutational analysis of the GLA gene in Mexican families with Fabry disease

    Indian Academy of Sciences (India)

    BIANCA ETHEL GUTIÉRREZ-AMAVIZCA; ANDREAS GAL; ROCÍO ORTÍZ-OROZCO; ULRICH ORTH; ERNESTO PRADO MONTES DE OCA; JAIME PAUL GUTIÉRREZ-AMAVIZCA; LUIS E. FIGUERA

    2017-03-01

    Fabry disease (FD) is a lysosomal storage disorder, which develops due to a deficiency in the hydrolytic enzyme, α-galactosidase A (α-Gal A). Alpha-Gal A hydrolyzes glycosphingolipid globotriaosylceramide (Gb3), and an α-Gal Adeficiency leads to Gb3 accumulation in tissues and cells in the body. This pathology is likely to involve multiple systems, but it is generally considered to affect primarily vascular endothelium. In this study, we investigated mutations in the GLA gene, which encodes α-Gal A, in Mexican families with FD. We included seven probands with FD that carried known mutations. We analysed pedigrees of the probands, and performed molecular screening in 65 relatives with the potential of carrying a GLA mutation. Five mutations (P40S, IVS4+4, G328V, R363H, R404del) were detected in seven unrelated Mexican familieswith the classic FD phenotype. Of the 65 relatives examined, 42 (64.6%) had a GLA gene mutation. In summary, among seven Mexican probands with FD, 65 relatives were at risk of carrying a known GLA mutation, and molecular screening identified 42 individuals with the mutation. Thus, our findings showed that it is important to perform molecular analysis in families with FD to detect mutations and to provide accurate diagnoses for individuals that could be affected.

  17. High Variability of Fabry Disease Manifestations in an Extended Italian Family

    Directory of Open Access Journals (Sweden)

    Giuseppe Cammarata

    2015-01-01

    Full Text Available Fabry disease (FD is an inherited metabolic disorder caused by partial or full inactivation of the lysosomal hydrolase α-galactosidase A (α-GAL. The impairment of α-GAL results in the accumulation of undegraded glycosphingolipids in lysosomes and subsequent cell and microvascular dysfunctions. This study reports the clinical, biochemical, and molecular characterization of 15 members of the same family. Eight members showed the exonic mutation M51I in the GLA gene, a disease-causing mutation associated with the atypical phenotype. The clinical history of this family highlights a wide phenotypic variability, in terms of involved organs and severity. The phenotypic variability of two male patients is not related to differences in α-GAL enzymatic activity: though both have no enzymatic activity, the youngest shows severe symptoms, while the eldest is asymptomatic. It is noticeable that for two female patients with the M51I mutation the initial clinical diagnosis was different from FD. One of them was diagnosed with Familial Mediterranean Fever, the other with Multiple Sclerosis. Overall, this study confirms that the extreme variability of the clinical manifestations of FD is not entirely attributable to different mutations in the GLA gene and emphasizes the need to consider other factors or mechanisms involved in the pathogenesis of Fabry Disease.

  18. Persistent increase in cardiac troponin I in Fabry disease: a case report

    Directory of Open Access Journals (Sweden)

    Schneider Christian

    2011-01-01

    Full Text Available Abstract Background Hypertrophic cardiomyopathy is a frequent manifestation in Fabry disease (FD - an X-linked lysosomal storage disorder caused by reduced activity of the enzyme α-galactosidase A. In FD an elevation of specific cardiac biomarkers, such as cardiac troponin I (cTNI has been reported in case of clinical manifestation suggestive of myocardial ischemia. In diagnosing acute myocardial infarction cTNI is considered the most reliable parameter. Case Presentation In the referred case we present a 59 years old female patient with the diagnosis of FD presenting with persistently increased cTNI level (lowest value 0.46 ng/ml, highest value 0.69 ng/ml; normal range Conclusions Our case report demonstrates a persistent cTNI release in FD with cardiac involvement. Proving the persistence in a symptom free interval, it might be related to a direct damage of myocytes. In FD cTNI could serve as a beneficial long term parameter providing new perspectives for screening strategies.

  19. Prognostic Indicators of Renal Disease Progression in Adults with Fabry Disease: Natural History Data from the Fabry Registry

    NARCIS (Netherlands)

    C. Wanner; J.P. Oliveira; A. Ortiz; M. Mauer; D.P. Germain; G.E. Linthorst; A.L. Serra; L. Marodi; R. Mignani; B. Cianciaruso; B. Vujkovac; R. Lemay; D. Beitner-Johnson; S. Waldek; D.G. Warnock

    2010-01-01

    Background and objectives: These analyses were designed to characterize renal disease progression in untreated adults with Fabry disease. Design, setting, participants, & measurements: Data from the Fabry Registry for 462 untreated adults (121 men and 341 women) who had at least two estimated GFR (e

  20. Alpha-Galactosidase A p.A143T, a non-Fabry disease-causing variant

    OpenAIRE

    Lenders, Malte; Weidemann, Frank; Kurschat, Christine; Canaan-Kühl, Sima; Duning, Thomas; Stypmann, Jörg; Schmitz, Boris; Reiermann, Stefanie; Krämer, Johannes; Blaschke, Daniela; Wanner, Christoph; Brand, Stefan-Martin; Brand, Eva

    2016-01-01

    Background Fabry disease (FD) is an X-linked multisystemic disorder with a heterogeneous phenotype. Especially atypical or late-onset type 2 phenotypes present a therapeutical dilemma. Methods To determine the clinical impact of the alpha-Galactosidase A (GLA) p.A143T/ c.427G > A variation, we retrospectively analyzed 25 p.A143T patients in comparison to 58 FD patients with other missense mutations. Results p.A143T patients suffering from stroke/ transient ischemic attacks had slightly decrea...

  1. Uric Acid as a Marker of Mortality and Morbidity in Fabry Disease.

    Science.gov (United States)

    Rob, Daniel; Marek, Josef; Dostálová, Gabriela; Goláň, Lubor; Linhart, Aleš

    2016-01-01

    Serum uric acid (UA) elevation is common in patients with cardiovascular, renal and metabolic diseases. However, no study to date has analysed the role of UA in Fabry disease (FD). To evaluate the association between serum UA levels and mortality and morbidity in FD. We conducted a post-hoc analysis of a prospectively followed-up cohort of 124 patients with genetically proven FD. Serum UA levels were acquired at baseline; clinical events and mortality were assessed during regular visits every 6 to 12 months. The primary endpoint was a composite of multiple secondary outcomes: all-cause mortality, adverse cardiovascular events, progression of renal dysfunction and stroke or transient ischaemic attack (TIA). Predictive value was assessed using the Cox proportional hazards model and the Kaplan Meyer estimator. During follow-up of 7.4 ± 3.7 years, 64 (52%) patients reached the primary combined endpoint. Overall, UA levels were significantly associated with combined outcome (p < 0.001) and remained independently associated after correcting for age, sex and estimated glomerular filtration rate (hazard ratio [HR] per 20 μmol/l increase 1.09, 95% confidence interval [95%CI] (1.00-1.19), p = 0.04). UA was associated with overall mortality in univariate analysis (p = 0.021); however, the association did not reach statistical significance after multivariate correction (HR per 20 μmol/l increase 1.07 95%CI 0.93-1.25, p = 0.32). Higher UA levels were also associated with cardiac adverse outcomes, progression of left ventricular hypertrophy and progression of renal dysfunction (ps < 0.001). No association was observed between UA levels and stroke or TIA (p = 0.323). Increased serum UA levels may represent an independent risk factor for adverse clinical outcomes in Fabry patients and are associated with all-cause mortality. UA is a widely available and cheap biomarker that may improve risk stratification of Fabry patients in clinical practice.

  2. Involvement of dorsal root ganglia in Fabry's disease.

    Science.gov (United States)

    Gadoth, N; Sandbank, U

    1983-08-01

    Bouts of shooting pain along the extremities are common in the early stages of Fabry's disease. No pathological explanation has been advanced to clarify the mechanism of such pain. In the present case neuronal storage of glycolipid was confined to dorsal root ganglia neurones only. It is suggested that this may explain the shooting pain in Fabry's disease. In hereditary sensory radicular neuropathy, familial dysautonomia, and tabes dorsalis, changes in dorsal root ganglia cells cause similar clinical signs and thus it may be concluded that shooting pains in Fabry's disease may be caused by damage to dorsal root ganglia neurones.

  3. Urinary Podocyte Loss Is Increased in Patients with Fabry Disease and Correlates with Clinical Severity of Fabry Nephropathy

    Science.gov (United States)

    Fall, Brent; Scott, C. Ronald; Mauer, Michael; Shankland, Stuart; Pippin, Jeffrey; Jefferson, Jonathan A.; Wallace, Eric; Warnock, David; Najafian, Behzad

    2016-01-01

    Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine (UPodo/g Cr) was 3.6 fold greater in Fabry patients (3,741 ± 2796; p = 0.001) than healthy subjects (1,040 ± 972). Fabry patients with normoalbuminuria and normoproteinuria had over 2-fold greater UPodo/g Cr than healthy subjects (p = 0.048). UPodo/gCr was inversely related to eGFR in male patients (r = -0.69, p = 0.003). UPodo/gCr was directly related to urine protein creatinine ratio (r = 0.33; p = 0.04) in all Fabry patients. These studies confirm increased podocyturia in Fabry disease, even when proteinuria and albuminuria are absent. Podocyturia correlates with clinical severity of Fabry nephropathy, and potentially may be of prognostic value. PMID:27992580

  4. Pedigree analysis of Mexican families with Fabry disease as a powerful tool for identification of heterozygous females.

    Science.gov (United States)

    Gutiérrez-Amavizca, B E; Orozco-Castellanos, R; R Padilla-Gutiérrez, J; Valle, Y; Figuera, L E

    2014-08-28

    Fabry disease (FD) is an X-linked lysosomal storage disease caused by α-galactosidase A deficiency; in contrast to other X-linked diseases, heterozygous females can be as affected as men. The construction and analysis of a family pedigree is a powerful tool to aid clinicians in diagnosis, establishment of inheritance pattern, and early detection of potentially affected relatives. The present study highlights the importance of pedigree analysis in families with FD for identifying other possibly affected relatives and investigating the clinical manifestations. This clinical report included 12 Mexican index cases with confirmed FD diagnosis. We constructed and analyzed their pedigree, and diagnosed FD in 24 affected relatives. Clinical features were similar to those reported for other populations. Pedigree analysis further identified an additional 30 women as possible carriers. We conclude that pedigree construction and analysis is a useful tool to help physicians detect and diagnose relatives at risk for FD, particularly heterozygous females, so that they can receive genetic counseling and early treatment. Mexican families with FD were similar to other populations reported in the literature, and our findings confirmed that heterozygous females can have signs and symptoms ranging from subtle manifestations to the classical severe presentation described in males.

  5. Autonomic skin responses in females with Fabry disease

    DEFF Research Database (Denmark)

    Møller, Anette Torvin; Bach, Flemming W.; Feldt-Rasmussen, Ulla

    2009-01-01

    dysfunction. This study examined peripheral autonomic nerve function in 19 female patients with Fabry disease and 19 sex and age-matched controls by measuring (1) sweat production following acetylcholine challenge; (2) the sympathetically mediated vasoconstrictor responses to inspiratory gasp, stress......, and the cold pressor test; and (3) cutaneous blood flow following capsaicin. The vasoconstrictor response to inspiratory gasp was increased in Fabry patients compared to controls (p = 0.03), while the response to cold and mental stress did not change. Female patients with Fabry disease had a reduced sweat...

  6. Fabry disease in patients with hypertrophic cardiomyopathy: a practical approach to diagnosis.

    Science.gov (United States)

    Seo, Jiwon; Kim, Minji; Hong, Geu-Ru; Kim, Dae-Seong; Son, Jang-Won; Cho, In Jeong; Shim, Chi Young; Chang, Hyuk-Jae; Ha, Jong-Won; Chung, Namsik

    2016-09-01

    This study aimed to develop a new set of screening criteria that is easily applicable and highly sensitive for the detection of patients at high risk of Fabry disease (FD) among hypertrophic cardiomyopathy (HCM) patients. We prospectively studied 273 consecutive unrelated patients who were referred to HCM clinic for unknown left ventricular hypertrophy. Among the 273 patients, we selected 65 high-risk patients who fulfilled at least one of our newly proposed screening criteria. All 273 patients were assayed for plasma α-galactosidase A (α-GAL A) activity. The new screening criteria were: (1) atypical HCM, (2) history or presence of documented arrhythmia, (3) short PR interval defined as <120 ms on electrocardiogram, and (4) symptoms of autonomic dysfunction. From this screening study, three unrelated patients (4.6%; 2 females and 1 male) were newly diagnosed with FD using α-GAL A activity and mutation analysis of the GLA gene. Using the screening method based on the newly proposed criteria, the prevalence of FD in our HCM population was 4.6% if at least one criterion was met and 18.8% if ⩾3 criteria were met. Therefore, our proposed criteria are easily applicable and highly sensitive for classifying patients at high risk of FD from HCM patients.

  7. PrEFiNe Plan: Strategic plan for Fabry diseases in Nephrology.

    Science.gov (United States)

    Del Pino, M D; Ortiz, A; Torra, R; Hernandez, D

    2016-01-01

    Renal failure is one of the main causes of death in patients with Fabry disease (FD). Due to the low prevalence of FD, delayed diagnosis and misdiagnosis, often the correct diagnosis is made when organ damage is already present. Early recognition of the disease would allow the prevention of severe complications and the premature death of patients with FD. We present here the PrEFiNE project, which includes a wide spectrum of activities with the aim of improve knowledge and diagnosis of FD. The project is sponsored by Shire Iberia (http://shireiberica.com/) From January 2016 to the end of 2017 several activities will be carried out, starting with a survey to evaluate current FD knowledge among nephrologists; in addition some studies to assess prevalence of this disease will be performed. One study will include patients receiving dialysis, another study will cover kidney transplant patients, and a pilot study in chronic kidney disease in stage 3-5 predialysis. Also planned is a pharmacoeconomic study to focus on burden of FD. At the same time medical education activities will be conducted both on line and on site. Plan for dissemination will include medical publications and diffusion to media. PrEFiNE Project will finish with the publication of a compilation book on FD in Nephrology including all planned activities and proposing recommendations based on results and detected unmet needs. PrEfiNE Plan will be coordinated by severa scientific committees, one at national level and 10 other regionals comittees, tha will be responsible to ensure the maximum scientific quality of proposed activities. An advisory board will supervise the project. PrEfiNE project will evaluate an action plan focused on improving FD knowledge to make necessary recommendations for an early recognition of the disease. In addition will generate a plan to improve previously undetected needs. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights

  8. Impaired Left Atrial Function in Fabry Disease: A Longitudinal Speckle-Tracking Echocardiography Study.

    Science.gov (United States)

    Pichette, Maxime; Serri, Karim; Pagé, Maude; Di, Lu Zhao; Bichet, Daniel G; Poulin, Frédéric

    2017-02-01

    Fabry disease (FD) is characterized by the accumulation of sphingolipids in multiple organs, including the left atrium. It is uncertain if the left atrial (LA) reservoir, conduit, and contractile functions evaluated by speckle-tracking echocardiography are affected in Fabry cardiomyopathy and whether enzyme replacement therapy can improve LA function. In this retrospective cohort study, LA strain, strain rates, and phasic LA volumes were studied in 50 patients with FD and compared with values in 50 healthy control subjects. All three LA phasic functions were altered. Peak positive strain (reservoir function) was 38.9 ± 14.9% versus 46.5 ± 10.9% (P = .004), and late diastolic strain (contractile function) was 12.6 ± 5.9% versus 15.6 ± 5.3% (P = .010). In 15 patients who started enzyme replacement therapy during the study, most of the LA parameters improved at 1-year follow-up (peak positive strain from 32.0 ± 13.5% to 38.0 ± 13.5%, P = .006), whereas there was a trend toward deterioration in 15 patients who never received treatment (peak positive strain from 47.3 ± 10.8% to 41.3 ± 9.3%, P = .058). Nine patients with FD (21%) experienced new-onset atrial fibrillation or stroke during 4-year follow-up. By univariate analysis, peak positive strain and early diastolic strain demonstrated significant associations with clinical events, surpassing conventional echocardiographic parameters and clinical characteristics. LA reservoir, conduit, and contractile functions by speckle-tracking echocardiography were all affected in FD. Enzyme replacement therapy improved LA function. LA strain parameters were associated with atrial fibrillation and stroke. Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  9. Lysosome-associated protein 1 (LAMP-1) and lysosome-associated protein 2 (LAMP-2) in a larger family carrier of Fabry disease.

    Science.gov (United States)

    Pereira, Ester M; do Monte, Semiramis J H; do Nascimento, Fernando F; de Castro, Jose A F; Sousa, Jackeline L M; Filho, Henrique C S A L C; da Silva, Raimundo N; Labilloy, Anatália; Monte Neto, José T; da Silva, Adalberto S

    2014-02-15

    This study investigated the potential relationship between the expression levels of lysosome-associated membrane proteins (LAMP) 1 and 2 and responses to enzyme replacement therapy (ERT) in the members of a single family with Fabry disease (FD). LAMP levels were assessed by flow cytometry in leukocytes from 17 FD patients who received an eight-month course of ERT course and 101 healthy individuals. We found that phagocytic cells from the FD patients had higher expression levels of both LAMP-1 and LAMP-2, relative to the levels in phagocytes from the healthy controls (p=0.001). Furthermore, the LAMP-1 and LAMP-2 levels in phagocytes from the FD carriers continuously decreased with ERT administration to reach levels similar to those in healthy controls. We suggest that LAMP-1 and LAMP-2 could be used as additional markers with which to assess ERT effectiveness in FD.

  10. Fabry disease: recent advances in pathology, diagnosis, treatment and monitoring

    Directory of Open Access Journals (Sweden)

    Hoffmann Björn

    2009-10-01

    Full Text Available Abstract Background In Fabry disease (α-galactosidase A deficiency accumulation of Globotriaosylceramide (Gb3 leads to progressive organ failure and premature death. The introduction of enzyme replacement therapy (ERT was the beginning of a new era in this disorder, and has prompted a broad range of research activities. This review aims to summarize recent developments and progress with high impact for Fabry disease. Methods A Pubmed analysis was performed using the search terms "Fabry disease", "Anderson-Fabry disease", "alpha-galactosidase A" and "Gb3". Of the given publications by 31st January 2009 only original articles recently published in peer reviewed journals were included for this review. Case reports were included only when they comprised a new aspect. In addition we included relevant conference abstracts when the results had not already been published as original articles. Results Apart from Gb3-accumulation cellular and organ specific damages may be related also to inflammatory and immunological consequences. It will be interesting whether this may lead to new therapeutic strategies in the treatment of Fabry disease. Since newborn screening is still difficult in Fabry disease, detection of patients in populations at risk is of great importance. Undiagnosed patients with Fabry disease may still be found in cohorts of subjects with renal diseases, cardiomyopathy and TIA or stroke. Efforts should be undertaken to identify these individuals and initialise ERT in order to hault disease progression. It has also been demonstrated that Gb3-accumulation leads to pre-clinical damages and it is believed that early treatment may be the only possibility so far to prevent irreversible organ damage.

  11. Non-invasive determination of myocardial lipid content in Fabry disease by {sup 1}H-MR spectroscopy

    Energy Technology Data Exchange (ETDEWEB)

    Petritsch, B.; Koestler, H.; Machann, W.; Horn, M.; Weng, A.M.; Goltz, J.P.; Hahn, D.; Beer, M. [Universitaetsklinikum Wuerzburg (Germany). Inst. fuer Roentgendiagnostik im ZOM; Niemann, M.; Weidemann, F.; Wanner, C. [Universitaetsklinikum Wuerzburg (Germany). Medizinische Klinik I

    2012-11-15

    Purpose: In Fabry disease (FD), a progressive deposition of sphingolipids is reported in different organs. The present study applied {sup 1}H magnetic resonance spectroscopy (MRS) to investigate the myocardial lipid content in FD. Materials and Methods: In patients (PTS, n = 15) with genetically proven FD, {sup 1}H MRS of the heart was acquired in the same examination as routine cardiac cine and late enhancement MR imaging. Healthy volunteers (n = 11) without history of cardiac disease served as control (CTL). Myocardial triglycerides in vivo were quantified in {sup 1}H MRS. Left ventricular (LV) ejection fraction (EF) and late enhancement were assessed for the determination of LV systolic function, and onset or absence of myocardial fibrosis. Results: All {sup 1}H MRS revealed resonances for intramyocardial triglycerides. Clinical parameters, e.g. EF (PTS 64 {+-} 2 % vs. CTL 61 {+-} 1 %) were similar in PTS and CTL or showed a non-significant trend (LV mass). Apart from a single patient with elevated myocardial triglycerides, no significant impact of Fabry disease on the triglyceride/water resonance ratio (PTS 0.47 {+-} 0.11 vs. CTL 0.52 {+-} 0.11 %) was observed in our patient cohort. Conclusion: A comprehensive cardiac evaluation of morphology, function as well as metabolism in Fabry PTS with suspected cardiac involvement is feasible in a single examination. No significant effect of myocardial triglyceride deposition could be observed in patients. The remarkably high myocardial triglyceride content in one patient with advanced FD warrants further studies in PTS with an extended history of the disease. (orig.)

  12. Arterial Wall Properties and Womersley Flow in Fabry Disease

    Directory of Open Access Journals (Sweden)

    Dimitriadis Emilios

    2002-01-01

    Full Text Available Abstract Background Fabry disease is an X-linked recessive lysosomal storage disease resulting in the cellular accumulation of globotriaosylceramide particularly globotriaosylceramide. The disease is characterized by a dilated vasculopathy with arterial ectasia in muscular arteries and arterioles. Previous venous plethysomographic studies suggest enhanced endothelium-dependent vasodilation in Fabry disease indicating a functional abnormality of resistance vessels. Methods We examined the mechanical properties of the radial artery in Fabry disease, a typical fibro-muscular artery. Eight control subjects and seven patients with Fabry disease had a right brachial arterial line placed allowing real time recording of intra-arterial blood pressure. Real time B-mode ultrasound recordings of the right radial artery were obtained simultaneously allowing calculation of the vessel wall internal and external diameter, the incremental Young's modulus and arterial wall thickness. By simultaneously measurement of the distal index finger-pulse oximetry the pulse wave speed was calculated. From the wave speed and the internal radial artery diameter the volume flow was calculated by Womersley analysis following truncation of the late diastolic phase. Results No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow. Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside. Both drugs however, elevated the mean arterial flow. Conclusions The current study suggests that no structural or mechanical abnormality exists in the vessel wall of fibro-muscular arteries in Fabry disease. This may indicate that a functional abnormality downstream to the conductance vessels is the dominant feature in

  13. A case of minimal change disease in a Fabry patient.

    Science.gov (United States)

    Zarate, Yuri A; Patterson, Larry; Yin, Hong; Hopkin, Robert J

    2010-03-01

    Fabry disease is an X-linked lysosomal storage disorder caused by mutations of the GLA gene and deficiency in alpha-galactosidase A activity. Glycosphingolipids accumulation causes renal injury that manifests early during childhood as tubular dysfunction and later in adulthood as proteinuria and renal insufficiency. Nephrotic syndrome as the first evidence of Fabry-related kidney damage is rare. We report the case of a teenager with known Fabry disease and normal renal function who developed acute nephrotic syndrome. He was found to have typical glycosphingolipids accumulation with no other findings suggestive of alternative causes of nephrotic syndrome on kidney biopsy. After treatment with enzyme replacement therapy and oral steroids, he went into complete remission from nephrotic syndrome, a response that is atypical for Fabry disease patients who develop heavy proteinuria as a result of longstanding disease and chronic renal injury. The nephrotic syndrome in this patient appears to have developed secondary to minimal change disease. We recommend considering immunotherapy in addition to enzyme replacement therapy in those patients with confirmed Fabry disease and acute nephrotic syndrome with clinical and microscopic findings suggestive of minimal change disease.

  14. Multicomponent nanoparticles as nonviral vectors for the treatment of Fabry disease by gene therapy

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    Ruiz de Garibay AP

    2012-10-01

    Full Text Available Aritz Pérez Ruiz de Garibay, Diego Delgado, Ana del Pozo-Rodríguez, María Ángeles Solinís, Alicia Rodríguez GascónPharmacokinetics, Nanotechnology and Gene Therapy Group, Pharmacy Faculty, University of the Basque Country UPV/EHU, Vitoria-Gasteiz, SpainPurpose: Gene-mediated enzyme replacement is a reasonable and highly promising approach for the treatment of Fabry disease (FD. The objective of the present study was to demonstrate the potential applications of solid lipid nanoparticle (SLN-based nonviral vectors for the treatment of FD.Methods: SLNs containing the pR-M10-αGal A plasmid that encodes the α-Galactosidase A (α-Gal A enzyme were prepared and their in vitro transfection efficacy was studied in Hep G2 cells. We also studied the cellular uptake of the vectors and the intracellular disposition of the plasmid.Results: The enzymatic activity of the cells treated with the vectors increased significantly relative to the untreated cells, regardless of the formulation assayed. When the SLNs were prepared with protamine or dextran and protamine, the activity of the α-Gal A enzyme by the transfected Hep G2 cells increased up to 12-fold compared to that of untreated cells.Conclusion: With this work we have revealed in Hep G2 cells the ability of a multicomponent system based on SLNs to act as efficient nonviral vectors to potentially correct low α-Gal A activity levels in FD with gene therapy.Keywords: solid lipid nanoparticles, Fabry disease, nonviral vectors, gene therapy

  15. Co-existence of Phenylketonuria and Fabry disease on a 3 year-old boy: case report

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    Bonapace Giuseppe

    2010-05-01

    Full Text Available Abstract Background The co-existence of two genetically distinct metabolic disorders in the same patient has rarely been reported. Phenylketonuria (PKU is an inborn error of the metabolism resulting from a phenylalanine hydroxylase deficiency. Fabry disease (FD is an X-linked lysosomal storage disorder due to a deficiency of the enzyme alpha-galactosidase A. Case presentation We report a case of a 3 year- old boy affected by classic PKU and FD, both confirmed by molecular data. The FD was suspected at the age of 21 months on the presence of non-specific GI symptoms (severe abdominal pain and periodically appearance of not specific episodes of gastroenteritis apparently non related to PKU. Conclusion This is the first report of co-existence of FD and PKU, two different congenital inborn of metabolism and in consideration of the prevalence of each disease this chance association is a very unusual event. The co-existence of this diseases made very difficult the correct interpretation of clinical symptoms as lack of appetite, severe abdominal pain and non-specific gastroenteritis episodes. Furthermore, this case report helps to define the early clinical phenotype of FD.

  16. Globotriaosylsphingosine accumulation and not alpha-galactosidase-A deficiency causes endothelial dysfunction in Fabry disease.

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    Mehdi Namdar

    Full Text Available BACKGROUND: Fabry disease (FD is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA resulting in the accumulation of globotriaosylsphingosine (Gb3 in a variety of tissues. While GLA deficiency was always considered as the fulcrum of the disease, recent attention shifted towards studying the mechanisms through which Gb3 accumulation in vascular cells leads to endothelial dysfunction and eventually multiorgan failure. In addition to the well-described macrovascular disease, FD is also characterized by abnormalities of microvascular function, which have been demonstrated by measurements of myocardial blood flow and coronary flow reserve. To date, the relative importance of Gb3 accumulation versus GLA deficiency in causing endothelial dysfunction is not fully understood; furthermore, its differential effects on cardiac micro- and macrovascular endothelial cells are not known. METHODS AND RESULTS: In order to assess the effects of Gb3 accumulation versus GLA deficiency, human macro- and microvascular cardiac endothelial cells (ECs were incubated with Gb3 or silenced by siRNA to GLA. Gb3 loading caused deregulation of several key endothelial pathways such as eNOS, iNOS, COX-1 and COX-2, while GLA silencing showed no effects. Cardiac microvascular ECs showed a greater susceptibility to Gb3 loading as compared to macrovascular ECs. CONCLUSIONS: Deregulation of key endothelial pathways as observed in FD vasculopathy is likely caused by intracellular Gb3 accumulation rather than deficiency of GLA. Human microvascular ECs, as opposed to macrovascular ECs, seem to be affected earlier and more severely by Gb3 accumulation and this notion may prove fundamental for future progresses in early diagnosis and management of FD patients.

  17. Fabry disease - current treatment and new drug development.

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    Motabar, Omid; Sidransky, Ellen; Goldin, Ehud; Zheng, Wei

    2010-07-23

    Fabry disease is a rare inherited lysosomal storage disorder caused by a partial or complete deficiency of α-galactosidase A (GLA), resulting in the storage of excess cellular glycosphingolipids. Enzyme replacement therapy is available for the treatment of Fabry disease, but it is a costly, intravenous treatment. Alternative therapeutic approaches, including small molecule chaperone therapy, are currently being explored. High throughput screening (HTS) technologies can be utilized to discover other small molecule compounds, including non-inhibitory chaperones, enzyme activators, molecules that reduce GLA substrate, and molecules that activate GLA gene promoters. This review outlines the current therapeutic approaches, emerging treatment strategies, and the process of drug discovery and development for Fabry disease.

  18. A case of Fabry's disease with congenital agammaglobulinemia.

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    Lee, Ki-Yeol; Jeon, Su-Young; Hong, Jin-Woo; Kim, Sung-Eun; Song, Ki-Hoon; Kim, Young-Hun; Kim, Ki-Ho

    2011-07-01

    Fabry's disease is an X-linked lysosomal storage disorder caused by abnormalities in the α-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues. It manifests as progressive multiple organ dysfunctions caused by the deposition of Gb3. On the other hand, congenital agammaglobulinemia is usually caused by mutations in Bruton's tyrosine kinase (Btk) gene with X-linked dominence, suppresses B cell maturation, and causes recurrent pyogenic infections. In former reports, the distance between the loci in the Xq22 region of the human X chromosome was found to be about 69 kilobases. A 23-yr-old man diagnosed with congenital agammaglobulinemia at age 5, showed typical clinical and laboratory and histopathological findings of Fabry's disease. The genetic basis of this combination of the two syndromes was studied in this patient. Here, we report a case of Fabry's disease with congenital agammaglobulinemia.

  19. Two-dimensional speckle tracking as a non-invasive tool for identification of myocardial fibrosis in Fabry disease.

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    Krämer, Johannes; Niemann, Markus; Liu, Dan; Hu, Kai; Machann, Wolfram; Beer, Meinrad; Wanner, Christoph; Ertl, Georg; Weidemann, Frank

    2013-06-01

    This cross-sectional study aimed to analyse myocardial deformation in patients with Fabry disease (FD) in order to evaluate speckle tracking as a method for non-invasive determination of myocardial fibrosis. Myocardial fibrosis is common in Fabry cardiomyopathy and is associated with disease progression and severe prognosis. In 101 consecutive Fabry patients (39.8 ± 12.9 years; 42 males), the quantitative measurement of myocardial fibrosis with magnetic resonance imaging was compared with regional myocardial deformation assessed by speckle-tracking imaging. Patients were analysed in relation to per cent of late-enhancement (LE)-positive areas of left-ventricular (LV) mass. Fifty-two patients (51%) displayed LE with a mean volume of 1.2 ± 1.8% of total LV mass. Predominantly basal lateral and posterior segments were affected. Patients with LE had lower global systolic longitudinal strain than those without (LE -14.8 ± 3.5% and -18.9 ± 2.1%, respectively; P speckle tracking, was predominantly caused by basal posterior (P = 0.049) and lateral (P = 0.005) segments and global systolic strain correlated with the amount of LE (r = 0.543; P 2%, n = 22) showed the lowest deformation values (-5.9 ± 8.4%) in basal postero-lateral segments when compared with those with mild (Speckle tracking can be used as a tool for the indirect evaluation of LE in FD.

  20. Fabry disease, a complex pathology not easy to diagnose

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    Paolo Colomba

    2015-12-01

    Full Text Available Fabry disease is a multisystemic lysosomal storage disorder, inherited in an X-linked manner. It is a defect of metabolism of the glycosphingolipids, due to the reduction or absence of the activity of lysosomal enzyme α-galactosidase A. This reduction of activity causes the storage of globotriaosylceramide and derivatives in the lysosomes, triggering a cascade of cellular events, mainly in vascular endothelium. These events are the responsible for the systemic clinical manifestations and the renal, cardiac and cerebrovascular complications, or a combination of them. The symptomatology can lead to the premature death of patient between the fourth or fifth decade of life. The first symptoms can occur at different ages, generally in childhood, with different severity and course. Fabry disease is suspected on the basis of clinical and anamnestic-familial data, and it is confirmed by enzymatic and genetic assays. However, Fabry disease could be a pathology more complex than previously considered, and the diagnostic tests that are currently in use could be not always sufficient to confirm the clinical diagnosis. Probably, other factors could be also involved in the onset of symptomatology. In the last years, the knowledge of the disease is considerably increased but other studies are necessary to make a prompt and reliable diagnosis. An early diagnosis of Fabry disease is essential for the beginning of the enzyme replacement therapy, which can contribute to arrest its progression and improve the quality of life of patients.

  1. Magnetic resonance spectroscopy in patients with Fabry and Gaucher disease

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    Gruber, S., E-mail: stephan@nmr.at [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Bogner, W. [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Stadlbauer, A. [MR Physics Group, Department of Radiology, Landesklinikum St. Poelten (Austria); Krssak, M. [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Bodamer, O. [Department of Pediatrics, Medical University of Vienna (Austria)

    2011-08-15

    Objective: Fabry and Gaucher diseases are rare progressive inherited disorders of glycosphingolipid metabolism that affect multiple organ systems. The aim of this study was to investigate evidence for metabolic changes in the central nervous system involvement using proton magnetic resonance spectroscopic imaging. Methods: Seven Fabry and eight Gaucher patients were included into this study. A two-dimensional, spectroscopic imaging method with an ultra-short echo-time of 11 ms was used at a 3 T whole body magnet. Absolute metabolic values were retrieved using internal water scaling. Results were compared, with sex- and age-matched controls. Results: In contrast to previous findings, absolute and relative metabolite values of N-acetyl-aspartate (NAA) or NAA/Creatine (Cr), Cr, Choline (Cho) or Cho/Cr and myo-Inositol (mI) or mI/Cr revealed no, differences between Fabry and Gaucher Type 1 (GD1) patients and controls. Average values were, 10.22, 6.32, 2.15 and 5.39 mMol/kg wet weight for NAA, Cr, Cho and mI, respectively. In this study, we found significantly decreasing NAA/Cho with increasing age in all three groups (Fabry, GD1, patients and healthy controls) (between 5 and 8% per decade). Conclusions: There were no changes of the quantified metabolites detected by MRS in normal appearing white matter. This study shows the importance of sex- and age-matched controls.

  2. Patients affected with Fabry disease have an increased incidence of progressive hearing loss and sudden deafness: an investigation of twenty-two hemizygous male patients

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    Chassaing Augustin

    2002-10-01

    Full Text Available Abstract Background Fabry disease (FD, OMIM 301500 is an X-linked inborn error of glycosphingolipid metabolism due to the deficient activity of alpha-galactosidase A, a lysosomal enzyme. While the progressive systemic deposition of uncleaved glycosphingolipids throughout the body is known to have protean clinical manifestations, few data are available regarding the cochlear involvement. Methods We non-invasively investigated cochlear functions in 22 consecutive hemizygous males (age 19–64 years, mean 39 affected with classic FD. Conventional audiometry, tympanometry, ABR audiometry, otoacoustic emissions were performed in all patients, together with medical history record and physical examination as part of an exhaustive baseline evaluation prior to enzyme replacement therapy. Results A total of 12 patients (54.5% with classic FD were found to have abnormal audition. Five patients had progressive hearing loss and seven patients (32% experienced sudden deafness. In addition, a hearing loss on high-tone frequencies was found in 7 out of the 10 remaining patients without clinical impairment, despite their young age at time of examination. The incidence of hearing loss appeared significantly increased in FD patients with kidney failure (P tinnitus aurium was also found in six patients (27%. Conclusion This is the first evidence of a high incidence of both progressive hearing loss and sudden deafness in a cohort of male patients affected with classic Fabry disease. The exact pathophysiologic mechanism(s of the cochlear involvement deserves further studies.

  3. The Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study

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    Kultigin Turkmen

    2016-12-01

    Full Text Available Background/Aims: Fabry disease is a treatable cause of chronic kidney disease (CKD characterized by a genetic deficiency of α-galactosidase A. European Renal Best Practice (ERBP recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. Methods: This prospective study assessed α-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. Results: Three (all males of 313 CKD patients (0.95% were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and started enzyme replacement therapy and 4 were female. The most frequent manifestations in male patients were fatigue (100%, tinnitus, vertigo, acroparesthesia, hypohidrosis, cornea verticillata and angiokeratoma (all 85%, heat intolerance (71%, and abdominal pain (57%. The most frequent manifestations in female patients were fatigue and cornea verticillata (50%, and tinnitus, vertigo and angiokeratoma (25%. Three patients had severe episodic abdominal pain attacks and proteinuria, and were misdiagnosed as familial Mediterranean fever. Conclusions: The prevalence of Fabry disease in selected CKD patients is in the range found among renal replacement therapy patients, but the disease is diagnosed at an earlier, treatable stage. These data support the ERBP recommendation to screen for Fabry disease in patients with CKD of unknown origin.

  4. Fabry disease presenting with sudden hearing loss and otosclerosis: a case report

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    Felisati Giovanni

    2012-04-01

    Full Text Available Abstract Introduction Fabry disease is an X-linked lysosomal storage disorder resulting in a multiple-system disorder with a wide spectrum of physical signs and symptoms, predominantly affecting the central and peripheral nervous systems, skin, heart, kidneys, and eyes. Case presentation We describe the case of a 26-year-old European Caucasian man who had Fabry disease and who presented with episodic sudden unilateral hearing loss and was treated with glucocorticoids, pentoxifylline, hyperbaric oxygen, and fluoride because of concomitant audiometric evidence of otosclerosis. This case demonstrates the partial and transient beneficial effect of standard treatment for sudden hearing loss not related to Fabry disease and analyzes the possible connection between typical Fabry disease inner-ear lesions and otosclerosis. Whereas hearing loss has been described in connection with Fabry disease, otosclerosis-associated hearing loss in Fabry disease has not yet been described. Conclusions Although progressive hearing loss in patients with Fabry disease seems to be influenced by replacement therapy, few data concerning treatment of sudden hearing loss are available. The lack of literature concerning the pathogenesis of the otological involvement in Fabry disease makes it impossible to identify a connection between the latter and otosclerosis. Therefore, this report may help to reinforce the importance of a thorough evaluation of hearing in patients with Fabry disease and may be of help with therapeutic decision-making.

  5. Fabry disease: a study of 6 hemizygous men and 5 heterozygous women with emphasis on dermatologic manifestations.

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    Larralde, Margarita; Boggio, Paula; Amartino, Hernán; Chamoles, Néstor

    2004-12-01

    To determine the significance of the dermatologic and systemic abnormalities found in 11 patients with Fabry disease (FD) which is an X-linked lysosomal storage disorder caused by the partial or complete deficiency of the alpha-galactosidase A enzyme. This defect leads to the accumulation of uncleaved glycosphingolipids throughout vascular endothelium and visceral tissues. Case series. Pediatric Dermatology Division, Ramos Mejia Hospital (primary care center) and Laboratory of Neurochemistry (referral center for metabolic diseases). Eleven patients with FD were studied: 6 hemizygous men (mean age, 23.0 years) and 5 heterozygous women (mean age, 49.4 years). Mucocutaneous angiokeratomas (AKs) were found in 5 (83%) of 6 hemizygotes and 4 (80%) of 5 heterozygotes. The AKs appeared at an average age of 13 years, affecting predominantly genitalia, back, elbows, and other frequently traumatized areas. All the hemizygotes and none of the heterozygotes suffered from hypohidrosis. Angiokeratomas on the trunk and oral mucosa without sweat abnormalities were detected in 80% of heterozygous women. All hemizygotic men presented with acral pain in childhood. We emphasize the value of early recognition of AKs and hypohidrosis as diagnostic clues to FD, a severe and progressive disorder.

  6. Avascular necrosis of bilateral femoral heads in a patient with Fabry's disease.

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    O'Neill, Francis

    2012-07-13

    The underlying cause of avascular necrosis (AVN) of the femoral head is often not apparent. We report the case of a 26 year old builder with a four month history of bilateral hip pain, and a diagnosis of bilateral femoral head avascular necrosis. Fabry\\'s disease was identified as the probable cause. Since 2001, enzyme replacement therapy for Fabry\\'s disease has become available, with a potential to influence the disease process, and this is of potential importance to clinicians treating AVN.

  7. Functional dyspepsia (FD) and non-erosive reflux disease (NERD): overlapping or discrete entities?

    LENUS (Irish Health Repository)

    Quigley, Eamonn M M

    2012-02-03

    As the incidence of both gastric cancer and peptic ulcer disease have declined, that of gastro-oesophageal reflux disease (GORD) and non-ulcer, or functional dyspepsia (FD) have reached virtually epidemic proportions. As we come to appreciate the expression of these disorders in the community, the real spectrum of each disease has become evident. FD and non-erosive reflux disease (NERD), the most prevalent manifestation of GORD, frequently overlap. Where then does GORD end and FD begin? Is it realistic, or even clinically relevant, to attempt a clear separation between these entities? These are more than issues of mere semantics; therapeutic options may be dictated by the classification of the patient as one or the other. Recent work indicates clearly that NERD is a heterogeneous disorder incorporating some patients who may well harbour subtle manifestations of oesophagitis and others who have entirely normal 24-hour pH studies. These differences may be crucial to the concept of NERD\\/FD overlap. While evidence in support of this concept is far from complete, it would appear that this overlap is most relevant to those NERD patients who do not exhibit abnormal esophageal acid exposure. These patients truly belong in the spectrum of functional gastrointestinal disorders rather than in GORD; attempts to shoe-horn these individuals into the spectrum of GORD will result in therapeutic disappointment and surgical disaster.

  8. Analysis of Placental Tissue in Fabry Disease With and Without Enzyme Replacement Therapy

    NARCIS (Netherlands)

    M.G. Bouwman; C.E.M. Hollak; M.A. van den Bergh Weerman; F.A. Wijburg; G.E. Linthorst

    2010-01-01

    There are only a few reports on the histology of placental tissue of pregnancies from mothers with Fabry disease. Fabry disease is a lysosomal disorder caused by alpha-galactosidase A deficiency. Extensive glycosphingolipid (GSL) accumulation in fetal and maternal placenta tissue obtained from a Fab

  9. Enfermedad de Fabry: Comunicación de ocho casos Fabry disease: Report of eight cases

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    M Palombo

    2011-12-01

    Full Text Available La Enfermedad de Fabry (EF constituye una alteración hereditaria del metabolismo de los glicoesfingolípidos, debida a la deficiencia parcial o completa de la enzima alfa-galactosidasa A. Es una enfermedad de transmisión genética ligada a X, que afecta universalmente a todas las etnias humanas con una incidencia comunicada de 1 cada 100.000 nacimientos, aunque es probable que esta cifra subestime la real prevalencia de la enfermedad, especialmente por el gran número de casos no diagnosticados. La EF se manifiesta en su forma más florida, en varones homocigotas que carecen completamente de actividad alfa-galactosidasa A, provocando una miríada de alteraciones, incluyendo anomalías renales (proteinuria progresiva e insuficiencia renal, cardiovasculares (cardiopatías, arritmias, accidentes cerebrovasculares, neurológicas (dolor acral y abdominal, y cocleo-vestibulares, entre las más importantes. Sin embargo, la afectación cutánea constituye la alteración más específica de la enfermedad y es en general, la que conduce a la sospecha diagnóstica. La EF no tratada reduce francamente la expectativa de vida de acuerdo a la severidad de la afectación renal y cardiovascular, si bien la terapia con reemplazo enzimático puede modificar e incluso detener el curso de la enfermedad. En 2010, una paciente de 28 años oriunda de la Provincia de Santa Fe, consultó porangioqueratomas, que condujeron al diagnóstico de enfermedad de Fabry. Desde entonces hemos estudiado y tratado a toda la familia, con ocho casos confirmados a la fecha y otros tantos en evaluación.Fabry disease (Online Mendelian Inheritance in Man No. 301500 is an X-linked inherited condition due to absence or reduction of ɑ galactosidase activity in lysosomes that results in accumulation of globotriaosylceramide and related neutral glycosphingolipids (storage disorder. It is estimated to occur in 1 in 40.000 to 117.000 live male births, although a more recent screening study in

  10. [Treatment of Fabry disease: Successes, failures, and expectations].

    Science.gov (United States)

    Lidove, Olivier; Barbey, Frédéric; Joly, Dominique

    2016-04-01

    Fabry disease, an X-linked lysosomal storage disease, results from α-galactosidase A deficiency. Two different recombinant enzyme treatments (algalsidase alpha agalsidase beta) have been available since 2001 to treat a disease that affects not only men but also women. Enzyme replacement therapy promotes cell clearance of susbtrate, and improves some clinical parameters (heart, kidney damage, pain, quality of life). However, there is no proven efficacy to date on central nervous system lesions, on cardiac morbidity and mortality, nor on renal damage beyond a certain stage (proteinuria>1g/day and/or estimated glomerular filtration rate<60mL/min/1.73m(2)). In this review, we discuss the potential benefit of an early intervention, the vascular protective measures to be associated with enzyme therapy and their rationale, and some alternative treatments under development, such as chaperones and substrate molecules inhibitors.

  11. Variations in plasma and urinary lipids in response to enzyme replacement therapy for Fabry disease patients by nanoflow UPLC-ESI-MS/MS.

    Science.gov (United States)

    Byeon, Seul Kee; Kim, Jin Yong; Lee, Jin-Sung; Moon, Myeong Hee

    2016-03-01

    A deficiency of α-galactosidase A causes Fabry disease (FD) by disrupting lipid metabolism, especially trihexosylceramide (THC). Enzyme replacement therapy (ERT) is clinically offered to FD patients in an attempt to lower the accumulated lipids. Studies on specific types of lipids that are directly or indirectly altered by FD are very scarce, even though they are crucial in understanding the biological process linked to the pathogenesis of FD. We performed a comprehensive lipid profiling of plasma and urinary lipids from FD patients with nanoflow liquid chromatography electrospray-ionization tandem mass spectrometry (nLC-ESI-MS/MS) and identified 129 plasma and 111 urinary lipids. Among these, lipids that exhibited alternations (>twofold) in patients were selected as targets for selected reaction monitoring (SRM)-based high-speed quantitation using nanoflow ultra-performance LC-ESI-MS/MS (nUPLC-ESI-MS/MS) and 31 plasma and 26 urinary lipids showed significant elevation among FD patients. Higher percentages of sphingolipids (SLs; 48% for plasma and 42% for urine) were highly elevated in patients; whereas, a smaller percentage of phospholipids (PLs; 15% for plasma and 13% for urine) were significantly affected. Even though α-galactosidase A is reported to affect THC only, the results show that other classes of lipids (especially SLs) are changed as well, indicating that FD not only alters metabolism of THC but various classes of lipids too. Most lipids showing significant increases in relative amounts before ERT decreased after ERT, but overall, ERT influenced plasma lipids more than urinary lipids.

  12. Fabry disease in genetic counseling practice: recommendations of the National Society of Genetic Counselors.

    Science.gov (United States)

    Bennett, Robin L; Hart, Kimberly A; O'Rourke, Erin; Barranger, John A; Johnson, Jack; MacDermot, Kay D; Pastores, Gregory M; Steiner, Robert D; Thadhani, Ravi

    2002-04-01

    The objective of this document is to provide health care professionals with recommendations for genetic counseling and testing of individuals with a suspected or confirmed diagnosis of Fabry disease, with a family history of Fabry disease, and those identified as female carriers of Fabry disease. These recommendations are the opinions of a multicenter working group of genetic counselors, medical geneticists, and other health professionals with expertise in Fabry disease counseling, as well as an individual with Fabry disease who is a founder of a Fabry disease patient advocacy group in the United States. The recommendations are U.S. Preventive Task Force Class III, and they are based on clinical experience, a review of pertinent English-language articles, and reports of expert committees. This document reviews the genetics of Fabry disease, the indications for genetic testing and interpretation of results, psychosocial considerations, and references for professional and patient resources. These recommendations should not be construed as dictating an exclusive course of management, nor does use of such recommendations guarantee a particular outcome. The professional judgment of a healthcare provider, familiar with the facts and circumstances of a specific case, will always supersede these recommendations.

  13. [Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome].

    Science.gov (United States)

    Undas, Anetta; Ryś, Donata; Wegrzyn, Wojciech; Musiał, Jacek

    2002-11-01

    A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.

  14. Clinical prodromes of neurodegeneration in Anderson-Fabry disease

    Science.gov (United States)

    Hughes, Derralynn; Milligan, Alan; Richfield, Linda; Reichmann, Heinz; Mehta, Atul; Schapira, Anthony H.V.

    2015-01-01

    Objective: To estimate the prevalence of prodromal clinical features of neurodegeneration in patients with Anderson-Fabry disease (AFD) in comparison to age-matched controls. Methods: This is a single-center, prospective, cross-sectional study in 167 participants (60 heterozygous females and 50 hemizygous males with genetically confirmed AFD, 57 age-matched controls) using a clinical screening program consisting of structured interview, quantitative tests of motor function, and assessments of cognition, depression, olfaction, orthostatic intolerance, pain, REM sleep behavior disorder, and daytime sleepiness. Results: In comparison to age-matched controls (mean age 48.3 years), patients with AFD (mean age 49.0 years) showed slower gait and transfer speed, poorer fine manual dexterity, and lower hand speed, which was independent of focal symptoms due to cerebrovascular disease. Patients with AFD were more severely affected by depression, pain, and daytime sleepiness and had a lower quality of life. These motor and nonmotor manifestations significantly correlated with clinical disease severity. However, patients with AFD did not reveal extrapyramidal motor features or signs of significant cognitive impairment, hyposmia, orthostatic intolerance, or REM sleep behavior disorder, which commonly precede later neurodegenerative disease. In our cohort, there were no differences in neurologic manifestations of AFD between heterozygous females and hemizygous males. Conclusions: Aside from cerebrovascular manifestations and small fiber neuropathy, AFD results in a distinct neurologic phenotype comprising poorer motor performance and specific nonmotor features. In contrast to functional loss of glucocerebrosidase in Gaucher disease, α-galactosidase deficiency in AFD is not associated with a typical cluster of clinical features prodromal for neurodegenerative diseases, such as Parkinson disease. PMID:25762709

  15. Agalsidase benefits renal histology in young patients with Fabry disease.

    Science.gov (United States)

    Tøndel, Camilla; Bostad, Leif; Larsen, Kristin Kampevold; Hirth, Asle; Vikse, Bjørn Egil; Houge, Gunnar; Svarstad, Einar

    2013-01-01

    The effect of early-onset enzyme replacement therapy on renal morphologic features in Fabry disease is largely unknown. Here, we evaluated the effect of 5 years of treatment with agalsidase alfa or agalsidase beta in 12 consecutive patients age 7-33 years (median age, 16.5 years). We performed renal biopsies at baseline and after 5 years of enzyme replacement therapy; 7 patients had additional biopsies after 1 and 3 years. After a median of 65 months, biopsy findings from all patients showed total clearance of glomerular endothelial and mesangial cell inclusions, and findings from 2 patients showed complete clearance of inclusions from epithelial cells of the distal tubule. The 4 patients who received the highest dose of agalsidase exhibited substantial clearance of podocyte inclusions, and the youngest patient had nearly complete clearance of these inclusions. Linear regression analysis showed a highly significant correlation between podocyte globotriaocylceramide clearance and cumulative agalsidase dose (r=0.804; P=0.002). Microalbuminuria normalized in five patients. In summary, long-term enzyme replacement therapy in young patients can result in complete globotriaocylceramide clearance of mesangial and glomerular endothelial cells across all dosage regimens, and clearance of podocyte inclusions is dose-dependent.

  16. The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and family studies

    Science.gov (United States)

    Ferreira, Susana; Ortiz, Alberto; Germain, Dominique P.; Viana-Baptista, Miguel; Gomes, António Caldeira; Camprecios, Marta; Fenollar-Cortés, Maria; Gallegos-Villalobos, Ángel; Garcia, Diego; García-Robles, José Antonio; Egido, Jesús; Gutiérrez-Rivas, Eduardo; Herrero, José Antonio; Mas, Sebastián; Oancea, Raluca; Péres, Paloma; Salazar-Martín, Luis Manuel; Solera-Garcia, Jesús; Alves, Helena; Garman, Scott C.; Oliveira, João Paulo

    2015-01-01

    Summary Lysosomal α-galactosidase A (α-Gal) is the enzyme deficient in Fabry disease (FD), an X-linked glycosphingolipidosis caused by pathogenic mutations affecting the GLA gene. The early-onset, multi-systemic FD classical phenotype is associated with absent or severe enzyme deficiency, as measured by in vitro assays, but patients with higher levels of residual α-Gal activity may have later-onset, more organ-restricted clinical presentations. A change in the codon 118 of the wild-type α-Gal sequence, replacing basic arginine by a potentially sulfhydryl-binding cysteine residue – GLA p.(Arg118Cys) –, has been recurrently described in large FD screening studies of high-risk patients. Although the Cys118 allele is associated with high residual α-Gal activity in vitro, it has been classified as a pathogenic mutation, mainly on the basis of theoretical arguments about the chemistry of the cysteine residue. However its pathogenicity has never been convincingly demonstrated by pathology criteria. We reviewed the clinical, biochemical and histopathology data obtained from 22 individuals of Portuguese and Spanish ancestry carrying the Cys118 allele, including 3 homozygous females. Cases were identified either on the differential diagnosis of possible FD manifestations and on case-finding studies (n=11; 4 males), or on unbiased cascade screening of probands’ close relatives (n=11; 3 males). Overall, those data strongly suggest that the GLA p.(Arg118Cys) variant does not segregate with FD clinical phenotypes in a Mendelian fashion, but might be a modulator of the multifactorial risk of cerebrovascular disease, since the allelic frequency in stroke patients was 0.0087 (p=0.0185 vs the general population). The Cys118 allelic frequency in healthy Portuguese adults (n=696) has been estimated as 0.001, therefore not qualifying for “rare” condition. PMID:25468652

  17. Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry

    Science.gov (United States)

    Ortiz, Alberto; Abiose, Ademola; Bichet, Daniel G; Cabrera, Gustavo; Charrow, Joel; Germain, Dominique P; Hopkin, Robert J; Jovanovic, Ana; Linhart, Aleš; Maruti, Sonia S; Mauer, Michael; Oliveira, João P; Patel, Manesh R; Politei, Juan; Waldek, Stephen; Wanner, Christoph; Yoo, Han-Wook; Warnock, David G

    2016-01-01

    Background Agalsidase β is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low α-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. In clinical trials, agalsidase β cleared glycolipid deposits from endothelial cells within 6 months; clearance from other cell types required sustained treatment. We hypothesised that there might be a ‘lag time’ to clinical benefit after initiating agalsidase β treatment, and analysed the incidence of severe clinical events over time in patients receiving agalsidase β. Methods The incidence of severe clinical events (renal failure, cardiac events, stroke, death) was studied in 1044 adult patients (641 men, 403 women) enrolled in the Fabry Registry who received agalsidase β (average dose 1 mg/kg every 2 weeks) for up to 5 years. Results The incidence of all severe clinical events was 111 per 1000 person-years (95% CI 84 to 145) during the first 6 months. After 6 months, the incidence decreased and remained stable within the range of 40–58 events per 1000 patient-years. The largest decrease in incidence rates was among male patients and those aged ≥40 years when agalsidase β was initiated. Conclusions Contrary to the expected increased incidence of severe clinical events with time, adult patients with Fabry disease had decreased incidence of severe clinical events after 6 months treatment with agalsidase β 1 mg/kg every 2 weeks. Trial registration number NCT00196742. PMID:26993266

  18. Plasma globotriaosylsphingosine: diagnostic value and relation to clinical manifestations of Fabry disease.

    Science.gov (United States)

    Rombach, S M; Dekker, N; Bouwman, M G; Linthorst, G E; Zwinderman, A H; Wijburg, F A; Kuiper, S; Vd Bergh Weerman, M A; Groener, J E M; Poorthuis, B J; Hollak, C E M; Aerts, J M F G

    2010-09-01

    Fabry disease is an X-linked lysosomal storage disorder due to deficiency of alpha-Galactosidase A, causing accumulation of globotriaosylceramide and elevated plasma globotriaosylsphingosine (lysoGb3). The diagnostic value and clinical relevance of plasma lysoGb3 concentration was investigated. All male and adult female patients with classical Fabry disease could be discerned by an elevated plasma lysoGb3. In young pre-symptomatic Fabry heterozygotes, lysoGb3 levels can be normal. Individuals carrying the R112H and P60L mutations, without classical Fabry symptoms, showed no elevated plasma lysoGb3. Multiple regression analysis showed that there is no correlation of plasma lysoGb3 concentration with total disease severity score in Fabry males. However, plasma lysoGb3 concentration did correlate with white matter lesions (odds ratio: 6.1 per 100 nM lysoGb3 increase (95% CI: 1.4-25.9, p=0.015). In females, plasma lysoGb3 concentration correlated with overall disease severity. Furthermore, plasma lysoGb3 level was related to left ventricular mass (19.5+/-5.5 g increase per 10 nM lysoGb3 increase; p=0.001). In addition, it was assessed whether lifetime exposure to lysoGb3 correlates with disease manifestations. Male Fabry patients with a high lysoGb3 exposure (>10,000 U), were moderately or severely affected, only one mildly. Female patients with a low exposure (1000 U showed disease complications. Plasma lysoGb3 is useful for the diagnosis of Fabry disease. LysoGb3 is an independent risk factor for development of cerebrovascular white matter lesions in male patients and left ventricular hypertrophy in females. Disease severity correlates with exposure to plasma lysoGb3.

  19. Positron Emission Tomography and Magnetic Resonance Imaging of the Brain in Fabry Disease

    DEFF Research Database (Denmark)

    Korsholm, Kirsten; Feldt-Rasmussen, Ulla; Granqvist, Henrik;

    2015-01-01

    risk of cerebrovascular disease at a young age in addition to heart and kidney failure. OBJECTIVE: The objective of this study was to assess brain function and structure in the Danish cohort of patients with Fabry disease in a prospective way using 18-fluoro-deoxyglucose (F-18 FDG) positron emission....... CONCLUSION: Our data indicated that, in patients with Fabry disease, MRI is the preferable clinical modality--if applicable--when monitoring cerebral status, as no additional major brain-pathology was detected with FDG-PET.......BACKGROUND: Fabry disease is a rare metabolic glycosphingolipid storage disease caused by deficiency of the lysosomal enzyme α-galactosidase A--leading to cellular accumulation of globotriasylceramide in different organs, vessels, tissues, and nerves. The disease is associated with an increased...

  20. Fabry Disease: A Turkish Case with a Novel Mutation and Dermatological Manifestations

    Directory of Open Access Journals (Sweden)

    Neslihan Onenli Mungan

    2015-03-01

    Full Text Available Fabry disease is a rare, X-linked disease, caused by the deficiency of lysosomal and #945;-galactosidase. Clinical fetaures are; acroparesthesia, unexplained fever, hypohidrosis and angiokeratomas. Untreated cases die early from cardiac complications, renal insuffiency or stroke. Currently there is no cure for Fabry disease, enzyme replacement therapy is the only choice in this progressive disease. A 9-year-old boy admitted to the Dermatology Clinic with reddish papular skin lesions, joint pain and anhydrosis. Hystological examination of the skin biopsy revealed angiokeratoma. There was no renal dysfunction or proteinuria. Biochemical confirmation of Fabry disease was made by determining the deficient leukocyte and #945;-galactosidase activity. Subsequently, the patient's molecular analysis was identified a novel nonsense mutation c. 785G>T in the GLA gene. Enzyme replacement therapy with agalsidase beta was started. He is on enzyme replacement therapy for 8 years, significant improvement was obtained in severity and frequency of pain crisis and fatigue. We report this case to emphasize the importance of early diagnosis of Fabry disease restricted to dermatological findings, especially before renal and cardiac involvement occurs, while enzyme replacement therapy is now available. Also this patient is one of the first Fabry patients under enzyme replacement therapy in Turkey. [Cukurova Med J 2015; 40(Suppl 1: 156-160

  1. Copious Podocyturia without Proteinuria and with Normal Renal Function in a Young Adult with Fabry Disease

    Science.gov (United States)

    Trimarchi, H.; Canzonieri, R.; Muryan, A.; Schiel, A.; Forrester, M.; Karl, A.; Lombi, F.; Andrews, J.; Pomeranz, V.; Rengel, T.; Zotta, E.

    2015-01-01

    The time for starting a patient with Fabry disease on enzyme replacement therapy is still a matter of debate, particularly when no overt classical clinical signs or symptoms are present. With respect to Fabry nephropathy, a dual problem coexists: the reluctance of many nephrologists to start enzyme replacement infusion until signs of renal disease appear as the appearance of proteinuria or an elevation in serum creatinine and the lack of validated biomarkers of early renal damage. In this regard, proteinuria is nowadays considered as an early and appropriate marker of kidney disease and of cardiovascular morbidity and mortality. However, in this report we demonstrate that podocyturia antedates the classical appearance of proteinuria and could be considered as an even earlier biomarker of kidney damage. Podocyturia may be a novel indication for the initiation of therapy in Fabry disease. PMID:26064721

  2. Copious Podocyturia without Proteinuria and with Normal Renal Function in a Young Adult with Fabry Disease

    Directory of Open Access Journals (Sweden)

    H. Trimarchi

    2015-01-01

    Full Text Available The time for starting a patient with Fabry disease on enzyme replacement therapy is still a matter of debate, particularly when no overt classical clinical signs or symptoms are present. With respect to Fabry nephropathy, a dual problem coexists: the reluctance of many nephrologists to start enzyme replacement infusion until signs of renal disease appear as the appearance of proteinuria or an elevation in serum creatinine and the lack of validated biomarkers of early renal damage. In this regard, proteinuria is nowadays considered as an early and appropriate marker of kidney disease and of cardiovascular morbidity and mortality. However, in this report we demonstrate that podocyturia antedates the classical appearance of proteinuria and could be considered as an even earlier biomarker of kidney damage. Podocyturia may be a novel indication for the initiation of therapy in Fabry disease.

  3. Fabry disease mimicking hypertrophic cardiomyopathy: genetic screening needed for establishing the diagnosis in women

    DEFF Research Database (Denmark)

    Havndrup, Ole; Christiansen, Michael; Stoevring, Birgitte

    2010-01-01

    AIMS: Fabry disease, an X-linked storage disorder caused by defective lysosomal enzyme alpha-galactosidase A activity, may resemble sarcomere-gene-associated hypertrophic cardiomyopathy (HCM). The 'cardiac variant' of Fabry disease which only affects the heart may be missed unless specifically...... tested for. METHODS AND RESULTS: We evaluated 90 consecutively recruited HCM probands and their relatives. Probands without sarcomere-gene mutations were tested for alpha-galactosidase A gene (GLA) mutations. Of the 90 families, 31 (34%) had sarcomere gene mutations and were therefore excluded...... without sarcomere gene mutations. GLA mutations were found in 3/90 (3%) of HCM families and in 2/20 (10%) of females without sarcomere-gene mutations. None of the probands presented other indices of Fabry disease. This, in combination with putative reversibility of cardiac changes by enzyme replacement...

  4. Receptor-mediated endocytosis of α-galactosidase A in human podocytes in Fabry disease

    DEFF Research Database (Denmark)

    Prabakaran, Thaneas; Nielsen, Rikke; Larsen, Jakob Vejby;

    2011-01-01

    Injury to the glomerular podocyte is a key mechanism in human glomerular disease and podocyte repair is an important therapeutic target. In Fabry disease, podocyte injury is caused by the intracellular accumulation of globotriaosylceramide. This study identifies in the human podocyte three endocy...

  5. Receptor-Mediated Endocytosis of α-Galactosidase A in Human Podocytes in Fabry Disease

    DEFF Research Database (Denmark)

    Prabakaran, Thaneas; Nielsen, Rikke; Larsen, Jakob Vejby;

    2011-01-01

    Injury to the glomerular podocyte is a key mechanism in human glomerular disease and podocyte repair is an important therapeutic target. In Fabry disease, podocyte injury is caused by the intracellular accumulation of globotriaosylceramide. This study identifies in the human podocyte three endocy...

  6. [Fabry disease: clinic and enzymatic diagnosis of homozygous and heterozygous. New therapeutic prospects].

    Science.gov (United States)

    Peces, R; Olea, T

    2002-01-01

    Fabry disease is an X-linked recessive lysosomal storage disorder caused by a partial or complete deficiency of alpha-galactosidase A. Intracellular accumulation of globotriaosylceramide, the glycolipid substrate of this enzyme, leads to severe painful neuropathy with progressive renal, cardiovascular, and cerebrovascular dysfunction and early death. Men are predominantly affected but many female carriers have similar clinical involvement, including increased risk of stroke. Physical stigmata, such as angiokeratomas in skin and mucous membranes and characteristic benign corneal abnormalities, facilitate identification of Fabry disease. The finding of a marked decreased activity of (alpha-galactosidase A in plasma, white blood cells or cultured skin fibroblasts confirms the diagnosis. Treatment thus far has been symptomatic only. Etiology-based therapies are being developed that include enzyme replacement therapy, gene therapy, and substrate deprivation. The recently completed double-blind, placebo-controlled trials of intravenous infusions of (alpha-galactosidase A in patients with Fabry disease demonstrated the safety and efficacy of this treatment. We report a family with Fabry disease composed of hemicygous and heterocygous. The propositus developed chronic renal failure and received a cadaver renal transplant, which remained with adequate renal function during 15 years.

  7. Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.

    Directory of Open Access Journals (Sweden)

    John Marshall

    Full Text Available Fabry disease is an X-linked glycosphingolipid storage disorder caused by a deficiency in the activity of the lysosomal hydrolase α-galactosidase A (α-gal. This deficiency results in accumulation of the glycosphingolipid globotriaosylceramide (GL-3 in lysosomes. Endothelial cell storage of GL-3 frequently leads to kidney dysfunction, cardiac and cerebrovascular disease. The current treatment for Fabry disease is through infusions of recombinant α-gal (enzyme-replacement therapy; ERT. Although ERT can markedly reduce the lysosomal burden of GL-3 in endothelial cells, variability is seen in the clearance from several other cell types. This suggests that alternative and adjuvant therapies may be desirable. Use of glucosylceramide synthase inhibitors to abate the biosynthesis of glycosphingolipids (substrate reduction therapy, SRT has been shown to be effective at reducing substrate levels in the related glycosphingolipidosis, Gaucher disease. Here, we show that such an inhibitor (eliglustat tartrate, Genz-112638 was effective at lowering GL-3 accumulation in a mouse model of Fabry disease. Relative efficacy of SRT and ERT at reducing GL-3 levels in Fabry mouse tissues differed with SRT being more effective in the kidney, and ERT more efficacious in the heart and liver. Combination therapy with ERT and SRT provided the most complete clearance of GL-3 from all the tissues. Furthermore, treatment normalized urine volume and uromodulin levels and significantly delayed the loss of a nociceptive response. The differential efficacies of SRT and ERT in the different tissues indicate that the combination approach is both additive and complementary suggesting the possibility of an improved therapeutic paradigm in the management of Fabry disease.

  8. Anti-BlyS antibody reduces the immune reaction against enzyme and enhances the efficacy of enzyme replacement therapy in Fabry disease model mice.

    Science.gov (United States)

    Sato, Yohei; Ida, Hiroyuki; Ohashi, Toya

    2017-02-02

    Formation of antibodies against a therapeutic enzyme is an important complication during enzyme replacement therapy (ERT) for lysosomal storage diseases. Fabry disease (FD) is caused by a deficiency of alpha-galactosidase (GLA), which results in the accumulation of globotriaosylceramide (GL-3). We have shown immune tolerance induction (ITI) during ERT in FD model mice by using an anti-B lymphocyte stimulator (anti-BlyS) antibody (belimumab). A single dose of the anti-BlyS antibody temporarily lowered the percentage of B cells and IgG antibody titer against recombinant human GLA. Administration of a low maintenance dose of the anti-BlyS antibody suppressed the B cell population and immunotolerance was induced in 20% of mice, but antibody formation could not be prevented. We then increased the maintenance dose of the anti-BlyS antibody and immunotolerance was induced in 50% of mice. Therapeutic enzyme distribution and clearance of GL-3 were also enhanced by a high maintenance dose of the anti-BlyS antibody.

  9. Functional and structural nerve fiber findings in heterozygote patients with Fabry disease

    DEFF Research Database (Denmark)

    Møller, Anette Torvin; Bach, Flemming Winther; Feldt-Rasmussen, Ulla;

    2009-01-01

    recently disease manifestations in female carriers of Fabry disease have been questioned. To explore the frequency of symptoms and the functional and structural involvement of the nervous system in female patients we examined the presence of pain, manifestations of peripheral neuropathy and nerve density...... in skin biopsies in 19 female patients with Fabry disease and 19 sex- and age-matched controls. Diaries, quantitative sensory testing, neurophysiologic tests and skin biopsies were performed. Daily pain was present in 63% of patients, with a median VAS score of 4.0. Tactile detection threshold...... and pressure pain threshold were lower and cold detection thresholds increased in patients. Sensory nerve action potential amplitude and maximal sensory conduction velocity were not different, whereas there was a highly significant reduction in intraepidermal nerve fiber density. We found no correlation...

  10. Screening for Fabry Disease in Left Ventricular Hypertrophy: Documentation of a Novel Mutation

    Energy Technology Data Exchange (ETDEWEB)

    Baptista, Ana, E-mail: baptista-ana@hotmail.com; Magalhães, Pedro; Leão, Sílvia; Carvalho, Sofia; Mateus, Pedro; Moreira, Ilídio [Centro Hospitalar de Trás-os-Montes e Alto Douro, Unidade de Vila Real (Portugal)

    2015-08-15

    Fabry disease is a lysosomal storage disease caused by enzyme α-galactosidase A deficiency as a result of mutations in the GLA gene. Cardiac involvement is characterized by progressive left ventricular hypertrophy. To estimate the prevalence of Fabry disease in a population with left ventricular hypertrophy. The patients were assessed for the presence of left ventricular hypertrophy defined as a left ventricular mass index ≥ 96 g/m{sup 2} for women or ≥ 116 g/m{sup 2} for men. Severe aortic stenosis and arterial hypertension with mild left ventricular hypertrophy were exclusion criteria. All patients included were assessed for enzyme α-galactosidase A activity using dry spot testing. Genetic study was performed whenever the enzyme activity was decreased. A total of 47 patients with a mean left ventricular mass index of 141.1 g/m{sup 2} (± 28.5; 99.2 to 228.5 g/m{sup 2}] were included. Most of the patients were females (51.1%). Nine (19.1%) showed decreased α-galactosidase A activity, but only one positive genetic test − [GLA] c.785G>T; p.W262L (exon 5), a mutation not previously described in the literature. This clinical investigation was able to establish the association between the mutation and the clinical presentation. In a population of patients with left ventricular hypertrophy, we documented a Fabry disease prevalence of 2.1%. This novel case was defined in the sequence of a mutation of unknown meaning in the GLA gene with further pathogenicity study. Thus, this study permitted the definition of a novel causal mutation for Fabry disease - [GLA] c.785G>T; p.W262L (exon 5)

  11. Altered dynamics of a lipid raft associated protein in a kidney model of Fabry disease.

    Science.gov (United States)

    Labilloy, Anatália; Youker, Robert T; Bruns, Jennifer R; Kukic, Ira; Kiselyov, Kirill; Halfter, Willi; Finegold, David; do Monte, Semiramis Jamil Hadad; Weisz, Ora A

    2014-02-01

    Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused by deficiency of the enzyme alpha-galactosidase A (α-gal A). These lipids are incorporated into the plasma membrane and intracellular membranes, with a preference for lipid rafts. Disruption of raft mediated cell processes is implicated in the pathogenesis of several human diseases, but little is known about the effects of the accumulation of glycosphingolipids on raft dynamics in the context of Fabry disease. Using siRNA technology, we have generated a polarized renal epithelial cell model of Fabry disease in Madin-Darby canine kidney cells. These cells present increased levels of Gb3 and enlarged lysosomes, and progressively accumulate zebra bodies. The polarized delivery of both raft-associated and raft-independent proteins was unaffected by α-gal A knockdown, suggesting that accumulation of Gb3 does not disrupt biosynthetic trafficking pathways. To assess the effect of α-gal A silencing on lipid raft dynamics, we employed number and brightness (N&B) analysis to measure the oligomeric status and mobility of the model glycosylphosphatidylinositol (GPI)-anchored protein GFP-GPI. We observed a significant increase in the oligomeric size of antibody-induced clusters of GFP-GPI at the plasma membrane of α-gal A silenced cells compared with control cells. Our results suggest that the interaction of GFP-GPI with lipid rafts may be altered in the presence of accumulated Gb3. The implications of our results with respect to the pathogenesis of Fabry disease are discussed.

  12. Renal histology before and after effective enzyme replacement therapy in a patient with classical Fabry's disease.

    Science.gov (United States)

    Hirashio, S; Taguchi, T; Naito, T; Maki, K; Ogata, S; Taniyama, K; Taniguchi, Y; Yorioka, N

    2009-05-01

    A 38-year-old man underwent renal biopsy because of proteinuria. It revealed swelling and vacuolation of glomerular epithelial cells, as well as myelin-like structures characteristic of Fabry's disease. Detection of decreased plasma activity of alpha-galactosidase A confirmed the diagnosis. Enzyme replacement therapy was provided with recombinant agalsidase-beta, resulting in improvement of his symptoms. When renal biopsy was repeated, specific staining for globotriaosylceramide showed that renal deposits were decreased by enzyme therapy.

  13. Safety and pharmacodynamic effects of a pharmacological chaperone on α-galactosidase A activity and globotriaosylceramide clearance in Fabry disease: report from two phase 2 clinical studies

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    Germain Dominique P

    2012-11-01

    Full Text Available Abstract Background Fabry disease (FD is a genetic disorder resulting from deficiency of the lysosomal enzyme α-galactosidase A (α-Gal A, which leads to globotriaosylceramide (GL-3 accumulation in multiple tissues. We report on the safety and pharmacodynamics of migalastat hydrochloride, an investigational pharmacological chaperone given orally at 150 mg every-other-day. Methods Two open-label uncontrolled phase 2 studies of 12 and 24 weeks (NCT00283959 and NCT00283933 in 9 males with FD were combined. At multiple time points, α-Gal A activity and GL-3 levels were quantified in blood cells, kidney and skin. GL-3 levels were also evaluated through skin and renal histology. Results Compared to baseline, increased α-Gal A activity of at least 50% was demonstrated in blood, skin and kidney in 6 of 9 patients. Patients’ increased α-Gal A activities paralleled the α-Gal A increases observed in vitro in HEK-293 cells transfected with the corresponding mutant form of the enzyme. The same 6 patients who demonstrated increases of α-Gal A activity also had GL-3 reduction in skin, urine and/or kidney, and had α-Gal A mutations that responded in transfected cells incubated with the drug. The 3 patients who did not show a consistent response in vivo had α-Gal A mutations that did not respond to migalastat HCl in transfected cells. Migalastat HCl was well tolerated. Conclusions Migalastat HCl is a candidate pharmacological chaperone that provides a novel genotype-specific treatment for FD. It enhanced α-Gal A activity and resulted in GL-3 substrate decrease in patients with responsive GLA mutations. Phase 3 studies are ongoing. Trial registration Clinicaltrial.gov: NCT00283959 and NCT00283933

  14. Home infusion program for Fabry disease: experience with agalsidase alfa in Argentina

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    Isaac Kisinovsky

    2013-02-01

    Full Text Available Fabry disease is an X-linked lysosomal storage disorder caused by inherited deficiency of the enzyme α-galactosidase A. Enzyme replacement treatment using agalsidase alfa significantly reduces pain, improves cardiac function and quality of life, and slows renal deterioration. Nevertheless, it is a life-long treatment which requires regular intravenous infusions and entails a great burden for patients. Our objective was to evaluate retrospectively the safety and tolerability of the home infusion of agalsidase alfa in patients with Fabry disease in Argentina. We evaluated all the patients with Fabry disease who received home infusion with agalsidase alfa 0.2 mg/kg between January 2005 and June 2011. The program included 87 patients; 51 males (mean age: 30 years and 36 females (mean age: 34 years. A total of 5229 infusions (mean: 59 per patient; range: 1-150 were administered. A total of 5 adverse reactions were seen in 5 patients (5.7% of patients and 0.9% of the total number of infusions. All were mild in severity and resolved by reducing the rate of infusion and by using antihistaminics. All these 5 patients were positive for IgG antibodies, but none of them presented IgE antibodies and none suffered an anaphylactic shock. In our group 18 patients were switched from agalsidase beta to agalsidase alfa without complications. Home infusion with agalsidase alfa is safe, well tolerated and is associated to high compliance.

  15. Fabry disease : Baseline medical characteristics of a cohort of 1765 males and females in the Fabry Registry

    NARCIS (Netherlands)

    Eng, C. M.; Fletcher, J.; Wilcox, W. R.; Waldek, S.; Scott, C. R.; Sillence, D. O.; Breunig, F.; Charrow, J.; Germain, D. P.; Nicholls, K.; Banikazemi, M.

    2007-01-01

    The Fabry Registry is a global observational research platform established to define outcome data on the natural and treated course of this rare disorder. Participating physicians submit structured longitudinal data to a centralized, confidential database. This report describes the baseline demograp

  16. Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease

    DEFF Research Database (Denmark)

    Hughes, Derralynn A; Nicholls, Kathleen; Shankar, Suma P

    2017-01-01

    therapy (ERT), stabilises specific mutant (amenable) forms of α-Gal to facilitate normal lysosomal trafficking. METHODS: The main objective of the 18-month, randomised, active-controlled ATTRACT study was to assess the effects of migalastat on renal function in patients with Fabry disease previously...... ventricular mass index decreased significantly with migalastat treatment (-6.6 g/m(2) (-11.0 to -2.2)); there was no significant change with ERT. Predefined renal, cardiac or cerebrovascular events occurred in 29% and 44% of patients in the migalastat and ERT groups, respectively. Plasma...... globotriaosylsphingosine remained low and stable following the switch from ERT to migalastat. PROs were comparable between groups. Migalastat was generally safe and well tolerated. CONCLUSIONS: Migalastat offers promise as a first-in-class oral monotherapy alternative treatment to intravenous ERT for patients with Fabry...

  17. COMPUTER ASSISTED RETINAL VESSEL TORTUOSITY EVALUATION IN NOVEL MUTATION FABRY DISEASE: Towards New Prognostic Markers.

    Science.gov (United States)

    San Román, Irene; Rodríguez, María-Elena; Caporossi, Orsola; Zoppetti, Claudia; Sodi, Andrea; Mecocci, Alessandro; López, David; Rodríguez, Beatriz; Gimeno, Juan-Ramón

    2017-03-01

    Fabry disease is a rare lysosomal storage disorder with systemic involvement. The authors report on a large Fabry family with GLA p.M187R mutation and exhaustive ophthalmologic assessment. Comprehensive systemic evaluation and genetic diagnosis were performed. Ophthalmologic evaluation included intraocular pressure/visual acuity measurement, refractometry, slit lamp examination, retinography, and optical coherence tomography. Three parameters quantified retinal vessel tortuosity: sum of angle metrics, product of angle distance, and triangular index. Calculations were semiautomatized using dedicated software. Ten individuals (2 males and 8 females) were described. Seventy-five percent had retinal vessel tortuosity. One hundred percent had cornea verticillata. Perimacular vessels were predominantly involved. The correlation between the right and left eye tortuosity measurements was very tight. A significant correlation between retinal vessel tortuosity and systemic severity measured by general Mainz Severity Score Index (MSSI), renal MSSI, and neurological MSSI but no cardiac MSSI was observed. Right sum of angle metrics value was an independent statistical predictor of the general-MSSI score in presence of age. p.M187R mutation causes a severe systemic and ophthalmologic phenotype, in both male and female patients. Semiautomatic assessment of retinal vessel tortuosity is an objective and reproducible tool. All three parameters of tortuosity are closely associated with Fabry severity scores. Studies of larger series are being awaited to establish the role of retinal vessel tortuosity as a noninvasive marker of disease progression.

  18. Skin-impedance in Fabry Disease: A prospective, controlled, non-randomized clinical study

    Directory of Open Access Journals (Sweden)

    Lidicker Jeffrey R

    2008-11-01

    Full Text Available Abstract Background We previously demonstrated improved sweating after enzyme replacement therapy (ERT in Fabry disease using the thermo-regularity sweat and quantitative sudomotor axon reflex tests. Skin-impedance, a measure skin-moisture (sweating, has been used in the clinical evaluation of burns and pressure ulcers using the portable dynamic dermal impedance monitor (DDIM system. Methods We compared skin impedance measurements in hemizygous patients with Fabry disease (22 post 3-years of bi-weekly ERT and 5 ERT naive and 22 healthy controls. Force compensated skin-moisture values were used for statistical analysis. Outcome measures included 1 moisture reading of the 100th repetitive reading, 2 rate of change, 3 average of 60–110th reading and 4 overall average of all readings. Results All outcome measures showed a significant difference in skin-moisture between Fabry patients and control subjects (p Conclusion The instrument portability, ease of its use, a relatively short time required for the assessment, and the fact that DDIM system was able to detect the difference in skin-moisture renders the instrument a useful clinical tool.

  19. Evaluación de pacientes con enfermedad de Fabry en la Argentina Evaluation of patients with Fabry disease in Argentina

    Directory of Open Access Journals (Sweden)

    2010-02-01

    Full Text Available La enfermedad de Fabry es un desorden lisosomal de transmisión ligada al cromosoma X debida al déficit de la enzima alfa galactosidasa A, con acumulación multisistémica de globotriaosilceramida y compromiso neurológico, gastrointestinal, cardíaco, renal, dermatológico y oftalmológico. Estudios recientes indican que las mujeres heterocigotas desarrollan síntomas similares a los de los varones, pero no existen en nuestro país datos comparativos respecto de la frecuencia relativa de manifestaciones clínicas, edad de inicio y gravedad entre hombres y mujeres con enfermedad de Fabry. Identificamos 59 pacientes adultos sintomáticos con enfermedad de Fabry: 32 varones (edad media: 34.8 años y 27 mujeres (edad media: 46.6 años. El diagnóstico se hizo por estudios enzimáticos en los hombres y genéticos en las mujeres. Se evaluó la frecuencia y la gravedad de las manifestaciones de la enfermedad. Las manifestaciones más frecuentes fueron acroparestesias, angioqueratomas, hipohidrosis y córnea verticilada; las tres primeras fueron estadísticamente más frecuentes en hombres, en los cuales la gravedad de estos síntomas fue significativamente mayor. Proteinuria e hipertrofia ventricular izquierda fueron hallazgos frecuentes tanto en hombres como en mujeres. Hubo una latencia prolongada entre la edad del inicio y la del diagnóstico de 14 años para varones y 30 para mujeres. La enfermedad de Fabry es una enfermedad subdiagnosticada y potencialmente letal que afecta a ambos sexos. La existencia de reemplazo enzimático obliga a identificar precozmente los síntomas y signos sugestivos de la enfermedad para realizar un diagnóstico y tratamiento precoces.Fabry disease is an X- linked lysosomal disorder due to deficient activity of the enzyme alpha galactosidase A which leads to multisystemic storage of globotriaosylceramide with neurologic, gastrointestinal, cardiac, renal, skin and ophtalmological involvement. Recent studies indicate

  20. Genetic Screening of Mutations Associated with Fabry Disease in a Nationwide Cohort of Juvenile Idiopathic Arthritis Patients

    Science.gov (United States)

    Gonçalves, Maria J.; Mourão, Ana F.; Martinho, António; Simões, Olívia; Melo-Gomes, José; Salgado, Manuel; Estanqueiro, Paula; Ribeiro, Célia; Brito, Iva; Fonseca, João E.; Canhão, Helena

    2017-01-01

    Fabry’s disease (FD) is a lysosomal storage disorder associated with an alpha-galactosidase A deficiency. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients with established diagnosis is unknown, but as musculoskeletal pain may be an important complaint at presentation, misdiagnosed cases are anticipated. With this study, we aim to calculate the frequency of FD-associated mutations in a cohort of JIA patients. Children with JIA from a national cohort were selected. Clinical and laboratorial information was recorded in the Portuguese rheumatic diseases register (http://Reuma.pt). Molecular genetic testing to detect GLA gene mutations was performed. After the multiplex polymerase chain reactions technique for DNA amplification, direct sequencing of the complete sequence of GLA gene was completed. From a cohort of 292 patients with JIA (188 females, 104 males), mutations were identified in 5 patients (all female). Four patients had the mutation D313Y, a rare GLA variant, which is associated with low enzymatic levels in plasma, but normal lysosomal levels. One patient presented the missense mutation R118C, which was previously described in Mediterranean patients with FD. This is the first screening of FD mutations in a cohort of JIA patients. No “classic” pathogenic FD mutations were reported. The late-onset FD-associated mutation, R118C, was found in a frequency of 0.34% (1/292). PMID:28299312

  1. Morphological features of iPS cells generated from Fabry disease skin fibroblasts using Sendai virus vector (SeVdp).

    Science.gov (United States)

    Kawagoe, Shiho; Higuchi, Takashi; Otaka, Manami; Shimada, Yohta; Kobayashi, Hiroshi; Ida, Hiroyuki; Ohashi, Toya; Okano, Hirotaka J; Nakanishi, Mahito; Eto, Yoshikatsu

    2013-08-01

    We generated iPS cells from human dermal fibroblasts (HDFs) of Fabry disease using a Sendai virus (SeVdp) vector; this method has been established by Nakanishi et al. for pathogenic evaluation. We received SeVdp vector from Nakanishi and loaded it simultaneously with four reprogramming factors (Klf4, Oct4, Sox2, and c-Myc) to HDFs of Fabry disease; subsequently, we observed the presence of human iPS-like cells. The Sendai virus nucleocapsid protein was not detected in the fibroblasts by RT-PCR analysis. Additionally, we confirmed an undifferentiated state, alkaline phosphatase staining, and the presence of SSEA-4, TRA-1-60, and TRA-1-81. Moreover, ultrastructural features of these iPS cells included massive membranous cytoplasmic bodies typical of HDFs of Fabry disease. Thus, we successfully generated human iPS cells from HDFs of Fabry disease that retained the genetic conditions of Fabry disease; also, these abnormal iPS cells could not be easily differentiated into mature cell types such as neuronal cells, cardiomyocytes, etc. because of a massive accumulation of membranous cytoplasmic bodies in lysosomes, possibly the persistent damages of intracellular architecture.

  2. The right ventricle in Fabry disease: natural history and impact of enzyme replacement therapy.

    Science.gov (United States)

    Niemann, Markus; Breunig, Frank; Beer, Meinrad; Herrmann, Sebastian; Strotmann, Jörg; Hu, Kai; Emmert, Andrea; Voelker, Wolfram; Ertl, Georg; Wanner, Christoph; Weidemann, Frank

    2010-12-01

    Storage of globotriaosylceramides is present in the left and right ventricles of patients with Fabry disease. Improvement of left ventricular morphology and function during enzyme replacement therapy (ERT) has previously been reported. To analyse the effects of long term ERT on right ventricular morphology and function. This was a prospective follow-up of 75 genetically confirmed consecutive Fabry patients for 3.1±1.8 years. According to treatment guidelines the natural history was followed in 18 patients, whereas 57 patients received ERT. Standard echocardiography, strain rate imaging for regional deformation of the right and left ventricle, and magnetic resonance tomography with late enhancement (LE) imaging for the detection of fibrosis were all performed at yearly intervals. Right ventricular hypertrophy was evident in 53 patients (71%) at baseline. A significant positive correlation was found between left and right ventricular wall thickness (r=0.76; pright ventricle. Patients with LE in the left ventricle presented with the lowest right ventricular deformation properties. In contrast to the left ventricle, there was no change in right ventricular wall thickness (baseline 6.9±1.6 mm vs follow-up 6.7±1.5 mm; p=0.44) or systolic strain rate (2.2±0.7/s vs 2.1±0.8/s; p=0.31) during follow-up with ERT. The degree of right ventricular involvement in Fabry disease is related to the left ventricular cardiomyopathy stage. ERT seems to have no direct impact on right ventricular morphology and function.

  3. Therapy of Fabry disease with pharmacological chaperones: from in silico predictions to in vitro tests

    Directory of Open Access Journals (Sweden)

    Cammisa Marco

    2011-10-01

    Full Text Available Abstract Background Fabry disease is a rare disorder caused by a large variety of mutations in the gene encoding lysosomal alpha-galactosidase. Many of these mutations are unique to individual families. Fabry disease can be treated with enzyme replacement therapy, but a promising novel strategy relies on small molecules, so called "pharmacological chaperones", which can be administered orally. Unfortunately only 42% of genotypes respond to pharmacological chaperones. Results A procedure to predict which genotypes responsive to pharmacological chaperones in Fabry disease has been recently proposed. The method uses a position-specific substitution matrix to score the mutations. Using this method, we have screened public databases for predictable responsive cases and selected nine representative mutations as yet untested with pharmacological chaperones. Mutant lysosomal alpha galactosidases were produced by site directed mutagenesis and expressed in mammalian cells. Seven out of nine mutations responded to pharmacological chaperones. Nineteen other mutations that were tested with pharmacological chaperones, but were not included in the training of the predictive method, were gathered from literature and analyzed in silico. In this set all five mutations predicted to be positive were responsive to pharmacological chaperones, bringing the percentage of responsive mutations among those predicted to be positive and not used to train the classifier to 86% (12/14. This figure differs significantly from the percentage of responsive cases observed among all the Fabry mutants tested so far. Conclusions In this paper we provide experimental support to an "in silico" method designed to predict missense mutations in the gene encoding lysosomal alpha galactosidase responsive to pharmacological chaperones. We demonstrated that responsive mutations can be predicted with a low percentage of false positive cases. Most of the mutations tested to validate the method

  4. Long Term Treatment with Enzyme Replacement Therapy in Patients with Fabry Disease.

    Science.gov (United States)

    Oder, Daniel; Nordbeck, Peter; Wanner, Christoph

    2016-01-01

    Anderson-Fabry disease is a potentially life-threatening hereditary lysosomal storage disorder taking origin in over 1,000 known pathogenic mutations in the alpha-galactosidase A encoding gene. Over the past 15 years, intravenous replacement therapy of the deficient alpha agalsidase A enzyme has been well-established retarding the progression of a multisystemic disease and organ involvement. Despite this innovative treatment approach, premature deaths still do occur. The response to enzyme replacement therapy (ERT) varies considerably and appears to depend on gender, genotype (classic or later onset/non-classic), stage of disease or age and agalsidase inhibition by anti-agalsidase antibodies. Early ERT treatment at young age, a personalized approach, and adjunctive therapies for specific disease manifestations appear to impact on prognosis and are currently favored with the expectance of more effective intravenous and oral treatments in the short future.

  5. Accidente cerebro-vascular en la enfermedad de Fabry: Algo más que una simple estenosis Stroke in Fabry disease: More than a simple stenosis

    Directory of Open Access Journals (Sweden)

    Juan Manuel Politei

    2006-10-01

    Full Text Available Se analiza la evidencia existente a la fecha sobre los mecanismos fisiopatológicos que pueden generar accidentes cerebrovasculares en la enfermedad de Fabry. Esta entidad es el resultado de la deficiencia de a-galactosidasa A, lo que resulta en depósito patológico de glicoesfingilípidos en distintas poblaciones celulares. Asociados a la insuficiencia renal y cardíaca, los accidentes cerebrovasculares pueden derivar en la muerte de los pacientes. Durante mucho tiempo el único mecanismo generador de daño vascular informado fue la oclusión vascular por depósito lipídico a nivel endotelial. En la actualidad se describen otros mecanismos. El advenimiento de la terapia de reemplazo enzimático ha generado gran expectativa en cuanto la posibilidad de reversión de estos mecanismos. Si bien la evidencia es escasa y son necesarios más estudios a largo plazo, algunos informes demuestran que luego de meses, el tratamiento ha logrado revertir algunos de los mecanismos implicados.The objective is to analyze the updated evidence on the physiopathological mechanisms that can generate cerebrovascular damage in Fabry disease. Fabry disease is the result of the deficiency of a-galactosidasa A, which causes pathological storage of glycosphingolipids, in different cells. Associated to renal and cardiac insufficiency, cerebrovascular complications can derive in the death of the patients. During a long time the only reported mechanism was the vascular occlusion by deposit of lipids at endothelial level. At the present time, other mechanisms are postulated. The arrival of enzyme replacement therapy has generated great expectation on the possibility of reversion of these alterations. Although the evidence is scarce and more long term studies are necessary, some reports demonstrate that after months, the treatment has managed to revert some of the mechanisms involved.

  6. Expert opinion on temporary treatment recommendations for Fabry disease during the shortage of enzyme replacement therapy (ERT)

    NARCIS (Netherlands)

    G.E. Linthorst; D.P. Germain; C.E.M. Hollak; D. Hughes; A. Rolfs; C. Wanner; A. Mehta

    2011-01-01

    The shortage of enzyme for treatment of Fabry disease has caused anxiety among patients, physicians and governments. Following a request from the European Medicines Agency, consensus was reached on the temporary prioritization of patients for treatment based on disease severity and potential reversi

  7. Monitoring renal function in children with Fabry disease: comparisons of measured and creatinine-based estimated glomerular filtration rate

    NARCIS (Netherlands)

    C. Tondel; U. Ramaswami; K.M. Aakre; F. Wijburg; M. Bouwman; E. Svarstad

    2010-01-01

    Methods. Eighty-two examinations were done in 42 children (24 boys, 18 girls) with Fabry disease from three different centres. The mean age was 12.3 years. GFR was measured with iohexol, Cr-51-EDTA or iothalamate, and the mean mGFR was 108 ml/min/1.73 m(2). Results. The widely used original Schwartz

  8. Recommendations for the inclusion of Fabry disease as a rare febrile condition in existing algorithms for fever of unknown origin.

    Science.gov (United States)

    Manna, Raffaele; Cauda, Roberto; Feriozzi, Sandro; Gambaro, Giovanni; Gasbarrini, Antonio; Lacombe, Didier; Livneh, Avi; Martini, Alberto; Ozdogan, Huri; Pisani, Antonio; Riccio, Eleonora; Verrecchia, Elena; Dagna, Lorenzo

    2017-07-19

    Fever of unknown origin (FUO) is a rather rare clinical syndrome representing a major diagnostic challenge. The occurrence of more than three febrile attacks with fever-free intervals of variable duration during 6 months of observation has recently been proposed as a subcategory of FUO, Recurrent FUO (RFUO). A substantial number of patients with RFUO have auto-inflammatory genetic fevers, but many patients remain undiagnosed. We hypothesize that this undiagnosed subgroup may be comprised of, at least in part, a number of rare genetic febrile diseases such as Fabry disease. We aimed to identify key features or potential diagnostic clues for Fabry disease as a model of rare genetic febrile diseases causing RFUO, and to develop diagnostic guidelines for RFUO, using Fabry disease as an example of inserting other rare diseases in the existing FUO algorithms. An international panel of specialists in recurrent fevers and rare diseases, including internists, infectious disease specialists, rheumatologists, gastroenterologists, nephrologists, and medical geneticists convened to review the existing diagnostic algorithms, and to suggest recommendations for arriving at accurate diagnoses on the basis of available literature and clinical experience. By combining specific features of rare diseases with other diagnostic considerations, guidelines have been designed to raise awareness and identify rare diseases among other causes of FUO. The proposed guidelines may be useful for the inclusion of rare diseases in the diagnostic algorithms for FUO. A wide spectrum of patients will be needed to validate the algorithm in different clinical settings.

  9. One Year of Enzyme Replacement Therapy Reduces Globotriaosylceramide Inclusions in Podocytes in Male Adult Patients with Fabry Disease

    Science.gov (United States)

    Najafian, Behzad; Tøndel, Camilla; Svarstad, Einar; Sokolovkiy, Alexey; Smith, Kelly; Mauer, Michael

    2016-01-01

    Fabry nephropathy is associated with progressive accumulation of globotriaosylceramide (GL3) in podocytes. Reducing this GL3 burden may reduce podocyte injury. Sensitive methods to quantify podocyte GL3 content may determine whether a given strategy can benefit podocytes in Fabry disease. We developed an unbiased electron microscopic stereological method to estimate the average volume of podocytes and their GL3 inclusions in 6 paired pre- and post-enzyme replacement therapy (ERT) biopsies from 5 men with Fabry disease. Podocyte GL3 content was regularly reduced (average 73%) after 11–12 months of ERT. This was not detectable using a semi-quantitative approach. Parallel to GL3 reduction, podocytes became remarkably smaller (average 63%). These reductions in podocyte GL3 content or size were not significantly correlated with changes in foot process width (FPW). However, FPW after ERT was significantly correlated with the magnitude of the decrease in podocyte GL3 content from baseline to 11–12 months of ERT. Also podocytes exocytosed GL3 inclusions, a phenomenon correlated with their reduction in their GL3 content. Demonstrable after11–12 months, reduction in podocyte GL3 content allows for early assessment of treatment efficacy and shorter clinical trials in Fabry disease. PMID:27081853

  10. Immunohistochemical diagnosis of Fabry nephropathy and localisation of globotriaosylceramide deposits in paraffin-embedded kidney tissue sections.

    Science.gov (United States)

    Valbuena, Carmen; Leitão, Dina; Carneiro, Fátima; Oliveira, João Paulo

    2012-02-01

    Fabry disease (FD) is a rare X-linked lysosomal storage disorder of glycosphingolipids, mostly globotriaosylceramide (Gb3). Proteinuric chronic kidney disease develops frequently, and recognition of Fabry nephropathy on a kidney biopsy may be the first clue to the underlying diagnosis. Since the accumulated glycosphingolipids are largely extracted by the paraffin-embedding procedure, the most characteristic feature of Fabry nephropathy on routine light microscopy (LM) is nonspecific cell vacuolization. To test whether residual Gb3 in kidney tissue might be exploited for the specific diagnosis of Fabry nephropathy, paraffin-embedded kidney biopsies of nine FD patients (one boy, four men, four women) and of a female carrier of a mild genetic mutation, with no evidence of Fabry nephropathy, were immunostained with an anti-Gb3 antibody. The adult biopsies were additionally co-stained with a lysosomal marker (anti-lysosomal-associated membrane protein 2 (anti-LAMP2) antibody). The distribution of Gb3 deposits was scored per cell type and compared to the histological scorings of glycosphingolipid inclusions on semi-thin sections. FD patients had residual Gb3 in all types of glomerular, tubular, interstitial and vascular kidney cells. The highest expression of LAMP2 was seen in tubular cells, but there were no meaningful associations between LAMP2 expression and prevalence of Gb3 deposits on different kidney cell types. The histological scorings of glycosphingolipid inclusions were relatively higher than the corresponding immunohistochemical scorings of Gb3 deposits. In the mildly affected female, Gb3 expression was limited to tubular cells, a pattern similar to controls. Gb3 immunostaining allows the specific diagnosis of Fabry nephropathy even in kidney biopsies routinely processed for LM.

  11. [Globosides as key players in the pathophysiology of Shiga toxin-associated acute kidney failure and Fabry disease].

    Science.gov (United States)

    Porubsky, S

    2014-11-01

    Globosides and their isomeric counterparts isoglobosides belong to the class of neutral glycosphingolipids with an as yet undefined physiological function. In the pathogenesis of human diseases, globosides play an important role as cellular receptors for Shiga toxins which are produced by certain strains of S. dysenteriae and E. coli. In order to elucidate the pathogenesis of Shiga toxin-associated kidney failure, we studied human kidney biopsies and animal models. Our work showed that in patients suffering from Shiga toxin-elicited kidney failure, no complement activation could be demonstrated by immunohistochemical analysis of kidney biopsies. Therefore, complement activation is unlikely to play a major role in mediating thrombotic microangiopathy on exposure to Shiga toxin. Moreover, analysis of the human biopsies and of a murine model of Shiga toxin-associated disease pinpointed acute tubular damage as an important and previously neglected contributor to acute kidney failure in patients infected with Shiga toxin-producing E. coli. Furthermore, globosides play a decisive role in the pathogenesis of Fabry disease which results from a decreased or absent activity of the lysosomal enzyme α-galactosidase A. The results on transgenic mice showed that in vital organs, such as the heart, kidneys and liver, it was possible to revert the phenotype of Fabry disease by eliminating the synthesis of globosides. This implicates that substrate reduction therapy through inhibition of globosides might represent a new therapeutic option for Fabry disease, all the more so as globosides seem to be dispensable.

  12. Postmortem diagnosis of Fabry disease with acromegaly and a unique vasculopathy.

    Science.gov (United States)

    Takao, Masaki; Mori, Taisuke; Orikasa, Hideki; Oh, Haengphil; Suzuki, Kinuko; Koto, Atsuo; Yamazaki, Kazuto

    2007-09-01

    A 44-year-old Japanese man with elevated growth hormone levels and gradual deterioration of mental and renal function was admitted to the hospital. With his deteriorated general condition and renal failure, the patient developed pulmonary thromboembolism and died of respiratory failure. Autopsy examination was conducted, which revealed abnormal accumulation or intracytoplasmic storage of lipid-rich material in the small blood vessels, kidney, heart, and nervous system. After postmortem pathologic studies, including light-microscopic histochemistry, electron microscopy, and biochemical analysis of the stored lipid contents, a final diagnosis of Fabry disease was made. Histopathologic examination revealed a unique vasculopathy characterized by the presence of abnormal intracytoplasmic lipid inclusions and vascular remodeling. With regard to the clinical presentation of acromegaly, hyperplasia but not adenomatous transformation of the acidophils of the anterior pituitary gland with immunohistochemical detection of growth hormone within the cells was noted. In this case, the complication of acromegaly with hyperplasia of the acidophilic cells of the anterior pituitary gland and the unique vasculopathy causing significant organ failure, mainly of the kidney, heart, and central nervous systems, possibly as a result of microcirculatory failure, are considered to be not incidental findings but to be intimately involved in the pathogenesis of Farby disease.

  13. The attenuated/late onset lysosomal storage disorders: Therapeutic goals and indications for enzyme replacement treatment in Gaucher and Fabry disease.

    Science.gov (United States)

    Hollak, Carla E M; Weinreb, Neal J

    2015-03-01

    Enzyme replacement therapies have been developed and authorized for commercial use for six different lysosomal storage disorders. For Gaucher disease, Fabry disease and mucopolysaccharidosis type 1, disease-specific treatments have been available for more than a decade. Although long term follow-up data are still sparse, therapeutic goals for patients with Gaucher disease and Fabry disease have been formulated and published for both adults and children. Without adaptation or modification, these goals are often applied in clinical research and in routine patient care across the entire phenotypic spectrum of disease, although in practice, patients commonly manifest high variability in clinical presentation and course of the illness. In this context, establishing goals for the follow-up and treatment of late onset/attenuated phenotypes is particularly challenging. In this chapter, we review current therapeutic goals for Gaucher disease and Fabry disease and discuss approaches for those with attenuated disease manifestations.

  14. Anderson-Fabry, the histrionic disease: from genetics to clinical management

    Directory of Open Access Journals (Sweden)

    Franco Cecchi

    2013-02-01

    Full Text Available Anderson-Fabry disease (AFD is an Xlinked lysosomal storage disorder of glycosphingolipid catabolism, due to deficiency or absence of a galactosidase A (α-gal A enzyme. The disease may affect males and females, the latter with an average 10 years delay. Metabolites storage (mostly Gb3 and lyso-Gb3 leads to progressive cellular and multiorgan dysfunction, with either early and late onset variable clinical manifestations that usually reduce quality of life and life expectancy. Heart and kidney failure, stroke and sudden death are the most devastating complications. AFD is always been considered a very rare disease, although new epidemiologic data, based on newborn screening, showed that AFD prevalence is probably underestimated and much higher than previously reported, especially for late-onset atypical phenotypes. Currently, the diagnosis may be easier and simpler by evaluating α-gal A enzyme activity and genetic analysis for GLA gene mutations on dried blood spot. While a marked α-gal A deficiency leads to diagnosis of AFD in hemizygous males, the molecular analysis is mandatory in heterozygous females. However, referral to a center with an expert multidisciplinary team is highly advisable, in order to ensure careful management and treatment of patients, based also on accurate molecular and biochemical data interpretation. While long-term efficacy of enzyme replacement therapy (ERT in advanced stage is still debated, increasing evidence shows greater efficacy of early treatment initiation. Concomitant, organ-specific therapy is also needed. New treatment approaches, such as chemical chaperone therapy, alone or in combination with ERT, are currently under investigation. The present review illustrates the major features of the disease, focusing also on biochemical and genetic aspects.

  15. Angiotensinogen promoter and angiotensinogen II receptor type 1 gene polymorphisms and incidence of ischemic stroke and neurologic phenotype in Fabry disease.

    Science.gov (United States)

    Altarescu, Gheona; Haim, Shimon; Elstein, Deborah

    2013-11-01

    Stroke and/or white matter lesions (WMLs) are significant in Fabry disease. Polymorphisms of angiotensinogen (AGT), AGT Promoter and angiotensinogen II receptor type 1 (AGTR1) are correlated with WMLs. We compared AGT. AGT Promoter and AGTR1 genotypes to stroke incidence, Fabry-specific [Mainz Severity Score Index (MSSI)] severity score, and neurologic sub-score (n-MSSI). Sixty-three Fabry patients and 49 matched controls plus historic controls were genotyped. Institutional Review Board approval was received. Results. C and/or CC alleles of AGT Promoter and AGTR1 were significantly correlated with stroke and n-MSSI. Findings are suggestive of role of AGT Promoter and AGTR1 genotypes in Fabry phenotypes.

  16. Inhibition of Intermediate-Conductance Calcium-Activated K Channel (KCa3.1) and Fibroblast Mitogenesis by α-Linolenic Acid and Alterations of Channel Expression in the Lysosomal Storage Disorders, Fabry Disease, and Niemann Pick C

    Science.gov (United States)

    Oliván-Viguera, Aida; Lozano-Gerona, Javier; López de Frutos, Laura; Cebolla, Jorge J.; Irún, Pilar; Abarca-Lachen, Edgar; García-Malinis, Ana J.; García-Otín, Ángel Luis; Gilaberte, Yolanda; Giraldo, Pilar; Köhler, Ralf

    2017-01-01

    The calcium/calmodulin-gated KCa3.1 channel regulates normal and abnormal mitogenesis by controlling K+-efflux, cell volume, and membrane hyperpolarization-driven calcium-entry. Recent studies suggest modulation of KCa3.1 by omega-3 fatty acids as negative modulators and impaired KCa3.1 functions in the inherited lysosomal storage disorder (LSD), Fabry disease (FD). In the first part of present study, we characterize KCa3.1 in murine and human fibroblasts and test the impact of omega-3 fatty acids on fibroblast proliferation. In the second, we study whether KCa3.1 is altered in the LSDs, FD, and Niemann-Pick disease type C (NPC). Our patch-clamp and mRNA-expression studies on murine and human fibroblasts show functional expression of KCa3.1. KCa currents display the typical pharmacological fingerprint of KCa3.1: Ca2+-activation, potentiation by the positive-gating modulators, SKA-31 and SKA-121, and inhibition by TRAM-34, Senicapoc (ICA-17043), and the negative-gating modulator, 13b. Considering modulation by omega-3 fatty acids we found that α-linolenic acid (α-LA) and docosahexanenoic acid (DHA) inhibit KCa3.1 currents and strongly reduce fibroblast growth. The α-LA-rich linseed oil and γ-LA-rich borage oil at 0.5% produce channel inhibition while α-LA/γ-LA-low oils has no anti-proliferative effect. Concerning KCa3.1 in LSD, mRNA expression studies, and patch-clamp on primary fibroblasts from FD and NPC patients reveal lower KCa3.1-gene expression and membrane expression than in control fibroblasts. In conclusion, the omega-3 fatty acid, α-LA, and α-LA/γ-LA-rich plant oils, inhibit fibroblast KCa3.1 channels and mitogenesis. Reduced fibroblast KCa3.1 functions are a feature and possible biomarker of cell dysfunction in FD and NPC and supports the concept that biased lipid metabolism is capable of negatively modulating KCa3.1 expression. PMID:28197106

  17. Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel

    Directory of Open Access Journals (Sweden)

    Sommer Claudia

    2011-05-01

    Full Text Available Abstract Background Fabry disease is an inherited metabolic disorder characterized by progressive lysosomal accumulation of lipids in a variety of cell types, including neural cells. Small, unmyelinated nerve fibers are particularly affected and small fiber peripheral neuropathy often clinically manifests at young age. Peripheral pain can be chronic and/or occur as provoked attacks of excruciating pain. Manifestations of dysfunction of small autonomic fibers may include, among others, impaired sweating, gastrointestinal dysmotility, and abnormal pain perception. Patients with Fabry disease often remain undiagnosed until severe complications involving the kidney, heart, peripheral nerves and/or brain have arisen. Methods An international expert panel convened with the goal to provide guidance to clinicians who may encounter unrecognized patients with Fabry disease on how to diagnose these patients early using simple diagnostic tests. A further aim was to offer recommendations to control neuropathic pain. Results We describe the neuropathy in Fabry disease, focusing on peripheral small fiber dysfunction - the hallmark of early neurologic involvement in this disorder. The clinical course of peripheral pain is summarized, and the importance of medical history-taking, including family history, is highlighted. A thorough physical examination (e.g., angiokeratoma, corneal opacities and simple non-invasive sensory perception tests could provide clues to the diagnosis of Fabry disease. Reported early clinical benefits of enzyme replacement therapy include reduction of neuropathic pain, and adequate management of residual pain to a tolerable and functional level can substantially improve the quality of life for patients. Conclusions Our recommendations can assist in diagnosing Fabry small fiber neuropathy early, and offer clinicians guidance in controlling peripheral pain. This is particularly important since management of pain in young patients with

  18. Lentivector Iterations and Pre-Clinical Scale-Up/Toxicity Testing: Targeting Mobilized CD34+ Cells for Correction of Fabry Disease

    Directory of Open Access Journals (Sweden)

    Ju Huang

    2017-06-01

    Full Text Available Fabry disease is a rare lysosomal storage disorder (LSD. We designed multiple recombinant lentivirus vectors (LVs and tested their ability to engineer expression of human α-galactosidase A (α-gal A in transduced Fabry patient CD34+ hematopoietic cells. We further investigated the safety and efficacy of a clinically directed vector, LV/AGA, in both ex vivo cell culture studies and animal models. Fabry mice transplanted with LV/AGA-transduced hematopoietic cells demonstrated α-gal A activity increases and lipid reductions in multiple tissues at 6 months after transplantation. Next we found that LV/AGA-transduced Fabry patient CD34+ hematopoietic cells produced even higher levels of α-gal A activity than normal CD34+ hematopoietic cells. We successfully transduced Fabry patient CD34+ hematopoietic cells with “near-clinical grade” LV/AGA in small-scale cultures and then validated a clinically directed scale-up transduction process in a GMP-compliant cell processing facility. LV-transduced Fabry patient CD34+ hematopoietic cells were subsequently infused into NOD/SCID/Fabry (NSF mice; α-gal A activity corrections and lipid reductions were observed in several tissues 12 weeks after the xenotransplantation. Additional toxicology studies employing NSF mice xenotransplanted with the therapeutic cell product demonstrated minimal untoward effects. These data supported our successful clinical trial application (CTA to Health Canada and opening of a “first-in-the-world” gene therapy trial for Fabry disease.

  19. Patients with Fabry Disease after Enzyme Replacement Therapy Dose Reduction and Switch-2-Year Follow-Up.

    Science.gov (United States)

    Lenders, Malte; Canaan-Kühl, Sima; Krämer, Johannes; Duning, Thomas; Reiermann, Stefanie; Sommer, Claudia; Stypmann, Jörg; Blaschke, Daniela; Üçeyler, Nurcan; Hense, Hans-Werner; Brand, Stefan-Martin; Wanner, Christoph; Weidemann, Frank; Brand, Eva

    2016-03-01

    Because of the shortage of agalsidase-β supply between 2009 and 2012, patients with Fabry disease either were treated with reduced doses or were switched to agalsidase-α. In this observational study, we assessed end organ damage and clinical symptoms with special focus on renal outcome after 2 years of dose-reduction and/or switch to agalsidase-α. A total of 89 adult patients with Fabry disease who had received agalsidase-β (1.0 mg/kg body wt) for >1 year were nonrandomly assigned to continue this treatment regimen (regular-dose group, n=24), to receive a reduced dose of 0.3-0.5 mg/kg and a subsequent switch to 0.2 mg/kg agalsidase-α (dose-reduction-switch group, n=28), or to directly switch to 0.2 mg/kg agalsidase-α (switch group, n=37) and were followed-up for 2 years. We assessed clinical events (death, myocardial infarction, severe arrhythmia, stroke, progression to ESRD), changes in cardiac and renal function, Fabry-related symptoms (pain, hypohidrosis, diarrhea), and disease severity scores. Determination of renal function by creatinine and cystatin C-based eGFR revealed decreasing eGFRs in the dose-reduction-switch group and the switch group. The Mainz Severity Score Index increased significantly in these two groups (P=0.02 and Pswitch or a direct switch to agalsidase-α showed a decline of renal function independent of the eGFR formula used.

  20. Long-term enzyme replacement therapy is associated with reduced proteinuria and preserved proximal tubular function in women with Fabry disease

    DEFF Research Database (Denmark)

    Prabakaran, Thaneas; Birn, Henrik; Bibby, Bo M;

    2014-01-01

    BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficiency of α-galactosidase A (α-Gal A) causes intracellular accumulations of globotriaosylceramide (GL-3) and related glycosphingolipids in all organs, including the kidney, often leading...... to end-stage renal failure. In women with Fabry disease, accumulation of GL-3 in the glomerular podocytes and other renal cells induces progressive, proteinuric nephropathy, but not as severe as in men. Enzyme replacement therapy (ERT) with recombinant α-Gal A reduces cellular GL-3 deposits in podocytes...

  1. Oftalmopatía fabry : nuevos marcadores pronósticos para una enfermedad sistémica= Fabry ophthalmopathy: new prognostic markers for a systemic disease

    OpenAIRE

    2016-01-01

    INTRODUCCIÓN: La enfermedad de Fabry es un raro error innato del metabolismo de los glicoesfingolípidos perteneciente al grupo más numeroso de las enfermedades por depósito lisosomal, las lipidosis. Se estima una prevalencia de 1/40000 varones. Sigue un patrón de herencia dominante ligado a X y se origina por mutaciones en el gen GLA (Xq221). Estas mutaciones resultan en una enzima lisosomal α-galactosidasa-A (AGA) con actividad deficiente. El depósito ubícuo de moléculas glicolipídicas...

  2. Home infusion program for Fabry disease: experience with agalsidase alfa in Argentina Programa de infusión domiciliaria para la enfermedad de Fabry: experiencia con agalsidasa alfa en la Argentina

    Directory of Open Access Journals (Sweden)

    Isaac Kisinovsky

    2013-02-01

    Full Text Available Fabry disease is an X-linked lysosomal storage disorder caused by inherited deficiency of the enzyme α-galactosidase A. Enzyme replacement treatment using agalsidase alfa significantly reduces pain, improves cardiac function and quality of life, and slows renal deterioration. Nevertheless, it is a life-long treatment which requires regular intravenous infusions and entails a great burden for patients. Our objective was to evaluate retrospectively the safety and tolerability of the home infusion of agalsidase alfa in patients with Fabry disease in Argentina. We evaluated all the patients with Fabry disease who received home infusion with agalsidase alfa 0.2 mg/kg between January 2005 and June 2011. The program included 87 patients; 51 males (mean age: 30 years and 36 females (mean age: 34 years. A total of 5229 infusions (mean: 59 per patient; range: 1-150 were administered. A total of 5 adverse reactions were seen in 5 patients (5.7% of patients and 0.9% of the total number of infusions. All were mild in severity and resolved by reducing the rate of infusion and by using antihistaminics. All these 5 patients were positive for IgG antibodies, but none of them presented IgE antibodies and none suffered an anaphylactic shock. In our group 18 patients were switched from agalsidase beta to agalsidase alfa without complications. Home infusion with agalsidase alfa is safe, well tolerated and is associated to high compliance.La enfermedad de Fabry es un trastorno de almacenamiento lisosomal hereditario ligado al cromosoma X ocasionado por el déficit de la enzima alfa galactosidasa A. La terapia de reemplazo enzimático utilizando agalsidasa alfa reduce significativamente el dolor, mejora la función cardíaca y la calidad de vida y enlentece el deterioro renal. Sin embargo, es un tratamiento de por vida que requiere infusiones intravenosas regulares y supone una gran carga para los pacientes. Nuestro objetivo fue evaluar retrospectivamente la

  3. Right ventricular involvement in patients with Fabry's disease and the effect of enzyme replacement therapy

    Energy Technology Data Exchange (ETDEWEB)

    Wuest, W. [Universitaetsklinikum Erlangen (Germany). Radiologisches Inst.; Machann, W.; Koestler, H.; Hahn, D.; Beer, M. [Universitaetsklinikum Wuerzburg (Germany). Inst. fuer Roentgendiagnostik; Breunig, F.; Weidemann, F.; Wanner, C. [Universitaetsklinikum Wuerzburg (Germany). Medizinische Klinik I

    2011-11-15

    According to echocardiography reports, Fabry cardiomyopathy not only affects the left ventricle (LV) but also the right ventricle (RV). Until now no MRI studies about the effect of enzyme replacement therapy (ERT) on the RV are available. We evaluated the effect of ERT on the RV. In this prospective trial 14 patients with genetically proven Fabry's disease were examined using a 1.5 T MR scanner before ERT and after 13 {+-} 1 months of ERT. All patients underwent cardiac MR imaging and the RV/LV cardiac morphology and function were analyzed. At baseline examination the values were as follows: RV mass 31 {+-} 6 g/m{sup 2}, end-diastolic volume (EDV) 88 {+-} 13 ml/m{sup 2}, end-systolic volume (ESV) 39 {+-} 9 ml/m{sup 2}, stroke volume (SV) 49 {+-} 7 ml/m{sup 2} and ejection fraction (EF) 56 {+-} 5 %. The RV mass and EDV decreased significantly after 13 {+-} 1 months on ERT (mass 27 {+-} 7 g/m{sup 2}, p < 0.05, EDV 76 {+-} 24 ml/m{sup 2}, p < 0.05), with no significant change of ESV (33 {+-} 13 ml/m{sup 2}), SV (43 {+-} 12 ml/m{sup 2}) and EF (57 {+-} 7 %). The LV mass (102 {+-} 26 g/m{sup 2} vs. 94 {+-} 27 g/m{sup 2}, p < 0.05), EDV (76 {+-} 13 ml/m{sup 2} vs. 66 {+-} 22 ml/m{sup 2}, p < 0.05) and ESV (29 {+-} 9 ml/m{sup 2} vs. 23 {+-} 9 ml/m{sup 2}, p < 0.05) decreased significantly while the EF (64 {+-} 7 % vs. 66 {+-} 5 %; p < 0.05) increased significantly. Besides the known beneficial effect on the LV, ERT improves RV mass and EDV. (orig.)

  4. Identification of an Allosteric Binding Site on Human Lysosomal Alpha-Galactosidase Opens the Way to New Pharmacological Chaperones for Fabry Disease

    Science.gov (United States)

    den-Haan, Helena; Pérez-Sánchez, Horacio; Del Prete, Rosita; Liguori, Ludovica; Cimmaruta, Chiara; Lukas, Jan; Andreotti, Giuseppina

    2016-01-01

    Personalized therapies are required for Fabry disease due to its large phenotypic spectrum and numerous different genotypes. In principle, missense mutations that do not affect the active site could be rescued with pharmacological chaperones. At present pharmacological chaperones for Fabry disease bind the active site and couple a stabilizing effect, which is required, to an inhibitory effect, which is deleterious. By in silico docking we identified an allosteric hot-spot for ligand binding where a drug-like compound, 2,6-dithiopurine, binds preferentially. 2,6-dithiopurine stabilizes lysosomal alpha-galactosidase in vitro and rescues a mutant that is not responsive to a mono-therapy with previously described pharmacological chaperones, 1-deoxygalactonojirimycin and galactose in a cell based assay. PMID:27788225

  5. Characterization of Early Disease Status in Treatment-Naive Male Paediatric Patients with Fabry Disease Enrolled in a Randomized Clinical Trial

    Science.gov (United States)

    Wijburg, Frits A.; Bénichou, Bernard; Bichet, Daniel G.; Clarke, Lorne A.; Dostalova, Gabriela; Fainboim, Alejandro; Fellgiebel, Andreas; Forcelini, Cassiano; An Haack, Kristina; Hopkin, Robert J.; Mauer, Michael; Najafian, Behzad; Scott, C. Ronald; Shankar, Suma P.; Thurberg, Beth L.; Tøndel, Camilla; Tylki-Szymańska, Anna; Ramaswami, Uma

    2015-01-01

    Trial Design This analysis characterizes the degree of early organ involvement in a cohort of oligo-symptomatic untreated young patients with Fabry disease enrolled in an ongoing randomized, open-label, parallel-group, phase 3B clinical trial. Methods Males aged 5–18 years with complete α-galactosidase A deficiency, without symptoms of major organ damage, were enrolled in a phase 3B trial evaluating two doses of agalsidase beta. Baseline disease characteristics of 31 eligible patients (median age 12 years) were studied, including cellular globotriaosylceramide (GL-3) accumulation in skin (n = 31) and kidney biopsy (n = 6; median age 15 years; range 13–17 years), renal function, and glycolipid levels (plasma, urine). Results Plasma and urinary GL-3 levels were abnormal in 25 of 30 and 31 of 31 patients, respectively. Plasma lyso-GL-3 was elevated in all patients. GL-3 accumulation was documented in superficial skin capillary endothelial cells (23/31 patients) and deep vessel endothelial cells (23/29 patients). The mean glomerular filtration rate (GFR), measured by plasma disappearance of iohexol, was 118.1 mL/min/1.73 m2 (range 90.4–161.0 mL/min/1.73 m2) and the median urinary albumin/creatinine ratio was 10 mg/g (range 4.0–27.0 mg/g). On electron microscopy, renal biopsy revealed GL-3 accumulation in all glomerular cell types (podocytes and parietal, endothelial, and mesangial cells), as well as in peritubular capillary and non-capillary endothelial, interstitial, vascular smooth muscle, and distal tubules/collecting duct cells. Lesions indicative of early Fabry arteriopathy and segmental effacement of podocyte foot processes were found in all 6 patients. Conclusions These data reveal that in this small cohort of children with Fabry disease, histological evidence of GL-3 accumulation, and cellular and vascular injury are present in renal tissues at very early stages of the disease, and are noted before onset of microalbuminuria and development of

  6. Rapid preparation of (3R,4S,5R) polyhydroxylated pyrrolidine-based libraries to discover a pharmacological chaperone for treatment of Fabry disease.

    Science.gov (United States)

    Cheng, Wei-Chieh; Wang, Jen-Hon; Yun, Wen-Yi; Li, Huang-Yi; Hu, Jia-Ming

    2017-01-27

    The rapid discovery of a pharmacological chaperone toward human α-Gal A for the treatment of Fabry disease is described. Two polyhydroxylated pyrrolidines with the (3R,4S,5R) configuration pattern underwent rapid substituent diversity by conjugating the primary aminomethyl moiety of each with a variety of carboxylic acids to generate two libraries (2 × 60 members). Our bioevaluation results showed one member with the (2R,3R,4S,5R) configuration pattern and bearing a 5-cyclohexylpentanoyl group as a substituent moiety possessed sufficient chaperoning capability to rescue α-Gal A activity in the lymphocyte of the N215S Fabry patient-derived cell line and other α-Gal A mutants in COS7 cells.

  7. Home infusion program with enzyme replacement therapy for Fabry disease: The experience of a large Italian collaborative group

    Directory of Open Access Journals (Sweden)

    D. Concolino

    2017-09-01

    We conclude that home infusions in eligible patients with FD are safe, contribute to improve treatment compliance and therapeutic clinical outcomes, and may have a positive impact on self-perceived QoL.

  8. Comprehensive clinical evaluation of a large Spanish family with Anderson-Fabry disease, novel GLA mutation and severe cardiac phenotype.

    Science.gov (United States)

    San Román-Monserrat, Irene; Moreno-Flores, Victoria; López-Cuenca, David; Rodríguez-González-Herrero, Elena; Guillén-Navarro, Encarna; Rodríguez-González-Herrero, Beatriz; Alegría-Fernández, Marisol; Poza-Cisneros, Gabriela; Piñero-Fernández, Juan A; Sornichero-Martínez, Javier; Gimeno-Blanes, Juan R

    2014-06-06

    Fabry disease is an X-linked multisystemic lysosomal-storage condition. We describe a large family with a novel GLA mutation: p.M187R/g7219 T>G. Anamnesis/physical-exam, blood/urine analysis, α-Gal-A activity and/or genetic study of at-risk individuals and multidisciplinary evaluation in confirmed cases. 4 males and 13 heterozygous-females displayed the mutation. Cardiac/renal/neurological disease was diagnosed at a mean age of 41/29/39 years in males and 51/56/46 years in females. Onset mean age was 20 years versus 42 years. 9/15 had cardiomyopathy. Delta wave suggestive of accessory pathway was identified in 1 male and 2 females. 1 female had cardiac arrest (ventricular fibrillation, 61 years). 2 females and 1 male died suddenly (63, 64 and 57 years). Cardiac-subscore of Mainz Severity-Score-Index was severe for males and females over 40 years. 4/15(26%) developed early renal disease. 2 males needed dialysis. 1 male died at 69 years in spite of kidney-heart transplant. We describe the largest genetically confirmed Spanish family using multidisciplinary evaluation and MSSI calculation. The novel mutation p.M187R/g7219 T>G is associated with a particularly malignant cardiac phenotype in males and females over 40 years. Severity was higher than that of the largest Spanish FOS-cohort. Short-PR with delta is being reported for the first time. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  9. Left Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry Cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Johannes Krämer

    Full Text Available In spite of several research studies help to describe the heart in Fabry disease (FD, the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations in geometry have not been systematically evaluated.In 74 FD patients (mean age 36±12 years; 45 females the extent of myocardial fibrosis and its progression were quantified using cardiac magnetic-resonance-imaging with late enhancement technique (LE. Results were compared to standard echocardiography complemented by 2D-speckle-tracking, 3D-sphericity-index (SI and standardized blood pressure measurement. At baseline, no patient received enzyme replacement therapy (ERT. After 51±24 months, a follow-up examination was performed.Systolic blood pressure (SBP was higher in patients with vs. without LE: 123±17 mmHg vs. 115±13 mmHg; P = 0.04. A positive correlation was found between SI and the amount of LE-positive myocardium (r = 0.51; P<0.001 indicating an association of higher SI in more advanced stages of the cardiomyopathy. SI at baseline was positively associated with the increase of LE-positive myocardium during follow-up. The highest SBP (125±19 mmHg and also the highest SI (0.32±0.05 was found in the subgroup with a rapidly increasing LE (ie, ≥0.2% per year; n = 16; P = 0.04. Multivariate logistic regression analysis including SI, SBP, EF, left ventricular volumes, wall thickness and NT-proBNP adjusted for age and sex showed SI as the most powerful parameter to detect rapid progression of LE (AUC = 0.785; P<0.05.LV geometry as assessed by the sphericity index is altered in relation to the stage of the Fabry cardiomyopathy. Although patients with FD are not hypertensive, the SBP has a clear impact on the progression of the cardiomyopathy.

  10. Kidney transplantation from a mother with unrecognized Fabry disease to her son with low α-galactosidase A activity: A 14-year follow-up without enzyme replacement therapy.

    Science.gov (United States)

    Odani, Keiko; Okumi, Masayoshi; Honda, Kazuho; Ishida, Hideki; Tanabe, Kazunari

    2016-07-01

    We report a case of kidney transplantation from mother to son, both of whom were likely to have had an unrecognized renal variant phenotype of Fabry disease. The patient was a 54-year-old man, with an unknown primary cause of end stage renal disease. He had no notable past medical history, other than end stage renal disease. He underwent living-related kidney transplantation from his mother at age 40 years. Foam cells in the glomeruli were identified on histology assessment of a 0-hour allograft biopsy, with zebra bodies identified in the glomerular visceral epithelial cells by electron microscopy. These findings were indicative of Fabry disease in the donated kidney. As a definitive diagnosis of Fabry's disease could not be confirmed, enzyme replacement therapy was not initiated. Thirteen years after kidney transplantation, the patient underwent left nephrectomy for a left renal tumour, with pathological findings of clear cell carcinoma, foam cells and zebra bodies in the native kidney. Detailed examinations identified low α-galactosidase A activity and mutation of the α-Gal A gene, confirming a diagnosis of a renal variant phenotype of Fabry disease. Histology of several allograft biopsies performed over the 14 years from the time of kidney transplantation revealed only moderate interstitial fibrosis and tubular atrophy, with no evidence of disease progression on electron microscopy, despite the presence of zebra bodies in the glomerular visceral epithelial cells.

  11. Surveying quality of life in patients with Fabry disease by the SF-36 scale%SF-36量表评估Fabry病患者生活质量的研究

    Institute of Scientific and Technical Information of China (English)

    欧阳彦; 潘晓霞; 王朝晖; 沈平雁; 王伟铭; 任红; 张文; 陈楠

    2014-01-01

    Objective To test the availability of the SF-36 scale for the Chinese patient with Fabry disease (FD),the quality of life(QOL) and its probable influence factors were analyzed.Methods The data were obtained from 50 healthy volunteers and 57 patients with FD enrolled in nephrology department of Ruijin hospital from Jan,2003 to Jan,2013.The SF-36 scale was used to evaluate the QOL of patients and to compare the difference between the patients and controls.Furthermore,the influencing factors were estimated by multiple linear regressions.Results Between the patients and controls,the differences of 8 dimensionalitiy's scores had statistical significance,which claimed that the reaction was sensitive.Especially,in the patient group,the Pearson correlation coefficient among each domain of the SF-36 was lower than its Cronbach's α coefficient (0.934),which indicated good internal consistency reliability.The two common factors were much the same to the theory assume,which illustrated the construct validity was available.Even the acceptability of the patient' group at 100% proved the scale was appropriate for patients with FD.In addition,in rolephysical (RP) and bodily pain (BP),the scores of the male patients were less than the females (P <0.01),declared that the QOL of the males was inferior to the females.In the physical function (PF) and the mental health (MH),the scores of the patients with angiokeratoma were less than the group without it,so the patients who had evident clinical symptoms more likely contributed to depression.Using multiple linear regression,age,gender and clinical types were chosen into regression equation by stepwise regression,and the main potential predictor was age.Conclusions The SF-36 scale applies to evaluate the QOL of patients with FD.It is critical to concern and manage the QOL of FD patients,especially in their mental health aspect.%目的 调查中国Fabry病患者生存质量(quality of life,QOL),分析其可能的影响因

  12. Investigation of multiple organ involvement in Fabry disease%法布里病患者多器官病变的临床特点分析

    Institute of Scientific and Technical Information of China (English)

    张巍; 康曼德; 赵亚雯; 李凡; 舒俊龙; 左越焕; 刘靖; 黄一宁; 袁云

    2015-01-01

    Objective To investigate incidence and clinical features of multiple organ involvement in Chinese patients with Fabry disease.Methods We collected 151 patients of 31 families with Fabry disease,all of whom were confirmed by classic pathology,decreased α-galactosidase A activity or GLA mutation from the year of 2011 to 2014 in Department of Neurology,Peking University First Hospital.The clinical data included incidence and onset of neuralgia,renal dysfunction,heart disease,hypertension and cerebral stroke.We calculate the incidence of these symptoms and analyze their gender difference using chisquare with SPSS 17.0 software.Results Among 31 families with Fabry disease,151 patients (82 males and 69 females) were affected,including 91 patients (60.26%) with neuralgia,62 patients (41.06%) with renal disease,33 patients (21.85%) with heart disease,33 patients (21.85%) with hypertension,and 21 patients (13.91%) with cerebral stroke.The peak incidence of neuralgia was from 10 to 20 years old,followed by renal disease from 30 to 40 years old,heart disease and stroke from 40 to 50 years old.There is no peak period for hypertension.Except for males with higher incidence of renal dysfunction than females (P < 0.05),there were no gender difference in the incidence of other organ involvement.Conclusion The incidence and onset of multiple organ dysfunction in Chinese patients with Fabry disease displayed special regularity.Gender difference appeared only in renal disease.%目的 总结我国法布里病患者多器官病变的发生率和临床表现特点.方法 收集北京大学第一医院神经内科2001-2014年依据病理改变、α-半乳糖苷酶活性A测定和GLA基因突变检查确诊的31个法布里病家系发病者的临床资料,统计其神经痛、肾脏病变、心脏病变、高血压病和脑卒中的发生率和起病时间,使用SPSS 17.0统计软件对男女患者不同器官损害的发生率进行x2检验,比较男女患者上述

  13. The Large Phenotypic Spectrum of Fabry Disease Requires Graduated Diagnosis and Personalized Therapy: A Meta-Analysis Can Help to Differentiate Missense Mutations

    Directory of Open Access Journals (Sweden)

    Valentina Citro

    2016-12-01

    Full Text Available Fabry disease is caused by mutations in the GLA gene and is characterized by a large genotypic and phenotypic spectrum. Missense mutations pose a special problem for graduating diagnosis and choosing a cost-effective therapy. Some mutants retain enzymatic activity, but are less stable than the wild type protein. These mutants can be stabilized by small molecules which are defined as pharmacological chaperones. The first chaperone to reach clinical trial is 1-deoxygalactonojirimycin, but others have been tested in vitro. Residual activity of GLA mutants has been measured in the presence or absence of pharmacological chaperones by several authors. Data obtained from transfected cells correlate with those obtained in cells derived from patients, regardless of whether 1-deoxygalactonojirimycin was present or not. The extent to which missense mutations respond to 1-deoxygalactonojirimycin is variable and a reference table of the results obtained by independent groups that is provided with this paper can facilitate the choice of eligible patients. A review of other pharmacological chaperones is provided as well. Frequent mutations can have residual activity as low as one-fourth of normal enzyme in vitro. The reference table with residual activity of the mutants facilitates the identification of non-pathological variants.

  14. The Large Phenotypic Spectrum of Fabry Disease Requires Graduated Diagnosis and Personalized Therapy: A Meta-Analysis Can Help to Differentiate Missense Mutations

    Science.gov (United States)

    Citro, Valentina; Cammisa, Marco; Liguori, Ludovica; Cimmaruta, Chiara; Lukas, Jan; Cubellis, Maria Vittoria; Andreotti, Giuseppina

    2016-01-01

    Fabry disease is caused by mutations in the GLA gene and is characterized by a large genotypic and phenotypic spectrum. Missense mutations pose a special problem for graduating diagnosis and choosing a cost-effective therapy. Some mutants retain enzymatic activity, but are less stable than the wild type protein. These mutants can be stabilized by small molecules which are defined as pharmacological chaperones. The first chaperone to reach clinical trial is 1-deoxygalactonojirimycin, but others have been tested in vitro. Residual activity of GLA mutants has been measured in the presence or absence of pharmacological chaperones by several authors. Data obtained from transfected cells correlate with those obtained in cells derived from patients, regardless of whether 1-deoxygalactonojirimycin was present or not. The extent to which missense mutations respond to 1-deoxygalactonojirimycin is variable and a reference table of the results obtained by independent groups that is provided with this paper can facilitate the choice of eligible patients. A review of other pharmacological chaperones is provided as well. Frequent mutations can have residual activity as low as one-fourth of normal enzyme in vitro. The reference table with residual activity of the mutants facilitates the identification of non-pathological variants. PMID:27916943

  15. Fabry-Perot interferometers

    CERN Document Server

    Mora-Hernandez, G

    1988-01-01

    This book describes the Fabry-Perot interferometer and its variants as well as its use, optimisation and applications. The author begins with an historical perspective on the development of the instrument. Because of the quantitative uses of the device, the text tends to be mostly mathematical in its treatment. However, there is also much practical detail on the use and optimization of the Fabry-Perot interferometer and discussion of its classical uses (such as in metrology) and its contemporary applications (such as in lasers). In addition the book contains a comprehensive bibliography summarizing the extensive literature on the subject. This book will appeal both to high-resolution practitioners, such as spectroscopists, and to the laser community, since the Fabrv-Perot is not only an integral part of the laser but is also usea to characterize its optical and spectroscopic behaviour.

  16. First experience of simultaneous PET/MRI for the early detection of cardiac involvement in patients with Anderson-Fabry disease

    Energy Technology Data Exchange (ETDEWEB)

    Nappi, Carmela; Altiero, Michele; Imbriaco, Massimo; Giudice, Caterina Anna; Spinelli, Letizia; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Nicolai, Emanuele; Aiello, Marco; Diomiaiuti, Claudio Tommaso [IRCCS SDN, Naples (Italy); Pisani, Antonio [University Federico II, Department of Public Health, Naples (Italy)

    2015-03-26

    Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with severe multiorgan dysfunction and premature death. Early diagnosis and treatment strategies play a key role in patient outcome. We investigated the potential role of hybrid PET/MR imaging in the assessment of early cardiac involvement in AFD patients. Thirteen AFD patients without cardiac symptoms and with normal left ventricular function underwent simultaneous cardiac PET/MR imaging after administration of {sup 18}F-FDG. Cardiac FDG uptake was quantified by measuring the standardized uptake value in 17 myocardial segments in each subject. The coefficient of variation (COV, i.e. the standard deviation divided by the average) of the uptake of the 17 segments was calculated as an index of heterogeneity in the heart. Six patients exhibited focal late gadolinium enhancement (LGE) indicating intramyocardial fibrosis, and four of these also had positive short inversion time inversion recovery (STIR) sequences. All patients with LGE and positive STIR MR images showed focal FDG uptake in the corresponding myocardial segments indicating inflammation. Of the seven patients with negative LGE and STIR images, five showed homogeneous FDG cardiac uptake and two showed heterogeneous FDG uptake. The COV was significantly greater in patients with focal FDG uptake (0.25 ± 0.02) than in those without (0.14 ± 0.07, p < 0.01). PET/MR imaging is clinically feasible for the early detection of cardiac involvement in patients with AFD. Further studies evaluating the role of hybrid PET/MR imaging in management of the disease in larger patient populations are warranted. (orig.)

  17. Relationship between left ventricular diastolic function and myocardial sympathetic denervation measured by {sup 123}I-meta-iodobenzylguanidine imaging in Anderson-Fabry disease

    Energy Technology Data Exchange (ETDEWEB)

    Spinelli, Letizia; Giudice, Caterina Anna; Imbriaco, Massimo; Trimarco, Bruno; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Pellegrino, Teresa [Institute of Biostructure and Bioimaging, National Council of Research, Naples (Italy); Pisani, Antonio; Riccio, Eleonora [University Federico II, Department of Public Health, Naples (Italy); Salvatore, Marco [IRCCS SDN, Naples (Italy)

    2016-04-15

    Whether cardiac sympathetic nervous function abnormalities may be present in patients with Anderson-Fabry disease (AFD) remains unexplored. We investigated the relationship between left ventricular (LV) function and cardiac sympathetic nervous function in patients with AFD. Twenty-five patients (12 men, mean age 43 ± 13 years) with genetically proved AFD and preserved LV ejection fraction and ten age and gender-matched control subjects underwent speckle tracking echocardiography and {sup 123}I-meta-iodobenzylguanidine (MIBG) imaging from which early and late heart to mediastinum (H/M) ratios and myocardial washout rate values were calculated. In AFD patients, a significant correlation between late H/M ratio and LV mass index (r = -61, p = 0.001), left atrial volume (r = -0.72, p < 0.001), systolic pulmonary artery pressure (r = -0.75, p < 0.001), and early diastolic untwisting rate (r = -0.66, p < 0.001) was found. Ten AFD patients exhibited a late H/M ratio below two fold standard deviation of control subjects (≤1.75). Patients showing late H/M ratio ≤ 1.75 had significantly higher LV mass index, relative wall thickness, left atrial volume and systolic pulmonary artery pressure, lower systolic longitudinal strain and an early diastolic untwisting rate compared to patients with late H/M ratio > 1.75. At multivariable linear regression analysis, early diastolic untwisting rate was the only independent predictor of late H/M ratio ≤ 1.75 (odds ratio 1.15, 95 % confidence interval 1.07-1.31, p < 0.05). The present findings provide the first demonstration of a cardiac sympathetic derangement in AFD patients with preserved LV ejection fraction, which is mostly related to LV diastolic dysfunction. (orig.)

  18. Klinefelter syndrome with fabry disease--a case of nondisjunction of the X-chromosome with sex-linked recessive mutation.

    Science.gov (United States)

    Sadick, Victoria J; Fietz, Michael J; Tchan, Michel C; Kovoor, Pramesh; Thomas, Liza; Sadick, Norman

    2014-12-01

    A 52 year-old male with Klinefelter syndrome presented with chest tightness and rapid atrial fibrillation with hypotension. His echocardiogram demonstrated symmetrical left ventricular hypertrophy with minimal diastolic dysfunction. Subsequent investigations confirmed the diagnosis of Fabry cardiomyopathy. This is the first reported case of Klinefelter syndrome with homozygous sex-linked recessive mutation presenting primarily with cardiac manifestation.

  19. Enzyme replacement therapy for Anderson-Fabry disease: A complementary overview of a Cochrane publication through a linear regression and a pooled analysis of proportions from cohort studies.

    Science.gov (United States)

    El Dib, Regina; Gomaa, Huda; Ortiz, Alberto; Politei, Juan; Kapoor, Anil; Barreto, Fellype

    2017-01-01

    Anderson-Fabry disease (AFD) is an X-linked recessive inborn error of glycosphingolipid metabolism caused by a deficiency of alpha-galactosidase A. Renal failure, heart and cerebrovascular involvement reduce survival. A Cochrane review provided little evidence on the use of enzyme replacement therapy (ERT). We now complement this review through a linear regression and a pooled analysis of proportions from cohort studies. To evaluate the efficacy and safety of ERT for AFD. For the systematic review, a literature search was performed, from inception to March 2016, using Medline, EMBASE and LILACS. Inclusion criteria were cohort studies, patients with AFD on ERT or natural history, and at least one patient-important outcome (all-cause mortality, renal, cardiovascular or cerebrovascular events, and adverse events) reported. The pooled proportion and the confidence interval (CI) are shown for each outcome. Simple linear regressions for composite endpoints were performed. 77 cohort studies involving 15,305 participants proved eligible. The pooled proportions were as follows: a) for renal complications, agalsidase alfa 15.3% [95% CI 0.048, 0.303; I2 = 77.2%, p = 0.0005]; agalsidase beta 6% [95% CI 0.04, 0.07; I2 = not applicable]; and untreated patients 21.4% [95% CI 0.1522, 0.2835; I2 = 89.6%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; b) for cardiovascular complications, agalsidase alfa 28% [95% CI 0.07, 0.55; I2 = 96.7%, p<0.0001]; agalsidase beta 7% [95% CI 0.05, 0.08; I2 = not applicable]; and untreated patients 26.2% [95% CI 0.149, 0.394; I2 = 98.8%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; and c) for cerebrovascular complications, agalsidase alfa 11.1% [95% CI 0.058, 0.179; I2 = 70.5%, p = 0.0024]; agalsidase beta 3.5% [95% CI 0.024, 0.046; I2 = 0%, p = 0.4209]; and untreated patients 18.3% [95% CI 0.129, 0.245; I2 = 95% p < 0.0001]. Effect differences favored agalsidase beta

  20. Genetic Disorders with Dyshidrosis: Ectodermal Dysplasia, Incontinentia Pigmenti, Fabry Disease, and Congenital Insensitivity to Pain with Anhidrosis.

    Science.gov (United States)

    Wataya-Kaneda, Mari

    2016-01-01

    Sweating is regulated by various neurohormonal mechanisms. A disorder in any part of the sweating regulatory pathways, such as the thermal center, neurotransmitters in the central to peripheral nerve, innervation of periglandular neurotransmission, and sweat secretion in the sweat gland itself, induces dyshidrosis. Therefore, hereditary disorders with dyshidrosis result from a variety of causes. These diseases have characteristic symptoms derived from each pathogenesis besides dyshidrosis. The information in this chapter is useful for the differential diagnosis of representative genetic disorders with dyshidrosis. © 2016 S. Karger AG, Basel.

  1. iSIGHT-FD scalability test report.

    Energy Technology Data Exchange (ETDEWEB)

    Clay, Robert L.; Shneider, Max S.

    2008-07-01

    The engineering analysis community at Sandia National Laboratories uses a number of internal and commercial software codes and tools, including mesh generators, preprocessors, mesh manipulators, simulation codes, post-processors, and visualization packages. We define an analysis workflow as the execution of an ordered, logical sequence of these tools. Various forms of analysis (and in particular, methodologies that use multiple function evaluations or samples) involve executing parameterized variations of these workflows. As part of the DART project, we are evaluating various commercial workflow management systems, including iSIGHT-FD from Engineous. This report documents the results of a scalability test that was driven by DAKOTA and conducted on a parallel computer (Thunderbird). The purpose of this experiment was to examine the suitability and performance of iSIGHT-FD for large-scale, parameterized analysis workflows. As the results indicate, we found iSIGHT-FD to be suitable for this type of application.

  2. Holographic Fabry-Perot spectrometer.

    Science.gov (United States)

    Martínez-Matos, O; Rodrigo, José A; Vaveliuk, P; Calvo, M L

    2011-02-15

    We propose a spectrum analyzer based on the properties of a hologram recorded with the field transmitted by a Fabry-Perot etalon. The spectral response of this holographic Fabry-Perot spectrometer (HFPS) is analytically investigated in the paraxial approximation and compared with a conventional Fabry-Perot etalon of similar characteristics. We demonstrate that the resolving power is twice increased and the free spectral range (FSR) is reduced to one-half. The proposed spectrometer could improve the operational performance of the etalon because it can exhibit high efficiency and it would be insensible to environmental conditions such as temperature and vibrations. Our analysis also extends to another variant of the HFPS based on holographic multiplexing of the transmitted field of a Fabry-Perot etalon. This device increases the FSR, keeping the same HFPS performance.

  3. Presymptomatic diagnosis of Fabry's disease

    DEFF Research Database (Denmark)

    Hasselbalch, Rasmus Bo; Madsen, Per Lav; Bundgaard, Henning;

    2016-01-01

    differential diagnoses in patients presenting with cardiac hypertrophy. In boys, onset has been reported in early childhood with complaints initially comprising neuropathic pain, reduced sweat production, and gastrointestinal symptoms. Later the cardiac, renal, and central nervous systems may become affected...

  4. Evaluation of the efficacy and safety of three dosing regimens of agalsidase alfa enzyme replacement therapy in adults with Fabry disease

    Science.gov (United States)

    Goláň, Lubor; Goker-Alpan, Ozlem; Holida, Myrl; Kantola, Ikka; Klopotowski, Mariusz; Kuusisto, Johanna; Linhart, Aleš; Musial, Jacek; Nicholls, Kathleen; Gonzalez-Rodriguez, Derlis; Sharma, Reena; Vujkovac, Bojan; Chang, Peter; Wijatyk, Anna

    2015-01-01

    Purpose Efficacy and safety of agalsidase alfa at 0.2 mg/kg weekly were compared with 0.2 mg/kg every other week (EOW). Exploratory analyses were performed for 0.4 mg/kg weekly. Patients and methods This was a 53-week, Phase III/IV, multicenter, open-label study (NCT01124643) in treatment-naïve adults (≥18 years) with Fabry disease. Inclusion criteria were left ventricular hypertrophy at baseline, defined as left ventricular mass indexed to height >50 g/m2.7 for males and >47 g/m2.7 for females. Primary endpoint was reduction of left ventricular mass indexed to height as assessed by echocardiography. Secondary endpoints included cardiac (peak oxygen consumption, 6-minute walk test, Minnesota Living with Heart Failure Questionnaire, New York Heart Association classification), renal (Modification of Diet in Renal Disease, estimated glomerular filtration rate), and biomarker (plasma globotriaosylceramide) assessments. Safety endpoints were adverse events and anti–agalsidase alfa antibodies. Results Twenty patients were randomized to 0.2 mg/kg EOW (mean age, 50.3 years; 70% male), 19 to 0.2 mg/kg weekly (51.8 years; 53% male), and 5 to 0.4 mg/kg weekly (49.4 years; 40% male). The mean change in left ventricular mass indexed to height by Week 53 in the 0.2-mg/kg EOW and weekly groups was 3.2 g/m2.7 and 0.5 g/m2.7, with no significant difference between groups. No clinically meaningful changes by Week 53 were found within or between the 0.2-mg/kg groups for peak oxygen consumption, 6-minute walk test, or Minnesota Living with Heart Failure Questionnaire. Two patients in each group improved by ≥1 New York Heart Association classification. No significant differences were found between 0.2 mg/kg EOW and weekly for mean change in estimated glomerular filtration rate (−1.21 mL/min/1.73 m2 vs −3.32 mL/min/1.73 m2) or plasma globotriaosylceramide (−1.05 nmol/mL vs −2.13 nmol/mL), respectively. Infusion-related adverse events were experienced by 25% and 21% in the

  5. The prevalent deep intronic c. 639+919 G>A GLA mutation causes pseudoexon activation and Fabry disease by abolishing the binding of hnRNPA1 and hnRNP A2/B1 to a splicing silencer

    DEFF Research Database (Denmark)

    Palhais, Bruno; Dembic, Maja; Sabaratnam, Rugivan;

    2016-01-01

    with the ESS is also able to inhibit inclusion of an unrelated pseudoexon in the FGB gene, and that also in the FGB context inactivation of the ESS by the c.639+919 G>A mutation causes pseudoexon activation, underscoring the universal nature of the ESS. Finally, we demonstrate that splice switching......Fabry disease is an X-linked recessive inborn disorder of the glycosphingolipid metabolism, caused by total or partial deficiency of the lysosomal α-galactosidase A enzyme due to mutations in the GLA gene. The prevalent c.639+919 G>A mutation in GLA leads to pathogenic insertion of a 57bp...... pseudoexon sequence from intron 4, which is responsible for the cardiac variant phenotype. In this study we investigate the splicing regulatory mechanism leading to GLA pseudoexon activation. Splicing analysis of GLA minigenes revealed that pseudoexon activation is influenced by cell-type. We demonstrate...

  6. Evaluation of the efficacy and safety of three dosing regimens of agalsidase alfa enzyme replacement therapy in adults with Fabry disease

    Directory of Open Access Journals (Sweden)

    Goláň L

    2015-07-01

    Full Text Available Lubor Goláň,1 Ozlem Goker-Alpan,2 Myrl Holida,3 Ikka Kantola,4 Mariusz Klopotowski,5 Johanna Kuusisto,6 Aleš Linhart,1 Jacek Musial,7 Kathleen Nicholls,8 Derlis Gonzalez-Rodriguez,9 Reena Sharma,10 Bojan Vujkovac,11 Peter Chang,12 Anna Wijatyk12 1First Faculty of Medicine, Department of Cardiovascular Medicine, Charles University, Prague, Czech Republic; 2Lysosomal Research and Treatment Unit, Fairfax, VA, USA; 3Stead Family Department of Pediatrics, Division of Medical Genetics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA; 4Division of Medicine, Turku University Hospital, Turku, Finland; 5Institute of Cardiology, Warsaw, Poland; 6Department of Medicine, Center for Medicine and Clinical Research, University of Eastern Finland and Kuopio University Hospital, Kuopio, Finland; 7Department of Internal Medicine, Jagiellonian University Medical College, Krakow, Poland; 8Department of Nephrology, Royal Melbourne Hospital and the University of Melbourne, VIC, Australia; 9Instituto Privado de Hematologia E Investigacion Clinica (IPHIC, Asuncion, Paraguay; 10Salford Royal NHS Foundation Trust, Salford, UK; 11General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia; 12Shire, Lexington, MA, USA Purpose: Efficacy and safety of agalsidase alfa at 0.2 mg/kg weekly were compared with 0.2 mg/kg every other week (EOW. Exploratory analyses were performed for 0.4 mg/kg weekly.Patients and methods: This was a 53-week, Phase III/IV, multicenter, open-label study (NCT01124643 in treatment-naïve adults (≥18 years with Fabry disease. Inclusion criteria were left ventricular hypertrophy at baseline, defined as left ventricular mass indexed to height >50 g/m2.7 for males and >47 g/m2.7 for females. Primary endpoint was reduction of left ventricular mass indexed to height as assessed by echocardiography. Secondary endpoints included cardiac (peak oxygen consumption, 6-minute walk test, Minnesota Living with Heart Failure Questionnaire, New

  7. Flat-detector computed tomography (FD-CT)

    Energy Technology Data Exchange (ETDEWEB)

    Kalender, Willi A.; Kyriakou, Yiannis [University of Erlangen-Nuernberg, Institute of Medical Physics, Erlangen (Germany)

    2007-11-15

    Flat-panel detectors or, synonymously, flat detectors (FDs) have been developed for use in radiography and fluoroscopy with the defined goal to replace standard X-ray film, film-screen combinations and image intensifiers by an advanced sensor system. FD technology in comparison to X-ray film and image intensifiers offers higher dynamic range, dose reduction, fast digital readout and the possibility for dynamic acquisitions of image series, yet keeping to a compact design. It appeared logical to employ FD designs also for computed tomography (CT) imaging. Respective efforts date back a few years only, but FD-CT has meanwhile become widely accepted for interventional and intra-operative imaging using C-arm systems. FD-CT provides a very efficient way of combining two-dimensional (2D) radiographic or fluoroscopic and 3D CT imaging. In addition, FD technology made its way into a number of dedicated CT scanner developments, such as scanners for the maxillo-facial region or for micro-CT applications. This review focuses on technical and performance issues of FD technology and its full range of applications for CT imaging. A comparison with standard clinical CT is of primary interest. It reveals that FD-CT provides higher spatial resolution, but encompasses a number of disadvantages, such as lower dose efficiency, smaller field of view and lower temporal resolution. FD-CT is not aimed at challenging standard clinical CT as regards to the typical diagnostic examinations; but it has already proven unique for a number of dedicated CT applications, offering distinct practical advantages, above all the availability of immediate CT imaging in the interventional suite or the operating room. (orig.)

  8. Clinical and magnetic resonance imaging studies of Anderson-Fabry disease: 2 cases report%表现为早发缺血性卒中的Fabry病二例

    Institute of Scientific and Technical Information of China (English)

    李小璇; 王璐; 张赟; 张巍; 洪道俊; 王朝霞; 高惠珍; 袁云

    2011-01-01

    Objective To report 2 cases of Fabry disease with early-onset ischemic stroke and investigate the clinical and image features. Methods The 2 patients developed dizziness, diplopia and progressive memory decline at 32 and 25 years of age respectively. The α-galactosidase A (GLA)activity was 4. 3 U in case 1 and 1. 0 U in case 2 ( normal range 100-500 U). Skin biopsy showed numerous membrane bounded osmiophilic laminar material in vascular smooth muscle cells and endothelial cells. GLA gene analysis revealed GLA exon 7 1033-1034 TC del in case 1 and GLA exon 3 466G > A in case 2. The 2 patients were evaluated by Mainz severity score index (MSSI) and cranial MRI. Results The general MSSI was 32 in case 1 and 16 in case 2, with the highest score of neurological score ( 11 and 14). Both cases showed multifocal infarcts in bilateral cerebellum, occipital lobe, basal ganglia, which were hypointensity on T1 WI and hyperintensity on T2WI. There were hyperintensity changes in the pulvinar and basal ganglia on T1 WI in case 2. Conclusions Cerebral ischemic stroke could appear as initial symptoms in Fabry disease,which predominantly involved the posterior circulation. There might be no dilation of basal-vertebral artery.%目的 报道以早发缺血性卒中为主要表现的2例Fabry病,分析其卒中表现规律和影像学改变特点.方法 2例Fabry病患者分别在32岁和25岁出现头晕和突发视物成双,伴随记忆力下降.α-半乳糖苷酶活性在例1为4.3 U,例2为1.0 U(正常值100~500 U).皮肤病理检查发现血管平滑肌细胞和内皮细胞内充满膜性包裹的板层样小体.α-半乳糖苷酶基因检查显示例1的7号外显子存在1033-1034 TC del突变,而例2存在3号外显子c.466G>A突变.对2例患者进行美茵滋严重度评分(MSSI)和头MRI检查.结果 例1的MSSI总分为32,其中神经系统评分最高(11分),例2的总分为16,其中神经系统评分最高(14分).MRI均表现为双侧小脑、枕叶、基

  9. Vascular Tortuosities of the Upper Eyelid: A New Clinical Finding in Fabry Patient Screening

    Directory of Open Access Journals (Sweden)

    Langis Michaud

    2013-01-01

    Full Text Available Purpose. To report a new clinical finding related to Fabry disease. Methods. Fabry subjects were enrolled in the study, matched for age and sex with healthy individuals as a control group. This is a prospective review of all upper lid pictures taken for every subject at their last visit. A 4-step grading scale is proposed to classify this new entity. Results. Group A (Fabry comprised 16 males and 22 females, aged 40 (±14 years on average. Group B (control comprised 7 males and 8 females, aged 37 (±12 Vessels tortuosity was identified on the external superior lid in 36 of the Fabry patients (94.7%, while none of the subjects in group B showed similar vessels tortuosity. In addition, microaneurysms (MAs were found in 10/38 group A subjects while none in group B presented a similar finding. The differences are highly significant. Conclusion. This paper proposes that blood vessels tortuosity on the upper eyelid be recognized as a new clinical entity for inclusion among the classic ocular manifestations of Fabry’s disease. Without evidence of any negative impact, it should be considered a benign sign contributing to evidence of suspected Fabry disease.

  10. Distributed Fabry-Perot Measurement System

    Institute of Scientific and Technical Information of China (English)

    WENG Jian-hua

    2003-01-01

    The theoretic analysis indicates that if the lengths of the cascaded and paralleled Fabry-Perot sensors are properly selected, the crosstalk can be well restricted.And the experiment simulation results agree with that of the theoretic analysis.

  11. The overlapped radial basis function-finite difference (RBF-FD) method: A generalization of RBF-FD

    Science.gov (United States)

    Shankar, Varun

    2017-08-01

    We present a generalization of the RBF-FD method that computes RBF-FD weights in finite-sized neighborhoods around the centers of RBF-FD stencils by introducing an overlap parameter δ ∈ (0 , 1 ] such that δ = 1 recovers the standard RBF-FD method and δ = 0 results in a full decoupling of stencils. We provide experimental evidence to support this generalization, and develop an automatic stabilization procedure based on local Lebesgue functions for the stable selection of stencil weights over a wide range of δ values. We provide an a priori estimate for the speedup of our method over RBF-FD that serves as a good predictor for the true speedup. We apply our method to parabolic partial differential equations with time-dependent inhomogeneous boundary conditions - Neumann in 2D, and Dirichlet in 3D. Our results show that our method can achieve as high as a 60× speedup in 3D over existing RBF-FD methods in the task of forming differentiation matrices.

  12. A Novel Rapid MALDI-TOF-MS-Based Method for Measuring Urinary Globotriaosylceramide in Fabry Patients

    Science.gov (United States)

    Alharbi, Fahad J.; Geberhiwot, Tarekegn; Hughes, Derralynn A.; Ward, Douglas G.

    2016-04-01

    Fabry disease is an X-linked lysosomal storage disorder caused by deficiency of α-galactosidase A, resulting in the accumulation of glycosphingolipids in various organs. Globotriaosylceramide (Gb3) and its isoforms and analogues have been identified and quantified as biomarkers of disease severity and treatment efficacy. The current study aimed to establish rapid methods for urinary Gb3 extraction and quantitation. Urine samples from 15 Fabry patients and 21 healthy control subjects were processed to extract Gb3 by mixing equal volumes of urine, methanol containing an internal standard, and chloroform followed by sonication and centrifugation. Thereafter, the lower phase was analyzed by MALDI-TOF MS and the relative peak areas of the internal standard and four major species of Gb3 determined. The results showed high reproducibility with intra- and inter-assay coefficients variation of 9.9% and 13.7%, respectively. The limit of detection was 0.15 ng/μL and the limit of quantitation was 0.30 ng/μL. Total urinary Gb3 levels in both genders of classic Fabry patients were significantly higher than in healthy controls (p < 0.0001). Gb3 levels in Fabry males were higher than in Fabry females (p = 0.08). We have established a novel assay for urinary total Gb3 that takes less than 15 min from start to finish.

  13. Basics principles of flat detector computed tomography (FD-CT); Grundlagen der Flachdetektor-CT (FD-CT)

    Energy Technology Data Exchange (ETDEWEB)

    Kyriakou, Y.; Struffert, T.; Doerfler, A.; Kalender, W.A. [Universitaet Erlangen-Nuernberg, Institut fuer Medizinische Physik, Erlangen (Germany)

    2009-09-15

    Flat detectors (FDs) have been developed for use in radiography and fluoroscopy to replace standard X-ray film, film-screen combinations and image intensifiers (II). In comparison to X-ray film and II, FD technology offers higher dynamic range, dose reduction, fast digital readout and the possibility for dynamic acquisitions of image series, yet keeping to a compact design. It appeared logical to employ FD designs also for computed tomography (CT) imaging. FDCT has meanwhile become widely accepted for interventional and intra-operative imaging using C-arm systems. Additionally, the introduction of FD technology was a milestone for soft-tissue CT imaging in the interventional suite which was not possible with II systems in the past. This review focuses on technical and performance issues of FD technology and its wide range of applications for CT imaging. FDCT is not aimed at challenging standard clinical CT as regards to the typical diagnostic examinations, but it has already proven unique for a number of dedicated CT applications offering distinct practical advantages, above all the availability of immediate CT imaging during an intervention. (orig.) [German] Flachdetektoren (FD) sind fuer die Anwendung in der Radiographie und Fluoroskopie entwickelt worden, um die damaligen Standards - Film-Folien-Kombinationen (FFK) und Bildverstaerker (BV) - zu ersetzen. Im Vergleich zu FFK und BV bietet die FD-Technologie eine hoehere Dynamik, Verzerrungsfreiheit und eine gesteigerte Dosiseffizienz. Weitere Vorteile sind die Anfertigung Serienaufnahmen, eine sofortige Digitalausgabe und eine kompakte Bauweise. Dies legt auch eine Anwendung von Flachdetektoren in der Computertomographie (CT) nahe. Inzwischen ist die FD-CT weitgehend in der interventionellen und intraoperativen Bildgebung etabliert - meist als C-Bogen-System. Die FD-Technologie hat erstmals die Weichteilbildgebung in der interventionellen Suite ermoeglicht, was mit BV-Systemen nicht moeglich war. Diese

  14. Functional diversification of FD transcription factors in rice, components of florigen activation complexes.

    Science.gov (United States)

    Tsuji, Hiroyuki; Nakamura, Hiroyuki; Taoka, Ken-ichiro; Shimamoto, Ko

    2013-03-01

    Florigen, a protein encoded by the FLOWERING LOCUS T (FT) in Arabidopsis and Heading date 3a (Hd3a) in rice, is the universal flowering hormone in plants. Florigen is transported from leaves to the shoot apical meristem and initiates floral evocation. In shoot apical cells, conserved cytoplasmic 14-3-3 proteins act as florigen receptors. A hexameric florigen activation complex (FAC) composed of Hd3a, 14-3-3 proteins, and OsFD1, a transcription factor, activates OsMADS15, a rice homolog of Arabidopsis APETALA1, leading to flowering. Because FD is a key component of the FAC, we characterized the FD gene family and their functions. Phylogenetic analysis of FD genes indicated that this family is divided into two groups: (i) canonical FD genes that are conserved among eudicots and non-Poaceae monocots; and (ii) Poaceae-specific FD genes that are organized into three subgroups: Poaceae FD1, FD2 and FD3. The Poaceae FD1 group shares a small sequence motif, T(A/V)LSLNS, with FDs of eudicots and non-Poaceae monocots. Overexpression of OsFD2, a member of the Poaceae FD2 group, produced smaller leaves with shorter plastochrons, suggesting that OsFD2 controls leaf development. In vivo subcellular localization of Hd3a, 14-3-3 and OsFD2 suggested that in contrast to OsFD1, OsFD2 is restricted to the cytoplasm through its interaction with the cytoplasmic 14-3-3 proteins, and interaction of Hd3a with 14-3-3 facilitates nuclear translocation of the FAC containing OsFD2. These results suggest that FD function has diverged between OsFD1 and OsFD2, but formation of a FAC is essential for their function.

  15. Characterization of Fabry mice treated with recombinant adeno-associated virus 2/8-mediated gene transfer

    Directory of Open Access Journals (Sweden)

    Choi Jin-Ok

    2010-04-01

    Full Text Available Abstract Background Enzyme replacement therapy (ERT with α-galactosidase A (α-Gal A is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease. However, ERT has limitations of a short half-life, requirement for frequent administration, and limited efficacy for patients with renal failure. Therefore, we investigated the efficacy of recombinant adeno-associated virus (rAAV vector-mediated gene therapy for a Fabry disease mouse model and compared it with that of ERT. Methods A pseudotyped rAAV2/8 vector encoding α-Gal A cDNA (rAAV2/8-hAGA was prepared and injected into 18-week-old male Fabry mice through the tail vein. The α-Gal A expression level and globotriaosylceramide (Gb3 levels in the Fabry mice were examined and compared with Fabry mice with ERT. Immunohistochemical and ultrastructural studies were conducted. Results Treatment of Fabry mice with rAAV2/8-hAGA resulted in the clearance of accumulated Gb3 in tissues such as liver, spleen, kidney, heart, and brain with concomitant elevation of α-Gal A enzyme activity. Enzyme activity was elevated for up to 60 weeks. In addition, expression of the α-Gal A protein was identified in the presence of rAAV2/8-hAGA at 6, 12, and 24 weeks after treatment. α-Gal A activity was significantly higher in the mice treated with rAAV2/8-hAGA than in Fabry mice that received ERT. Along with higher α-Gal A activity in the kidney of the Fabry mice treated with gene therapy, immunohistochemical studies showed more α-Gal A expression in the proximal tubules and glomerulus, and less Gb3 deposition in Fabry mice treated with this gene therapy than in mice given ERT. The α-gal A gene transfer significantly reduced the accumulation of Gb3 in the tubules and podocytes of the kidney. Electron microscopic analysis of the kidneys of Fabry mice also showed that gene therapy was more effective than ERT. Conclusions The rAAV2/8-hAGA mediated α-Gal A gene

  16. Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients

    NARCIS (Netherlands)

    B.E. Smid; S.M. Rombach; J.M.F.G. Aerts; S. Kuiper; M. Mirzaian; H.S. Overkleeft; B.J.H.M. Poorthuis; C.E.M. Hollak; J.E.M. Groener; G.E. Linthorst

    2011-01-01

    ABSTRACT: BACKGROUND: Enzyme replacement therapy is currently the only approved therapy for Fabry disease. From June 2009 on, viral contamination of Genzyme's production facility resulted in a worldwide shortage of agalsidase beta leading to involuntary dose reductions (approved dose 1 mg/kg/eow, re

  17. Neurological aspects of Gaucher and Fabry disease

    NARCIS (Netherlands)

    Biegstraaten, M.

    2011-01-01

    Momenteel zijn er meer dan vijftig stapelingsziekten bekend, waarvan de ernst zeer uiteen loopt. Sommige aandoeningen worden gekenmerkt door ernstige mentale retardatie, neurologische problemen en overlijden op kinderleeftijd; andere vormen hebben nauwelijks een lagere levensverwachting. Ook in de l

  18. Molecular Characterization of FT and FD homologs from Eriobotrya deflexa Nakai forma koshunensis

    Directory of Open Access Journals (Sweden)

    Ling eZhang

    2016-01-01

    Full Text Available In angiosperms, regulation of flowering is a vital process for successful reproduction. To date, the molecular mechanism of flowering is well studied in the model plant, Arabidopsis, in which key genes such as FLOWERING LOCUST (FTor FD have been identified to regulate flowering. However, the flowering mechanisms are still largely unknown in fruit trees like loquat. To this end, we first cloned one FT- and two FD-like genes from the loquat (E. deflexa Nakai f. koshunensis and referred to as EdFT, EdFD1 and EdFD2, respectively. Phylogenetic analysis has shown that EdFT, EdFD1 and EdFD2 are conserved during the evolution process. EdFT is mainly expressed in reproductive tissues (e.g. flower buds, flowers and fruits, while EdFD1 and EdFD2 are mainly expressed in apical buds including leaf buds and flower buds. EdFT is localized in the whole cell, while EdFD1 or EdFD2 is localized in the nucleus. Ectopic expression of EdFT, EdFD1 and EdFD2 in Arabidopsis results in early flowering. In addition, we have also revealed that the EdFT interacts with both EdFD1 and EdFD2. Overall, these data suggest that the EdFT, EdFD1 and EdFD2 are the functional homologs of FT and FD, respectively, which might act together to regulate loquat flowering through a similar mechanism found in Arabidopsis.

  19. 21 CFR 866.5540 - Immunoglobulin G (Fd fragment specific) immunological test system.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Immunoglobulin G (Fd fragment specific... Test Systems § 866.5540 Immunoglobulin G (Fd fragment specific) immunological test system. (a) Identification. An immunoglobulin G (Fd fragment specific) immunological test system is a device that consists...

  20. 21 CFR 74.1705 - FD&C Yellow No. 5.

    Science.gov (United States)

    2010-04-01

    ... listing the color additive using the names FD&C Yellow No. 5 and tartrazine. The label shall bear a statement such as “Contains FD&C Yellow No. 5 (tartrazine) as a color additive” or “Contains color additives including FD&C Yellow No. 5 (tartrazine).” The labels of certain drug products subject to this...

  1. 以慢性肾炎综合征为主要表现的Fabry病2例报道并文献复习%Fabry disease with chronic nephritis syndrome as the main manifestation and literature study:2 cases report

    Institute of Scientific and Technical Information of China (English)

    廖武琼; 李典耕; 谢大洋; 陈仆; 李清刚; 陈香美

    2015-01-01

    Objective To investigate the clinical manifestation and renal pathology of Fabry disease which characteri zed with chronic nephritis syndrome as the main manifestation. Methods Retrospectively review the 2 cases of Fabry disease, which characterized as chronic nephritis syndrome as the main manifestation, the clinical signs and pathology were analyzed, and review of the relevant literature to master the methods of diagnosis and treatment. Results One case was male patient, the other was female patient, and onset age was 46 years old and 44 years old, 2 cases showed proteinuria, hematuria, hyperten-sion, and no characteristic of extra-renal manifestations. Under the light microscope, glomerular sclerosis rates were 30. 8%and 32. 1% respectively, significant swelling of glomerular podocytes, visible vacuoles formed in the cytoplasm, renal tubular multifocal atrophic were found. Dense and irregular osmiophilic myelin bodies also can be found. There is no effective cure methods for Fabry disease, combined with renal damage, the treatment mainly was to protect the kidney function, reducing protein, control symptom, the main medicine are ARB, such as losartan potassium, leflunomide. Conclusion Fabry disease is a rare genetic chromosome X diseases, renal damage is one of the most common clinical manifestation, under microscopic osmiophilic myelin bodies is the pathological finding, symptomatic treatment with enzyme replacement therapy are the main treatment, and combined with the genetics of appropriate intervention to block the genetics of the disease.%目的:探讨以慢性肾炎综合征为主要表现的Fabry病临床表现和肾脏病理学特点。方法回顾性分析收治的以慢性肾炎综合征为主要表现的Fabry病2例,分析其临床体征及病理学特征,同时复习相关文献掌握其诊断及治疗方法。结果男女患者各1例,发病年龄分别为46岁和44岁,2例均表现为蛋白尿、血尿、高血压,而无特征性的肾外表现

  2. Fourier Transform Fabry-Perot Interferometer

    Science.gov (United States)

    Snell, Hilary E.; Hays, Paul B.

    1992-01-01

    We are developing a compact, rugged, high-resolution remote sensing instrument with wide spectral scanning capabilities. This relatively new type of instrument, which we have chosen to call the Fourier-Transform Fabry-Perot Interferometer (FT-FPI), is accomplished by mechanically scanning the etalon plates of a Fabry-Perot interferometer (FPI) through a large optical distance while examining the concomitant signal with a Fourier-transform analysis technique similar to that employed by the Michelson interferometer. The FT-FPI will be used initially as a ground-based instrument to study near-infrared atmospheric absorption lines of trace gases using the techniques of solar absorption spectroscopy. Future plans include modifications to allow for measurements of trace gases in the stratosphere using spectral lines at terahertz frequencies.

  3. An Archetype Semi-Ring Fabry-Perot (SRFP) Resonator

    Science.gov (United States)

    Taghavi-Larigani, Shervin; VanZyl, Jakob

    2009-01-01

    We introduce and demonstrate the generation of a novel resonator, termed Semi-Ring Fabry-Perot (SRFP), that exhibits unique features, such as, its use of one plane mirror, allowing the SRFP to be easily fabricated as a symmetrical device. In addition to its unique features, it exhibits advantages of ring and Fabry-Perot resonators: 1) compared to a ring resonator that only allows a transmitted intensity, the Semi-Ring Fabry-Perot (SRFP) supports standing waves, allowing both a reflected and transmitted intensity; 2) the reflected light spectrum of the SRFP resonator is much narrower than similar Fabry-Perot, implying higher finesse.

  4. 21 CFR 201.20 - Declaration of presence of FD&C Yellow No. 5 and/or FD&C Yellow No. 6 in certain drugs for human...

    Science.gov (United States)

    2010-04-01

    ... eye, containing FD&C Yellow No. 5 as a color additive using the names FD&C Yellow No. 5 and tartrazine... “Contains FD&C Yellow No. 5 (tartrazine) as a color additive” or “Contains color additives including FD&C Yellow No. 5 (tartrazine)”. The labels of certain drug products subject to this labeling requirement...

  5. THE RELATIONSHIP BETWEEN THE SPONTANEOUS NOCTURNALEPISODES OF ALKALINIZATION AND AUTONOMIC NERVOUSFUNCTION ON FD PATIENTS

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To study the relationship between the spontaneous nocturnal episodes of alkalinization and the autonomic nerve system function and vagal function. Methods 24-hour intragastric pH was measured and auto nomic and vagal function was measured with the time domain analyses of heart rate variability in 20 patients with functional dyspepsia but without diseases of the cardiovascular system. Results 13 of 20 had the nocturnal episodes of alkalinization. The total 24-hour SDNN and rMSSD were normal in 20 subjects with FD. There was no significant dif ference (P >0. 05) in the comparison of the total SDNN and rMSSD of the 2 groups with alkalinization and without alkalinization. The 2 groups both had higher PNN50s in the nocturnal time, and there was no significant difference (P >0. 05). Conclusion The results suggest that the total autonomic nerve function and vagal function of patients with FD are normal, vagal activities of the 2 groups are both increased in the nocturnal period. The reason for the nocturnal episodes of alkalinization is not a decrease of vagal activity with a subsequent decrease of secretion.

  6. Optimized equivalent staggered-grid FD method for elastic wave modelling based on plane wave solutions

    Science.gov (United States)

    Yong, Peng; Huang, Jianping; Li, Zhenchun; Liao, Wenyuan; Qu, Luping; Li, Qingyang; Liu, Peijun

    2017-02-01

    In finite-difference (FD) method, numerical dispersion is the dominant factor influencing the accuracy of seismic modelling. Various optimized FD schemes for scalar wave modelling have been proposed to reduce grid dispersion, while the optimized time-space domain FD schemes for elastic wave modelling have not been fully investigated yet. In this paper, an optimized FD scheme with Equivalent Staggered Grid (ESG) for elastic modelling has been developed. We start from the constant P- and S-wave speed elastic wave equations and then deduce analytical plane wave solutions in the wavenumber domain with eigenvalue decomposition method. Based on the elastic plane wave solutions, three new time-space domain dispersion relations of ESG elastic modelling are obtained, which are represented by three equations corresponding to P-, S- and converted-wave terms in the elastic equations, respectively. By using these new relations, we can study the dispersion errors of different spatial FD terms independently. The dispersion analysis showed that different spatial FD terms have different errors. It is therefore suggested that different FD coefficients to be used to approximate the three spatial derivative terms. In addition, the relative dispersion error in L2-norm is minimized through optimizing FD coefficients using Newton's method. Synthetic examples have demonstrated that this new optimal FD schemes have superior accuracy for elastic wave modelling compared to Taylor-series expansion and optimized space domain FD schemes.

  7. Small-fibre neuropathy in female Fabry patients: reduced allodynia and skin blood flow after topical capsaicin

    DEFF Research Database (Denmark)

    Møller, Anette Torvin; Feldt-Rasmussen, Ulla; Rasmussen, Åse K.;

    2006-01-01

    affected. Recently, attention has been drawn to female patients whether they also show signs of nerve involvement. An early sign of the disease is painful small-fibre neuropathy. The aim of this study was to evaluate a small-fibre dysfunction in female Fabry patients by using capsaicin applied topically....... The response to capsaicin was evaluated by laser Doppler imaging. We found that the female Fabry patients had a significantly smaller increase in blood flow (p = 0.0003) after capsaicin application. The area of static mechanical allodynia and dynamic mechanical hyperalgesia was also significantly smaller (p...

  8. Physics Simulations of fluids - a brief overview of Phoenix FD

    CERN Document Server

    CERN. Geneva; Nikolov, Svetlin

    2014-01-01

    The presentation will briefly describe the simulation and rendering of fluids with Phoenix FD, and then proceed into implementation details. We will present our methods of parallelizing the core simulation algorithms and our utilization of the GPU. We will also show how we take advantage of computational fluid dynamics specifics in order to speed up the preview and final rendering, thus achieving a quick pipeline for the creation of various visual effects. About the speakers Ivaylo Iliev is a Senior Software developer at Chaos Group and is the creator of the Phoenix FD simulator for fluid effects. He has a strong interest in physics and has worked on military simulators before focusing on visual effects. He has a Master?s degree from the Varna Technical University. Svetlin Nikolov is a Senior Software developer at Chaos Group with keen interest in physics and artificial intelligence and 7 years of experience in the software industry. He comes from a game development background with a focu...

  9. A high resolution scanning fabry-perot for OSIRIS

    Directory of Open Access Journals (Sweden)

    A. Bernal

    2007-01-01

    Full Text Available Following the directives of the workshop on the interferometric mode of OSIRIS, the Mexican team has received support to ac- quire a scanning Fabry-Perot interferometer for OSIRIS. In this presentation we will try to define which Fabry-Perot will be selected as well as which filters need to be acquired. We will also discuss the acquisition software

  10. Ophthalmic experience over 10 years in an observational nationwide Danish cohort of Fabry patients with access to enzyme replacement

    DEFF Research Database (Denmark)

    Fledelius, Hans C.; Sandfeld, Lisbeth; Rasmussen, Åse Krogh

    2015-01-01

    PURPOSE: Enzyme replacement therapy (ERT) was offered from year 2001 to patients with Fabry disease. The ophthalmic experience was analysed, as part of a general 10-year status. METHODS: A retrospective observational series comprising 39 patients (25 females, 14 males) closely followed by the end...

  11. An economic Fabry-Perot wavelength reference

    Science.gov (United States)

    Fżrész, Gábor; Glenday, Alex; Latham, Christian

    2014-07-01

    Precision radial velocity (PRV) measurements are key in studying exoplanets, and so are wavelength calibrators in PRV instruments. ThAr lamps offer an affordable but somewhat limited solution for the visible passband. Laser frequency combs are ideal calibrators, except the (still) narrow wavelength coverage and large price tag. White light Fabry-Perot (FP) calibrators offer frequency-comb like properties in a more affordable and less complicated package1. Using a commercial solid FP etalon and off-the shelf components we have constructed an economic FP calibrator suitable for observatories on a smaller budget.

  12. Glass transition of repulsive charged rods (fd-viruses).

    Science.gov (United States)

    Kang, Kyongok

    2014-05-14

    It has recently been shown that suspensions of long and thin charged fibrous viruses (fd) form a glass at low ionic strengths. The corresponding thick electric double layers give rise to long-ranged repulsive electrostatic interactions, which lead to caging and structural arrest at concentrations far above the isotropic-nematic coexistence region. Structural arrest and freezing of the orientational texture are found to occur at the same concentration. In addition, various types of orientational textures are equilibrated below the glass transition concentration, ranging from a chiral-nematic texture with a large pitch (of about 100 μm), an X-pattern, and a tightly packed domain texture, consisting of helical domains with a relatively small pitch (of about 10 μm) and twisted boundaries. The dynamics of both particles as well as the texture are discussed, below and above the glass transition. Dynamic light scattering correlation functions exhibit two dynamical modes, where the slow mode is attributed to the elasticity of helical domains. On approach of the glass-transition concentration, the slow mode increases in amplitude, while as the amplitudes of the fast and slow mode become equal at the glass transition. Finally, interesting features of the "transient" behaviors of charged fd-rod glass are shown as the initial caging due to structural arrest, the propagation of flow originating from stress release, and the transition to the final metastable glass state. In addition to the intensity correlation function, power spectra are presented as a function of the waiting time, at the zero-frequency limit that may access to the thermal anomalities in a charged system.

  13. Protocol and methodology of the Stroke in Young Fabry Patients (sifap1) study: a prospective multicenter European study of 5,024 young stroke patients aged 18-55 years.

    LENUS (Irish Health Repository)

    Rolfs, Arndt

    2011-01-01

    Stroke in the young has not been thoroughly investigated with most previous studies based on a small number of patients from single centers. Furthermore, recent reports indicate that Fabry disease may be a significant cause for young stroke. The primary aim of our study was to determine the prevalence of Fabry disease in young stroke patients, while the secondary aim was to describe patterns of stroke in young patients.

  14. Interleukin-18 deteriorates Fabry cardiomyopathy and contributes to the development of left ventricular hypertrophy in Fabry patients with GLA IVS4+919 G>A mutation

    Science.gov (United States)

    Huang, Wei-Lin; Chou, Shih-Jie; Chang, Wei-Chao; Chang, Yuh-Lih; Leu, Hsin-Bang; Chen, Kuan-Hsuan; Wang, Kang-Ling; Lai, Ying-Hsiu; Liu, Yung-Yang; Lu, Kai-Hsi; Li, Hsin-Yang; Sung, Yen-Jen; Jong, Yuh-Jyh; Chen, Yann-Jang; Chen, Chung-Hsuan; Yu, Wen-Chung

    2016-01-01

    Rationale A high incidence of GLA IVS4+919 G>A mutation in patients with Fabry disease of the later-onset cardiac phenotype, has been reported in Taiwan. However, suitable biomarkers or potential therapeutic surrogates for Fabry cardiomyopathy (FC) in such patients under enzyme replacement treatment (ERT) remain unknown. Objective Using FC patients carrying IVS4+919 G>A mutation, we constructed an induced pluripotent stem cell (iPSC)-based disease model to investigate the pathogenetic biomarkers and potential therapeutic targets in ERT-treated FC. Results and methods The iPSC-differentiated cardiomyocytes derived from FC-patients (FC-iPSC-CMs) carried IVS4+919 G>A mutation recapitulating FC characteristics, including low α-galactosidase A enzyme activity, cellular hypertrophy, and massive globotriaosylceramide accumulation. Microarray analysis revealed that interleukin-18 (IL-18), a pleiotropic cytokine involved in various myocardial diseases, was the most highly upregulated marker in FC-iPSC-CMs. Meanwhile, IL-18 levels were found to be significantly elevated in the culture media of FC-iPSC-CMs and patients’ sera. Notably, the serum IL-18 levels were highly paralleled with the progression of left ventricular hypertrophy in Fabry patients receiving ERT. Finally, using FC-iPSC-CMs as in vitro FC model, neutralization of IL-18 with specific antibodies combined with ERT synergistically reduced the secretion of IL-18 and the progression of cardiomyocyte hypertrophy in FC-iPSC-CMs. Conclusion Our data demonstrated that cardiac IL-18 and circulating IL-18 are involved in the pathogenesis of FC and LVH. IL-18 may be a novel marker for evaluating ERT efficacy, and targeting IL-18 might be a potential adjunctive therapy combined with ERT for the treatment of advanced cardiomyopathy in FC patients with IVS4+919 G>A mutation. PMID:27888626

  15. Fabry-Perot resonance of water waves.

    Science.gov (United States)

    Couston, Louis-Alexandre; Guo, Qiuchen; Chamanzar, Maysamreza; Alam, Mohammad-Reza

    2015-10-01

    We show that significant water wave amplification is obtained in a water resonator consisting of two spatially separated patches of small-amplitude sinusoidal corrugations on an otherwise flat seabed. The corrugations reflect the incident waves according to the so-called Bragg reflection mechanism, and the distance between the two sets controls whether the trapped reflected waves experience constructive or destructive interference within the resonator. The resulting amplification or suppression is enhanced with increasing number of ripples and is most effective for specific resonator lengths and at the Bragg frequency, which is determined by the corrugation period. Our analysis draws on the analogous mechanism that occurs between two partially reflecting mirrors in optics, a phenomenon named after its discoverers Charles Fabry and Alfred Perot.

  16. Line-imaging Fabry-Perot interferometer

    Energy Technology Data Exchange (ETDEWEB)

    Mathews, A.R.; Warnes, R.H.; Hemsing, W.F.; Whittemore, G.R.

    1990-01-01

    A method for measuring the velocity history of a line element on a shock-loaded solid has been demonstrated. Light from single-frequency laser is focused through a cylindrical lens to a line on a moving target. The return Doppler-shifted image is passed through a Fabry-Perot interferometer. Because only specific combinations of incident light angle and frequency can pass through the interferometer the output is an incomplete image of the moving target appearing as a set of fringes. This image is focused onto an electronic streak camera and swept in time. The fringe pattern changes with time as the target surface moves, allowing determination of velocity for each point on the target that forms a fringe. Because the velocity can only be measured at the fringe positions, it is necessary to use an interpolating polynomial to obtain a continuous function of time and velocity along the sampled lien. 9 refs., 7 figs.

  17. The many facets of the Fabry-Perot

    CERN Document Server

    Sanchez-Soto, Luis L; Leuchs, Gerd

    2016-01-01

    We address the response, both in amplitude and intensity, of a Fabry-Perot from a variety of viewpoints. These complementary pictures conspire to achieve a comprehensive and consistent theory of the operation of this system.

  18. Enhanced DNA-directed effects of FdUMP[10] compared to 5FU.

    Science.gov (United States)

    Gmeiner, William H; Trump, Eric; Wei, Cui

    2004-01-01

    FdUMP[N] molecules and conjugates are much more effective at inhibiting the proliferation of human tumor cells than is the widely used anticancer drug 5-fluorouracil (5FU). We have evaluated the inhibition of thymidylate synthase (TS), the extent of DNA damage, cell cycle arrest, and the induction of apoptosis by FdUMP[10] and 5FU in the human colorectal cancer cell line HT29. The magnitude and duration of TS inhibition following exposure of HT29 cells to FdUMP[10] at 1 x 10(-8) M was greater than that which occurred following exposure of these cells to 5FU at 1 x 10(-6) M. FdUMP[10] exposure also resulted in much more extensive DNA damage to HT29 cells than occurred following exposure to 100-fold higher concentrations of 5FU. Although exposure of HT29 cells to both drugs resulted in S-phase arrest, more complete accumulation of cells in S-phase was achieved following FdUMP[10] exposure at much lower drug concentrations. FdUMP[10] was also much more effective at inducing apoptosis in HT29 cells than was 5FU. The results are consistent with FdUMP[10] being much more efficient that 5FU at inducing DNA damage that results in apoptotic cell death in colon cancer cells.

  19. Exposure estimate for FD&C colour additives for the US population

    Science.gov (United States)

    Folmer, Daniel E.; Lee, Hyoung S.; Butts, Kyla M.; Carberry, Susan E.

    2016-01-01

    Dietary exposures to the seven food, drug, and cosmetic (FD&C) colour additives that are approved for general use in food in the United States were estimated for the US population (aged 2 years and older), children (aged 2–5 years) and teenage boys (aged 13–18 years) based on analytical levels of the FD&C colour additives in foods. Approximately 600 foods were chosen for analysis, based on a survey of product labels, for the levels of FD&C colour additives. Dietary exposure was estimated using both 2-day food consumption data from the combined 2007–10 National Health and Nutrition Examination Survey (NHANES) and 10–14-day food consumption data from the 2007–10 NPD Group, Inc. National Eating Trends – Nutrient Intake Database (NPD NET-NID). Dietary exposure was estimated at the mean and 90th percentile using three different exposure scenarios: low exposure, average exposure and high exposure, to account for the range in the amount of each FD&C colour additive for a given food. For all populations and all exposure scenarios, the highest cumulative eaters-only exposures in food were determined for FD&C Red No. 40, FD&C Yellow No. 5 and FD&C Yellow No. 6. In addition, the eaters-only exposure was estimated for individual food categories in order to determine which food categories contributed the most to the exposure for each FD&C colour additive. Breakfast Cereal, Juice Drinks, Soft Drinks, and Frozen Dairy Desserts/Sherbet (also referred to as Ice Cream, Frozen Yogurt, Sherbet (including Bars, Sticks, Sandwiches)) were the major contributing food categories to exposure for multiple FD&C colour additives for all three populations. PMID:27092991

  20. On the role of polynomials in RBF-FD approximations: I. Interpolation and accuracy

    Science.gov (United States)

    Flyer, Natasha; Fornberg, Bengt; Bayona, Victor; Barnett, Gregory A.

    2016-09-01

    Radial basis function-generated finite difference (RBF-FD) approximations generalize classical grid-based finite differences (FD) from lattice-based to scattered node layouts. This greatly increases the geometric flexibility of the discretizations and makes it easier to carry out local refinement in critical areas. Many different types of radial functions have been considered in this RBF-FD context. In this study, we find that (i) polyharmonic splines (PHS) in conjunction with supplementary polynomials provide a very simple way to defeat stagnation (also known as saturation) error and (ii) give particularly good accuracy for the tasks of interpolation and derivative approximations without the hassle of determining a shape parameter. In follow-up studies, we will focus on how to best use these hybrid RBF polynomial bases for FD approximations in the contexts of solving elliptic and hyperbolic type PDEs.

  1. The GREGOR Fabry-P\\'erot Interferometer

    CERN Document Server

    Puschmann, K G; Kneer, F; Erdogan, N Al; Balthasar, H; Bauer, S M; Beck, C; González, N Bello; Collados, M; Hahn, T; Hirzberger, J; Hofmann, A; Louis, R E; Nicklas, H; Okunev, O; Pillet, V Martínez; Popow, E; Seelemann, T; Volkmer, R; Wittmann, A D; Woche, M

    2012-01-01

    The GREGOR Fabry-P\\'erot Interferometer (GFPI) is one of three first-light instruments of the German 1.5-meter GREGOR solar telescope at the Observatorio del Teide, Tenerife, Spain. The GFPI uses two tunable etalons in collimated mounting. Thanks to its large-format, high-cadence CCD detectors with sophisticated computer hard- and software it is capable of scanning spectral lines with a cadence that is sufficient to capture the dynamic evolution of the solar atmosphere. The field-of-view (FOV) of 50" x 38" is well suited for quiet Sun and sunspot observations. However, in the vector spectropolarimetric mode the FOV reduces to 25" x 38". The spectral coverage in the spectroscopic mode extends from 530-860 nm with a theoretical spectral resolution R of about 250,000, whereas in the vector spectropolarimetric mode the wavelength range is at present limited to 580-660 nm. The combination of fast narrow-band imaging and post-factum image restoration has the potential for discovery science concerning the dynamic Su...

  2. A fully differential OTA with dynamic offset cancellation in 28nm FD-SOI process

    Science.gov (United States)

    Jaworski, Zbigniew

    2016-12-01

    This papers presents a classic fully differential operational transconductance amplifier (FDOTA) implemented in industrial 28 nm FD-SOI (Fully-Depleted SOI) technology. A novel approach to minimized the FDOTA offset voltage is proposed. The solution employs the unique feature of FD-SOI technology - back-gate biasing - combined with modern compensation methodology. The proposed method results in considerable design overhead. However, this offset cancellation approach is very effective and allows to improve FDOTA performance when classic techniques reach their limits.

  3. Survey on the prevalence of GERD and FD based on the Montreal definition and the Rome III criteria among patients presenting with epigastric symptoms in Japan.

    Science.gov (United States)

    Ohara, Shuichi; Kawano, Tatsuyuki; Kusano, Motoyasu; Kouzu, Teruo

    2011-05-01

    The present survey aimed to clarify the prevalence of gastroesophageal reflux disease (GERD) and functional dyspepsia (FD) in patients presenting with epigastric symptoms in Japan based on the Montreal definition and the Rome III criteria, respectively, and to determine the degree of overlap between the two disease entities and the validity of using these Western-developed diagnostic criteria in Japan. Patients presenting with epigastric symptoms for whom the first upper gastrointestinal endoscopy was scheduled from April through August 2007 at 55 institutions were asked to complete a questionnaire to ascertain the type, frequency, and severity of epigastric symptoms. The prevalence of esophageal mucosal damage was also determined from endoscopic findings. A total of 1,076 patients were included in the analysis population. There was a high degree of coincidence for all symptoms, with the mean number of symptoms per patient of 2.8. With strict application of the Montreal and Rome III definitions, symptomatic GERD accounted for 15.6% (168 patients), whereas FD accounted for 10.3% (111 patients), and the overlap between GERD and FD symptoms was less than 10%. However, when frequency and severity alone were considered in more broadly defined criteria, the overlap between GERD and FD symptoms was 30-40%. A highly specific disease classification is possible when the Montreal definition and the Rome III criteria are strictly applied. On the other hand, the present survey highlighted a problem with the criteria whereby a definitive diagnosis could not be made in a substantial number of patients. This problem will require further research.

  4. Development of Rotary Balance System in FD09 Wind Tunnel%FD09风洞旋转天平试验系统研制

    Institute of Scientific and Technical Information of China (English)

    黄浩; 张永升; 刘丹

    2014-01-01

    For the purpose of analyzing and predicting spin characteristics of aircraft ,the rotary balance testing in wind tunnel is mainly used to measure aerodynamic forces acting on aircraft model rotating around the longitudi‐nal axis of wind axes system .For this case ,the rotary balance system is developed in FD09 low speed wind tun‐nel .The design feature and performance index of rotary balance system are introduced .Verification and com‐parison by using SDM (Standard Dynamic Model) and fighter plane model are performed .The results indicate that the rotary balance system of FD09 wind tunnel is stable and reliable ,the repetition of test curves of FD09 wind tunnel are agree well with the reference curves ,while the test curves of FD09 wind tunnel are more smoothly than reference curves ,and the aerodynamic coefficients yielded by the rotary balance system of FD09 wind tunnel have high accuracy .The rotary balance system of FD09 wind tunnel can be put into the project tests and aerodynamic research .%为了分析和预测飞机的尾旋特性,一般通过旋转天平风洞试验测定飞机模型在不同姿态角时绕风轴以不同旋转速率作等速旋转状态下的气动特性。针对上述情况,研制 FD09低速风洞旋转天平试验系统,介绍该旋转天平试验系统的设计特点、性能指标,并进行SDM标模和战斗机模型对比验证。结果表明:本试验系统工作稳定可靠,试验结果与参考曲线有较好的重复性,并且本试验系统试验曲线的光滑性要更好一些,同时本试验系统给出的试验数据精度较高,可以用于开展型号试验及相关空气动力学研究。

  5. Calibrating echelle spectrographs with Fabry-Perot etalons

    CERN Document Server

    Bauer, Florian F; Reiners, Ansgar

    2015-01-01

    Over the past decades hollow-cathode lamps have been calibration standards for spectroscopic measurements. Advancing to cm/s radial velocity precisions with the next generation of instruments requires more suitable calibration sources with more lines and less dynamic range problems. Fabry-Perot interferometers provide a regular and dense grid of lines and homogeneous amplitudes making them good candidates for next generation calibrators. We investigate the usefulness of Fabry-Perot etalons in wavelength calibration, present an algorithm to incorporate the etalon spectrum in the wavelength solution and examine potential problems. The quasi periodic pattern of Fabry-Perot lines is used along with a hollow-cathode lamp to anchor the numerous spectral features on an absolute scale. We test our method with the HARPS spectrograph and compare our wavelength solution to the one derived from a laser frequency comb. The combined hollow-cathode lamp/etalon calibration overcomes large distortion (50 m/s) in the wavelengt...

  6. Time Delay Properties of a Fabry-Perot Interferometer

    Institute of Scientific and Technical Information of China (English)

    YUAN Shi; MAN Wei-Ning; YU Jin; GAO Jin-Yue

    2001-01-01

    The time delay properties of a Fabry-Perot interferometer are investigated. We found that the group velocity of light through a Fabry-Perot interferometer can be reduced to 10-4 of the light speed in vacuum and the time delay is 210ns, when the reflectivity is 0.999 and the distance between two mirrors is 1 cm. The system is analogous to the recently proposed one-dimensional photonic band-gap structures with a defect [Zhu et al. Opt.Commun. 174(2000)139].

  7. Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients

    Directory of Open Access Journals (Sweden)

    Hollak Carla EM

    2011-10-01

    Full Text Available Abstract Background Enzyme replacement therapy is currently the only approved therapy for Fabry disease. From June 2009 on, viral contamination of Genzyme's production facility resulted in a worldwide shortage of agalsidase beta leading to involuntary dose reductions (approved dose 1 mg/kg/eow, reduced dose 0.5 mg/kg/m, or switch to agalsidase alpha (administered dose 0.2 mg/kg/eow. An assessment report from the European Medicines Agency (EMA raised serious concerns about an increase in adverse events at lower dosages of agalsidase beta. We determined the influence of the shortage on clinical event incidence and the most sensitive biochemical marker (lysoGb3 in Dutch Fabry patients. Methods The incidence of clinical events per person per year was calculated from start of agalsidase beta treatment until the shortage, and was compared to the incidence of clinical events during the shortage period. In addition, plasma lysoGb3, eGFR, quality of life (SF-36 and brief pain inventory (BPI questionnaires were analysed. Results All thirty-five Dutch Fabry patients using agalsidase beta (17 males were included. Mean clinical event incidence was unchanged: 0.15 events per person per year before versus 0.15 during the shortage (p = 0.68. In total 28 clinical events occurred in 14 patients during 4.6 treatment years, compared to 7 events in 6 patients during the 1.3 year shortage period. eGFR and BPI scores were not significantly altered. Two SF-36 subscales were significantly but minimally reduced in females. In males, lysoGb3 increased with a median of 8.1 nM (range 2.5 - 29.2 after 1 year of shortage (p = 0.001. Increases in lysoGb3 were found in both patients switching to agalsidase alpha and on a reduced agalsidase beta dose. Antibody status, treatment duration or clinical event incidence showed no clear correlation to lysoGb3 increases. Conclusions No increase in clinical event incidence was found in the adult Dutch Fabry cohort during the agalsidase

  8. Mathematical model of Fabry-Perot cavity filter%WDM设计中的Fabry-Perot腔传递函数模型

    Institute of Scientific and Technical Information of China (English)

    孙雅东; 武良春

    2003-01-01

    作为WDM(Wave Division Multiplexing)一个重要的器件,Fabry-Perot腔的相关设计和算法不断更新.笔者提出一种新的数学模型:把Fabry-Perot作为系统的一个参数对待,将信号与系统的知识纳入Fabry-Perot腔中,用系统的方法(梅森公式)求解Fabry-Perot腔的传递函数,避免了繁琐的矩阵运算,这样易于理解和优化设计.

  9. Synchronized oscillations of dimers in biphasic charged fd-virus suspensions

    Science.gov (United States)

    Kang, K.; Piao, S. H.; Choi, H. J.

    2016-08-01

    Micron-sized colloidal spheres that are dispersed in an isotropic-nematic biphasic host suspension of charged rods (fd-virus particles) are shown to spontaneously form dimers, which exhibit a synchronized oscillatory motion. Dimer formation is not observed in the monophase of isotropic and nematic suspensions. The synchronized oscillations of dimers are connected to the inhomogeneous state of the host suspension of charged rods (fd viruses) where nematic domains are in coexistence with isotropic regions. The synchronization of oscillations occurs in bulk states, in the absence of an external field. With a low field strength of an applied electric field, the synchronization is rather reduced, but it recovers again when the field is turned off. In this Rapid Communication, we report this observation as an example of the strange attractor, occurring in the mixture of PS (polystyrene) dimers in an isotropic-nematic coexistence biphasic fd-virus network. Furthermore, we highlight that the synchronization of PS-dimer oscillations is the result of a global bifurcation diagram, driven by a delicate balance between the short-attractive "twisted" interaction of PS dimers and long-ranged electrostatic repulsive interactions of charged fd rods. The interest is then in the local enhancement of "twist-nematic" elasticity in reorientation of the dimer oscillations. An analysis of image-time correlations is provided with the data movies and Fourier transforms of averaged orientations for the synchronized oscillations of dimers in the biphasic I -N coexistence concentration of charged fd-virus suspensions.

  10. Efficient fdCas9 Synthetic Endonuclease with Improved Specificity for Precise Genome Engineering

    KAUST Repository

    Aouida, Mustapha

    2015-07-30

    The Cas9 endonuclease is used for genome editing applications in diverse eukaryotic species. A high frequency of off-target activity has been reported in many cell types, limiting its applications to genome engineering, especially in genomic medicine. Here, we generated a synthetic chimeric protein between the catalytic domain of the FokI endonuclease and the catalytically inactive Cas9 protein (fdCas9). A pair of guide RNAs (gRNAs) that bind to sense and antisense strands with a defined spacer sequence range can be used to form a catalytically active dimeric fdCas9 protein and generate double-strand breaks (DSBs) within the spacer sequence. Our data demonstrate an improved catalytic activity of the fdCas9 endonuclease, with a spacer range of 15–39 nucleotides, on surrogate reporters and genomic targets. Furthermore, we observed no detectable fdCas9 activity at known Cas9 off-target sites. Taken together, our data suggest that the fdCas9 endonuclease variant is a superior platform for genome editing applications in eukaryotic systems including mammalian cells.

  11. A Fabry-Perot Solid Etalon for Teaching.

    Science.gov (United States)

    Bruce, P. J.; And Others

    1986-01-01

    Describes a solid etalon Fabry-Perot interferometer, discussing free spectral range, instrumental finesse, and temperature effects. Provides schematic of temperature control/display circuit. Explains use of 100 millimeter camera lens and 10 power micrometer eyepiece for resolving rings and measure diameters. (JM)

  12. A Coaxial Cable Fabry-Perot Interferometer for Sensing Applications

    Directory of Open Access Journals (Sweden)

    Ming Luo

    2013-11-01

    Full Text Available This paper reports a novel coaxial cable Fabry-Perot interferometer for sensing applications. The sensor is fabricated by drilling two holes half-way into a coaxial cable. The device physics was described. The temperature and strain responses of the sensor were tested. The measurement error was calculated and analyzed.

  13. Development of the Fabry-Perot Spectrometer Application

    Science.gov (United States)

    Browne, Kathryn

    2015-01-01

    Methane is a greenhouse gas with global warming effects 20 times more detrimental than carbon dioxide. Currently, only aircraft missions measure methane and do not provide continuous monitoring, This presentation will cover the Fabry-Perot spectrometer which will provide continuous monitoring of methane. It will also cover the development of the software used to extract and process the data the spectrometer collects.

  14. Gamma irradiation of Fabry-Perot interband cascade lasers

    Energy Technology Data Exchange (ETDEWEB)

    Myers, Tanya L.; Cannon, Bret D.; Brauer, Carolyn S.; Canedy, Chadwick L.; Kim, Chul Soo; Kim, Mijin; Merritt, Charles D.; Bewley, William W.; Vurgaftman, Igor; Meyer, Jerry R.

    2017-09-20

    Two Fabry-Perot interband cascade lasers (ICLs) were exposed to Cobalt-60 gamma rays for a dosage of 500 krad(Si) each, which is higher than is typically encountered in space applications. The ICLs do not show any significant changes in threshold current or slope efficiency, suggesting the suitability of ICLs for use in radiation environments.

  15. Silicon Carbide Mounts for Fabry-Perot Interferometers

    Science.gov (United States)

    Lindemann, Scott

    2011-01-01

    Etalon mounts for tunable Fabry- Perot interferometers can now be fabricated from reaction-bonded silicon carbide structural components. These mounts are rigid, lightweight, and thermally stable. The fabrication of these mounts involves the exploitation of post-casting capabilities that (1) enable creation of monolithic structures having reduced (in comparison with prior such structures) degrees of material inhomogeneity and (2) reduce the need for fastening hardware and accommodations. Such silicon carbide mounts could be used to make lightweight Fabry-Perot interferometers or could be modified for use as general lightweight optical mounts. Heretofore, tunable Fabry-Perot interferometer structures, including mounting hardware, have been made from the low-thermal-expansion material Invar (a nickel/iron alloy) in order to obtain the thermal stability required for spectroscopic applications for which such interferometers are typically designed. However, the high mass density of Invar structures is disadvantageous in applications in which there are requirements to minimize mass. Silicon carbide etalon mounts have been incorporated into a tunable Fabry-Perot interferometer of a prior design that originally called for Invar structural components. The strength, thermal stability, and survivability of the interferometer as thus modified are similar to those of the interferometer as originally designed, but the mass of the modified interferometer is significantly less than the mass of the original version.

  16. Variable Free Spectral Range Spherical Mirror Fabry-Perot Interferometer

    CERN Document Server

    Kerner, K; Yashchuk, V V; Budker, D; Kerner, Katherine; Rochester, Simon M.; Yashchuk, Valeriy V.

    2003-01-01

    A spherical Fabry-Perot interferometer with adjustable mirror spacing is used to produce interference fringes with frequency separation (c/2L)/N, N=2-15. The conditions for observation of these fringes are derived from the consideration of the eigenmodes of the cavity with high transverse indices.

  17. A Demonstration of TIA Using FD-SOI CMOS OPAMP for Far-Infrared Astronomy

    Science.gov (United States)

    Nagase, Koichi; Wada, Takehiko; Ikeda, Hirokazu; Arai, Yasuo; Ohno, Morifumi; Hanaoka, Misaki; Kanada, Hidehiro; Oyabu, Shinki; Hattori, Yasuki; Ukai, Sota; Suzuki, Toyoaki; Watanabe, Kentaroh; Baba, Shunsuke; Kochi, Chihiro; Yamamoto, Keita

    2016-07-01

    We are developing a fully depleted silicon-on-insulator (FD-SOI) CMOS readout integrated circuit (ROIC) operated at temperatures below ˜ 4 K. Its application is planned for the readout circuit of high-impedance far-infrared detectors for astronomical observations. We designed a trans-impedance amplifier (TIA) using a CMOS operational amplifier (OPAMP) with FD-SOI technique. The TIA is optimized to readout signals from a germanium blocked impurity band (Ge BIB) detector which is highly sensitive to wavelengths of up to ˜ 200 \\upmu m. For the first time, we demonstrated the FD-SOI CMOS OPAMP combined with the Ge BIB detector at 4.5 K. The result promises to solve issues faced by conventional cryogenic ROICs.

  18. Functional Characterization of Phalaenopsis aphrodite Flowering Genes PaFT1 and PaFD.

    Science.gov (United States)

    Jang, Seonghoe; Choi, Sang-Chul; Li, Hsing-Yi; An, Gynheung; Schmelzer, Elmon

    2015-01-01

    We show that the key flowering regulators encoded by Phalaenopsis aphrodite FLOWERING LOCUS T1 (PaFT1) and PaFD share high sequence homologies to these from long-day flowering Arabidopsis and short-day flowering rice. Interestingly, PaFT1 is specifically up-regulated during flowering inductive cooling treatment but is not subjected to control by photoperiod in P. aphrodite. Phloem or shoot apex-specific expression of PaFT1 restores the late flowering of Arabidopsis ft mutants. Moreover, PaFT1 can suppress the delayed flowering caused by SHORT VEGATATIVE PHASE (SVP) overexpression as well as an active FRIGIDA (FRI) allele, indicating the functional conservation of flowering regulatory circuit in different plant species. PaFT1 promoter:GUS in Arabidopsis showed similar staining pattern to that of Arabidopsis FT in the leaves and guard cells but different in the shoot apex. A genomic clone or heat shock-inducible expression of PaFT1 is sufficient to the partial complementation of the ft mutants. Remarkably, ectopic PaFT1 expression also triggers precocious heading in rice. To further demonstrate the functional conservation of the flowering regulators, we show that PaFD, a bZIP transcription factor involved in flowering promotion, interacts with PaFT1, and PaFD partially complemented Arabidopsis fd mutants. Transgenic rice expressing PaFD also flowered early with increased expression of rice homologues of APETALA1 (AP1). Consistently, PaFT1 knock-down Phalaenopsis plants generated by virus-induced gene silencing exhibit delayed spiking. These studies suggest functional conservation of FT and FD genes, which may have evolved and integrated into distinct regulatory circuits in monopodial orchids, Arabidopsis and rice that promote flowering under their own inductive conditions.

  19. Functional Characterization of Phalaenopsis aphrodite Flowering Genes PaFT1 and PaFD.

    Directory of Open Access Journals (Sweden)

    Seonghoe Jang

    Full Text Available We show that the key flowering regulators encoded by Phalaenopsis aphrodite FLOWERING LOCUS T1 (PaFT1 and PaFD share high sequence homologies to these from long-day flowering Arabidopsis and short-day flowering rice. Interestingly, PaFT1 is specifically up-regulated during flowering inductive cooling treatment but is not subjected to control by photoperiod in P. aphrodite. Phloem or shoot apex-specific expression of PaFT1 restores the late flowering of Arabidopsis ft mutants. Moreover, PaFT1 can suppress the delayed flowering caused by SHORT VEGATATIVE PHASE (SVP overexpression as well as an active FRIGIDA (FRI allele, indicating the functional conservation of flowering regulatory circuit in different plant species. PaFT1 promoter:GUS in Arabidopsis showed similar staining pattern to that of Arabidopsis FT in the leaves and guard cells but different in the shoot apex. A genomic clone or heat shock-inducible expression of PaFT1 is sufficient to the partial complementation of the ft mutants. Remarkably, ectopic PaFT1 expression also triggers precocious heading in rice. To further demonstrate the functional conservation of the flowering regulators, we show that PaFD, a bZIP transcription factor involved in flowering promotion, interacts with PaFT1, and PaFD partially complemented Arabidopsis fd mutants. Transgenic rice expressing PaFD also flowered early with increased expression of rice homologues of APETALA1 (AP1. Consistently, PaFT1 knock-down Phalaenopsis plants generated by virus-induced gene silencing exhibit delayed spiking. These studies suggest functional conservation of FT and FD genes, which may have evolved and integrated into distinct regulatory circuits in monopodial orchids, Arabidopsis and rice that promote flowering under their own inductive conditions.

  20. Electrical Changes in Resting, Exercise, and Holter Electrocardiography in Fabry Cardiomyopathy.

    Science.gov (United States)

    Krämer, Johannes; Nordbeck, Peter; Störk, Stefan; Ritter, Christian; Ertl, Georg; Wanner, Christoph; Weidemann, Frank

    2015-10-27

    In Fabry cardiomyopathy, little is known about the interaction between its key feature of myocardial replacement fibrosis and changes in resting, Holter, and exercise electrocardiography (-ECG). Resting ECG, 24-h Holter ECG, and exercise ECG were performed in 95 patients (50 women) with Fabry disease, staged using cardiac magnetic resonance imaging to measure left ventricular fibrosis. With resting ECG, T alterations were seen in patients with cardiac fibrosis, while time intervals and rhythm were unchanged (except for a longer QRS duration in patients with severe fibrosis). All patients with severe fibrosis showed T inversion, ST alteration, or both. With Holter ECG, maximum and minimum heart rate did not differ with fibrosis severity. Patients without fibrotic tissue showed less ventricular premature beats (VPB) (median 5/24 h) compared to those with mild (median 11/24 h) or severe fibrosis (median 115/24 h; P 10 mmHg) occurred in some patients with no (3/41) or mild fibrosis (3/34) but not in patients with severe fibrosis (0/20; P Holter ECG. During exercise ECG, advanced cardiomyopathy is characterized by chronotropic incompetence with limitations on blood pressure and heart rate increase.

  1. 21 CFR 74.705 - FD&C Yellow No. 5.

    Science.gov (United States)

    2010-04-01

    ... butter, cheese, and ice cream, shall specifically declare the presence of FD&C Yellow No. 5 by listing...&C Yellow No. 5 is principally the trisodium salt of 4,5-dihydro-5-oxo-1-(4-sulfophenyl)-4- -1H..., or a salt of this carboxylic acid. The resulting dye is purified and isolated as the sodium salt....

  2. A DLM/FD method for fluid/flexible-body interactions

    NARCIS (Netherlands)

    Yu, Zhaosheng

    2005-01-01

    In this study, we extended the distributed-Lagrange-multiplier/fictitious-domain (DLM/FD) formulation of Glowinski et al. [Int. J. Multiphase Flow 25 (1999) 755] for the fluid/rigid-body interactions to deal with the fluid/flexible-body interactions by replacing Newton’s equations of motion for the

  3. 77 FR 12610 - Pre-Disaster Emergency Declaration Requests, FD 010-4

    Science.gov (United States)

    2012-03-01

    ... SECURITY Federal Emergency Management Agency Pre-Disaster Emergency Declaration Requests, FD 010-4 AGENCY...: The Federal Emergency Management Agency (FEMA) is accepting comments on Pre-Disaster Emergency... upon the date of issuance. This policy applies to pre-disaster emergency declaration requests from all...

  4. The Arabidopsis Floral Repressor BFT DelaysFlowering by Competing with FT for FD Bindingunder High Salinity

    Institute of Scientific and Technical Information of China (English)

    2014-01-01

    Soil salinity is one of the most serious agricultural problems that significantly reduce crop yields in the aridand semi-arid regions. It influences various phases of plant growth and developmental processes, such as seed germina-tion, leaf and stem growth, and reproductive propagation. Salt stress delays the onset of flowering in many plant spe-cies. We have previously reported that the Arabidopsis BROTHER OF FT AND TFL1 (BFT) acts as a floral repressor undersalt stress. However, the molecular mechanisms underlying the BFT function in the salt regulation of flowering inductionis unknown. In this work, we found that BFT delays flowering under high salinity by competing with FLOWERING LOCUST (FT) for binding to the FD transcription factor. The flowering time of FD-deficient fd-2 mutant was insensitive to highsalinity. BFT interacts with FD in the nucleus via the C-terminal domain of FD, which is also required for the interactionof FD with FT, and interferes with the FT-FD interaction. These observations indicate that BFT constitutes a distinct saltstress signaling pathway that modulates the function of the FT-FD module and possibly provides an adaptation strategythat fine-tunes photoperiodic flowering under high salinity.

  5. Variations on the "Blue-Bottle" Demonstration Using Food Items That Contain FD&C Blue #1

    Science.gov (United States)

    Staiger, Felicia A.; Peterson, Joshua P.; Campbell, Dean J.

    2015-01-01

    Erioglaucine dye (FD&C Blue #1) can be used instead of methylene blue in the classic "blue-bottle" demonstration. Food items containing FD&C Blue #1 and reducing species such as sugars can therefore be used at the heart of this demonstration, which simply requires the addition of strong base such as sodium hydroxide lye.

  6. The finite-difference time-domain (FD-TD) method for electromagnetic scattering and interaction problems

    Science.gov (United States)

    Taflove, A.; Umashankar, K. R.

    1987-01-01

    The formulation and recent applications of the finite-difference time-domain (FD-TD) method for the numerical modeling of electromagnetic scattering and interaction problems are considered. It is shown that improvements in FD-TD modeling concepts and software implementation often make it a preferable choice for structures which cannot be easily treated by conventional integral equations and asymptotic approaches. Recent FD-TD modeling validations in research areas including coupling to wires and wire bundles in free space and cavities, scattering from surfaces in relativistic motion, inverse scattering, and radiation condition theory, are reviewed. Finally, the advantages and disadvantages of FD-TD, and guidelines concerning when FD-TD should and should not be used in high-frequency electromagnetic modeling problems, are summarized.

  7. Single-SectionFabry-Perot Mode-Locked Semiconductor Lasers

    Directory of Open Access Journals (Sweden)

    Weiguo Yang

    2011-01-01

    Full Text Available We present a review of the theoretical models and experimental verification of the single-section Fabry-Perot mode-locked semiconductor lasers based on multiple-spatial-mode (MSM coupling. The mode-locked operation at the repetition rates of 40 GHz and higher and the pulse width of a few picoseconds are confirmed by the intensity autocorrelation, the fast photo detection and RF spectrum, and the optical spectral interference measurement of ultrafast pulse. The spatial mode coupling theory of single-section Fabry-Perot mode-locked semiconductor lasers is also reviewed, and the results are compared with the experimental observations. The small signal modulation response of these lasers, which exhibits high-frequency responses well beyond the relaxation oscillation resonance limit, is also modeled theoretically, and the simulation is verified by the experimental measurements.

  8. Transforming Fabry-Perot resonances into a Tamm mode

    CERN Document Server

    Durach, Maxim

    2012-01-01

    We propose a novel photonic structure composed of metal nanolayer, Bragg mirror and metal nanolayer. The structure supports resonances that are transitional between Fabry-Perot and Tamm modes. When the dielectric contrast of the DBR is removed these modes are a pair of conventional Fabry-Perot resonances. They spectrally merge into a Tamm mode at high contrast. Such behavior differs from the results for structures supporting Tamm modes reported earlier. The optical properties of the structure in the frequency range of the DBR stop band, including highly beneficial 50% transmittivity through thick structures, are determined by the introduced in the paper hybrid resonances. The results can find a wide range of photonic applications.

  9. Curvature sensor based on a Fabry-Perot interferometer

    Science.gov (United States)

    Monteiro, Catarina; Ferreira, Marta S.; Kobelke, Jens; Schuster, Kay; Bierlich, Jörg; Frazão, Orlando

    2016-05-01

    A curvature sensor based on a Fabry-Perot interferometer is proposed. A capillary tube of silica is fusion spliced between two single mode fibers, producing a Fabry-Perot cavity. The light propagates in air, when passing through the capillary tube. Two different cavities are subjected to curvature and temperature. The cavity with shorter length shows insensitivity to both measurands. The larger cavity shows two operating regions for curvature measurement, where a linear response is shown, with a maximum sensitivity of 18.77pm/m-1 for the high curvature radius range. When subjected to temperature, the sensing head produces a similar response for different curvature radius, with a sensitivity of 0.87pm/°C.

  10. Optical power equalization using Fabry-Perot semiconductor optical amplifier

    Institute of Scientific and Technical Information of China (English)

    Yun Ling; Kun Qiu; Wei Zhang; Ying Pang

    2006-01-01

    A novel scheme of optical power equalization based on Fabry-Perot semiconductor optical amplifier (FPSOA) is proposed. Because of the gain characteristic of FP-SOA, real-time controlling mechanism according to input optical power is aborted in the scheme. The simulations show that 10-dB pulse peak power variation can be clamped in less than i dB. The influences of injecting current, pulse periods, and pulse width are discussed.

  11. Treatment of Fabry's Disease with the Pharmacologic Chaperone Migalastat

    DEFF Research Database (Denmark)

    Germain, Dominique P; Hughes, Derralynn A; Nicholls, Kathleen

    2016-01-01

    a significant treatment effect: 13 of 32 patients (41%) who received migalastat and 9 of 32 patients (28%) who received placebo had a response at 6 months (P=0.30). Among patients with suitable mutant α-galactosidase who received migalastat for up to 24 months, the annualized changes from baseline...

  12. Fabry's Disease: Case Series and Review of Literature

    African Journals Online (AJOL)

    case series of five patient's along with the review of literature is presented here. Keywords: ... underwent a renal transplant biopsy to ascertain the cause of ... A 16-year-old male presented in March 2002 with a fever of unknown origin and splenomegaly. ... There was no evidence of tuft necrosis/crescent formation or.

  13. Fabry-Perot腔滤波器的数学模型%The Mathematical Model of the Fabry-Perot Filter

    Institute of Scientific and Technical Information of China (English)

    齐永兴; 陈树强; 刘元安; 钱宗珏; 赵国谦

    2004-01-01

    将信号与系统的知识应用于Fabry-Perot(FP)腔滤波器的分析和设计中,避免了繁琐的矩阵运算,而且FP腔滤波器同整个系统紧密地联系在一起,使其成为一个以传递函数为特征的器件,更易于理解和优化设计.

  14. Controlled surface adsorption of fd filamentous phage by tuning of the pH and the functionalization of the surface

    Science.gov (United States)

    Jeon, Dae-Young; Hwang, Kyung Hoon; Park, So-Jeong; Kim, Yun-Jeong; Joo, Min-Kyu; Ahn, Seung-Eon; Kim, Gyu-Tae; Nam, Chang-Hoon

    2011-03-01

    The surface adsorption of fd filamentous phage (fd phage) dispersed in different solution pHs was investigated with functionalized SiO2/Si substrates. The fd phages at high pH (˜9.0) were well-adsorbed on the SiO2/Si surface that was functionalized by 3-aminopropyltriethoxysilane, whereas those at low pH (˜3.0) were well-adsorbed on the cleaned SiO2/Si surface. The high affinity of the carboxylic acid groups (COO-) at high pH (˜9.0) was attributed to the fact that they give a higher adsorption to the positively charged amine groups (NH3+) on the surface of the substrate, similar to the effect of H3O+ at low pH (˜3.0) in a solution on the surface of the hydroxyl groups on the substrate (OH-). Interestingly, the aligned structures of the fd phage at intermediate pH (˜7.0), caused by the locally positively charged coat protein of the fd phage and the shear forces along the washing and blowing direction, were identified. The effective spring constant of the fd phage bundles was estimated to be 0.672 N/m using a force-distance curve. Our results offer prerequisite information for the bottom-up assembly in SiO2/Si substrates using the fd phage in bionanoelectronics.

  15. Dual role of tree florigen activation complex component FD in photoperiodic growth control and adaptive response pathways.

    Science.gov (United States)

    Tylewicz, Szymon; Tsuji, Hiroyuki; Miskolczi, Pál; Petterle, Anna; Azeez, Abdul; Jonsson, Kristoffer; Shimamoto, Ko; Bhalerao, Rishikesh P

    2015-03-10

    A complex consisting of evolutionarily conserved FD, flowering locus T (FT) proteins is a regulator of floral transition. Intriguingly, FT orthologs are also implicated in developmental transitions distinct from flowering, such as photoperiodic control of bulbing in onions, potato tuberization, and growth cessation in trees. However, whether an FT-FD complex participates in these transitions and, if so, its mode of action, are unknown. We identified two closely related FD homologs, FD-like 1 (FDL1) and FD-like 2 (FDL2), in the model tree hybrid aspen. Using gain of function and RNAi-suppressed FDL1 and FDL2 transgenic plants, we show that FDL1 and FDL2 have distinct functions and a complex consisting of FT and FDL1 mediates in photoperiodic control of seasonal growth. The downstream target of the FT-FD complex in photoperiodic control of growth is Like AP1 (LAP1), a tree ortholog of the floral meristem identity gene APETALA1. Intriguingly, FDL1 also participates in the transcriptional control of adaptive response and bud maturation pathways, independent of its interaction with FT, presumably via interaction with abscisic acid insensitive 3 (ABI3) transcription factor, a component of abscisic acid (ABA) signaling. Our data reveal that in contrast to its primary role in flowering, FD has dual roles in the photoperiodic control of seasonal growth and stress tolerance in trees. Thus, the functions of FT and FD have diversified during evolution, and FD homologs have acquired roles that are independent of their interaction with FT.

  16. Precision Measurement of B(D+ -> mu+ nu) and the Pseudoscalar Decay Constant fD+

    CERN Document Server

    Eisenstein, B I; Mehrabyan, S; Lowrey, N; Selen, M; White, E J; Wiss, J; Mitchell, R E; Shepherd, M R; Besson, D; Pedlar, T K; Cronin-Hennessy, D; Gao, K Y; Hietala, J; Kubota, Y; Klein, T; Lang, B W; Poling, R; Scott, A W; Zweber, P; Dobbs, S; Metreveli, Z; Seth, K K; Tomaradze, A G; Libby, J; Martin, L; Powell, A; Wilkinson, G; Ecklund, K M; Love, W; Savinov, V; Méndez, H; Ge, J Y; Miller, D H; Shipsey, I P J; Xin, B; Adams, G S; Anderson, M; Cummings, J P; Danko, I; Hu, D; Moziak, B; Napolitano, J; He, Q; Insler, J; Muramatsu, H; Park, C S; Thorndike, E H; Yang, F; Artuso, M; Blusk, S; Khalil, S; Li, J; Menaa, N; Mountain, R; Nisar, S; Randrianarivony, K; Sultana, N; Skwarnicki, T; Stone, S; Wang, J C; Zhang, L M; Bonvicini, G; Cinabro, D; Dubrovin, M; Lincoln, A; Naik, P; Rademacker, J; Asner, D M; Edwards, K W; Reed, J; Briere, R A; Ferguson, T; Tatishvili, G; Vogel, H; Watkins, M E; Rosner, J L; Alexander, J P; Cassel, D G; Duboscq, J E; Ehrlich, R; Fields, L; Galik, R S; Gibbons, L; Gray, R; Gray, S W; Hartill, D L; Heltsley, B K; Hertz, D; Hunt, J M; Kandaswamy, J; Kreinick, D L; Kuznetsov, V E; Ledoux, J; Mahlke-Krüger, H; Mohapatra, D; Onyisi, P U E; Patterson, J R; Peterson, D; Riley, D; Ryd, A; Sadoff, A J; Shi, X; Stroiney, S; Sun, W M; Wilksen, T; Athar, S B; Patel, R; Yelton, J; Rubin, P

    2008-01-01

    We measure the branching ratio of the purely leptonic decay of the D+ meson with unprecedented precision as B(D+ -> mu+ nu) = (3.82 +/- 0.32 +/- 0.09)x10^(-4), using 818/pb of data taken on the psi(3770) resonance with the CLEO-c detector at the CESR collider. We use this determination to derive a value for the pseudoscalar decay constant fD+, combining with measurements of the D+ lifetime and assuming |Vcd| = |Vus|. We find fD+ = (205.8 +/- 8.5 +/- 2.5) MeV. The decay rate asymmetry [B(D+ -> mu+ nu)-B(D- -> mu- nu)]/[B(D+ -> mu+ nu)+B(D- -> mu- nu)] = 0.08 +/- 0.08, consistent with no CP violation. We also set 90% confidence level upper limits on B(D+ -> tau+ nu) e+ nu) < 8.8x10^(-6).

  17. Non-gravitational Perturbations and Virtual Impactors: the case of asteroid 2009 FD

    CERN Document Server

    Spoto, F; Farnocchia, D; Chesley, S R; Micheli, M; Valsecchi, G B; Perna, D; Hainaut, O

    2014-01-01

    Asteroid 2009 FD could impact Earth between 2185 and 2196. The long term propagation to the possible impacts and the intervening planetary encounters make 2009 FD one of the most challenging asteroids in terms of hazard assessment. To compute accurate impact probabilities we model the Yarkovsky effect by using the available physical characterization of 2009 FD and general properties of the Near Earth Asteroid population. We perform the hazard assessment with two independent methods: the first method is a generalization of the standard impact monitoring algorithms in use by NEODyS and Sentry, while the second one is based on a Monte Carlo approach. Both methods generate orbital samples in a 7 dimensional space that includes orbital elements and the parameter characterizing the Yarkovsky effect. The highest impact probability is $2.7 \\times 10^{-3}$ for an impact during the 2185 Earth encounter. Impacts after 2185 corresponding to resonant returns are possible, the most relevant being in 2190 with a probability...

  18. Effect of retS gene on antibiotics production in Pseudomonas fluorescens FD6.

    Science.gov (United States)

    Zhang, Qingxia; Xiao, Qi; Xu, Jingyou; Tong, Yunhui; Wen, Jia; Chen, Xijun; Wei, Lihui

    2015-11-01

    A hybrid sensor kinase termed RetS (regulator of exopolysaccharide and Type III secretion) controls expression of numerous genes in Pseudomonas aeruginosa. To investigate the function of RetS in P. fluorescens FD6, the retS gene was disrupted. Genetic inactivation of retS resulted in enhanced production of 2, 4-diacetylphloroglucinol, pyrrolnitrin, and pyoluteorin. The retS mutant also exhibited significant increase in phlA-lacZ, prnA-lacZ, and pltA-lacZ transcription levels, influencing expression levels of the small regulatory RNAs RsmX and RsmZ. In the gacSretS double mutant, all the phenotypic changes caused by the retS deletion were reversed to the level of gacS single mutant. Furthermore, the retS mutation drastically elevated biofilm formation and improved the colonization ability of strain FD6 on wheat rhizospheres. Based on these results, we proposed that RetS negatively controlled the production of antibiotics through the Gac/Rsm pathway in P. fluorescens FD6.

  19. A Fabry-Perot interferometer system for high-speed velocity measurement

    NARCIS (Netherlands)

    Cheng, L.K.; Bruinsma, A.J.A.; Prinse, W.C.; Smorenburg, C.

    1997-01-01

    The Fabry-Perot Velocity Interferometer System (F-PVIS) is designed and built for measuring the Doppler shift of light by recording positional changes in the interferometric pattern behind the Fabry-Perot interferometer. The velocity of a surface can be deduced from the Doppler shift which is caused

  20. Wide-angle spectral imaging using a Fabry-Perot interferometer

    NARCIS (Netherlands)

    Strauch, M.; Livshits, I.L.; Bociort, F.; Urbach, H.P.

    2015-01-01

    We show that wide-angle spectral imaging can be achieved with compact and cost-effective devices using Fabry-Pérot interferometers. Designs with a full field of view of 90°, in which the Fabry-Pérot interferometer is mounted either in front of an imaging lens system or behind a telecentric lens syst

  1. A Fabry-Perot interferometer system for high-speed velocity measurement

    NARCIS (Netherlands)

    Cheng, L.K.; Bruinsma, A.J.A.; Prinse, W.C.; Smorenburg, C.

    1997-01-01

    The Fabry-Perot Velocity Interferometer System (F-PVIS) is designed and built for measuring the Doppler shift of light by recording positional changes in the interferometric pattern behind the Fabry-Perot interferometer. The velocity of a surface can be deduced from the Doppler shift which is caused

  2. Holographic liquid crystal polarization grating with Fabry-Perot structure.

    Science.gov (United States)

    Sakamoto, Moritsugu; Yamaguchi, Haruki; Noda, Kohei; Sasaki, Tomoyuki; Kawatsuki, Nobuhiro; Ono, Hiroshi

    2016-03-15

    A holographic liquid crystal polarization grating with a Fabry-Perot structure was developed. Because of its resonant structure, the device offers high levels of control of the diffraction properties of incident-polarized light beams, depending on the resonance conditions. The diffracted light beams are emitted in both the reflection and transmission directions, and the device thus works as a multibranch polarization grating with double optical paths, unlike a conventional polarization grating. These device features were experimentally demonstrated and were also explained theoretically.

  3. Review: Research progress on Fabry-Perot resonator antenna

    Institute of Scientific and Technical Information of China (English)

    Zhen-guo LIU; Zhi-chen GE; Xi-yuan CHEN

    2009-01-01

    The Fabry-Perot resonator (FPR) antenna has found wide applications in microwave and millimeter waves and recently attracted considerable interest, in this paper, a summary of planar and cylindrical structures, analytic models and research development is presented, and a comparison between these structures and analytic models is made, showing that such analytic models as the FP cavity mode, electromagnetic band gap (EBG) defect mode, transmission line mode, and leaky-wave mode are consistent when applied to analyze this type of resonator antenna. Some interesting topics under recent research, including dual or multi-band, improvement of gain bandwidth, low profile and beam control, are surveyed.

  4. A stable fiber-based Fabry-Perot cavity

    CERN Document Server

    Steinmetz, T; Colombe, Y; Hunger, D; Hänsch, T W; Warburton, R J; Reichel, J

    2006-01-01

    We report the development of a fiber-based, tunable optical cavity with open access. The cavity is of the Fabry-Perot type and is formed with miniature spherical mirrors positioned on the end of single- or multi-mode optical fibers by a transfer technique which involves lifting a high-quality mirror from a smooth convex substrate, either a ball lens or micro-lens. The cavities typically have a finesse of $\\sim 1,000$ and a mode volume of 600 $\\mu$m$^3$. We demonstrate the detection of small ensembles of cold Rb atoms guided through such a cavity on an atom chip.

  5. Experimental measurement of photothermal effect in Fabry-Perot cavities

    CERN Document Server

    De Rosa, M L; Cerdonio, M; Pinard, M; Marin, F

    2002-01-01

    We report the experimental observation of the photothermal effect. The measurements are performed by modulating the laser power absorbed by the mirrors of two high-finesse Fabry-Perot cavities. The results are very well described by a recently proposed theoretical model [M. Cerdonio, L. Conti, A. Heidmann and M. Pinard, Phys. Rev. D 63 (2001) 082003], confirming the correctness of such calculations. Our observations and quantitative characterization of the photothermal effect demonstrate its critical importance for high sensitivity interferometric displacement measurements, as those necessary for gravitational wave detection.

  6. Triggering DTH and CTL Activity by fd Filamentous Bacteriophages: Role of CD4+ T Cells in Memory Responses

    Directory of Open Access Journals (Sweden)

    Giovanna Del Pozzo

    2010-01-01

    Full Text Available The ability of fd bacteriophage particles to trigger different arms of the immune system has been previously shown by us with particular emphasis on the ability of phages to raise CTL responses in vitro and in vivo. Here we show that fd virions in the absence of adjuvants are able to evoke a DTH reaction mediated by antigen specific CD8+ T cells. In addition, we analyzed the induction of CTL responses in mice depleted of CD4+ T cells, and we observed that short-term secondary CTL responses were induced in the absence of CD4+ T cells while induction of long-term memory CTLs required the presence of CD4+ T lymphocytes. These results examine the cellular mechanism at the basis of fd efficiency and provide new elements to further validate the use of fd particles for eliciting and monitoring antigen-specific CTLs.

  7. Hemodynamic Changes Caused by Flow Diverters in Rabbit Aneurysm Models: Comparison of Virtual and Realistic FD Deployments Based on Micro-CT Reconstruction.

    Science.gov (United States)

    Xu, Jinyu; Deng, Benqiang; Fang, Yibin; Yu, Ying; Cheng, Jiyong; Wang, Shengzhang; Wang, Kuizhong; Liu, Jian-Min; Huang, Qinghai

    2013-01-01

    Adjusting hemodynamics via flow diverter (FD) implantation is emerging as a novel method of treating cerebral aneurysms. However, most previous FD-related hemodynamic studies were based on virtual FD deployment, which may produce different hemodynamic outcomes than realistic (in vivo) FD deployment. We compared hemodynamics between virtual FD and realistic FD deployments in rabbit aneurysm models using computational fluid dynamics (CFD) simulations. FDs were implanted for aneurysms in 14 rabbits. Vascular models based on rabbit-specific angiograms were reconstructed for CFD studies. Real FD configurations were reconstructed based on micro-CT scans after sacrifice, while virtual FD configurations were constructed with SolidWorks software. Hemodynamic parameters before and after FD deployment were analyzed. According to the metal coverage (MC) of implanted FDs calculated based on micro-CT reconstruction, 14 rabbits were divided into two groups (A, MC >35%; B, MC 0.05). The normalized mean WSS in Group A after realistic FD implantation was significantly lower than that of Group B. All parameters in Group B exhibited no significant difference between realistic and virtual FDs. This study confirmed MC-correlated differences in hemodynamic parameters between realistic and virtual FD deployment.

  8. A 10 Gs/s latched comparator with dynamic offset cancellation in 28nm FD-SOI process

    Science.gov (United States)

    Jaworski, Zbigniew

    2016-12-01

    This papers presents a high-speed, latched comparator implemented in industrial 28 nm FD-SOI technology. A novel approach to counter the mismatch is proposed. The solution employs trimming the threshold voltage by means of modulating of back-gate polarization of FD-SOI transistors. The comparator is a first step towards the design of a complete 4-bit FLASH analog-to-digital converter, with a sampling frequency of 10 GHz.

  9. 可调Fabry-Perot腔滤波器结构%On the Structure of Fabry-Perot Tunable Optical Filter

    Institute of Scientific and Technical Information of China (English)

    于多多

    2006-01-01

    设计了C波段可调光纤Fabry-Perot腔光滤波器,并对其进行了参数特性及影响因素的分析.器件在较宽的自由光谱区内有良好的滤光特性,可调谐范围广,优于绝大部分其他类型的光滤波器,并且具有较高的精细度特性.

  10. 光纤Fabry-Perot腔振动传感器%Fiber Optical Fabry-Perot Vibration Sensor

    Institute of Scientific and Technical Information of China (English)

    江毅; 刘莉

    2003-01-01

    用频谱分析的方法对光纤Fabry-Perot腔传感器的输出信号进行解调.在正弦振动条件下,用干涉信号的3次谐波与基次波的功率比直接计算出振动幅度,可以简单地解调出信号.这一技术能够满足工程中精度要求不是很高的场合应用.

  11. Error analysis of Fabry-Perot Filter%Fabry-Perot滤光器的误差分析

    Institute of Scientific and Technical Information of China (English)

    常亮; 刘忠

    2007-01-01

    本文介绍了影响Fabry-Perot滤光器性能的几个重要因素,并对其产生的误差进行了分析,主要有相位误差,温度变化对系统产生的误差,光线入射角度偏移所带来的误差以及平板平行度误差和平板面形精度对系统的影响等.

  12. Fiber-coupled short Fabry-Perot resonators

    Energy Technology Data Exchange (ETDEWEB)

    Stone, J.; Marcuse, D. (AT and T Bell Labs., Holmdel, NJ (USA))

    1989-05-01

    Fabry-Perot resonators intended as filters in wavelength-multiplexed optical communications systems may have to be very short (on the order of 10 {mu}m) in order to increase their free spectral range. Short, yet tunable cavities can be designed as air gaps between two fibers placed in close proximity with highly reflecting mirrors deposited on their ends. However, an air-gap resonator with plane mirrors between closely spaced fiber ends may yield low throughout because of the poor match between the modes of typical single-mode fibers and the resonant mode in the air-gap cavity. The throughput can be improved by confining the resonant mode by means of a hollow dielectric tube placed inside the resonator. This paper compares short fiber-coupled Fabry-Parot resonators with and without an inserted hollow dielectric waveguide and derives expressions for their transmission losses. The authors show that the throughput of both types of resonator can be improved significantly by using a special fiber with large mode size to couple to the resonator. The special fiber is then spliced to a conventional single-mode fiber. They conclude that the resonator with an inserted hollow dielectric waveguide offers increased throughput for resonators with high finesse.

  13. Transforming Fabry-Pérot resonances into a Tamm mode

    Science.gov (United States)

    Durach, Maxim; Rusina, Anastasia

    2012-12-01

    We propose an optical structure composed of two metal nanolayers enclosing a distributed Bragg reflector (DBR) mirror. The structure is an open photonic system whose bound modes are coupled to external radiation. We apply the special theoretical treatment based on inversion symmetry of the structure to classify its resonances. We show that the structure supports resonances transitional between Fabry-Pérot modes and Tamm plasmons. When the dielectric contrast of the DBR is removed these modes are a pair of conventional Fabry-Pérot resonances. They spectrally merge into a Tamm mode at high contrast. The optical properties of the structure in the frequency range of the DBR stop band, including highly beneficial 50% transmittivity through thick structures with sub-skin-depth metal films, are determined by the hybrid quasinormal modes of the open nonconservative structure under consideration. The results can find a broad range of applications in photonics and optoelectronics, including the possibility of coherent control over optical fields in the class of structures similar to the one proposed here.

  14. Hydrocarbon gas detection with microelectromechanical Fabry-Perot interferometer

    Science.gov (United States)

    Mannila, Rami; Tuohiniemi, Mikko; Mäkynen, Jussi; Näkki, Ismo; Antila, Jarkko

    2013-05-01

    VTT Technical Research Centre of Finland has developed microelectromechanical (MEMS) Fabry-Perot interferometer (FPI) for hydrocarbon measurements. Fabry-Perot interferometer is a structure where is two highly reflective surfaces separated by a tunable air gap. The MEMS FPI is a monolithic device, i.e. it is made entirely on one substrate in a batch process, without assembling separate pieces together. The gap is adjusted by moving the upper mirror with electrostatic force, so there are no actual moving parts. The manufactured MEMS FPIs have been characterized. The tuning wavelength range of the MEMS FPI is 2.8-3.5 μm and its spectral resolution is 50-60 nm. VTT has designed and manufactured a handheld size demonstrator device based on the technology presented in this abstract. This device demonstrates gas detecting by measuring cigarette lighter gas and various plastic materials transmission spectra. The demonstrator contains light source, gas cell, MEMS FPI, detector and control electronics. It is connected to a laptop by USB connection, additional power supply or connection is not needed.

  15. Fiber Current Sensor Based on Fabry-Perot%基于光纤Fabry-Perot的电流传感器

    Institute of Scientific and Technical Information of China (English)

    刘京诚; 姚小芳; 陈小强; 刘俊

    2007-01-01

    利用光纤抗电磁干扰、绝缘性好, 体积小等特性,提出一种新型的光纤电流传感器. 该传感器将悬臂梁结构和光纤Fabry-Perot相结合,基于材料力学理论和电磁感应原理,利用Fabry-Perot干涉仪原理来实现对电流的测量. 在解调方式上采用了相位解调法,分析了该传感器的工作原理及光纤F-P的测量原理,建立了光纤F-P腔的数学模型,推导出电流检测理论公式,并进行了相关实验,证明该电流传感器具有绝缘性好、结构简单、灵敏度高等优点.

  16. Crosstalk Enhancement in 32 nm FD SOI MOSFET using HR Substrate and Multilayer BOX

    Directory of Open Access Journals (Sweden)

    Parisa Tavanazadeh

    2011-06-01

    Full Text Available In this paper, the crosstalk in 32 nm UTB SOI MOSFET is examined by using a new structure, a high resistivity substrate, and a multilayer BOX (SiO2-Diamond. The electrical and thermal characteristics of the conventional SOI and a multilayer BOX SOI are compared, and it is concluded that parasitic capacitances and crosstalk are improved by incorporating multilayer BOX to a HR Substrate. In a conventional HR FD SOI, crosstalk is approximately -121dB, while by incorporating multilayer BOX substrate and increasing the thickness of the Diamond to 100nm, crosstalk can be reduced by 20%.

  17. Focused ion beam post-processing of optical fiber Fabry-Perot cavities for sensing applications.

    Science.gov (United States)

    André, Ricardo M; Pevec, Simon; Becker, Martin; Dellith, Jan; Rothhardt, Manfred; Marques, Manuel B; Donlagic, Denis; Bartelt, Hartmut; Frazão, Orlando

    2014-06-01

    Focused ion beam technology is combined with chemical etching of specifically designed fibers to create Fabry-Perot interferometers. Hydrofluoric acid is used to etch special fibers and create microwires with diameters of 15 μm. These microwires are then milled with a focused ion beam to create two different structures: an indented Fabry-Perot structure and a cantilever Fabry-Perot structure that are characterized in terms of temperature. The cantilever structure is also sensitive to vibrations and is capable of measuring frequencies in the range 1 Hz - 40 kHz.

  18. A novel Michelson Fabry-Perot hybrid interference sensor based on the micro-structured fiber

    Science.gov (United States)

    Zhang, Yaxun; Zhang, Yu; Wang, Zhenzhen; Liu, Zhihai; Wei, Yong; Zhao, Enming; Yang, Xinghua; Zhang, Jianzhong; Yang, Jun; Yuan, Libo

    2016-09-01

    We propose and demonstrate a novel Michelson Fabry-Perot hybrid fiber interference sensor. By integrating a Michelson interferometer in a two-core fiber and a Fabry-Perot interferometer in a micro silica-capillary, we produce the Michelson Fabry-Perot hybrid interference sensor. Owing to the structure characteristic of the micro-structured fiber, this hybrid fiber interference sensor can achieve the measurement of the axial strain and radial bending simultaneously. The measurement sensitivity of the axial train is 0.015 nm/με and the measurement sensitivity of the radial bending is 1.393 nm/m-1.

  19. Two-Color Fabry-Perot Laser Diode with THz Primary Mode Spacing

    CERN Document Server

    O'Brien, S; Buckley, K; Fehse, R; Amann, A; O'Reilly, E P; Barry, L P; Anandarajah, P; Patchell, J; O'Gorman, J

    2006-01-01

    A class of multiwavelength Fabry-Perot lasers is introduced where the spectrum is tailored through a non-periodic patterning of the cavity effective index. The cavity geometry is obtained using an inverse scattering approach and can be designed such that the spacing of discrete Fabry-Perot lasing modes is limited only by the bandwidth of the inverted gain medium. A specific two-color semiconductor laser with a mode spacing in the THz regime is designed, and measurements are presented demonstrating the simultaneous oscillation of the two wavelengths. The extension of the Fabry-Perot laser concept described presents significant new possibilities in laser cavity design.

  20. Fabry-perot multilayers for enhancing the diffraction efficiency of ion-implanted gratings.

    Science.gov (United States)

    Escoubas, L; Flory, F O; Lemarchand, F; Drouard, E; Roux, L; Tisserand, S; Albrand, G

    2001-04-01

    Enhancement of the free-space diffraction efficiency of gratings made by titanium-ion implantation is demonstrated both theoretically and experimentally. Indeed, by insertion of a grating into a multilayer dielectric Fabry-Perot cavity, the diffraction efficiency can be increased to as much as 24 times that of a single grating. The sensitivity of the diffraction efficiency to the optogeometrical parameters of the grating or of the Fabry-Perot cavity is discussed. Moreover, a process for performance of a phase grating inside a Fabry-Perot cavity is described, and experimental results concerning efficiency measurements are compared with computed values for various grating periods.

  1. Fabry-Perot Diaphragm Fiber Optic Sensor (DFOS for Acoustic Detection

    Directory of Open Access Journals (Sweden)

    Yan SUN

    2007-10-01

    Full Text Available A diaphragm fiber optic sensor (DFOS solely based on Fabry-Perot multiple beam interference has been designed and fabricated with micro-electric mechanical system (MEMS technology. The silicon diaphragm with an embossed center was designed with an interference gap width kept accurately. The DFOS was verified to be a truly and purely Fabry-Perot device via a critical test. Parallel testing with a Piezoelectric (PZT sensor showed that the DFOS had high sensitivity. The Fabry-Perot DFOS also demonstrated excellent performance in on-line monitoring of Partial Discharge (PD in power transformers.

  2. Non-destructive, preclinical evaluation of root canal anatomy of human teeth with flat-panel detector volume CT (FD-VCT); Zerstoerungsfreie praeklinische Evaluation der Wurzelkanalanatomie menschlicher Zaehne mittels Flaechendetektor-Volumen-CT (FD-VCT)

    Energy Technology Data Exchange (ETDEWEB)

    Heidrich, G.; Hassepass, F.; Dullin, C.; Grabbe, E. [Universitaetsklinikum Goettingen, Abt. Diagnostische Radiologie (Germany); Attin, T.; Hannig, C. [Universitaetsklinikum Goettingen, Abt. fuer Zahnerhaltung, Praeventive Zahnheilkunde und Paradontologie (Germany)

    2005-12-15

    Purpose: Successful endodontic diagnostics and therapy call for adequate depiction of the root canal anatomy with multimodal diagnostic imaging. The aim of the present study is to evaluate visualization of the endodont with flat-panel detector volume CT (FD-VCT). Materials and methods: 13 human teeth were examined with the prototype of a FD-VCT. After data acquisition and generation of volume data sets in volume rendering technology (VRT), the findings obtained were compared to conventional X-rays and cross-section preparations of the teeth. Results: The anatomical structures of the endodont such as root canals, side canals and communications between different root canals as well as dentricles could be detected precisely with FD-VCT. The length of curved root canals was also determined accurately. The spatial resolution of the system is around 140 {mu}m. Only around 73% of the main root canals detected with FD-VCT and 87% of the roots could be visualized with conventional dental X-rays. None of the side canals, shown with FD-VCT, was detectable on conventional X-rays. In all cases the enamel and dentin of the teeth could be well delineated. No differences in image quality could be discerned between stored and freshly extracted teeth, or between primary and adult teeth. (orig.)

  3. Three-Dimensional Complex Padé FD Migration: Splitting and Corrections

    Directory of Open Access Journals (Sweden)

    D. Mondini

    2012-01-01

    Full Text Available Three-dimensional wave-equation migration techniques are still quite expensive because of the huge matrices that need to be inverted. Several techniques have been proposed to reduce this cost by splitting the full 3D problem into a sequence of 2D problems. To reduce errors, the Li correction is applied at regular multiples of depth extrapolation increment. We compare the performance of splitting techniques in wave propagation for 3D finite-difference (FD migration in terms of image quality and computational cost. We study the behaviour of the complex Padé approximation in combination with two- and alternating four-way splitting, that is, splitting into the coordinate directions at one depth and the diagonal directions at the next depth level. We also extend the Li correction for use with the complex Padé expansion and diagonal directions. From numerical examples in inhomogeneous media, we conclude that alternate four-way splitting is the most cost-effective strategy to reduce numerical anisotropy in complex Padé 3D FD migration.

  4. Fabry-Perot Observations of HH 1/2

    Directory of Open Access Journals (Sweden)

    A. Riera

    2005-01-01

    Full Text Available Presentamos nuevas observaciones Fabry-Perot del sistema HH1/2 en la l nea de H. Se han obtenidos los perfiles de las l neas, y los mapas de velocidad radial y de dispersi n de velocidades a partir de los canales de velocidad de los objetos HH 1 y HH 2. La distribuci on espacial de la velocidad radial de ambos objetos (HH 1, HH 2 presenta material desplazado al rojo hacia la fuente, y material desplazado al azul alej ndose de la fuente. Un modelo que contempla la presencia de emisi n directa+dispersada con tres condensaciones emisoras reproduce cualitativamente la distribuci n espacial de la velocidad radial y de la dispersi n de velocidades de HH 2.

  5. Millimeter-long fiber Fabry-Perot cavities.

    Science.gov (United States)

    Ott, Konstantin; Garcia, Sebastien; Kohlhaas, Ralf; Schüppert, Klemens; Rosenbusch, Peter; Long, Romain; Reichel, Jakob

    2016-05-01

    We demonstrate fiber Fabry-Perot (FFP) cavities with concave mirrors that can be operated at cavity lengths as large as 1.5 mm without significant deterioration of the finesse. This is achieved by using a laser dot machining technique to shape spherical mirrors with ultralow roughness and employing single-mode fibers with large mode area for good mode matching to the cavity. Additionally, in contrast to previous FFPs, these cavities can be used over an octave-spanning frequency range with adequate coatings. We also show directly that shape deviations caused by the fiber's index profile lead to a finesse decrease as observed in earlier attempts to build long FFP cavities, and show a way to overcome this problem.

  6. Fiber Fabry-Perot interferometer for curvature sensing

    Science.gov (United States)

    Monteiro, Catarina S.; Ferreira, Marta S.; Silva, Susana O.; Kobelke, Jens; Schuster, Kay; Bierlich, Jörg; Frazão, Orlando

    2016-07-01

    A curvature sensor based on an Fabry-Perot (FP) interferometer was proposed. A capillary silica tube was fusion spliced between two single mode fibers, producing an FP cavity. Two FP sensors with different cavity lengths were developed and subjected to curvature and temperature. The FP sensor with longer cavity showed three distinct operating regions for the curvature measurement. Namely, a linear response was shown for an intermediate curvature radius range, presenting a maximum sensitivity of 68.52 pm/m-1. When subjected to temperature, the sensing head produced a similar response for different curvature radii, with a sensitivity varying from 0.84 pm/°C to 0.89 pm/°C, which resulted in a small cross-sensitivity to temperature when the FP sensor was subjected to curvature. The FP cavity with shorter length presented low sensitivity to curvature.

  7. Fourier transform spectrometer based on Fabry-Perot interferometer.

    Science.gov (United States)

    Al-Saeed, Tarek A; Khalil, Diaa A

    2016-07-10

    We analyze the Fourier transform spectrometer based on a symmetric/asymmetric Fabry-Perot interferometer. In this spectrometer, the interferogram is obtained by recording the intensity as a function of the interferometer length. Then, we recover the spectrum by applying the discrete Fourier transform (DFT) directly on the interferogram. This technique results in spectral harmonic overlap and fictitious wavenumber components outside the original spectral range. For this purpose, in this work, we propose a second method to recover the spectrum. This method is based on expanding the DFT of the interferogram and the spectrum by a Haar or box function. By this second method, we recovered the spectrum and got rid of the fictitious spectral components and spectral harmonic overlap.

  8. Millimeter-long Fiber Fabry-Perot cavities

    CERN Document Server

    Ott, Konstantin; Kohlhaas, Ralf; Schüppert, Klemens; Rosenbusch, Peter; Long, Romain; Reichel, Jakob

    2016-01-01

    We demonstrate fiber Fabry-Perot (FFP) cavities with concave mirrors that can be operated at cavity lengths as large as 1.5mm without significant deterioration of the finesse. This is achieved by using a laser dot machining technique to shape spherical mirrors with ultralow roughness and employing single-mode fibers with large mode area for good mode matching to the cavity. Additionally, in contrast to previous FFPs, these cavities can be used over an octave-spanning frequency range with adequate coatings. We also show directly that shape deviations caused by the fiber's index profile lead to a finesse decrease as observed in earlier attempts to build long FFP cavities, and show a way to overcome this problem.

  9. A Fabry-Perot Microcavity for Diamond-Based Photonics

    CERN Document Server

    Janitz, Erika; Dimock, Mark; Bourassa, Alexandre; Sankey, Jack; Childress, Lilian

    2015-01-01

    Open Fabry-Perot microcavities represent a promising route for achieving a quantum electrodynamics (cavity-QED) platform with diamond-based emitters. In particular, they offer the opportunity to introduce high purity, minimally fabricated material into a tunable, high quality factor optical resonator. Here, we demonstrate a fiber-based microcavity incorporating a thick (> 10 {\\mu}m) diamond membrane with a finesse of 17,000, corresponding to a quality factor Q ~ $10^6$. Such minimally fabricated, thick samples can contain optically stable emitters similar to those found in bulk diamond. We observe modified microcavity spectra in the presence of the membrane, and develop analytic and numerical models to describe the effect of the membrane on cavity modes, including loss and coupling to higher-order transverse modes. We estimate that a Purcell enhancement of approximately 20 should be possible for emitters within the diamond in this device, and provide evidence that better diamond surface treatments and mirror ...

  10. Intrinsic Fabry-Perot optical fiber sensors and their multiplexing

    Science.gov (United States)

    Wang, Anbo

    2007-12-11

    An intrinsic Fabry-Perot optical sensor includes a thin film sandwiched between two fiber ends. When light is launched into the fiber, two reflections are generated at the two fiber/thin film interfaces due to a difference in refractive indices between the fibers and the film, giving rise to the sensor output. In another embodiment, a portion of the cladding of a fiber is removed, creating two parallel surfaces. Part of the evanescent fields of light propagating in the fiber is reflected at each of the surfaces, giving rise to the sensor output. In a third embodiment, the refractive index of a small portion of a fiber is changed through exposure to a laser beam or other radiation. Interference between reflections at the ends of the small portion give rise to the sensor output. Multiple sensors along a single fiber are multiplexed using an optical time domain reflectometry method.

  11. Photoacoustic imaging using an 8-beam Fabry-Perot scanner

    Science.gov (United States)

    Huynh, Nam; Ogunlade, Olumide; Zhang, Edward; Cox, Ben; Beard, Paul

    2016-03-01

    The planar Fabry Perot (FP) photoacoustic scanner has been shown to provide exquisite high resolution 3D images of soft tissue structures in vivo to depths up to approximately 10mm. However a significant limitation of current embodiments of the concept is low image acquisition speed. To increase acquisition speed, a novel multi-beam scanner architecture has been developed. This enables a line of equally spaced 8 interrogation beams to be scanned simultaneously across the FP sensor and the photoacoustic signals detected in parallel. In addition, an excitation laser operating at 200Hz was used. The combination of parallelising the detection and the high pulse repetition frequency (PRF) of the excitation laser has enabled dramatic reductions in image acquisition time to be achieved. A 3D image can now be acquired in 10 seconds and 2D images at video rates are now possible.

  12. Diffractively coupled Fabry-Perot resonator with power-recycling

    CERN Document Server

    Britzger, Michael; Kroker, Stefanie; Brückner, Frank; Burmeister, Oliver; Kley, Ernst-Bernhard; Tünnermann, Andreas; Danzmann, Karsten; Schnabel, Roman

    2011-01-01

    We demonstrate the optical coupling of two cavities without light transmission through a substrate. Compared to a conventional coupling component, that is a partially transmissive mirror, an all-reflective coupler avoids light absorption in the substrate and therefore associated thermal problems, and even allows the use of opaque materials with possibly favourable mechanical and thermal properties. Recently, the all-reflective coupling of two cavities with a low-efficiency 3-port diffraction grating was theoretically investigated. Such a grating has an additional (a third) port. However, it was shown that the additional port does not necessarily decrease the bandwidth of the coupled cavities. Such an all-reflective scheme for cavity coupling is of interest in the field of gravitational wave detection. In such detectors light that is resonantly enhanced inside the so-called power-recycling cavity is coupled to (kilometre-scale) Fabry-Perot resonators representing the arms of a Michelson interferometer. In orde...

  13. Fiber Fabry-Perot cavity with high finesse

    CERN Document Server

    Hunger, David; Colombe, Yves; Deutsch, Christian; Hänsch, Theodor W; Reichel, Jakob

    2010-01-01

    We have realized a fiber-based Fabry-Perot cavity with CO2 laser-machined mirrors. It combines very small size, high finesse F>=130000, small waist and mode volume, and good mode matching between the fiber and cavity modes. This combination of features is a major advance for cavity quantum electrodynamics (CQED), as shown in recent CQED experiments with Bose-Einstein condensates enabled by this cavity [Y. Colombe et al., Nature 450, 272 (2007)]. It should also be suitable for a wide range of other applications, including coupling to solid-state emitters, gas detection at the single-particle level, fiber-coupled single-photon sources and high-resolution optical filters with large stopband.

  14. Fiber Fabry-Perot interferometer for curvature sensing

    Science.gov (United States)

    Monteiro, Catarina S.; Ferreira, Marta S.; Silva, Susana O.; Kobelke, Jens; Schuster, Kay; Bierlich, Jörg; Frazão, Orlando

    2016-12-01

    A curvature sensor based on an Fabry-Perot (FP) interferometer was proposed. A capillary silica tube was fusion spliced between two single mode fibers, producing an FP cavity. Two FP sensors with different cavity lengths were developed and subjected to curvature and temperature. The FP sensor with longer cavity showed three distinct operating regions for the curvature measurement. Namely, a linear response was shown for an intermediate curvature radius range, presenting a maximum sensitivity of 68.52 pm/m-1. When subjected to temperature, the sensing head produced a similar response for different curvature radii, with a sensitivity varying from 0.84 pm/°C to 0.89 pm/°C, which resulted in a small cross-sensitivity to temperature when the FP sensor was subjected to curvature. The FP cavity with shorter length presented low sensitivity to curvature.

  15. Fabry-Perot enhanced Faraday rotation in graphene.

    Science.gov (United States)

    Ubrig, Nicolas; Crassee, Iris; Levallois, Julien; Nedoliuk, Ievgeniia O; Fromm, Felix; Kaiser, Michl; Seyller, Thomas; Kuzmenko, Alexey B

    2013-10-21

    We demonstrate that giant Faraday rotation in graphene in the terahertz range due to the cyclotron resonance is further increased by constructive Fabry-Perot interference in the supporting substrate. Simultaneously, an enhanced total transmission is achieved, making this effect doubly advantageous for graphene-based magneto-optical applications. As an example, we present far-infrared spectra of epitaxial multilayer graphene grown on the C-face of 6H-SiC, where the interference fringes are spectrally resolved and a Faraday rotation up to 0.15 radians (9°) is attained. Further, we discuss and compare other ways to increase the Faraday rotation using the principle of an optical cavity.

  16. Micromachined fiber optic Fabry-Perot underwater acoustic probe

    Science.gov (United States)

    Wang, Fuyin; Shao, Zhengzheng; Hu, Zhengliang; Luo, Hong; Xie, Jiehui; Hu, Yongming

    2014-08-01

    One of the most important branches in the development trend of the traditional fiber optic physical sensor is the miniaturization of sensor structure. Miniature fiber optic sensor can realize point measurement, and then to develop sensor networks to achieve quasi-distributed or distributed sensing as well as line measurement to area monitoring, which will greatly extend the application area of fiber optic sensors. The development of MEMS technology brings a light path to address the problems brought by the procedure of sensor miniaturization. Sensors manufactured by MEMS technology possess the advantages of small volume, light weight, easy fabricated and low cost. In this paper, a fiber optic extrinsic Fabry-Perot interferometric underwater acoustic probe utilizing micromachined diaphragm collaborated with fiber optic technology and MEMS technology has been designed and implemented to actualize underwater acoustic sensing. Diaphragm with central embossment, where the embossment is used to anti-hydrostatic pressure which would largely deflect the diaphragm that induce interferometric fringe fading, has been made by double-sided etching of silicon on insulator. By bonding the acoustic-sensitive diaphragm as well as a cleaved fiber end in ferrule with an outer sleeve, an extrinsic Fabry-Perot interferometer has been constructed. The sensor has been interrogated by quadrature-point control method and tested in field-stable acoustic standing wave tube. Results have been shown that the recovered signal detected by the sensor coincided well with the corresponding transmitted signal and the sensitivity response was flat in frequency range from 10 Hz to 2kHz with the value about -154.6 dB re. 1/μPa. It has been manifest that the designed sensor could be used as an underwater acoustic probe.

  17. Demonstrations Using a Fabry-Perot. I. Multiple-Slit Interference

    Science.gov (United States)

    Roychoudhuri, Chandrasekhar

    1975-01-01

    Describes a demonstration technique for showing multiple-slit interference patterns with the use of a Fabry-Perot etalon and a laser beam. A simple derivation of the analytical expression for such fringes is presented. (Author/CP)

  18. Fabry-Perot Based Ranging Interferometer Receiver for High Spectral Resolution Lidar Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Michigan Aerospace Corporation (MAC) is pleased to present the following Phase II proposal for a Fabry-Perot Based Interferometer Receiver for the High Spectral...

  19. A Novel Technique to Measure Gain Spectrum for Fabry-Pérot Semiconductor Lasers

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    A novel gain measurement technique based on the integration of the measured amplified spontaneous emission spectrum multiplying a phase function over one longitudinal mode interval is proposed for Fabry-Perot semiconductor lasers.

  20. Sinusoidal Phase-Modulating Fabry-Perot Interferometer for Angular Displacement Measurement

    Institute of Scientific and Technical Information of China (English)

    Caini Zhang; Xiangzhao Wang

    2003-01-01

    In this paper, a sinusoidal phase-modulating Fabry-Perot interferometer is proposed to measure angular displacement.The usefulness of the interferometer is demonstrated by simulations and experiments.

  1. Compact High-Resolution Broad-Band Terahertz Fabry-Perot Spectrometer Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Our objective is to develop a compact scanning Fabry-Perot spectrometer, for satellite far-infrared astronomy and Earth remote sensing, that operates at wavelengths...

  2. Sinusoidal Phase-Modulating Fabry-Perot Interferometer for Angular Displacement Measurement

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    In this paper, a sinusoidal phase-modulating Fabry-Perot interferometer is proposed to measure angular displacement. The usefulness of the interferometer is demonstrated by simulations and experiments.

  3. Rugged Low Temperature Actuators for Tunable Fabry Perot Optical Filters Project

    Data.gov (United States)

    National Aeronautics and Space Administration — During our Phase I SBIR research, we propose to integrate a novel low-temperature large-strain actuator technology into Fabry-Perot optical filters. The resulting...

  4. Simultaneous quantification of 5-FU, 5-FUrd, 5-FdUrd, 5-FdUMP, dUMP and TMP in cultured cell models by LC-MS/MS.

    Science.gov (United States)

    Carli, Delphine; Honorat, Mylène; Cohen, Sabine; Megherbi, Mehdi; Vignal, Bruno; Dumontet, Charles; Payen, Léa; Guitton, Jérôme

    2009-10-01

    To specifically quantify several metabolites of 5-fluorouracil (5-FU) and two endogenous monophosphate nucleotides, we developed an original method based on a liquid chromatography-tandem mass spectrometry (LC-MS/MS). This assay allowed the determination of: (i) the intracellular production of 5-fluoro-2'-deoxyuridine-5'-monophosphate (5-FdUMP) from 5-FU or 5-fluoro-2'-deoxyuridine (5-FdUrd), (ii) the impact of 5-FdUMP concentration on the intracellular 2'-deoxyuridine-5'-monophosphate (dUMP)/thymidine-5'-monophosphate (TMP) ratio, and (iii) the secretion extent of 5-FdUMP and 5-FU from human cultured cells by ABC transporters. Under our experimental conditions, cells were incubated with 5-FU or 5-FUrd. Then, cellular proteins were precipitated by methanol. This procedure provided high extraction recovery. In addition, to measure 5-FU and 5-FdUMP secretion from cells, we carried out quantification of these molecules in culture medium. Media were either directly injected (5-FU) or underwent a solid phase extraction using Oasis Wax extraction cartridge (5-FdUMP). Separation of analytes was performed on a dC18 Atlantis 3.5microm, (100mmx2.1mm i.d) column with isocratic mode using ammonium formate buffer/methanol/water (5/5/90, v/v) as mobile phase. The run time did not exceed 6.2min. The analytes were ionized in an electrospray interface under negative ion mode. We validated the method over a range of 2.5-150ngmL(-1) according to the compounds. Intra- and inter-assay variability was lower than 10% over seven days. All compounds were stable in cells or in culture medium when samples were stored at -20 degrees C for at least two weeks, and after three freeze-thaw cycles. No matrix effect was observed in both media.

  5. Engineering-reflected phase in Fabry-Perot sensors with resonant mirrors.

    Science.gov (United States)

    Gellineau, Antonio; Wong, Yu-Po; Solgaard, Olav

    2013-12-01

    Fabry-Perot cavities made with photonic crystal (PC) mirrors and other resonant structures exhibit nontraditional characteristics due to the strong wavelength dependence of their reflected phase. This Letter describes how engineering the phase of PC mirrors enables sensors that are tolerant to variations in laser center frequency and line width. Reflection spectra measurements of Fabry-Perot cavities made with PC mirrors were collected as a function of wavelength and cavity length, providing experimental verification of theory and simulations.

  6. Compact imaging spectrometer combining Fourier transform spectroscopy with a Fabry-Perot interferometer.

    Science.gov (United States)

    Pisani, Marco; Zucco, Massimo

    2009-05-11

    An imaging spectrometer based on a Fabry-Perot interferometer is presented. The Fabry-Perot interferometer scans the mirror distance up to contact and the intensity modulated light signal is transformed using a Fourier Transform based algorithm, as the Michelson based Fourier Transform Spectrometers does. The resulting instrument has the advantage of a compact, high numerical aperture, high luminosity hyperspectral imaging device. Theory of operation is described along with one experimental realization and preliminary results.

  7. Familial Dysautonomia (FD Human Embryonic Stem Cell Derived PNS Neurons Reveal that Synaptic Vesicular and Neuronal Transport Genes Are Directly or Indirectly Affected by IKBKAP Downregulation.

    Directory of Open Access Journals (Sweden)

    Sharon Lefler

    Full Text Available A splicing mutation in the IKBKAP gene causes Familial Dysautonomia (FD, affecting the IKAP protein expression levels and proper development and function of the peripheral nervous system (PNS. Here we found new molecular insights for the IKAP role and the impact of the FD mutation in the human PNS lineage by using a novel and unique human embryonic stem cell (hESC line homozygous to the FD mutation originated by pre implantation genetic diagnosis (PGD analysis. We found that IKBKAP downregulation during PNS differentiation affects normal migration in FD-hESC derived neural crest cells (NCC while at later stages the PNS neurons show reduced intracellular colocalization between vesicular proteins and IKAP. Comparative wide transcriptome analysis of FD and WT hESC-derived neurons together with the analysis of human brains from FD and WT 12 weeks old embryos and experimental validation of the results confirmed that synaptic vesicular and neuronal transport genes are directly or indirectly affected by IKBKAP downregulation in FD neurons. Moreover we show that kinetin (a drug that corrects IKBKAP alternative splicing promotes the recovery of IKAP expression and these IKAP functional associated genes identified in the study. Altogether, these results support the view that IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons. This function seems to be mostly affected in FD-hESC derived PNS neurons probably reflecting some PNS neuronal dysfunction observed in FD.

  8. Familial Dysautonomia (FD) Human Embryonic Stem Cell Derived PNS Neurons Reveal that Synaptic Vesicular and Neuronal Transport Genes Are Directly or Indirectly Affected by IKBKAP Downregulation.

    Science.gov (United States)

    Lefler, Sharon; Cohen, Malkiel A; Kantor, Gal; Cheishvili, David; Even, Aviel; Birger, Anastasya; Turetsky, Tikva; Gil, Yaniv; Even-Ram, Sharona; Aizenman, Einat; Bashir, Nibal; Maayan, Channa; Razin, Aharon; Reubinoff, Benjamim E; Weil, Miguel

    2015-01-01

    A splicing mutation in the IKBKAP gene causes Familial Dysautonomia (FD), affecting the IKAP protein expression levels and proper development and function of the peripheral nervous system (PNS). Here we found new molecular insights for the IKAP role and the impact of the FD mutation in the human PNS lineage by using a novel and unique human embryonic stem cell (hESC) line homozygous to the FD mutation originated by pre implantation genetic diagnosis (PGD) analysis. We found that IKBKAP downregulation during PNS differentiation affects normal migration in FD-hESC derived neural crest cells (NCC) while at later stages the PNS neurons show reduced intracellular colocalization between vesicular proteins and IKAP. Comparative wide transcriptome analysis of FD and WT hESC-derived neurons together with the analysis of human brains from FD and WT 12 weeks old embryos and experimental validation of the results confirmed that synaptic vesicular and neuronal transport genes are directly or indirectly affected by IKBKAP downregulation in FD neurons. Moreover we show that kinetin (a drug that corrects IKBKAP alternative splicing) promotes the recovery of IKAP expression and these IKAP functional associated genes identified in the study. Altogether, these results support the view that IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons. This function seems to be mostly affected in FD-hESC derived PNS neurons probably reflecting some PNS neuronal dysfunction observed in FD.

  9. A Study of the Chaos of Non-linear Fabry-Perot Resonator%含非线性介质Fabry-Perot腔混沌研究

    Institute of Scientific and Technical Information of China (English)

    袁圣付; 姜宗福

    2000-01-01

    根据混沌动力学的基本理论,计算了含非线性介质Fabry-Perot腔动力学方程在其参数下的Lyapunov指数分布图.利用Lyapunov指数和动力学特性的对应关系,分析出非线性Fabry-Perot腔在各种参数下的动力学特性、稳态参数区域面积和混沌转换机制.

  10. Influence of intensity loss in the cavity of a folded Fabry-Perot interferometer on interferometric signals.

    Science.gov (United States)

    Shyu, Lih-Horng; Chang, Chung-Ping; Wang, Yung-Cheng

    2011-06-01

    Fabry-Perot interferometer is often used for the micro-displacement, because of its common optical path structure being insensitive to the environmental disturbances. Recently, the folded Fabry-Perot interferometer has been investigated for displacement measurements in large ranges. The advantages of a folded Fabry-Perot interferometer are insensitive to the tilt angle and higher optical resolution. But the design of the optical cavity has become more and more complicated. For this reason, the intensity loss in the cavity will be an important parameter for the distribution of the interferometric intensity. To obtain a more accurate result of such interferometer utilized for displacement measurements, the intensity loss of the cavity in the fabricated folded Fabry-Perot interferometer and the modified equation of the folded Fabry-Perot interferometer will be described. According to the theoretical and experimental results, the presented model is available for the analysis of displacement measurements by a folded Fabry-Perot interferometer.

  11. Kinetic characterization of a glycoside hydrolase family 44 xyloglucanase/endoglucanase from Ruminococcus flavefaciens FD-1.

    Science.gov (United States)

    Warner, Christopher D; Go, Rica M; García-Salinas, Carolina; Ford, Clark; Reilly, Peter J

    2011-01-05

    Two forms of Ruminococcus flavefaciens FD-1 endoglucanase B, a member of glycoside hydrolase family 44, one with only a catalytic domain and the other with a catalytic domain and a carbohydrate binding domain (CBM), were produced. Both forms hydrolyzed cellotetraose, cellopentaose, cellohexaose, carboxymethylcellulose (CMC), birchwood and larchwood xylan, xyloglucan, lichenan, and Avicel but not cellobiose, cellotriose, mannan, or pullulan. Addition of the CBM increased catalytic efficiencies on both CMC and birchwood xylan but not on xyloglucan, and it decreased rates of cellopentaose and cellohexaose hydrolysis. Catalytic efficiencies were much higher on xyloglucan than on other polysaccharides. Hydrolysis rates increased with increasing cellooligosaccharide chain length. Cellotetraose hydrolysis yielded only cellotriose and glucose. Hydrolysis of cellopentaose gave large amounts of cellotetraose and glucose, somewhat more of the former than of the latter, and much smaller amounts of cellobiose and cellotriose. Cellohexaose hydrolysis yielded much more cellotetraose than cellobiose and small amounts of glucose and cellotriose, along with a low and transient amount of cellopentaose.

  12. FD_BH: a program for simulating electromagnetic waves from a borehole antenna

    Science.gov (United States)

    Ellefsen, Karl J.

    2002-01-01

    Program FD_BH is used to simulate the electromagnetic waves generated by an antenna in a borehole. The model representing the antenna may include metallic parts, a coaxial cable as a feed to the driving point, and resistive loading. The program is written in the C programming language, and the program has been tested on both the Windows and the UNIX operating systems. This Open-File Report describes • The contents and organization of the Zip file (section 2). • The program files, the installation of the program, the input files, and the execution of the program (section 3). • Address to which suggestions for improving the program may be sent (section 4).

  13. On the role of polynomials in RBF-FD approximations: II. Numerical solution of elliptic PDEs

    Science.gov (United States)

    Bayona, Victor; Flyer, Natasha; Fornberg, Bengt; Barnett, Gregory A.

    2017-03-01

    RBF-generated finite differences (RBF-FD) have in the last decade emerged as a very powerful and flexible numerical approach for solving a wide range of PDEs. We find in the present study that combining polyharmonic splines (PHS) with multivariate polynomials offers an outstanding combination of simplicity, accuracy, and geometric flexibility when solving elliptic equations in irregular (or regular) regions. In particular, the drawbacks on accuracy and stability due to Runge's phenomenon are overcome once the RBF stencils exceed a certain size due to an underlying minimization property. Test problems include the classical 2-D driven cavity, and also a 3-D global electric circuit problem with the earth's irregular topography as its bottom boundary. The results we find are fully consistent with previous results for data interpolation.

  14. Estimated Daily Intake and Safety of FD&C Food Colour Additives in the US Population.

    Science.gov (United States)

    Bastaki, Maria; Farrell, Thomas; Bhusari, Sachin; Bi, Xiaoyu; Scrafford, Carolyn

    2017-03-23

    A refined exposure assessment was undertaken to calculate the Estimated Daily Intake (EDI) of the seven FD&C straight colour additives and five FD&C colour lakes ("synthetic" food colours) approved in the United States (US). The EDIs were calculated for the US population as a whole and specific age groups, including children 2-5 y, children 6-12 y, adolescents 13-18 y, and adults 19+ y. Actual use data were collected from an industry survey of companies who are users of these colour additives in a variety of products, with additional input from food colour manufacturers. Food consumption data were obtained from the National Health and Nutrition Examination Survey (NHANES). The assessment was further refined by adjusting the intake to more realistic scenarios based on the fraction of products containing colour within specific food categories using data provided by the Mintel International Group LTD. The results of the analysis indicate that a) the use levels reported by industry are consistent with the concentrations measured analytically by the US Food and Drug Administration; b) exposure to food colour additives in the US by average and high intake consumers is well below the Acceptable Daily Intake (ADI) of each colour additive as published by the Joint WHO/FAO Committee on Food Additives (JECFA) and allows wide margins of safety; and c) for children to reach the intake assumed in the Southampton study, they would have to consume at least 30 times more foods and beverages daily than the high consumers (95%) and the foods would have to contain four different colours at maximum use levels, a clearly unrealistic scenario. It is concluded that food colour use as currently practiced in the US is safe and does not result in excessive exposure to the population, even at conservative ranges of food consumption and levels of use.

  15. Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase.

    Directory of Open Access Journals (Sweden)

    David G Warnock

    Full Text Available Migalastat HCl (AT1001, 1-Deoxygalactonojirimycin is an investigational pharmacological chaperone for the treatment of α-galactosidase A (α-Gal A deficiency, which leads to Fabry disease, an X-linked, lysosomal storage disorder. The currently approved, biologics-based therapy for Fabry disease is enzyme replacement therapy (ERT with either agalsidase alfa (Replagal or agalsidase beta (Fabrazyme. Based on preclinical data, migalastat HCl in combination with agalsidase is expected to result in the pharmacokinetic (PK enhancement of agalsidase in plasma by increasing the systemic exposure of active agalsidase, thereby leading to increased cellular levels in disease-relevant tissues. This Phase 2a study design consisted of an open-label, fixed-treatment sequence that evaluated the effects of single oral doses of 150 mg or 450 mg migalastat HCl on the PK and tissue levels of intravenously infused agalsidase (0.2, 0.5, or 1.0 mg/kg in male Fabry patients. As expected, intravenous administration of agalsidase alone resulted in increased α-Gal A activity in plasma, skin, and peripheral blood mononuclear cells (PBMCs compared to baseline. Following co-administration of migalastat HCl and agalsidase, α-Gal A activity in plasma was further significantly increased 1.2- to 5.1-fold compared to agalsidase administration alone, in 22 of 23 patients (95.6%. Importantly, similar increases in skin and PBMC α-Gal A activity were seen following co-administration of migalastat HCl and agalsidase. The effects were not related to the administered migalastat HCl dose, as the 150 mg dose of migalastat HCl increased α-Gal A activity to the same extent as the 450 mg dose. Conversely, agalsidase had no effect on the plasma PK of migalastat. No migalastat HCl-related adverse events or drug-related tolerability issues were identified.ClinicalTrials.gov NCT01196871.

  16. VARIABILITY OF FORCED OSCILLATION (SIEMENS SIREGNOST FD-5) MEASUREMENTS OF TOTAL RESPIRATORY RESISTANCE IN PATIENTS AND HEALTHY-SUBJECTS

    NARCIS (Netherlands)

    GIMENO, F; VANDERWEELE, LT; KOETER, GH; DEMONCHY, JGR; VANALTENA, R

    1993-01-01

    The reproducibility of total respiratory resistance (R(rs)) measured with a simplified forced oscillatory method (Siemens Siregnost FD 5) was measured and compared with that of slow inspiratory vital capacity (IVC) and forced expiratory volume in one second (FEV1). The former technique has the advan

  17. Processing and functional display of the 86 kDa heterodimeric penicillin G acylase on the surface of phage fd

    NARCIS (Netherlands)

    Verhaert, R.M D; van Duin, J; Quax, Wim

    1999-01-01

    The large heterodimeric penicillin G acylase from Alcaligenes faecalis was displayed on the surface of phage fd. We fused the coding sequence (alpha subunit-internal peptide-beta subunit) to the gene of a phage coat protein. A modified g3p signal sequence was used to direct the polypeptide to the pe

  18. A 3-D RBF-FD elliptic solver for irregular boundaries: modeling the atmospheric global electric circuit with topography

    Directory of Open Access Journals (Sweden)

    V. Bayona

    2015-04-01

    Full Text Available A numerical model based on Radial Basis Function-generated Finite Differences (RBF-FD is developed for simulating the Global Electric Circuit (GEC within the Earth's atmosphere, represented by a 3-D variable coefficient linear elliptic PDE in a spherically-shaped volume with the lower boundary being the Earth's topography and the upper boundary a sphere at 60 km. To our knowledge, this is (1 the first numerical model of the GEC to combine the Earth's topography with directly approximating the differential operators in 3-D space, and related to this (2 the first RBF-FD method to use irregular 3-D stencils for discretization to handle the topography. It benefits from the mesh-free nature of RBF-FD, which is especially suitable for modeling high-dimensional problems with irregular boundaries. The RBF-FD elliptic solver proposed here makes no limiting assumptions on the spatial variability of the coefficients in the PDE (i.e. the conductivity profile, the right hand side forcing term of the PDE (i.e. distribution of current sources or the geometry of the lower boundary.

  19. Intratumoral pharmacokinetic analysis by 19F-magnetic resonance spectroscopy and cytostatic in vivo activity of gemcitabine (dFdC) in two small cell lung cancer xenografts

    DEFF Research Database (Denmark)

    Kristjansen, P E; Quistorff, B; Spang-Thomsen, M;

    1993-01-01

    BACKGROUND: Gemcitabine, 2'2'difluoro-deoxycytidine (dFdC), has shown activity in several preclinical models, and presently the compound is being clinically evaluated in patients with lung cancer and other solid tumors. DESIGN: The cytostatic in vivo activity of dFdC was tested in the two human...... the same single dose of the drug....

  20. Modified Fabry-Perot interferometric method for waveguide loss measurement.

    Science.gov (United States)

    Taebi, Sareh; Khorasaninejad, Mohammadreza; Saini, Simarjeet Singh

    2008-12-10

    An extension to the Fabry-Perot interferometric method is demonstrated to calculate the optical loss and the reflectivity for optical waveguides simultaneously. The method uses an excitation of the waveguide with a broadband amplified spontaneous emission source (a superluminescent diode in our case) and curve fitting to account for the change of input power, thereby simplifying the measurement procedure. The use of a broadband source as opposed to tunable lasers allows for simultaneous measurements over multiple wavelengths and decreased sensitivity to reflections in the cavity. Further, waveguides of different lengths are measured to calculate the optical loss and the reflectivity simultaneously. It is shown that, if the value for reflectivity is assumed, there could be a large error in the measurement of loss especially for short waveguides. Optical loss for ridge waveguides is measured and compared by using a tunable laser as the input source. The method can be used for a generic case where it is suspected that the input power changes during the measurement.

  1. Active Sensing with Fabry-Perot Infrared Interferometers

    Science.gov (United States)

    Huang, Jin; Gosangi, Rakesh; Gutierrez-Osuna, Ricardo

    2011-09-01

    In this article, we describe an active-sensing framework for infrared (IR) spectroscopy. The goal is to generate a sequence of wavelengths that best discriminates among chemicals. Unlike feature-selection strategies, the sequence is selected on-the-fly as the device acquires data. The framework models the problem as a Partially Observable Markov Decision Process (POMDP), which is solved by a greedy myopic algorithm. In previous work [1], we had applied this framework to temperature-modulated metal oxide sensor. Here, we adapt the framework to a tunable IR sensor based on Fabry-Perot interferometers (FPI). FPIs provide a low-cost alternative to traditional Fourier Transform Infrared Spectroscopy (FTIR), though at the expense of a narrower effective range and lower spectral resolution. Here, we first test whether the framework can scale up to large problems consisting 27 chemicals with 60 dimensions; our previous work on metal oxide sensors employed three chemicals and 7 dimensions. For this purpose, FPI spectra are simulated from FTIR. Then we validate the framework experimentally on 3 chemicals using a prototype instrument based on FPIs. These preliminary results are encouraging and indicate that the framework is able to solve classification problems of reasonable size.

  2. Optical fiber Fabry-Perot interferometer for microorganism growth detection

    Science.gov (United States)

    Liu, Xiaohui; Jiang, Mingshun; Sui, Qingmei; Luo, Shuyang; Geng, Xiangyi

    2016-07-01

    An optical fiber Fabry-Perot interferometer (FPI) based on hollow-core photonic crystal fiber (HCPCF) for microorganism growth detection is proposed and demonstrated. The FPI is formed by splicing both ends of a short section of HCPCF to SMFs and cleaving the SMF pigtail to a proper length. By measuring the fringe contrast of interference pattern, the refractive index (RI) changes of analyte during microorganism growth can be obtained. RI response of the sensor was investigated theoretically and experimentally. It shows linear response with sensitivity of -136 dB/RIU and good repeatability. Temperature response was also tested and the result confirms the low temperature cross-sensitivity of the sensor. Detection of yeast growth in liquid medium by the FPI sensor was conducted and the result shows the characteristic of typical yeast growth curve. With its advantages of high RI sensitivity, low temperature cross-sensitivity, capability for real-time measurement and so on, this FPI sensor has great potential in biosensing.

  3. Dielectric Fabry-Perot mirror coatings for infrared astronomy

    Science.gov (United States)

    Trauger, J.

    1984-01-01

    A number of Fabry-Perot (FP) coating designs have been developed specifically for IR astronomy. These have been optimized for uniform reflectance over five contiguous spectral ranges of particular astronomical interest between 1 and 5 microns. Wavelength coverage in individual designs is given by (maximum wavelength/minimum wavelength) greater than 1.4, over which reflective finesse is maintained near 35. Consideration has been made for the peculiar requirements of FP coatings. Visual alignment and testing of the FP has been made convenient by bringing the reflective finesse of the coating at 632.8 nm to the same value as the main IR reflection band. For example, a calcium fluoride FP can be aligned and tested at 632.8-nm He-Ne laser line, with the assurance of a corresponding finesse and spectral resolving power over its IR (3.6-5.1-micron) range. In addition, the FP can be used for IR spectroscopy while being simultaneously monitored and/or actively controlled at the laser wavelength. The potential for the coating itself to degrade the effective surface flatness of the FP through either mechanical stress or thickness nonuniformities is minimized by reducing to a minimum the number and thickness of coating layers. Several of these coatings have been fabricated and are actively in use at a number of observatories.

  4. Fabry-Perot Interferometer for Column CO2

    Science.gov (United States)

    Heaps, William S.; Kawa, Randolph; Bhartia, P. K. (Technical Monitor)

    2002-01-01

    Global atmospheric CO2 measurements are essential to resolving significant discrepancies in our understanding of the global carbon budget and, hence, humankind's role in global climate change. The science measurement requirements for CO2 are extremely demanding (precision approx. 0.3%). No atmospheric chemical species has ever been measured from space with this precision. We are developing a novel application of a Fabry-Perot interferometer to detect spectral absorption of reflected sunlight by CO2 and O2 in the atmosphere. Preliminary design studies indicate that the method will be able to achieve the sensitivity and signal-to-noise detection required to measure column CO2 at the target specification. The objective of this program is to construct a prototype instrument for deployment on an aircraft to test the instrument performance and our ability to retrieve the data in the real atmosphere. To date we have assembled a laboratory bench system to begin testing the optical and electronic components. We are also measuring signal and noise levels in actual sunlight to evaluate component performance.

  5. Fabry-Perot Interferometer for Column CO2: Airborne

    Science.gov (United States)

    Kawa, S. R.; Heaps, W. S.; Mao, J.; Andrews, A. E.; Burris, J. F.; Miodek, M.; Georgieva, E.

    2002-01-01

    Global atmospheric CO2 measurements are essential to resolving significant discrepancies in our understanding of the global carbon budget and, hence, humankind's role in global climate change. The science measurement requirements for CO2 are, however, extremely demanding (precision approximately 0.3%). We are developing a novel application of a Fabry-Perot interferometer to detect spectral absorption of reflected sunlight by CO2 and O2 in the atmosphere that should be able to achieve sufficient sensitivity and signal-to-noise to measure column CO2 at the target specification. We are currently constructing a prototype instrument for deployment on aircraft. The aircraft version will measure total column CO2 and CO2 below the aircraft as well as O2, which allows normalization of CO2 column amounts for varying surface height and pressure. This instrument will be a valuable asset in carbon budget field studies as well as a useful tool for evaluating existing and future space-based CO2 measurements. We will present the instrument concept, sensitivity calculations, and the results of testing a bench system in the laboratory and outdoors on the ground. We will also discuss our plan for deployment on the aircraft and potential flight applications to the CO2 budget problem.

  6. Demonstration of Fabry-Perot interferometric spectrometry technology

    Science.gov (United States)

    Petersen, T. V.; Makel, D. B.; Thurman, C.

    1993-06-01

    As rocket engine components experience wear or failure, anomalous materials may be entrained in the plume. Historically, visible plume anomalies have preceded many rocket engine failures, some of which have been catastrophic. Development of a small, rugged, high-speed, high resolution Fabry-Perot interferometer (FPI) based spectrometer capable of detecting the spectral signatures of eroding engine components during rocket engine test and/or flight operations is described. An operational plume spectrometer fabricated with miniaturized optics has been successfully tested. An extensive test series was conducted to define the limits of the spectrometer with respect to time-response and resolution. The data collected during testing were correlated with measurements obtained using sensitive ground equipment in order to benchmark the spectrometer's performance against a known device. The FPI demonstrated the reliability required for a flight instrument by functioning satisfactorily at or near the rocket engine test stand environment. Several of the optical components are interchangeable, allowing collection of a greater variety of plume signals. Also, the FPI's high resolution capabilities suggest it is suitable for application to both absorption and emission spectroscopy.

  7. Performance of a dual Fabry-Perot cavity refractometer.

    Science.gov (United States)

    Egan, Patrick F; Stone, Jack A; Hendricks, Jay H; Ricker, Jacob E; Scace, Gregory E; Strouse, Gregory F

    2015-09-01

    We have built and characterized a refractometer that utilizes two Fabry-Perot cavities formed on a dimensionally stable spacer. In the typical mode of operation, one cavity is held at vacuum, and the other cavity is filled with nitrogen gas. The differential change in length between the cavities is measured as the difference in frequency between two helium-neon lasers, one locked to the resonance of each cavity. This differential change in optical length is a measure of the gas refractivity. Using the known values for the molar refractivity and virial coefficients of nitrogen, and accounting for cavity length distortions, the device can be used as a high-resolution, multi-decade pressure sensor. We define a reference value for nitrogen refractivity as n-1=(26485.28±0.3)×10(-8) at p=100.0000  kPa, T=302.9190  K, and λ(vac)=632.9908  nm. We compare pressure determinations via the refractometer and the reference value to a mercury manometer.

  8. Equation of state measurements using single Fabry-Perot velocimeter

    Science.gov (United States)

    Saxena, A. K.; Kaushik, T. C.; Rawool, A. M.; Gupta, Satish C.

    2012-07-01

    Accurate measurement of projectile and free surface velocity of target is a crucial part of any high pressure physics experiments involving hyper velocity impact. Fabry Perot velocimeter (FPV) is a widely accepted technique for single surface velocity measurements. In the present work a possibility of using single FPV for two surface velocity measurements is experimentally explored. The laser light is launched in such a way that it illuminates both the surfaces under study and reflected light coming from the flyer as well as from the target is used to generate interference fringes. Therefore by recording fringe displacements due to Doppler shift in reflected light corresponding to movement of both the surfaces at different time instants, velocity profile of both target and projectile can be computed. This technique has been utilized for equation of state measurements of polyurethane based retro-reflective tape at 12 GPa and aluminum at 15 GPa. Measured peak particle velocities at the target-glass interface have been found in good agreement with the reported equation of state data.

  9. Fabry-Perot干涉腔型光纤声发射传感器%Fiber-optic acoustic emission sensors with dual Fabry-Perot interferometric cavities

    Institute of Scientific and Technical Information of China (English)

    赵江海; 章小建

    2015-01-01

    设计了一种新型的双Fabry-Perot腔光纤传感器用于材料损伤引起的声发射信号检测.基于低细度Fabry-Perot腔多光束干涉原理,建立了光纤Fabry-Perot传感器检测声发射信号的数学模型.分析双Fabry-Perot腔正交点稳定工作机制,设计并制作了具有双Fabry-Perot腔结构的光纤声发射传感器来保持传感器正交点的稳定性.通过模拟AE信号检测与热应力干扰实验对设计的传感器进行了实验验证.实验一通过冲击振动与折断铅笔芯产生两类模拟声发射信号,使用商用的压电传感器和光纤传感器进行对比实验,结果表明光纤传感器能够成功检测到两类模拟声发射信号,灵敏度为12.9 nm,带宽达到30 kHz.实验二对传感器进行工作点稳定测试,结果表明双F-P腔的控制机制能够保证传感器工作在正交点,解决了传感器输出信号衰减的问题.

  10. Neuropsychiatric manifestations of Fahr′s disease pathogenesis and potential for treatment

    Directory of Open Access Journals (Sweden)

    Raheel Mushtaq

    2013-01-01

    Full Text Available Fahr′s disease (FD is a rare neuropsychiatric disease consisting of bilateral basal ganglia calcification with neurological, cognitive, and psychiatric manifestations. We report here a sporadic case of FDs with its neuropsychology.

  11. Neuropsychiatric manifestations of Fahr′s disease pathogenesis and potential for treatment

    OpenAIRE

    Raheel Mushtaq; Sheikh Shoib; M. S. V. K Raju; Nilesh Naphade; Tabindah Shah; Alka Pawar

    2013-01-01

    Fahr's disease (FD) is a rare neuropsychiatric disease consisting of bilateral basal ganglia calcification with neurological, cognitive, and psychiatric manifestations. We report here a sporadic case of FDs with its neuropsychology.

  12. Population Pharmacokinetics of Gemcitabine and dFdU in Pancreatic Cancer Patients Using an Optimal Design, Sparse Sampling Approach.

    Science.gov (United States)

    Serdjebi, Cindy; Gattacceca, Florence; Seitz, Jean-François; Fein, Francine; Gagnière, Johan; François, Eric; Abakar-Mahamat, Abakar; Deplanque, Gael; Rachid, Madani; Lacarelle, Bruno; Ciccolini, Joseph; Dahan, Laetitia

    2017-06-01

    Gemcitabine remains a pillar in pancreatic cancer treatment. However, toxicities are frequently observed. Dose adjustment based on therapeutic drug monitoring might help decrease the occurrence of toxicities. In this context, this work aims at describing the pharmacokinetics (PK) of gemcitabine and its metabolite dFdU in pancreatic cancer patients and at identifying the main sources of their PK variability using a population PK approach, despite a sparse sampled-population and heterogeneous administration and sampling protocols. Data from 38 patients were included in the analysis. The 3 optimal sampling times were determined using KineticPro and the population PK analysis was performed on Monolix. Available patient characteristics, including cytidine deaminase (CDA) status, were tested as covariates. Correlation between PK parameters and occurrence of severe hematological toxicities was also investigated. A two-compartment model best fitted the gemcitabine and dFdU PK data (volume of distribution and clearance for gemcitabine: V1 = 45 L and CL1 = 4.03 L/min; for dFdU: V2 = 36 L and CL2 = 0.226 L/min). Renal function was found to influence gemcitabine clearance, and body surface area to impact the volume of distribution of dFdU. However, neither CDA status nor the occurrence of toxicities was correlated to PK parameters. Despite sparse sampling and heterogeneous administration and sampling protocols, population and individual PK parameters of gemcitabine and dFdU were successfully estimated using Monolix population PK software. The estimated parameters were consistent with previously published results. Surprisingly, CDA activity did not influence gemcitabine PK, which was explained by the absence of CDA-deficient patients enrolled in the study. This work suggests that even sparse data are valuable to estimate population and individual PK parameters in patients, which will be usable to individualize the dose for an optimized benefit to risk ratio.

  13. Fast 3d Hybrid Seismic Modeling: Ray-fd Approach For Elastic Models With Locally Complex Structures

    Science.gov (United States)

    Oprsal, I.; Brokesova, J.; Faeh, D.; Giardini, D.

    Hybrid approaches may find broad applications wherever full source, path,and site effects modeling methods are too expensive. A new efficient hybrid method allowing to compute seismic wavefield in large 3D elastic models containing a complex local structure embedded in a large, but considerably simpler, structure is designed. This hybrid method combines the ray approach in the large simple structure with the finite difference (FD) approach in the local complex structure. The hybrid method is based on two successive steps. In the 1st one, the source and path information is carried by wavefield propagating in the large simple structure. This wavefield, calculated by the ray method, is incident at the points along a two-fold formal boundary (excitation box, EB) surrounding that part of the model which is to be replaced by the complex medium in the 2nd step. 3D rays are necessary due to ar- bitrary source-EB configuration, even in case the 1st step structure is less dimensional (2D, 1D, homogeneous). Along EB, the ray endpoints may be distributed sparsely thanks to relative simplicity of the structure. This reduces computer time requirements and also the size of the excitation file saved on the disk. The ray wavefield along EB provides (after interpolation in space and time) the input for the second step consisting in calculating the complete wavefield by the 3D FD method on irregular grids. The FD computational domain contains the EB and its close vicinity. The 2nd step model differs from the 1st step model only inside the EB where the local complex structure is inserted. To verify the consistency between the 1st and the 2nd step binding, the 2nd step computation can be performed on (unchanged) 1st step model ('replication test'). This should give the same wavefield as the 1st step inside, and zero wavefield outside the EB. The EB remains fully permeable for all waves propagating within the FD domain. Provided the 1st step structure does not contain too many layers

  14. Two-dimensional photonic-crystal-based Fabry-Perot etalon.

    Science.gov (United States)

    Ho, Chong Pei; Pitchappa, Prakash; Kropelnicki, Piotr; Wang, Jian; Cai, Hong; Gu, Yuandong; Lee, Chengkuo

    2015-06-15

    We demonstrate the design, fabrication, and characterization of a polycrystalline-silicon-based photonic crystal Fabry-Perot etalon, which is aimed to work in the mid-infrared wavelengths. The highly reflective mirrors required in a Fabry-Perot etalon are realized by freestanding polycrystalline-silicon-based photonic crystal membranes with etched circular air holes. A peak reflection of 96.4% is observed at 3.60 μm. We propose a monolithic CMOS-compatible fabrication process to configure two such photonic crystal mirrors to be in parallel to form a Fabry-Perot etalon; a filtered transmission centered at 3.51 μm is observed. The quality factor measured is around 300, which is significantly higher than in existing works. This creates the possibility of using such devices for high-resolution applications such as gas sensing and hyperspectral imaging.

  15. Enhanced random lasing in ZnO nanocombs assisted by Fabry-Perot resonance.

    Science.gov (United States)

    Chen, Yungting; Chen, Yangfang

    2011-04-25

    The ultraviolet random lasing behavior of an ensemble of ZnO nanocombs has been demonstrated. It is found that the Fabry-Perot resonance induced by nanocomb geometry can greatly enhance random lasing action with a low threshold condition. Besides, the emission spectra exhibit few sharp lasing peaks with a full width at half maximum (FWHM) of less than 0.3 nm and a narrow background emission with a FWHM of about 5 nm. Cathodoluminescence mapping images are utilized to analyze the Fabry-Perot resonance phenomenon. The resonant effect on the lasing system is further confirmed by nanocombs with different resonant cavity lengths. The unique lasing behavior induced by the simultaneous occurrence of Fabry-Perot resonance and random laser action shown here may open up a new possibility for the creation of highly efficient light emitting devices.

  16. Distributed torsion sensor based on cascaded coaxial cable Fabry-Perot interferometers

    Science.gov (United States)

    Cheng, Baokai; Zhu, Wenge; Hua, Liwei; Liu, Jie; Li, Yurong; Nygaard, Runar; Xiao, Hai

    2016-07-01

    Cascaded coaxial cable Fabry-Perot interferometers (FPI) are studied and demonstrated for distributed torsion measurement. Multiple weak reflectors are implemented on a coaxial cable so that any two consecutive reflectors can form a Fabry-Perot cavity. By fixing the cable sensor in a helical form on a shaft, the distributed torsion of the shaft can be measured by the cascaded Fabry-Perot cavities. A test on a single section shows that the sensor has a linear response with a sensitivity of 1.834 MHz (rad/m)-1 in the range of twisted rate from 0 to 8.726 rad m-1. The distributed torsion sensing capability is useful in drilling process monitoring, structure health monitoring and machine failure detection.

  17. Improved spectral characteristics of 980 nm broad area slotted Fabry-Perot diode lasers

    Institute of Scientific and Technical Information of China (English)

    Gao Zhuo; Wang Jun; Xiong Cong; Liu Yuanyuan; Liu Suping; Ma Xiaoyu

    2012-01-01

    A novel broad area slotted Fabry-Perot diode laser is designed and fabricated.Using a new semianalytical method,we introduce effective refractive index perturbations in the form of etched slot features into a conventional 980 nm broad area Fabry-Perot cavity,and the spectral characteristics of the device are expected to be noticeably improved.A low density of slot features is formed by using standard optical lithography and inductively coupled plasma dry etching.The experimental results show that the full spectral width at half-maximum is less than 0.4 nm,meanwhile,the thermal shift of the emission spectrum is decreased from 0.26 to 0.07 nm/℃ over a temperature range of 10 to 60 ℃.The improved spectral characteristics of the device are proved to be attributed to such slotted Fabry-Perot laser structures.

  18. High Finesse Fiber Fabry-Perot Cavities: Stabilization and Mode Matching Analysis

    CERN Document Server

    Gallego, Jose; Alavi, Seyed Khalil; Alt, Wolfgang; Martinez-Dorantes, Miguel; Meschede, Dieter; Ratschbacher, Lothar

    2015-01-01

    Fiber Fabry-Perot cavities, formed by micro-machined mirrors on the end-facets of optical fibers, are used in an increasing number of technical and scientific applications, where they typically require precise stabilization of their optical resonances. Here, we study two different approaches to construct fiber Fabry-Perot resonators and stabilize their length for experiments in cavity quantum electrodynamics with neutral atoms. A piezo-mechanically actuated cavity with feedback based on the Pound-Drever-Hall locking technique is compared to a novel rigid cavity design that makes use of the high passive stability of a monolithic cavity spacer and employs thermal self-locking and external temperature tuning. Furthermore, we present a general analysis of the mode matching problem in fiber Fabry-Perot cavities, which explains the asymmetry in their reflective line shapes and has important implications for the optimal alignment of the fiber resonators. Finally, we discuss the issue of fiber-generated background ph...

  19. Fiber Fabry-Perot interferometer with controllable temperature sensitivity.

    Science.gov (United States)

    Zhang, Xinpu; Peng, Wei; Zhang, Yang

    2015-12-01

    We proposed a fiber taper based on the Fabry-Perot (FP) interferometer structure with controllable temperature sensitivity. The FP interferometer is formed by inserting a segment of tapered fiber tip into the capillary and subsequently splicing the other end of the capillary to a single-mode fiber (SMF), the tapered fiber endface, and the spliced face form the FP cavity. Through controlling the inserted tapered fiber length, a series of FP interferometers were made. Because the inserted taper tip has the degree of freedom along the fiber axial, when the FP interferometer is subjected to temperature variation, the thermal expansion of the fiber taper tip will resist the FP cavity length change caused by the evolution of capillary length, and we can control the temperature sensitivity by adjusting the inserted taper length. In this structure, the equivalent thermal expansion coefficient of the FP interferometer can be defined; it was used to evaluate the temperature sensitivity of the FP interferometer, which provides an effective method to eliminate the temperature effect and to enhance other measurement accuracy. We fabricated the FP interferometers and calibrated their temperature characters by measuring the wavelength shift of the resonance dips in the reflection spectrum. In a temperature range of 50°C to 150°C, the corresponding temperature sensitivities can be controlled between 0 and 1.97 pm/°C when the inserted taper is between 75 and 160 μm. Because of its controllable temperature sensitivity, ease of fabrication, and low cost, this FP interferometer can meet different temperature sensitivity requirements in various application areas, especially in the fields which need temperature insensitivity.

  20. Probing a self-assembled fd virus membrane with a microtubule

    Science.gov (United States)

    Xie, Sheng; Pelcovits, Robert A.; Hagan, Michael F.

    2016-06-01

    The self-assembly of highly anisotropic colloidal particles leads to a rich variety of morphologies whose properties are just beginning to be understood. This article uses computer simulations to probe a particle-scale perturbation of a commonly studied colloidal assembly, a monolayer membrane composed of rodlike fd viruses in the presence of a polymer depletant. Motivated by experiments currently in progress, we simulate the interaction between a microtubule and a monolayer membrane as the microtubule "pokes" and penetrates the membrane face-on. Both the viruses and the microtubule are modeled as hard spherocylinders of the same diameter, while the depletant is modeled using ghost spheres. We find that the force exerted on the microtubule by the membrane is zero either when the microtubule is completely outside the membrane or when it has fully penetrated the membrane. The microtubule is initially repelled by the membrane as it begins to penetrate but experiences an attractive force as it penetrates further. We assess the roles played by translational and rotational fluctuations of the viruses and the osmotic pressure of the polymer depletant. We find that rotational fluctuations play a more important role than the translational ones. The dependence on the osmotic pressure of the depletant of the width and height of the repulsive barrier and the depth of the attractive potential well is consistent with the assumed depletion-induced attractive interaction between the microtubule and viruses. We discuss the relevance of these studies to the experimental investigations.

  1. A Three-Dimensional DRAM Using Floating Body Capacitance Cells in an FD-SOI Process

    Directory of Open Access Journals (Sweden)

    Xuelian Liu

    2013-12-01

    Full Text Available This paper describes a three- dimensional DRAM in which the floating body capacitance (FBC of a fully depleted SOI (FD-SOI device is used as a storage node. This 1T DRAM lends itself particularly well to a 3D wafer-to-wafer bonding process because of the absence of deep etched and filled trench capacitor structure, and the improved thickness control tolerance in wafer thinning. A novel three-tier, 3D, 1T embedded DRAM is presented that can be vertically integrated with a microprocessor, achieving low cost, high-density on-chip main memory. A 394Kbits test chip has been designed and fabricated using the Lincoln Labs 3-Tier 3D 0.18um fully depleted SOI CMOS process where an earlier (and previously reported successful 3D SRAM was obtained. The measured retention time under holding conditions in this 180 nm process is greater than 10 ms. The test chip measures an access time of 50 ns and operates at 10 MHz.

  2. Analysis of ITMS System Impact Mechanism in Beijing Based on FD and Traffic Entropy

    Directory of Open Access Journals (Sweden)

    Ailing Huang

    2012-01-01

    Full Text Available Although more attention has been attracted to benefit evaluation of Intelligent Transportation Systems (ITS deployment, how ITS impact the traffic system and make great effects is little considered. As a subsystem of ITS, in this paper, Intelligent Transportation Management System (ITMS is studied with its impact mechanism on the road traffic system. Firstly, the correlative factors between ITMS and the road traffic system are presented and 3 positive feedback chains are defined. Secondly, we introduce the theory of Fundamental Diagram (FD and traffic system entropy to demonstrate the correlative relationship between ITMS and feedback chains. The analyzed results show that ITMS, as a negative feedback factor, has damping functions on the coupling relationship of all 3 positive feedback chains. It indicates that with its deployment in Beijing, ITMS has impacted the improvement of efficiency and safety for the road traffic system. Finally, related benefits brought by ITMS are presented corresponding to the correlative factors, and effect standards are identified for evaluating ITMS comprehensive benefits.

  3. Magnetoresistance mobility characterization in advanced FD-SOI n-MOSFETs

    Science.gov (United States)

    Shin, Minju; Shi, Ming; Mouis, Mireille; Cros, Antoine; Josse, Emmanuel; Mukhopadhyay, Sutirha; Piot, Benjamin; Kim, Gyu-Tae; Ghibaudo, Gérard

    2015-01-01

    In this work, we applied the magnetoresistance (MR) characterization technique on n-type FD-SOI devices from a 14 nm-node technology. A notable advantage of MR is that it can probe the sub-threshold region, where Coulomb scattering influence is unscreened, while classical methods are validated to the strong inversion regime. At first, we discuss the influence of series resistance depending on gate bias, gate stack and temperature in this technology. Secondly, for long channel devices, we show that Coulomb scattering plays no significant role below threshold voltage at room temperature, in spite of the presence of a high-k/metal gate stack. MR-mobility (μMR) measurements were also performed in interface coupling conditions in order to further assess the role of the high-k/metal gate stack on transport properties and to analyze back bias induced mobility variations, depending on temperature range. Finally, the comparative study of low field effective mobility (μ0) and μMR shows that critical gate length of mobility degradation can be overestimated by using μ0 at low temperature due to a lack of ability of Y-function method to capture unscreened Coulomb scattering.

  4. Pea VEGETATIVE2 Is an FD Homolog That Is Essential for Flowering and Compound Inflorescence Development.

    Science.gov (United States)

    Sussmilch, Frances C; Berbel, Ana; Hecht, Valérie; Vander Schoor, Jacqueline K; Ferrándiz, Cristina; Madueño, Francisco; Weller, James L

    2015-04-01

    As knowledge of the gene networks regulating inflorescence development in Arabidopsis thaliana improves, the current challenge is to characterize this system in different groups of crop species with different inflorescence architecture. Pea (Pisum sativum) has served as a model for development of the compound raceme, characteristic of many legume species, and in this study, we characterize the pea VEGETATIVE2 (VEG2) locus, showing that it is critical for regulation of flowering and inflorescence development and identifying it as a homolog of the bZIP transcription factor FD. Through detailed phenotypic characterizations of veg2 mutants, expression analyses, and the use of protein-protein interaction assays, we find that VEG2 has important roles during each stage of development of the pea compound inflorescence. Our results suggest that VEG2 acts in conjunction with multiple FLOWERING LOCUS T (FT) proteins to regulate expression of downstream target genes, including TERMINAL FLOWER1, LEAFY, and MADS box homologs, and to facilitate cross-regulation within the FT gene family. These findings further extend our understanding of the mechanisms underlying compound inflorescence development in pea and may have wider implications for future manipulation of inflorescence architecture in related legume crop species.

  5. A FD/DAMA network architecture for the first generation land mobile satellite services

    Science.gov (United States)

    Yan, T.-Y.; Wang, C.; Cheng, U.; Dessouky, K.; Rafferty, W.

    1989-01-01

    A frequency division/demand assigned multiple access (FD/DAMA) network architecture for the first-generation land mobile satellite services is presented. Rationales and technical approaches are described. In this architecture, each mobile subscriber must follow a channel access protocol to make a service request to the network management center before transmission for either open-end or closed-end services. Open-end service requests will be processed on a blocked call cleared basis, while closed-end requests will be processed on a first-come-first-served basis. Two channel access protocols are investigated, namely, a recently proposed multiple channel collision resolution scheme which provides a significantly higher useful throughput, and the traditional slotted Aloha scheme. The number of channels allocated for either open-end or closed-end services can be adaptively changed according to aggregated traffic requests. Both theoretical and simulation results are presented. Theoretical results have been verified by simulation on the JPL network testbed.

  6. Matlab 稳健回归法在 FD 3019γ测井仪校准中的应用%The Application of Matlab Robust Regression in the Calibration of FD 3019

    Institute of Scientific and Technical Information of China (English)

    何涛

    2016-01-01

    简要介绍了 FD 3019γ测井仪的校准方法,即 Matlab 稳健回归法及其抗差性。通过一个实例对比了 Matlab 稳健回归法和最小二乘法建立的一元线性回归方程,结果表明 Matlab 稳健回归法较之最小二乘法更为准确。%The calibration method of FD 301 9 gamma logging instrument was introduced in this paper and discussed the Matlab robust regression function and its robustness.Comparison between the estab-lished linear Matlab robust regression and least squares regression equation shew that Matlab robust re-gression method is superior to the least squares method in accuracy.

  7. High-temperature fiber-optic Fabry-Perot interferometric sensors

    Energy Technology Data Exchange (ETDEWEB)

    Ding, Wenhui; Jiang, Yi; Gao, Ran, E-mail: bitjy@bit.edu.cn [School of Optoelectronics, Beijing Institute of Technology, Beijing 100081 (China); Liu, Yuewu [Key Laboratory for Mechanics in Fluid Solid Coupling Systems, Institute of Mechanics, Chinese Academy of Science, Beijing 100190 (China)

    2015-05-15

    A photonic crystal fiber (PCF) based high-temperature fiber-optic sensor is proposed and experimentally demonstrated. The sensor head is a Fabry-Perot cavity manufactured with a short section of endless single-mode photonic crystal fiber (ESM PCF). The interferometric spectrum of the Fabry-Perot interferometer is collected by a charge coupled device linear array based micro spectrometer. A high-resolution demodulation algorithm is used to interrogate the peak wavelengths. Experimental results show that the temperature range of 1200 °C and the temperature resolution of 1 °C are achieved.

  8. High-temperature fiber-optic Fabry-Perot interferometric sensors.

    Science.gov (United States)

    Ding, Wenhui; Jiang, Yi; Gao, Ran; Liu, Yuewu

    2015-05-01

    A photonic crystal fiber (PCF) based high-temperature fiber-optic sensor is proposed and experimentally demonstrated. The sensor head is a Fabry-Perot cavity manufactured with a short section of endless single-mode photonic crystal fiber (ESM PCF). The interferometric spectrum of the Fabry-Perot interferometer is collected by a charge coupled device linear array based micro spectrometer. A high-resolution demodulation algorithm is used to interrogate the peak wavelengths. Experimental results show that the temperature range of 1200 °C and the temperature resolution of 1 °C are achieved.

  9. Negative Goos-H(a)nchen Effect in Thin-Film Fabry-Perot Filter

    Institute of Scientific and Technical Information of China (English)

    LI Ming-Yu; LIU Xu; MA Xin; LI Yi-Yu; GU Pei-Fu

    2007-01-01

    We investigate the Goos-Hanchen effect of a Gaussian light beam reflected by the thin-film Fabry-Perot filter. It is shown that the Goos-Hanchen shift can be either negative or positive. The Gaussian-beam analysis and stationary phase method are introduced to calculate the lateral shift between the incident beam and the reflected beam at different wavelengths and to analyse the Goos-Hanchen effect in the thin-film Fabry-Perot filter. The effect of the incident beam diameter is also discussed.

  10. The Three-Arm Michelson-Fabry-Pérot Detector for Gravitational Waves

    Institute of Scientific and Technical Information of China (English)

    Chao-Guang Huang; Yong-Gui Li; Ning Zhu

    2016-01-01

    A three-arm Michelson-Fabry-Pérot detector for gravitational waves is designed.It consists of three Michelson Fabry-Pérot interferometers,one for each pair of arms.The new detector can be used to confirm whether the gravitational waves are in general relativity polarization states and to set the strong constraints on non-GR gravitational wave polarization states.By the new detectors,the angular resolution of sources can be improved significantly.With the new detector,it is easier to search for and confirm a gravitational wave signal in the observation data.

  11. Period Doubling in a Fabry-Perot Laser Diode Subject to Optical Pulse Injection

    Institute of Scientific and Technical Information of China (English)

    ZHAO Yue-Peng; WANG Yun-Cai; ZHANG Ming-Jiang; AN Yi; WANG Ji-Long

    2007-01-01

    Experimental study and numerical simulations of the period doubling of injected optical pulses in Fabry-Perot laser diodes are presented. In our experiments, the period doubling is achieved within a wide input frequency range and the period doubling of the injected optical pulses with 6.32 GHz repetition rate is investigated in detail. The obtained experimental results indicate that period doubling occurs at an appropriate injected optical power level when the bias current of the Fabry-Perot laser diode is located in lower ranges. Moreover, the experimental observed features have been numerically demonstrated by using a coupled rate-equation model. Numerical simulations are consistent with the experimental results.

  12. Displacement-noise-free gravitational-wave detection with two Fabry-Perot cavities

    CERN Document Server

    Rakhubovsky, Andrey A

    2008-01-01

    We propose two detuned Fabry-Perot cavities, each pumped through both the mirrors, positioned in line as a toy model of the gravitational-wave (GW) detector free from displacement noise of the test masses. It is demonstrated that the noise of cavity mirrors can be completely excluded in a proper linear combination of the cavities output signals. This model is illustrated by a simplified round trip model (without Fabry-Perot cavities). We show that in low-frequency region the obtained displacement-noise-free response signal is stronger than the one of the interferometer recently proposed by S.Kawamura and Y.Chen.

  13. Model of Fabry-Pérot-type electromagnetic modes of a cylindrical nanowire

    DEFF Research Database (Denmark)

    Bordo, Vladimir

    2010-01-01

    The rigorous theory of normal electromagnetic modes of a cylindrical nanowire of finite length is developed. The exact integral equation which determines the solution of Maxwell's equations obeying the boundary conditions at the whole nanowire surface is derived. The nanowire normal (Fabry......-Pérot) modes are defined as non-trivial solutions of the source-free equation. The approach is considered in more detail for elongated nanowires whose length is much larger than their diameter. The resonance condition obtained for a single-mode nanowire resembles the formula for the Fabry-Pérot resonator...

  14. Fiber-Optic Anemometer Based on Silicon Fabry-Perot Interferometer

    Science.gov (United States)

    2015-11-05

    f, oo •• " • ~ OII Ct’ THIS f’ORM CANCELS AND SUPERSEOES All. P~EVIOUS \\I!:RSI Fiber-optic anemometer based on silicon Fabry-Pérot...monitoring, etc. In this paper, we propose a new anemometer which consists of a Fabry-Pérot interferometer (FPI) implemented using a thin silicon mounted...on the tip of an optical fiber. The anemometer takes advantage of the superior thermal and optical properties of silicon. Silicon is transparent to

  15. Decreased vibrational susceptibility of Fabry-Perot cavities via designs of geometry and structural support

    Institute of Scientific and Technical Information of China (English)

    Yang Tao; Li Wen-Bo; Zang Er-Jun; Chen Li-Sheng

    2007-01-01

    Ultra-stable optical cavities are widely used for laser frequency stabilization. In these experiments the laser performance relies on the length stability of the Fabry-Perot cavities. Vibration-induced deformation is one of the dominant factors that affect the stability of ultra-stable optical cavities. We have quantitatively analysed the elastic deformation of Fabry-Perot cavities with various shapes and mounting configurations. Our numerical result facilitates a novel approach for the design of ultra-stable cavities that are insensitive to vibrational perturbations. This approach can be applied to many experiments such as laser frequency stabilization, high-precision laser spectroscopy, and optical frequency standards.

  16. Study on Fabry-Perot interference optic fiber temperature sensor%Fabry-Perot干涉式光纤温度传感器的研究

    Institute of Scientific and Technical Information of China (English)

    曹满婷

    2001-01-01

    分析了温度对相位的调制作用以及Fabry-Perot干涉结构检测相位变化的原理,提出了一种具有高灵敏度和高分辨率的相位调制型全光纤结构,并进行了系统的噪声分析

  17. Optic Fiber Fabry-Perot Interferometer Applied to Measuring Temperature and Displacement%测量温度和位移的光纤Fabry-Perot干涉仪

    Institute of Scientific and Technical Information of China (English)

    陈国霖; 戴福隆; 周辛庚

    2002-01-01

    本文描述了两种光纤Fabry-Peort干涉仪的原理、结构和制造方法.采用光频调制方法用以辨别干涉时域条纹的移动方向,实现了对温度和位移的测量.另外还介绍了条纹计数检测的电路原理.

  18. On the improvement of DC analog characteristics of FD SOI transistors by using asymmetric self-cascode configuration

    Science.gov (United States)

    de Souza, Michelly; Flandre, Denis; Doria, Rodrigo Trevisoli; Trevisoli, Renan; Pavanello, Marcelo Antonio

    2016-03-01

    This paper demonstrates the improvement of DC analog performance of FD SOI transistors provided by the adoption of asymmetric self-cascode (A-SC) configuration. It consists of two transistors connected in series with gates shortened, acting as a single device. The doping concentration of the two transistors in the structure is different, leading to higher threshold voltage of the transistor at the source side of the composite structure than that of the transistor at the drain side. By reducing the doping concentration level at the channel of the transistor at drain side of the composite structure, forcing it to work in saturation, part of the applied drain bias is absorbed and does not reach the transistor close to the source, which is the main responsible for the overall device characteristics. As a result, larger drain current level and transconductance are obtained in comparison to symmetric self-cascode (where both transistors present same doping level) apart from promoting output conductance reduction. The transconductance, output conductance, Early voltage, and intrinsic voltage gain are used as figures of merit to demonstrate and validate the advantages of the proposed structure. The influence of channel length and doping concentration are also evaluated. The A-SC configuration is fully compatible with any standard FD SOI MOSFET technology with multiple threshold voltages. A simulation analysis demonstrates the feasibility of the proposed asymmetric structure in a UTBB FD SOI technology.

  19. A New Method of processing the Fabry-Perot Interference Fringes%一种新的Fabry-Perot干涉条纹处理方法

    Institute of Scientific and Technical Information of China (English)

    鄂非; 高秋艳; 艾勇

    2009-01-01

    介绍了一种提取Fabry-Perot(法布里-帕罗)干涉条纹圆心点坐标和条纹半径的新方法.首先对干涉图像依次进行二值化处理,对所得到的条纹强度曲线进行均平滤波和自适应滤波,根据条纹灰度值强度余弦函数分布的特点,对条纹灰度值数据进行最小二乘法拟合,获得条纹强度峰值坐标,通过精确的迭代算法,进而获得Fabry-Perot干涉条纹圆心点的坐标;然后再对干涉条纹进行圆周积分,从而可以确定每级Fabry-Perot干涉条纹的半径长度.该方法可提高计算精度,减小计算误差.

  20. Performance Analysis of Compact FD-MIMO Antenna Arrays in a Correlated Environment

    KAUST Repository

    Nadeem, Qurrat-Ul-Ain

    2017-03-06

    Full dimension multiple-input-multiple-output (FDMIMO) is one of the key technologies proposed in the 3rd Generation Partnership Project (3GPP) for the fifth generation (5G) communication systems. The reason can be attributed to its ability to yield significant performance gains through the deployment of active antenna elements at the base station in the vertical as well as the conventional horizontal directions, enabling several elevation beamforming strategies. The resulting improvement in spectral efficiency largely depends on the orthogonality of the sub-channels constituting the FD-MIMO system. Accommodating a large number of antenna elements with sufficient spacing poses several constraints for practical implementation, making it imperative to consider compact antenna arrangements that minimize the overall channel correlation. Two such configurations considered in this work are the uniform linear array (ULA) and the uniform circular array (UCA) of antenna ports, where each port is mapped to a group of physical antenna elements arranged in the vertical direction. The generalized analytical expression for the spatial correlation function (SCF) for the UCA is derived, exploiting results on spherical harmonics and Legendre polynomials. The mutual coupling between antenna dipoles is accounted for and the resulting SCF is also presented. The second part of this work compares the spatial correlation and mutual information (MI) performance of the ULA and UCA configurations in the 3GPP 3D urban-macro and urban-micro cell scenarios, utilizing results from Random Matrix Theory (RMT) on the deterministic equivalent of the MI for the Kronecker channel model. Simulation results study the performance patterns of the two arrays as a function of several channel and array parameters and identify applications and environments suitable for the deployment of each array.

  1. Podocitúria na doença de Fabry

    Directory of Open Access Journals (Sweden)

    Ester Miranda Pereira

    2016-03-01

    Full Text Available Resumo Introdução: A doença de Fabry (DF é uma desordem lisossômica ligada ao cromossomo X ocasionada por mutações no gene que codifica a enzima lisossômica α-galactosidase A (α-GAL. A redução ou ausência da atividade dessa enzima leva ao acúmulo progressivo de gb3. A doença renal é uma importante consequência clínica da acumulação de Gb3. Podócito é o tipo celular mais afetado na doença renal, que mostra apenas uma resposta parcial à Terapia de Reposição Enzimática. Além disso, a disfunção podocitária é a principal contribuinte para a perda progressiva da função renal e pode ser encontrada alterada mesmo antes do início da microalbuminúria. Assim, a podocitúria na DF pode ser uma ferramenta importante para prever a doença renal. Objetivo: O objetivo deste estudo foi quantificar a excreção urinária de podócitos em pacientes com DF (V269M, n = 14 e controles saudáveis (n = 40, e relacioná-las com as variáveis sexo, idade, tempo de terapia e a razão albumina: creatinina (AUC. Métodos: Podócitos urinários foram identificados utilizando imunofluorescência para podocalixina e DAPI. O número de células podocalixina positivo foi contado e o número médio foi utilizado (faixa normal 0-0.6 podócitos/mL. Resultados: O número médio de podócitos na urina de pacientes com DF foi significativamente maior do que os controles saudáveis (p < 0.0001. Observou-se uma correlação positiva entre podocitúria e AUC (p = 0.004; r2 = 0.6417. Conclusão: A podocitúria pode ser uma ferramenta adicional para avaliar a progressão da doença renal em pacientes que se espera que tenha um fenótipo mais agressivo.

  2. Effects of disturbance on regeneration of Abies fabri forests at the upper reaches of the Yangtze River

    Institute of Scientific and Technical Information of China (English)

    Gao Jia-rong; Gao Yang

    2007-01-01

    Mudflow is the principal disturbance in Abies fabri forests. In the Gongga Mountain areas of the upper reaches of the Yangtze River, the intensities and periodicity of different scale mudflows vary. Small-scale mudflows are more frequent, occurring every one or two years while large-scale mudflows may occur once in more than one hundred years. Through a field study of A. fabri forests during different stages of growth, we analyzed their structural characteristics and discovered that after different sizes of mudflow, poplar and birch often occupy the dominant canopy at the expense of the slow growing A. fabri, for only a small number of saplings are A. fabri that occurs in the first regeneration stage. However, a large number of seed resources can be found in mature A.fabri forests and as a unique regeneration species, A. fabri will gradually replace all the other species and form a stable community of strong shade-tolerant trees. Because of the intimate relationship between growing conditions and soil and water conservation at the upper reaches of the Yangtze River, we should carry out some artificial measurements to control and promote the slow regeneration process of A. fabri.

  3. Simultaneous wavelength and orbital angular momentum demultiplexing using tunable MEMS-based Fabry-Perot filter

    DEFF Research Database (Denmark)

    Lyubopytov, Vladimir; Porfirev, Alexey P.; Gurbatov, Stanislav O.

    2017-01-01

    In this paper, we experimentally demonstrate simultaneous wavelength and orbital angular momentum (OAM) multiplexing/demultiplexing of 10 Gbit/s data streams using a new on-chip micro-component-tunable MEMS-based Fabry-Perot filter integrated with a spiral phase plate. In the experiment, two...

  4. Broadband Measurement of Coating Thermal Noise in Rigid Fabry-Perot Cavities

    CERN Document Server

    Chalermsongsak, Tara; Hall, Evan D; Arai, Koji; Gustafson, Eric K; Adhikari, Rana X

    2014-01-01

    We report on the relative length fluctuation of two fixed-spacer Fabry-Perot cavities with mirrors fabricated from silica/tantala dielectric coatings on fused silica substrates. By locking a laser to each cavity and reading out the beat note $\\hat{\

  5. A Lossy Fabry-perot Based Optical Filter for Natural Gas Analysis

    NARCIS (Netherlands)

    Ayerden, N.P.; Ghaderi, M.; De Graaf, G.; Wolffenbuttel, R.F.

    2014-01-01

    A set-up for optical gas composition measurement based on absorption spectroscopy is composed of a white light source, a gas cell and a spectrometer. The Fabry-Perot optical filter is suitable for miniaturization of this system, as it is composed of only two reflectors with a transparent layer in-be

  6. High precision planar waveguide propagation loss measurement technique using a Fabry-Perot cavity

    DEFF Research Database (Denmark)

    Feuchter, Thomas; Thirstrup, Carsten

    1994-01-01

    A high precision measurement technique for characterizing the propagation loss in silica low-loss optical waveguides, based on measuring the contrast of a Fabry-Perot cavity, is demonstrated. The cavity consists of the waveguide coupled to two polarization-maintaining fibers, each end facet coated...

  7. All-Optical Switching Using Fabry-Perot Laser Diodes(Invited paper)

    Institute of Scientific and Technical Information of China (English)

    P. K. A. Wai; L. Y. Chan; H. Y. Tam

    2003-01-01

    In this paper, we investigate all-optical packet switching using a multi-wavelength mutual injection-locked Fabry-Perot laser diode. We observe error-free packet-switching of a 10 Gb/s signal with an extinction ratio of 16.9.

  8. A Novel Extrinsic Fiber-Optic Fabry-Perot Strain Sensor System Based on Optical Amplification

    Institute of Scientific and Technical Information of China (English)

    Yun-Jiang Rao; Jian Jiang; Zheng-Lin Ran

    2003-01-01

    A novel extrinsic fiber-optic Fabry-Perot interferometric strain sensor system is demonstrated based on the simultaneous use of the amplified spontaneous emission and optical amplification. The improvement of 3~4 orders of magnitude in signal level can be achieved.

  9. Influence of laser frequency noise on scanning Fabry-Perot interferometer based laser Doppler velocimetry

    DEFF Research Database (Denmark)

    Rodrigo, Peter John; Pedersen, Christian

    2014-01-01

    n this work, we study the performance of a scanning Fabry-Perot interferometer based laser Doppler velocimeter (sFPILDV) and compare two candidate 1.5 um single-frequency laser sources for the system – a fiber laser (FL) and a semiconductor laser (SL). We describe a straightforward calibration pr...

  10. Single resonance monolithic Fabry-Perot filters formed by volume Bragg gratings and multilayer dielectric mirrors.

    Science.gov (United States)

    Lumeau, Julien; Koc, Cihan; Mokhun, Oleksiy; Smirnov, Vadim; Lequime, Michel; Glebov, Leonid B

    2011-05-15

    A new class of Fabry-Perot filters produced by a multilayer dielectric mirror deposited on top of a reflecting volume Bragg grating is described. The first fabricated prototype for the 852 nm region demonstrates a 30 pm bandwidth, 90+% transmission at resonance, and a good agreement with theoretical simulation. © 2011 Optical Society of America

  11. Enhanced Transmission in Rolled-up Hyperlenses utilizing Fabry-Pe\\'rot Resonances

    CERN Document Server

    Kerbst, Jochen; Rottler, Andreas; Koitmäe, Aune; Bröll, Markus; Stemmann, Andrea; Heyn, Christian; Heitmann, Detlef; Mendach, Stefan

    2011-01-01

    We experimentally demonstrate that the transmission though rolled-up metal/semiconductor hyperlenses can be enhanced at desired frequencies utilizing Fabry-P\\'erot resonances. By means of finite difference time domain simulations we prove that hyperlensing occurs at frequencies of high transmission.

  12. A Ray-tracing Method to Analyzing Modulated Planar Fabry-Perot Antennas

    DEFF Research Database (Denmark)

    Hougs, Mikkel Dahl; Kim, Oleksiy S.; Breinbjerg, Olav

    2015-01-01

    A new approach for fast modelling of Fabry-Perot antennas with modulated partially reflective surfaces (PRS) using ray-tracing is proposed. For validation of the method, a configuration is introduced which consists of a cavity with a modulated PRS, fed internally by a magnetic dipole. The PRS con...

  13. A Novel Extrinsic Fiber-Optic Fabry-Perot Strain Sensor System Based on Optical Amplification

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    A novel extrinsic fiber-optic Fabry-Perot interferometric strain sensor system is demonstrated based on the simultaneous use of the amplified spontaneous emission and optical amplification. The improvement of 3~4 orders of magnitude in signal level can be achieved.

  14. A Ray-tracing Method to Analyzing Modulated Planar Fabry-Perot Antennas

    DEFF Research Database (Denmark)

    Hougs, Mikkel Dahl; Kim, Oleksiy S.; Breinbjerg, Olav

    2015-01-01

    A new approach for fast modelling of Fabry-Perot antennas with modulated partially reflective surfaces (PRS) using ray-tracing is proposed. For validation of the method, a configuration is introduced which consists of a cavity with a modulated PRS, fed internally by a magnetic dipole. The PRS...

  15. Development of tunable Fabry-Perot spectral camera and light source for medical applications

    Science.gov (United States)

    Kaarre, M.; Kivi, S.; Panouillot, P. E.; Saari, H.; Mäkynen, J.; Sorri, I.; Juuti, M.

    2013-05-01

    VTT has developed a fast, tunable Fabry-Perot (FP) filter component and applied it in making small, lightweight spectral cameras and light sources. One application field where this novel technology is now tested is medical field. A demonstrator has been made to test the applicability of FP based spectral filtering in the imaging of retina in visible light wavelength area.

  16. Folded Fabry-Perot quasi-optical ring resonator diplexer Theory and experiment

    Science.gov (United States)

    Pickett, H. M.; Chiou, A. E. T.

    1983-01-01

    Performance of folded Fabry-Perot quasi-optical ring resonator diplexers with different geometries of reflecting surfaces is investigated both theoretically and experimentally. Design of optimum surface geometry for minimum diffraction, together with the figure of merit indicating improvement in performance, are given.

  17. All-Optical Switching Using Fabry-Perot Laser Diodes (Invited paper)

    Institute of Scientific and Technical Information of China (English)

    P.; K.; A.; Wai; L.; Y.; Chan; H.; Y.; Tam

    2003-01-01

    In this paper, we investigate all-optical packet switching using a multi-wavelength mutual injection-locked Fabry-Perot laser diode. We observe error-free packet-switching of a 10 Gb/s signal with an extinction ratio of 16.9.

  18. Large-area Fabry-Perot modulator based on electro-optic polymers

    DEFF Research Database (Denmark)

    Benter, Nils; Bertram, Ralph Peter; Soergel, Elisabeth

    2006-01-01

    We present a large-area electro-optic Fabry-Perot modulator utilizing a photoaddressable bis-azo polymer placed between two dielectric mirrors with an open aperture of 2 cm. A modulation efficientcy of 1% at an effective modulation voltage of 20 V for a wavelength of 1.55 mymeter is demonstrated...

  19. Investgation of the risk factors of FD in Beijing university students%北京在校大学生功能性消化不良相关危险因素的调查

    Institute of Scientific and Technical Information of China (English)

    胡景; 杨云生; 彭丽华; 孙刚; 郭旭; 王巍峰

    2009-01-01

    生FD的相关危险因素.%Objective To investigate the possible risk factors of functional dyspepsia(FD) in Beijing uni-versity students. Methods 490764 random samples were selected. A self-reported questionnaire containing ROME Ⅲ questionnaire and other questions regarding food habit, studying style, past history, ere was delivered to all students. The factors related to FD were compared between the two groups. Rosults To distribute ques-tionnaire 9800,call in 8608. The response rate is 87.8% ,the qualification rate is 80.8%. A total of 602 youth were divided into FD group (110 cases) and control group (492 cases without any symptoms related to FD).The differences between the two groups were found as follows: ①Indexes in height, weight and body mass in FD group were significantly lower than those in the control group (P < 0.05, P < 0.01). ②The occurrence rate of engorgement, being choosy in food, often eating omophagia, cold drinking, overheated food, pungent food, dry and hard food, milk food, dietetic restraint, and wine drinking in FD group was significantly higher than that in the control group (P<0.01). ③The cases in FD group had significantly less time for relaxation, sleep, physical activities, rest on weekends than those in the control group (P<0.01). Mueh more cases in FD group liked to stay up studying and felt stress and press in study than those in the control group (P< 0.05). ④More cases had the history of food allergy, frequent oral administration of drugs, surgical trauma and operation, dysentery, gastroenteritis,liver and gall and pancreas diseases and the similar familial symptoms of FD in FD group than those in the control group (P < 0.05). ⑤Logistic regression analysis showed FD was positively correlated with being choosy in food, lesser movement time, smoking, frequent oral administration of drugs, history of surgical trauma and operation, history of gastroenteritis, history of similar familial symptoms,but negatively with sleep time. Conclusion The above mentioned

  20. Calcium-dependent protein kinases responsible for the phosphorylation of a bZIP transcription factor FD crucial for the florigen complex formation.

    Science.gov (United States)

    Kawamoto, Nozomi; Sasabe, Michiko; Endo, Motomu; Machida, Yasunori; Araki, Takashi

    2015-02-09

    Appropriate timing of flowering is critical for reproductive success and necessarily involves complex genetic regulatory networks. A mobile floral signal, called florigen, is a key molecule in this process, and flowering locus T (FT) protein is its major component in Arabidopsis. FT is produced in leaves, but promotes the floral transition in the shoot apex, where it forms a complex with a basic region/leucine-zipper (bZIP) transcription factor, FD. Formation of the florigen complex depends on the supposed phosphorylation of FD; hitherto, however, the responsible protein kinase(s) have not been identified. In this study, we prepared protein extracts from shoot apices of plants around the floral transition, and detected a protein kinase activity that phosphorylates a threonine residue at position 282 of FD (FD T282), which is a crucial residue for the complex formation with FT via 14-3-3. The kinase activity was calcium-dependent. Subsequent biochemical, cellular, and genetic analyses showed that three calcium-dependent protein kinases (CDPKs) efficiently phosphorylate FD T282. Two of them (CPK6 and CPK33) are expressed in shoot apical meristem and directly interact with FD, suggesting they have redundant functions. The loss of function of one CDPK (CPK33) resulted in a weak but significant late-flowering phenotype.

  1. Influence of the Fineness of Optical Fiber Fabry-Perot Resonant Cavity on the Phase Sensitivity%光纤Fabry-Perot谐振腔的精细度对相位灵敏度的影响

    Institute of Scientific and Technical Information of China (English)

    康崇; 王政平; 黄宗军; 吴强; 韩占国

    2005-01-01

    分析了光纤Fabry-Perot谐振腔的工作原理及其工作点的选择问题,推导了谐振腔的光学相位灵敏度与精细度的关系,给出了理论公式及曲线,并与光纤Math-Zehnder干涉进行了对比,指出了在相位灵敏度上两者的巨大差距,证明了光纤Fabry-Perot干涉的独特优点.介绍了光纤Fabry-Perot干涉的实验系统,提出了检测及计算Fabry-Perot谐振腔有效精细度的方法,实验结果与理论仿真基本吻合.

  2. Wildlife disease elimination and density dependence

    KAUST Repository

    Potapov, A.

    2012-05-16

    Disease control by managers is a crucial response to emerging wildlife epidemics, yet the means of control may be limited by the method of disease transmission. In particular, it is widely held that population reduction, while effective for controlling diseases that are subject to density-dependent (DD) transmission, is ineffective for controlling diseases that are subject to frequency-dependent (FD) transmission. We investigate control for horizontally transmitted diseases with FD transmission where the control is via culling or harvest that is non-selective with respect to infection and the population can compensate through DD recruitment or survival. Using a mathematical model, we show that culling or harvesting can eradicate the disease, even when transmission dynamics are FD. Eradication can be achieved under FD transmission when DD birth or recruitment induces compensatory growth of new, healthy individuals, which has the net effect of reducing disease prevalence by dilution. We also show that if harvest is used simultaneously with vaccination, and there is high enough transmission coefficient, application of both controls may be less efficient than vaccination alone. We illustrate the effects of these control approaches on disease prevalence for chronic wasting disease in deer where the disease is transmitted directly among deer and through the environment.

  3. 基于Fabry-Perot干涉测量技术的大坝安全监测系统探究%Research on Monitoring System for Dam Safety Based on Fabry-Perot Interferometry Technology

    Institute of Scientific and Technical Information of China (English)

    周柏兵; 朱劭宇; 谢福临; 王振平

    2006-01-01

    Fabry-Perot干涉测量技术的原理及基于该技术的光纤传感系统作了介绍,分析了Fabry-Perot干涉测量技术原理并推导了有关计算公式,展望此类技术在大坝安全监测中的应用.

  4. Measurements of the phase shift on reflection for low-order infrared Fabry-Perot interferometer dielectric stack mirrors.

    Science.gov (United States)

    Mielke, S L; Ryan, R E; Hilgeman, T; Lesyna, L; Madonna, R G; Van Nostrand, W C

    1997-11-01

    A simple technique based on a Fizeau interferometer to measure the absolute phase shift on reflection for a Fabry-Perot interferometer dielectric stack mirror is described. Excellent agreement between the measured and predicted phase shift on reflection was found. Also described are the salient features of low-order Fabry-Perot interferometers and the demonstration of a near ideal low-order (1-10) Fabry-Perot interferometer through minimizing the phase dispersion on reflection of the dielectric stack. This near ideal performance of a low-order Fabry-Perot interferometer should enable several applications such as compact spectral imagers for solid and gas detection. The large free spectral range of such systems combined with an active control system will also allow simple interactive tuning of wavelength agile laser sources such as CO(2) lasers, external cavity diode lasers, and optical parametric oscillators.

  5. Simultaneous measurement of pressure and temperature by employing Fabry-Perot interferometer based on pendant polymer droplet.

    Science.gov (United States)

    Sun, Bing; Wang, Yiping; Qu, Junle; Liao, Changrui; Yin, Guolu; He, Jun; Zhou, Jiangtao; Tang, Jian; Liu, Shen; Li, Zhengyong; Liu, Yingjie

    2015-02-01

    We investigated a novel and ultracompact polymer-capped Fabry-Perot interferometer, which is based on a polymer capped on the endface of a single mode fiber (SMF). The proposed Fabry-Perot interferometer has advantages of easy fabrication, low cost, and high sensitivity. The variation of the Fabry-Perot cavity length can be easily controlled by using the motors of a normal arc fusion splicer. Moreover, the enhanced mechanical strength of the Fabry-Perot interferometer makes it suitable for high sensitivity pressure and temperature sensing in harsh environments. The proposed interferometer exhibits a wavelength shift of the interference fringes that corresponds to a temperature sensitivity of 249 pm/°C and a pressure sensitivity of 1130 pm/MPa, respectively, around the wavelength of 1560 nm.

  6. Recent Progress in Multiparameter Measurement Based on Extrinsic Fiber-Optic Fabry-Perot Interferometers and Fiber Gratings

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    This paper presents a review of recent progress in simultaneous measurement of multiparameters including strain, temperature, vibration, transverse load, based on the combinations of extrinsic fiber-optic Fabry-Perot interferometers and fiber gratings.

  7. Determination of Absolute Plate Spacing for the Fabry-Perot Subassembly of the Thermosphere Imager for Global Observations Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Task 1: rad test AD7746 component for failure dosage for use in GEO Task 2: Algorithm for determining absolute plate spacing of the TIGO Fabry-Perot plates,...

  8. Identification of the biocontrol bacterial strain FD6 and antimicrobial study of this bacterium against tomato grey mould pathogen Botrytis cinerea%生防细菌FD6的鉴定及其对番茄灰霉病菌的作用机制

    Institute of Scientific and Technical Information of China (English)

    常琳; 李倩; 童蕴慧; 徐敬友; 张清霞

    2011-01-01

    Antagonistic bacterium FD6 was isolated from the canola rhizosphere in Fujian Province.In order to define the inhibitory mechanism and ability of the strain FD6 to Botrytis cinerea,the causal agent of grey mould on tomato,was detected using microscope concave slides method and on detached tomato leaves.The strain FD6 was identified based on 16S rDNA BLAST,the analysis of physiological and biochemical characterization.The antibiotics production of bacterial strain FD6 was analyzed by PCR amplification,thin layer chromatography(TLC) and TLC-bioautography.The results showed that the bacterial culture of strain FD6 significantly inhibited the spore germination with an inhibition rate of 99% for B.cinerea and 59.7% efficiency to control grey mould on detached tomato leaves,whereas the cultural filtrate of the strain FD6 only showed a low inhibition rate of 31%.The 16S rDNA sequence of the strain FD6 shared the 99% similarities with the members in Pseudomonas and the minimal genetic distance with Pseudomonas fluorescnes.Combined with the physiological and biochemical characteristics,the strain FD6 was identified as P.fluorescens.Strain FD6 produced multiple antifungal factors,including pyrrolnitrin,2,4-diacetylphloroglucinol,pyoluterorin,siderphore,hydrogen cyanide and extracellular proteinase,but not phenazine-1-carboxylic acid.The pyrrolnitrin inhibited directly the spore germination and mycelial growth of B.cinerea.%拮抗细菌FD6分离自福建闽侯青口青菜根围土壤,采用凹玻片法和离体叶片接种法测定菌株FD6的抑菌能力,经16S rDNA序列比对和相关生理生化性状分析,对生防细菌FD6进行了鉴定,并通过PCR扩增、薄层层析、薄层色谱生物自显影等探讨FD6产生抗生素的种类及其抑菌效果。结果表明,FD6的细菌悬浮液可显著抑制灰霉病菌分生孢子萌发,抑制率达99%,FD6培养滤液的抑制率仅为31%;细菌悬浮液对番茄灰霉病防效达59.7%。FD6的16S rDNA序列与

  9. Nanocrystallization of the Fd3m Ti2Ni-type phase in Hf-based metallic glasses.

    Science.gov (United States)

    Louzguine, D V; Ko, M S; Ranganathan, S; Inoue, A

    2001-06-01

    Three ternary and four quaternary hafnium-based alloys have been rapidly solidified, and the devitrification of the resultant metallic glasses has been studied to evaluate the influence of composition on the products. The formation of metastable and stable Fd3m (Pearson symbol cF96) Ti2Ni was evident whenever the alloy composition in the stable equilibrium diagrams showed this phase. The replacement of nickel by iron led to the appearance of this phase in preference to the icosahedral quasicrystal. Several common features of the amorphous alloys that form either nanoscale icosahedral or cF96 Ti2Ni-type phases on devitrification are discussed and summarized.

  10. A [2Fe-2S] ferredoxin (FdVI) is essential for growth of the photosynthetic bacterium Rhodobacter capsulatus.

    OpenAIRE

    Armengaud, J.; Meyer, C.; Jouanneau, Y

    1997-01-01

    The physiological function of Rhodobacter capsulatus FdVI, a [2Fe-2S] ferredoxin, was investigated by the cloning, sequence analysis, and mutagenesis of its structural gene, called fdxE. The DNA region surrounding fdxE was mapped, and the nucleotide sequence of a 4.2-kb fragment was determined. fdxE is preceded by a sequence that is very similar to a sigma54 recognition site and is followed by a putative transcription stop signal, suggesting that fdxE forms a separate cistron. Two open readin...

  11. The F/D ratio and meson-baryon couplings from QCD sum rules II 13.75.Gx; 12.38.Lg; 11.55.Hx; QCD sum rules; Meson-baryon couplings; SU(3); F/D ratio

    CERN Document Server

    Kim, H; Oka, M; Lee, S H

    2000-01-01

    Using QCD sum rules, we compute the diagonal meson-baryon couplings, pi NN , eta NN , pi XI XI , eta XI XI , pi SIGMA SIGMA and eta SIGMA SIGMA , from the baryon-baryon correlation function with a meson: i integral d sup 4 x e sup i sup q supcentre dot sup x . The calculations are performed to leading order in p submu by considering the two separate Dirac structures, i gamma sub 5 gamma submu p supmu and gamma sub 5 sigma submu subnu q supmu p supnu separately. We first improve the previous sum rule calculations on these Dirac structures for the pi NN coupling by including three-particle pion wave functions of twist 4 and then extend the formalism to calculate the other couplings, eta NN , pi XI XI , eta XI XI , pi SIGMA SIGMA and eta SIGMA SIGMA . In the SU(3) symmetric limit, we identify the terms responsible for the F/D ratio in the OPE by matching the obtained couplings with their SU(3) relations. Depending on the Dirac structure considered, we find different identifications for the F/D ratio. The coupli...

  12. Near surface geophysics: application of FD-EMI sounding to the study of historical resources

    Science.gov (United States)

    Manstein, Yu. A.; Manstein, A. K.; Scozzari, A.

    2009-04-01

    Sounding with alternating electromagnetic fields has gained a growing attention and a broad usage during the last three decades, including Frequency Domain Electromagnetic Induction (FD-EMI) sounding methods. A portable electromagnetic sensor (EMS-NEMFIS), developed at IPGG (Siberian Branch of the Russian Academy of Sciences), which is based on such principle, is described in this work. Among the various application areas of such an instrument, focus in this work is given to the near-surface investigation of historical resources. The description of the device goes through the following steps: - Architecture of the instrument - Signal extraction principle - Tests and characterization Then, experiences made by using frequency-domain EMI soundings for geophysical applications in archaeology are presented, in order to assess the capability of the approach in such operative framework. In particular, case studies from the South Altay mountains and from Siberia have been selected to be shown in this context. The burial mounds of Pazyryk culture, dated 2500-3000 B.C., can be found over the wide area of South Altay mountains in Russia, Mongolia and China. This nomadic civilization belongs to the group of Mediterranean cultures. These people stayed in the Altay mountains for quite a short time - just a couple of centuries. Maybe they escaped from Europe due to Alexander Makedonsky wars or to some other unknown reason, and then went back to Europe. They burial mounds were kept safe because the wooden funeral cameras were buried into permafrost. However, recently, due to global warming, some of those cameras were melt, leading to a decay process. The information about presence of the ice lens inside of the mound is vital for decision to excavate the mound or not. Dozens of such a mounds were explored using NEMFIS during the years 2005 - 2007. Estimation of presence of the ice in some of them helped to find few good conserved burial cameras and safe a lot of resources for

  13. Photonic crystal fibre Brillouin laser based on Bragg grating Fabry-Perot cavity

    Institute of Scientific and Technical Information of China (English)

    Geng Dan; Yang Dong-Xiao; Shen Guo-Feng; Zhang Xian-Min

    2008-01-01

    A photonic crystal fibre Brillouin laser based on fibre Bragg grating Fabry-Perot cavity is presented. A highly nonlinear photonic crystal fibre 25 m in length is used as Brillouin gain medium and fibre Bragg grating Fabry-Perot cavity is chosen in order to enhance the laser conversion efficiency and suppress the higher-order Stokes waves. The laser reaches the threshold at input power of 35 mW, and the experimental laser conversion efficiency achieves 18% of the input power of 140 mW and does not show higher-order Stokes waves. A photonic crystal fibre BriUouin laser withshorter fibre length and lower threshold is experimentally realized.

  14. Sensing Properties of a Fabry-Perot Dielectric Structure and Dimer Nanoparticles

    Directory of Open Access Journals (Sweden)

    A. Polemi

    2012-01-01

    Full Text Available We investigate the use of a Fabry-Perot dielectric structure combined with differently shaped nanoparticles for Surface Enhanced Raman Scattering. In particular, we show how an ideal two-layer Fabry-Perot configuration enhances the local surface field of silver nanoparticles positioned on the surface of the structure. We develop the concept using disc dimers and then extend the discussion to bowtie nanoparticles. The structure is excited by a single emitter, which couples to the nanoparticles through the dielectric layers, producing a wide aperture field that can be used to excite multiple dimers. We show how an array of nanoparticles can be properly arranged in order to increase the total scattering signal generated from the structure. The layered geometry produces robust field properties in between nanoparticles, making the overall sensing characteristics less sensitive to the interparticle seperation distance and incident polarization.

  15. Coherent electron transparent tunneling through a single barrier within a Fabry-Perot cavity

    Science.gov (United States)

    Stolle, Jason; Baum, Chaz; Amann, Ryan; Haman, Ryan; Call, Tanner; Li, Wei

    2016-07-01

    Electromagnetic wave and quantum DeBroglie wave have many parallels between each other. We investigate the quantum mechanical counterpart of electromagnetic resonant tunneling through a non-absorbing metal layer. It is confirmed that an electron also has transparent transmission through a single barrier within a Fabry-Perot like cavity. This tunneling structure is actually a distortion of the Fabry-Perot echelon. We find that for a specific resonant electron energy, the cavity length is related to the electron's DeBroglie wavelength; and the single barrier can be located at a series positions with an interval equal to a half of the DeBroglie wavelength, not just at the center of the cavity. This tunneling phenomenon will have novel applications in quantum devices such as the resonant tunneling diode and scanning tunneling microscope. The results of this paper should also have impact on related electromagnetic research and application.

  16. Design and Fabrication of Integrated Fabry-Perot Type Color Reflector for Reflective Displays.

    Science.gov (United States)

    Cho, Seong M; Cheon, Sang Hoon; Kim, Tae-Youb; Ah, Chil Seong; Song, Juhee; Ryu, Hojun; Chu, Hye Yong

    2016-05-01

    A Fabry-Perot type integrated color reflector, with red/blue/green colors as subpixels, was designed and fabricated with Si substrate. Ag films were used as reflective mirror layers, SiO2 films were used as Fabry-Perot cavity layers and W films were used as partially reflective layers for the cavity. To minimize the effects of the thickness variation of the oxide cavity layers, the structure of the color reflector was optimized, and the differential deposition scheme was devised and applied in the fabrication process. The integrated color reflector was successfully fabricated with the proposed fabrication scheme. The measured white reflectance was > 45% in the visible spectrum range and -49% at 550 nm wavelength. The fabricated reflector had moderate color gamut of 17% of the National Television System Committee (NTSC) standard and it showed very high white reflectivity. The fabricated color reflector is expected to be applicable to reflective displays.

  17. Reflectivity enhanced refractive index sensor based on a fiber-integrated Fabry-Perot microresonator.

    Science.gov (United States)

    Wieduwilt, T; Dellith, J; Talkenberg, F; Bartelt, H; Schmidt, M A

    2014-10-20

    We discuss a fiber-integrated refractive index sensor with strongly improved detection performance. The resonator has been implemented by means of focused-ion beam milling of a step index fiber and shows a sensitivity of about 1.15µm/RIU. Coating the resonator walls led to a strongly improved mirror reflectivity by a factor of about 26. Design rules for device optimization and a detailed mathematical analysis are discussed, revealing that the sensor operates as an optimized Fabry-Perot resonator. We also show that the performance of such kind of Fabry-Perot sensors is, in general, limited by the detection limit function - a quantity depending on the cavitiy's finesse and on the measurement capabilities used.

  18. Silicon-based on-chip electrically tunable sidewall Bragg grating Fabry-Perot filter.

    Science.gov (United States)

    Zhang, Weifeng; Ehteshami, Nasrin; Liu, Weilin; Yao, Jianping

    2015-07-01

    We report the design, fabrication, and testing of a silicon-based on-chip electrically tunable sidewall Bragg grating Fabry-Perot filter. Spectral measurement shows that the filter has a narrow notch in reflection of approximately 46 pm, a Q-factor of 33,500, and an extinction ratio of 16.4 dB. DC measurement shows that the average central wavelength shift rates with forward and reverse bias are -1.15  nm/V and 4.2  pm/V, respectively. Due to strong light confinement in the Fabry-Perot cavity, the electro-optic frequency response shows that the filter has a 3-dB modulation bandwidth of ∼5.6  GHz. The performance of using the filter to perform modulation of a 3.5  Gb/s2(7)-1 nonreturn-to-zero pseudorandom binary sequence is evaluated.

  19. Resolution limits of extrinsic Fabry-Perot interferometric displacement sensors utilizing wavelength scanning interrogation.

    Science.gov (United States)

    Ushakov, Nikolai; Liokumovich, Leonid

    2014-08-10

    The factors limiting the resolution of displacement sensors based on the extrinsic Fabry-Perot interferometer were studied. An analytical model giving the dependency of extrinsic Fabry-Perot interferometric (EFPI) resolution on the parameters of an optical setup and a sensor interrogator was developed. The proposed model enables one to either estimate the limit of possible resolution achievable with a given setup, or derive the requirements for optical elements and/or a sensor interrogator necessary for attaining the desired sensor resolution. An experiment supporting the analytical derivations was performed, demonstrating a large dynamic measurement range (with cavity length from tens of microns to 5 mm), a high baseline resolution (from 14 pm), and good agreement with the model.

  20. Analyzing the temperature sensitivity of Fabry-Perot sensor using multilayer graphene diaphragm.

    Science.gov (United States)

    Li, Cheng; Liu, Qianwen; Peng, Xiaobin; Fan, Shangchun

    2015-10-19

    A miniature Fabry-Perot interferometric sensor with an ultra-high temperature sensitivity was constructed by using an approximate 8-layer graphene diaphragm. The extremely thin diaphragm was transferred onto the endface of a ferrule with an inner diameter of 125 μm, and van der Waals interactions between the graphene diaphragm and its substrate created a low finesse Fabry-Perot interferometer with a cavity length of 42.86 μm. Temperature testing demonstrated a temperature-induced cavity length change of 352 nm/°C with a good linearity in the range of 20-60 °C. The result conformed well to the proposed analytical models relating to thermal expansion of trapped gas, thermal-optical property of graphene diaphragm and deflection behavior of bulged graphene blister. However, the ultra-thin diaphragm exhibited a small deflection deformation characteristic due to the applied higher loads.

  1. 两种提取Fabry-Perot干涉条纹圆心的新方法

    Institute of Scientific and Technical Information of China (English)

    陈龙; 黎玉雯; 朱昭华

    2014-01-01

    本文介绍两种提取Fabry-Perot干涉条纹圆心点坐标的新方法。首先对干涉图像依次进行二值化处理,对所得到的条纹强度曲线进行均平滤波和自适应滤波,根据条纹灰度值强度余弦函数分布的特点,对条纹灰度值数据进行最小二乘法拟合,获得条纹强度峰值坐标,然后通过本文介绍的算法,进而获得Fabry-Perot干涉条纹圆心点的坐标。

  2. Manipulating the optical bistability at terahertz frequency in the Fabry-Perot cavity with graphene.

    Science.gov (United States)

    Jiang, Leyong; Guo, Jun; Wu, Leiming; Dai, Xiaoyu; Xiang, Yuanjiang

    2015-11-30

    We investigate theoretically the optical bistability from a Fabry-Perot cavity with graphene in the terahertz (THz) frequency. It is demonstrated that the optical bistablility in this cavity can be realized due to the electric field enhancement and the giant third-order nonlinear conductivity of graphene. The optical bistable behavior is strongly dependent on the transmission amplitude of the mirror and the position of the graphene in the cavity. It is especially important that the hysterical behaviors of the transmitted light rely on the optical conductivity of graphene, making the Fabry-Perot cavity to be a good candidate for dynamic tunable optical bistable device in the THz frequencies, owing to the possibility of high tunability of graphene conductivity by means of external electrostatic or magnetostatic field.

  3. On-Chip High-Finesse Fabry-Perot Microcavities for Optical Sensing and Quantum Information

    Directory of Open Access Journals (Sweden)

    Mohammad H. Bitarafan

    2017-07-01

    Full Text Available For applications in sensing and cavity-based quantum computing and metrology, open-access Fabry-Perot cavities—with an air or vacuum gap between a pair of high reflectance mirrors—offer important advantages compared to other types of microcavities. For example, they are inherently tunable using MEMS-based actuation strategies, and they enable atomic emitters or target analytes to be located at high field regions of the optical mode. Integration of curved-mirror Fabry-Perot cavities on chips containing electronic, optoelectronic, and optomechanical elements is a topic of emerging importance. Micro-fabrication techniques can be used to create mirrors with small radius-of-curvature, which is a prerequisite for cavities to support stable, small-volume modes. We review recent progress towards chip-based implementation of such cavities, and highlight their potential to address applications in sensing and cavity quantum electrodynamics.

  4. All-reflective Michelson, Sagnac, and Fabry-Perot interferometers based on grating beam splitters.

    Science.gov (United States)

    Sun, K X; Byer, R L

    1998-04-15

    All-reflective Michelson, Sagnac, and Fabry-Perot interferometers based on grating beam splitters are experimentally demonstrated at a wavelength of 1064 nm. A 1200-groove/mm grating diffracting 0 and -1 orders with an efficiency of 48.2% for each order was used as a near-50/50 beam splitter. The all-reflective Sagnac and Michelson interferometers were formed by reintroducing both of the diffracted beams back to the grating. The Fabry-Perot interferometer was formed in a Littrow configuration by using a 1700-groove/mm grating with a blazing efficiency of 91% as a cavity coupler. These interferometers encompass all the fundamental configurations of all-reflective laser interferometric gravitational-wave detectors, promising improved wave-front quality by avoiding volume thermal effects in transmissive optics under high-power laser illumination.

  5. Optical Analog-to-digital Conversion Scheme Based on Tunable Fabry-Perot Resonator

    Institute of Scientific and Technical Information of China (English)

    LI Zheng

    2007-01-01

    Proposed is an interference type of optical analog-to-digital conversion(ADC). The refractive index of Fabry-Perot cavity changes with different voltages. The Fabry-Perot resonator converts electronic intensity into light wavelength through selecting lights of different wavelengthes. The parameters of the scheme are acquired with the transmission matrix of optical element and the time of steady-state light field. The maximum sampling speedes of 4-bit, 6-bit, 7-bit, 8-bit and 9-bit(ADC) are 1.695×1010 count/s, 4.33×109 count/s, 2.38×109 count/s, 1.24×109 count/s and 5.9×108 count/s, respectively.

  6. Chronology of Fabry-Perot Interferometer Fiber-Optic Sensors and Their Applications: A Review

    Directory of Open Access Journals (Sweden)

    Md. Rajibul Islam

    2014-04-01

    Full Text Available Optical fibers have been involved in the area of sensing applications for more than four decades. Moreover, interferometric optical fiber sensors have attracted broad interest for their prospective applications in sensing temperature, refractive index, strain measurement, pressure, acoustic wave, vibration, magnetic field, and voltage. During this time, numerous types of interferometers have been developed such as Fabry-Perot, Michelson, Mach-Zehnder, Sagnac Fiber, and Common-path interferometers. Fabry-Perot interferometer (FPI fiber-optic sensors have been extensively investigated for their exceedingly effective, simple fabrication as well as low cost aspects. In this study, a wide variety of FPI sensors are reviewed in terms of fabrication methods, principle of operation and their sensing applications. The chronology of the development of FPI sensors and their implementation in various applications are discussed.

  7. A hybrid Fabry-Perot/Michelson interferometer sensor using a dual asymmetric core microstructured fiber

    Science.gov (United States)

    Frazão, O.; Silva, S. F.; Viegas, J.; Baptista, J. M.; Santos, J. L.; Roy, P.

    2010-02-01

    A hybrid Fabry-Perot/Michelson interferometer sensor using a dual asymmetric core microstructured fiber is demonstrated. The hybrid interferometer presents three waves. Two parallel Fabry-Perot cavities with low finesse are formed between the splice region and the end of a dual-core microstructured fiber. A Michelson configuration is obtained by the two small cores of the microstructured fiber. The spectral response of the hybrid interferometer presents two pattern fringes with different frequencies due to the respective optical path interferometers. The hybrid interferometer was characterized in strain and temperature presenting different sensitivity coefficients for each topology. Due to these characteristics, this novel sensing head is able to measure strain and temperature, simultaneously.

  8. Temperature-independent refractometer based on fiber-optic Fabry-Perot interferometer

    Science.gov (United States)

    Li, Jiacheng; Qiao, Xueguang; Wang, Ruohui; Rong, Qiangzhou; Bao, Weijia; Shao, Zhihua; Yang, Tingting

    2016-04-01

    A miniature fiber-optic refractometer based on Fabry-Perot interferometer (FPI) has been proposed and experimentally demonstrated. The sensing head consists of a short section of photonics crystal fiber (PCF) spliced to a single mode fiber (SMF), in which the end-face of the PCF is etched to remove holey structure with hydrofluoric (HF) acid. A Fabry-Perot interference spectrum is achieved based on the reflections from the fusion splicing interface and the end-face of the core of PCF. The interference fringe is sensitive to the external refractive index (RI) with an intensity-referenced sensitivity of 358.27 dB/RIU ranging from 1.33 to 1.38. The sensor has also been implemented for the concentration measurement of λ-phage DNA solution. In addition, the dip intensity is insensitive to the ambient temperature variation, making it a good candidate for temperature-independent bio-sensing area.

  9. Active vibration suppression in a suspended Fabry-Pérot cavity.

    Science.gov (United States)

    Canuto, Enrico

    2006-07-01

    This paper is concerned with active vibration suppression in a suspended Fabry-Pérot cavity, employed as the sensor in an innovative thrust-stand, called Nanobalance. The Nanobalance aims to exploit the sensitivity of in-vacuum Fabry-Pérot interferometers to sub-nanometric displacements in order to measure thrust (active and the passive) suspended to an athermic spacer. The thruster under test is mounted on the active pendulum and an equal dummy thruster is mounted on the passive one for balancing. The objective of the paper is to suppress the beat motion centered on the mean pendulum natural frequency (10-14 Hz depending on the thruster under test) without affecting the measurement bandwidth (2 Hz) where thrust has to be measured. Beat motion arises because of small pendulum imbalances excited by ground noise. Relevant digital control strategies and experimental results will be presented and discussed.

  10. Understanding the concept of resolving power in the Fabry-Perot interferometer using a digital simulation

    Energy Technology Data Exchange (ETDEWEB)

    Juvells, I [Departament de Fisica Aplicada i Optica, Universitat de Barcelona, Diagonal 647, 08028 Barcelona (Spain); Carnicer, A [Departament de Fisica Aplicada i Optica, Universitat de Barcelona, Diagonal 647, 08028 Barcelona (Spain); Ferre-Borrull, J [Departament d' Enginyeria Electronica, Universitat Rovira i Virgili, Electrica i Automatica. Av. Paisos Catalans 26, Campus Sescelades 43007 Tarragona (Spain); MartIn-Badosa, E [Departament de Fisica Aplicada i Optica, Universitat de Barcelona, Diagonal 647, 08028 Barcelona (Spain); Montes-Usategui, M [Departament de Fisica Aplicada i Optica, Universitat de Barcelona, Diagonal 647, 08028 Barcelona (Spain)

    2006-09-01

    The resolution concept in connection with the Fabry-Perot interferometer is difficult to understand for undergraduate students enrolled in physical optics courses. The resolution criterion proposed in textbooks for distinguishing equal intensity maxima and the deduction of the resolving power equation is formal and non-intuitive. In this paper, we study the practical meaning of the resolution criterion and resolution power using a computer simulation of a Fabry-Perot interferometer. The light source in the program has two monochromatic components, the wavelength difference being tunable by the user. The student can also adjust other physical parameters so as to obtain different simulation results. By analysing the images and graphics of the simulation, the resolving power concept becomes intuitive and understandable.

  11. The low coherence Fabry-Pérot interferometer with diamond and ZnO layers

    Science.gov (United States)

    Majchrowicz, D.; Den, W.; Hirsch, M.

    2016-09-01

    The authors present a fiber-optic Fabry-Pérot interferometer built with the application of diamond and zinc oxide (ZnO) thin layers. Thin ZnO films were deposited on the tip of a standard telecommunication single-mode optical fiber (SMF- 28) while the diamond layer was grown on the plate of silicon substrate. Investigated ZnO layers were fabricated by atomic layer deposition (ALD) and the diamond films were deposited using Microwave Plasma Enhanced Chemical Vapor Deposition (μPE CVD) system. Different thickness of layers was examined. The measurements were performed for the fiber-optic Fabry-Pérot interferometer working in the reflective mode. Spectra were registered for various thicknesses of ZnO layer and various length of the air cavity. As a light source, two superluminescent diodes (SLD) with central wavelength of 1300 nm and 1550 nm were used in measurement set-up.

  12. A VO-compatible Information System for 3D Fabry-Perot Data

    Science.gov (United States)

    Surace, C.; Jegouzot, I.; Granet, Y.; Amram, P.; Balkowski, C.; Carignan, C.; Epinat, B.; Fenouilet, T.; Marcelin, M.

    2009-09-01

    In the astronomical world, Fabry-Perot (FP) data are very specific. Using a scanning Fabry-Perot, extended line emitting targets can be observed with a large field of view, within a short spectral range (from 5 to 100 Å) and with high resolutions (from 1,000 to 20,000). Observation data cubes allow us to derive and to focus mostly on emission line flux maps, velocity maps and velocity dispersion maps. Specific tools are needed to process and analyze FP data and make results available to the community. We present a VO-compliant Information System (http://fabryperot.oamp.fr/) platform dedicated to the 3D FP data. A multi-criteria interface based on SItools 4.0 is available and Cone Search, SIAP, (SIAP2) and Characterization are being implemented. A discussion of ``Added value Tools'' to be implemented has begun. These might include projection of galaxies at high redshift, Tully Fisher diagrams, etc.

  13. Diaphragm based long cavity Fabry-Perot fiber acoustic sensor using phase generated carrier

    Science.gov (United States)

    Liu, Bin; Lin, Jie; Liu, Huan; Ma, Yuan; Yan, Lei; Jin, Peng

    2017-01-01

    A diaphragm based long cavity Fabry-Perot interferometric fiber acoustic sensor is proposed. The Fabry-Perot cavity is formed by a flat fiber facet and an ultra-thin silver diaphragm with a 6-meter long fiber inserted in the cavity. A narrow-linewidth ring-cavity erbium-doped fiber laser is applied to demodulate the sensing signal in the phase generated carrier algorithm. Experimental results have demonstrated that the phase sensitivity is about -140 dB re 1 rad/μPa at 2 kHz. The noise equivalent acoustic signal level is 60.6 μPa/Hz1/2 and the dynamic range is 65.1 dB-SPL at 2 kHz. The sensor is suitable for sensing of weak acoustic signals.

  14. Chronology of Fabry-Perot interferometer fiber-optic sensors and their applications: a review.

    Science.gov (United States)

    Islam, Md Rajibul; Ali, Muhammad Mahmood; Lai, Man-Hong; Lim, Kok-Sing; Ahmad, Harith

    2014-04-24

    Optical fibers have been involved in the area of sensing applications for more than four decades. Moreover, interferometric optical fiber sensors have attracted broad interest for their prospective applications in sensing temperature, refractive index, strain measurement, pressure, acoustic wave, vibration, magnetic field, and voltage. During this time, numerous types of interferometers have been developed such as Fabry-Perot, Michelson, Mach-Zehnder, Sagnac Fiber, and Common-path interferometers. Fabry-Perot interferometer (FPI) fiber-optic sensors have been extensively investigated for their exceedingly effective, simple fabrication as well as low cost aspects. In this study, a wide variety of FPI sensors are reviewed in terms of fabrication methods, principle of operation and their sensing applications. The chronology of the development of FPI sensors and their implementation in various applications are discussed.

  15. High Gain Slot Array with Fabry-Perot Cavity Feeding Circuit

    Directory of Open Access Journals (Sweden)

    Halim Boutayeb

    2016-01-01

    Full Text Available A new approach for designing slot arrays using a Fabry-Perot cavity for the feeding circuit is presented. The proposed array has simpler and smaller feeding circuit compared to conventional feeding networks that have multiple dividers or combiners. The dividers and combiners are usually sources of losses. In addition, the profile of the proposed array is not limited by the half-wavelength resonance condition that exists for Fabry-Perot resonator antennas based on partially reflecting surfaces. The operating frequency is not sensitive to the profile of the antenna. A small profile can be achieved without the utilization of an artificial magnetic conductor or a substrate with high dielectric constant. To validate the proposed approach, full-wave numerical results are presented at 5.8 GHz showing good impedance matching, a high gain of about 22 dB, and an efficiency of 76%.

  16. Fabry-Perot Study in the Orion Nebula (M 42: Protoplanetary Disks

    Directory of Open Access Journals (Sweden)

    Eduardo de la Fuente

    2003-01-01

    Full Text Available Se presenta un resumen de los resultados cinemáticas obtenidos del estudio Fabry-Perot en Hα de algunos proplyds en la nebulosa de Orión. Estos resultados se presentan en detalle en de la Fuente et al. (2003 a,b. Se obtienen velocidades sistémicas, tasas de perdida de masa, edad de los discos y perfiles de velocidad radial. Encontramos que la interferometria Fabry-Perot constituye una técnica efectiva para la detección de proplyds. Se discuten algunos aspectos astrobiológicos de estos resultados que ilustramos usando los proplyds 168-326, 167-317, 163-317, 158-323, 158-327 y 161-314.

  17. An analytical study on bistability of Fabry-Perot semiconductor optical amplifiers

    Science.gov (United States)

    Wang, Gang; Chen, Shuqiang; Yang, Huajun

    2016-09-01

    Optical bistabilities have been considered to be useful for sensor applications. As a typical nonlinear device, Fabry-Perot semiconductor optical amplifiers (FPSOAs) exhibit bistability under certain conditions. In this paper, the bistable characteristics in FPSOAs are investigated theoretically. Based on Adams's relationship between the incident optical intensity I in and the z-independent average intracavity intensity I av, an analytical expression of the bistable loop width in SOAs is derived. Numerical simulations confirm the accuracy of the analytical result.

  18. A SENSITIVE AND STABLE CONFOCAL FABRY-PEROT INTERFEROMETER FOR SURFACE ULTRASONIC VIBRATION DETECTION

    Institute of Scientific and Technical Information of China (English)

    DING HONG-SHENG; TONG LI-GE; CHEN GENG-HUA

    2001-01-01

    A new confocal Fabry-Pérot interferometer (CFPI) has been constructed. By using both of the conjugate rays,the sensitivity of the system was doubled. Moreover, the negative feedback control loop of a single-chip microcomputer (MCS-51) was applied to stabilize the working point at an optimum position. The system has been used in detecting the piezoelectric ultrasonic vibration on the surface of an aluminium sample.

  19. Analysis of a Piezo Electric Driver Circuit for Use in a Fabry-Perot Interferometer

    Directory of Open Access Journals (Sweden)

    Maithya J. Mutuku

    2013-09-01

    Full Text Available The design and fabrication of piezo electric driver circuit is presented and analysed. The output voltage which is a triangular wave voltage and frequency of the driver circuit were measured and set at 80 V peak to peak and an output frequency of 1 KHz. A photo detector circuit which receives the output beam from the confocal Fabry- perot interferometer (CFPI through the photodiode is as well presented

  20. Reduction of CCD observations made with a scanning Fabry--Perot interferometer. III. Wavelength scale refinement

    CERN Document Server

    Moiseev, A V

    2015-01-01

    We describe the recent modifications to the data reduction technique for observations acquired with the scanning Fabry-Perot interferometer (FPI) mounted on the 6-m telescope of the Special Astrophysical Observatory that allow the wavelength scale to be correctly computed in the case of large mutual offsets of studied objects in interferograms. Also the parameters of the scanning FPIs used in the SCORPIO-2 multimode focal reducer are considered.

  1. Fabry-Perot micro-structured polymer optical fibre sensors for opto-acoustic endoscopy

    DEFF Research Database (Denmark)

    Broadway, Christian; Gallego, Daniel; Woyessa, Getinet;

    2015-01-01

    Opto-Acoustic Endoscopy (OAE) requires sensors with a high sensitivity and small physical dimensions in order to facilitate integration into an endoscope of less than 1mm in diameter. We present fibre Bragg grating (FBG) and Fabry- Perot intrinsic fibre sensors for ultrasound detection. We presen...... and a simplification of the detection regime for an optimal interferometric OAE configuration. © (2015) COPYRIGHT Society of Photo-Optical Instrumentation Engineers (SPIE). Downloading of the abstract is permitted for personal use only....

  2. Quantum transport in bilayer graphene. Fabry-Perot interferences and proximity-induced superconductivity

    Energy Technology Data Exchange (ETDEWEB)

    Du, Renjun

    2015-10-30

    Bilayer graphene (BLG) p-n junctions made of hBN-BLG-hBN (hexagonal boron nitride) heterostructures enable ballistic transport over long distances. We investigate Fabry-Perot interferences, and detect that the bilayer-like anti-Klein tunneling transits into single-layer-like Klein tunneling when tuning the Fermi level towards the band edges. Furthermore, the proximity-induced superconductivity has been studied in these devices with Al leads.

  3. Topological Boundary States in 1D: An Effective Fabry-Perot Model

    CERN Document Server

    Levy, Eli

    2016-01-01

    We present a general and useful method to predict the existence, frequency, and spatial properties of gap states in photonic (and other) structures with a gapped spectrum. This method is established using the scattering approach. It offers a viewpoint based on a geometrical Fabry-Perot model. We demonstrate the capabilities of this model by predicting the behaviour of topological edge states in quasi-periodic structures. A proposition to use this model in Casimir physics is presented.

  4. Fabry-Perot-based Fourier-transform hyperspectral imaging allows multi-labeled fluorescence analysis.

    Science.gov (United States)

    Pisani, Marco; Zucco, Massimo

    2014-05-10

    We demonstrate the ability of our hyperspectral imaging device, based on a scanning Fabry-Perot interferometer, to obtain a single hyper-image of a sample marked with different fluorescent molecules, and to unambiguously discriminate them by observing their spectral fingerprints. An experiment carried out with cyanines, fluorescein, and quantum dots emitting in the yellow-orange region, demonstrates the feasibility of multi-labeled fluorescence microscopy without the use of multiple filter sets or dispersive means.

  5. Fiber-Tip Fabry-Perot Interferometric Sensor based on a Thin Silver Film

    Science.gov (United States)

    2012-11-01

    interferometer (FPI), named after French physicists Charles Fabry and Alfred Perot, is typically formed by two reflecting surfaces or mirrors. It has been...utilized for sensing applications for a long time 13,14 . The interference is caused by waves successively reflected between the two parallel...Film Assume the film act as a free vibrating circular plate clamped rigidly at the edge. Its lowest resonant frequency f0 is expressed as follows

  6. Low voltage logic circuits exploiting gate level dynamic body biasing in 28 nm UTBB FD-SOI

    Science.gov (United States)

    Taco, Ramiro; Levi, Itamar; Lanuzza, Marco; Fish, Alexander

    2016-03-01

    In this paper, the recently proposed gate level body bias (GLBB) technique is evaluated for low voltage logic design in state-of-the-art 28 nm ultra-thin body and box (UTBB) fully-depleted silicon-on-insulator (FD-SOI) technology. The inherent benefits of the low-granularity body-bias control, provided by the GLBB approach, are emphasized by the efficiency of forward body bias (FBB) in the FD-SOI technology. In addition, the possibility to integrate PMOS and NMOS devices into a single common well configuration allows significant area reduction, as compared to an equivalent triple well implementation. Some arithmetic circuits were designed using GLBB approach and compared to their conventional CMOS and DTMOS counterparts under different running conditions at low voltage regime. Simulation results shows that, for 300 mV of supply voltage, a 4 × 4-bit GLBB Baugh Wooley multiplier allows performance improvement of about 30% and area reduction of about 35%, while maintaining low energy consumption as compared to the conventional CMOS ⧹ DTMOS solutions. Performance and energy benefits are maintained over a wide range of process-voltage-temperature (PVT) variations.

  7. Phase behaviour in complementary DNA-coated gold nanoparticles and fd-viruses mixtures: a numerical study.

    Science.gov (United States)

    Chiappini, Massimiliano; Eiser, Erika; Sciortino, Francesco

    2017-01-01

    A new gel-forming colloidal system based on a binary mixture of fd-viruses and gold nanoparticles functionalized with complementary DNA single strands has been recently introduced. Upon quenching below the DNA melt temperature, such a system results in a highly porous gel state, that may be developed in a new functional material of tunable porosity. In order to shed light on the gelation mechanism, we introduce a model closely mimicking the experimental one and we explore via Monte Carlo simulations its equilibrium phase diagram. Specifically, we model the system as a binary mixture of hard rods and hard spheres mutually interacting via a short-range square-well attractive potential. In the experimental conditions, we find evidence of a phase separation occurring either via nucleation-and-growth or via spinodal decomposition. The spinodal decomposition leads to the formation of small clusters of bonded rods and spheres whose further diffusion and aggregation leads to the formation of a percolating network in the system. Our results are consistent with the hypothesis that the mixture of DNA-coated fd-viruses and gold nanoparticles undergoes a non-equilibrium gelation via an arrested spinodal decomposition mechanism.

  8. Polymer waveguide Fabry-Perot resonator for high-frequency ultrasound detection.

    Science.gov (United States)

    Tadayon, Mohammad Amin; Baylor, Martha-Elizabeth; Ashkenazi, Shai

    2014-12-01

    Piezoelectric technology is the backbone of most medical ultrasound imaging arrays; however, signal transduction efficiency severely deteriorates in scaling the technology to element size smaller than 0.1 mm, often required for high-frequency operation (>20 MHz). Optical sensing and generation of ultrasound has been proposed and studied as an alternative technology for implementing sub-millimeter size arrays with element size down to 10 μm. The application of thin polymer film Fabry-Perot resonators has been demonstrated for high-frequency ultrasound detection; however, their sensitivity is limited by light diffraction loss. Here, we introduce a new method to increase the sensitivity of an optical ultrasound receiver by utilizing a waveguide between the mirrors of the Fabry-Perot resonator. This approach eliminates diffraction loss from the cavity, and therefore the finesse is only limited by mirror loss and absorption. By applying this method, we have achieved noise equivalent pressure of 178 Pa over a bandwidth of 30 MHz or 0.03 Pa/Hz1/2, which is about 20-fold better than a similar device without a waveguide. The finesse of the tested Fabry-Perot resonator was around 200. This result is 5 times higher than the finesse measured in the same device outside the waveguide region.

  9. FABSOAR--A Fabry-Perot Spectrometer for Oxygen A-band Research Final Technical Report

    Energy Technology Data Exchange (ETDEWEB)

    Watchorn, Steven

    2010-09-10

    Because this was a Phase I project, it did not add extensively to the body of A-band knowledge. There was no basic research performed on that subject. The principal addition was that a mechanical and optical design for a triple-etalon Fabry-Perot interferometer (FABSOAR) capable of A-band sensing was sketched out and shown to be within readily feasible instrument fabrication parameters. The parameters for the proposed triple-etalon Fabry-Perot were shown to be very similar to existing Fabry-Perots built by Scientific Solutions. The mechanical design for the FABSOAR instrument incorporated the design of previous Scientific Solutions imagers, condensing the three three-inch-diameter etalons into a single, sturdy tube. The design allowed for the inclusion of a commercial off-the-shelf (COTS) filter wheel and a thermocooled CCD detector from Andor. The tube has supports to mount to a horizontal or vertical opticaltable surface, and was to be coupled to a Scientific Solutions pointing head at the Millstone Hill Observatory in Massachusetts for Phase II calibration and testing.

  10. Differential Radiometers Using Fabry-Perot Interferometric Technique for Remote Sensing of Greenhouse Gases

    Science.gov (United States)

    Georgieva, Elena M.; Heaps,William S.; Wilson, Emily L.

    2007-01-01

    A new type of remote sensing radiometer based upon the Fabry-Perot interferometric technique has been developed at NASA's Goddard Space Flight Center and tested from both ground and aircraft platform. The sensor uses direct or reflected sunlight and has channels for measuring column concentration of carbon dioxide at 1570 nm, oxygen lines sensitive to pressure and temperature at 762 and 768 nm, and water vapor (940 nm). A solid Fabry-Perot etalon is used as a tunable narrow bandpass filter to restrict the measurement to the gas of interest's absorption bands. By adjusting the temperature of the etalon, which changes the index of refraction of its material, the transmission fringes can be brought into nearly exact correspondence with absorption lines of the particular species. With this alignment between absorption lines and fringes, changes in the amount of a species in the atmosphere strongly affect the amount of light transmitted by the etalon and can be related to gas concentration. The technique is applicable to different chemical species. We have performed simulations and instrument design studies for CH4, "Cot isotope, and CO detection. Index Terms- Absorbing media, Atmospheric measurements, Fabry-Perot interferometers, Optical interferometry, Remote sensing.

  11. Indium phosphide all air-gap Fabry-Pérot filters for near-infrared spectroscopic applications

    Science.gov (United States)

    Ullah, A.; Butt, M. A.; Fomchenkov, S. A.; Khonina, S. N.

    2016-08-01

    Food quality can be characterized by noninvasive techniques such as spectroscopy in the Near Infrared wavelength range. For example, 930 -1450 nm wavelength range can be used to detect diseases and differentiate between meat samples. Miniaturization of such NIR spectrometers is useful for quick and mobile characterization of food samples. Spectrometers can be miniaturized, without compromising the spectral resolution, using Fabry-Pérot (FP) filters consisting of two highly reflecting mirrors with a central cavity in between. The most commonly used mirrors in the design of FP filters are Distributed Bragg Reflections (DBRs) consisting of alternating high and low refractive index material pairs, due to their high reflectivity compared to metal mirrors. However, DBRs have high reflectivity for a selected range of wavelengths known as the stopband of the DBR. This range is usually much smaller than the sensitivity range of the spectrometer detector. Therefore, a bandpass filter is usually required to restrict wavelengths outside the stopband of the FP DBRs. Such bandpass filters are difficult to design and implement. Alternatively, high index contrast materials must be can be used to broaden the stopband width of the FP DBRs. In this work, Indium phosphide all air-gap filters are proposed in conjunction with InGaAs based detectors. The designed filter has a wide stopband covering the entire InGaAs detector sensitivity range. The filter can be tuned in the 950-1450 nm with single mode operation. The designed filter can hence be used for noninvasive meat quality control.

  12. Vascular risk factors, vascular disease, lipids and lipid targets in patients with familial dysbetalipoproteinemia : A European cross-sectional study

    NARCIS (Netherlands)

    Koopal, C.; Retterstol, K.; Sjouke, B.; Hovingh, G. K.; Ros, E.; de Graaf, J.; Dullaart, R. P. F.; Bertolini, S.; Visseren, F. L. J.

    2015-01-01

    Background: Familial dysbetalipoproteinemia (FD), also known as type III hyperlipoproteinemia, is a genetic dyslipidemia characterized by elevated very low density lipoprotein (VLDL) and chylomicron remnant particles that confers increased risk of cardiovascular disease (CVD). The objective of this

  13. Research on FD-OCT structure and perimetry of primary open angle glaucoma%原发性开角型青光眼FD-OCT结构与视野损害的研究

    Institute of Scientific and Technical Information of China (English)

    乔宝笛; 戴涛; 帖彪; 杜敏

    2015-01-01

    目的 应用频域光学相关生物扫描(FD-OCT)与视野检查,研究早期发现与随访原发性开角型青光眼的敏感指标.方法 选择确诊为健康对照组25例50眼、可疑开角型青光眼组23例32眼及原发性开角型青光眼组26例39眼行FD-OCT及视野检查,测量FD-OCT各项参数及视野平均光敏感度缺损值(MD),三组组间差异比较采用单因素方差分析,三组组间两两差异比较采用q检验分析,平均视网膜神经纤维层厚度值(retinal nerval fiber layer,RNFL)与MD的关系选择Pearson相关分析.结果 经单因素方差分析,除了盘沿容积、视盘深度显示三组之间差别无统计学意义,其他参数均显示三组之间的差异有显著性意义(P<0.01).经q检验,视杯面积、盘沿面积、杯盘比、线性杯盘比、视杯容积和RNFL三组两两组间对比差异均有统计学意义(P<0.05),视盘面积和视盘宽度显示原发性开角型青光眼组与其他两组差异均有统计学意义(P<0.05).经Pearson相关分析,三组MD与RNFL均呈负的直线相关(P=0.000).结论 D-OCT可测量各项视盘参数及RNFL,结合视野测量的MD,是早期诊断和长期随访青光眼进展的重要敏感指标.

  14. The Donald W. Reynolds Consortium for Faculty Development to Advance Geriatrics Education (FD~AGE): a model for dissemination of subspecialty educational expertise.

    Science.gov (United States)

    Heflin, Mitchell T; Bragg, Elizabeth J; Fernandez, Helen; Christmas, Colleen; Osterweil, Dan; Sauvigné, Karen; Warshaw, Gregg; Cohen, Harvey Jay; Leipzig, Rosanne; Reuben, David B; Durso, Samuel C

    2012-05-01

    Most U.S. medical schools and training programs lack sufficient faculty expertise in geriatrics to train future physicians to care for the growing population of older adults. Thus, to reach clinician-educators at institutions and programs that have limited resources for enhancing geriatrics curricula, the Donald W. Reynolds Foundation launched the Faculty Development to Advance Geriatrics Education (FD~AGE) program. This consortium of four medical schools disseminates expertise in geriatrics education through support and training of clinician-educators. The authors conducted this study to measure the effects of FD~AGE. Program leaders developed a three-pronged strategy to meet program goals: FD~AGE offers (1) advanced fellowships in clinical education for geriatricians who have completed clinical training, (2) mini-fellowships and intensive courses for faculty in geriatrics, teaching skills, and curriculum development, and (3) on-site consultations to assist institutions with reviewing and redesigning geriatrics education programs. FD~AGE evaluators tracked the number and type of participants and conducted interviews and follow-up surveys to gauge effects on learners and institutions. Over six years (2004-2010), FD~AGE trained 82 fellows as clinician-educators, hosted 899 faculty scholars in mini-fellowships and intensive courses, and conducted 65 site visits. Participants taught thousands of students, developed innovative curricula, and assumed leadership roles. Participants cited as especially important to program success expanded knowledge, improved teaching skills, mentoring, and advocacy. The FD~AGE program represents a unique model for extending concentrated expertise in geriatrics education to a broad group of faculty and institutions to accelerate progress in training future physicians.

  15. Functional Inactivation of Putative Photosynthetic Electron Acceptor Ferredoxin C2 (FdC2) Induces Delayed Heading Date and Decreased Photosynthetic Rate in Rice.

    Science.gov (United States)

    Zhao, Juan; Qiu, Zhennan; Ruan, Banpu; Kang, Shujing; He, Lei; Zhang, Sen; Dong, Guojun; Hu, Jiang; Zeng, Dali; Zhang, Guangheng; Gao, Zhenyu; Ren, Deyong; Hu, Xingming; Chen, Guang; Guo, Longbiao; Qian, Qian; Zhu, Li

    2015-01-01

    Ferredoxin (Fd) protein as unique electron acceptor, involved in a variety of fundamental metabolic and signaling processes, which is indispensable for plant growth. The molecular mechanisms of Fd such as regulation of electron partitioning, impact of photosynthetic rate and involvement in the carbon fixing remain elusive in rice. Here we reported a heading date delay and yellowish leaf 1 (hdy1) mutant derived from Japonica rice cultivar "Nipponbare" subjected to EMS treatment. In the paddy field, the hdy1 mutant appeared at a significantly late heading date and had yellow-green leaves during the whole growth stage. Further investigation indicated that the abnormal phenotype of hdy1 was connected with depressed pigment content and photosynthetic rate. Genetic analysis results showed that the hdy1 mutant phenotype was caused by a single recessive nuclear gene mutation. Map-based cloning revealed that OsHDY1 is located on chromosome 3 and encodes an ortholog of the AtFdC2 gene. Complementation and overexpression, transgenic plants exhibited the mutant phenotype including head date, leaf color and the transcription levels of the FdC2 were completely rescued by transformation with OsHDY1. Real-time PCR revealed that the expression product of OsHDY1 was detected in almost all of the organs except root, whereas highest expression levels were observed in seeding new leaves. The lower expression levels of HDY1 and content of iron were detected in hdy1 than WT's. The FdC2::GFP was detected in the chloroplasts of rice. Real-time PCR results showed that the expression of many photosynthetic electron transfer related genes in hdy1 were higher than WT. Our results suggest that OsFdC2 plays an important role in photosynthetic rate and development of heading date by regulating electron transfer and chlorophyll content in rice.

  16. Functional Inactivation of Putative Photosynthetic Electron Acceptor Ferredoxin C2 (FdC2 Induces Delayed Heading Date and Decreased Photosynthetic Rate in Rice.

    Directory of Open Access Journals (Sweden)

    Juan Zhao

    Full Text Available Ferredoxin (Fd protein as unique electron acceptor, involved in a variety of fundamental metabolic and signaling processes, which is indispensable for plant growth. The molecular mechanisms of Fd such as regulation of electron partitioning, impact of photosynthetic rate and involvement in the carbon fixing remain elusive in rice. Here we reported a heading date delay and yellowish leaf 1 (hdy1 mutant derived from Japonica rice cultivar "Nipponbare" subjected to EMS treatment. In the paddy field, the hdy1 mutant appeared at a significantly late heading date and had yellow-green leaves during the whole growth stage. Further investigation indicated that the abnormal phenotype of hdy1 was connected with depressed pigment content and photosynthetic rate. Genetic analysis results showed that the hdy1 mutant phenotype was caused by a single recessive nuclear gene mutation. Map-based cloning revealed that OsHDY1 is located on chromosome 3 and encodes an ortholog of the AtFdC2 gene. Complementation and overexpression, transgenic plants exhibited the mutant phenotype including head date, leaf color and the transcription levels of the FdC2 were completely rescued by transformation with OsHDY1. Real-time PCR revealed that the expression product of OsHDY1 was detected in almost all of the organs except root, whereas highest expression levels were observed in seeding new leaves. The lower expression levels of HDY1 and content of iron were detected in hdy1 than WT's. The FdC2::GFP was detected in the chloroplasts of rice. Real-time PCR results showed that the expression of many photosynthetic electron transfer related genes in hdy1 were higher than WT. Our results suggest that OsFdC2 plays an important role in photosynthetic rate and development of heading date by regulating electron transfer and chlorophyll content in rice.

  17. Synthesis of wavelet envelope in 2-D random media having power-law spectra: comparison with FD simulations

    Science.gov (United States)

    Sato, Haruo; Fehler, Michael C.

    2016-10-01

    The envelope broadening and the peak delay of the S-wavelet of a small earthquake with increasing travel distance are results of scattering by random velocity inhomogeneities in the earth medium. As a simple mathematical model, Sato proposed a new stochastic synthesis of the scalar wavelet envelope in 3-D von Kármán type random media when the centre wavenumber of the wavelet is in the power-law spectral range of the random velocity fluctuation. The essential idea is to split the random medium spectrum into two components using the centre wavenumber as a reference: the long-scale (low-wavenumber spectral) component produces the peak delay and the envelope broadening by multiple scattering around the forward direction; the short-scale (high-wavenumber spectral) component attenuates wave amplitude by wide angle scattering. The former is calculated by the Markov approximation based on the parabolic approximation and the latter is calculated by the Born approximation. Here, we extend the theory for the envelope synthesis of a wavelet in 2-D random media, which makes it easy to compare with finite difference (FD) simulation results. The synthetic wavelet envelope is analytically written by using the random medium parameters in the angular frequency domain. For the case that the power spectral density function of the random velocity fluctuation has a steep roll-off at large wavenumbers, the envelope broadening is small and frequency independent, and scattering attenuation is weak. For the case of a small roll-off, however, the envelope broadening is large and increases with frequency, and the scattering attenuation is strong and increases with frequency. As a preliminary study, we compare synthetic wavelet envelopes with the average of FD simulation wavelet envelopes in 50 synthesized random media, which are characterized by the RMS fractional velocity fluctuation ε = 0.05, correlation scale a = 5 km and the background wave velocity V0 = 4 km s-1. We use the radiation

  18. Diversity and strain specificity of plant cell wall degrading enzymes revealed by the draft genome of Ruminococcus flavefaciens FD-1.

    Directory of Open Access Journals (Sweden)

    Margret E Berg Miller

    Full Text Available BACKGROUND: Ruminococcus flavefaciens is a predominant cellulolytic rumen bacterium, which forms a multi-enzyme cellulosome complex that could play an integral role in the ability of this bacterium to degrade plant cell wall polysaccharides. Identifying the major enzyme types involved in plant cell wall degradation is essential for gaining a better understanding of the cellulolytic capabilities of this organism as well as highlighting potential enzymes for application in improvement of livestock nutrition and for conversion of cellulosic biomass to liquid fuels. METHODOLOGY/PRINCIPAL FINDINGS: The R. flavefaciens FD-1 genome was sequenced to 29x-coverage, based on pulsed-field gel electrophoresis estimates (4.4 Mb, and assembled into 119 contigs providing 4,576,399 bp of unique sequence. As much as 87.1% of the genome encodes ORFs, tRNA, rRNAs, or repeats. The GC content was calculated at 45%. A total of 4,339 ORFs was detected with an average gene length of 918 bp. The cellulosome model for R. flavefaciens was further refined by sequence analysis, with at least 225 dockerin-containing ORFs, including previously characterized cohesin-containing scaffoldin molecules. These dockerin-containing ORFs encode a variety of catalytic modules including glycoside hydrolases (GHs, polysaccharide lyases, and carbohydrate esterases. Additionally, 56 ORFs encode proteins that contain carbohydrate-binding modules (CBMs. Functional microarray analysis of the genome revealed that 56 of the cellulosome-associated ORFs were up-regulated, 14 were down-regulated, 135 were unaffected, when R. flavefaciens FD-1 was grown on cellulose versus cellobiose. Three multi-modular xylanases (ORF01222, ORF03896, and ORF01315 exhibited the highest levels of up-regulation. CONCLUSIONS/SIGNIFICANCE: The genomic evidence indicates that R. flavefaciens FD-1 has the largest known number of fiber-degrading enzymes likely to be arranged in a cellulosome architecture. Functional

  19. Quantitative MR Markers and Psychiatric Symptoms in a Patient with Fahr Disease.

    Science.gov (United States)

    Lo Buono, Viviana; Corallo, Francesco; Costa, Antonio; Bramanti, Placido; Marino, Silvia

    2015-06-21

    Fahr's disease (FD), or primitive idiopathic calcification of the basal ganglia, is a rare neurodegenerative syndrome characterized by the presence of idiopathic bilateral and symmetrical cerebral calcifications. We describe the case of 43-year-old woman presenting with psychiatric symptoms, disorganized behavior, and migraine. Magnetic resonance imaging (MRI) examination showed basal ganglia calcifications. In addition, we analyzed the cortical brain volume and noted cortical atrophy. Extensive etiological clinico-biological assessment allowed us to exclude known causes of brain calcifications and to diagnose Fahr disease (FD). Neurological symptoms associated with psychiatric manifestations are not uncommon in FD. Purely psychiatric presentations are possible, as demonstrated by the present case, although there have been very few cases reported. To date, no studies related to the brain atrophy in FD have been reported.

  20. Production and purification of a-amylase from Aspergillus niger 33-19 CNMN FD 02A mutant form

    Directory of Open Access Journals (Sweden)

    Alexandra CILOCI

    2012-05-01

    Full Text Available From mutant micelial strain Aspergillus niger 33-19 CNMN FD 02A, through alcohol ethylic precipitation of cultural liquid, amylolytic preparation Amilonigrin AS was isolated with 10x degree of purity and a specific activity of 138.3U/mg proteins. α-Amylase from 20mM Tris-HCl extract of Amilonigrin AS was purified to homogeneity by PD-10 column gel filtration and HiTrap TM Q column ion exchange chromatography. A trial for the purification of α-amylase resulted in an enzyme specific activity of 199.68U/mg protein with purification fold 8.9. The analyses of purified α-amylase for molecular weight was carried out by SDSPAGE electrophoresis, with revealed two polypeptide bands estimated to be 66 and 40.5kDa, probably being two α-amylaseizoforms.

  1. Reconfigurable ultra-thin film GDNMOS device for ESD protection in 28 nm FD-SOI technology

    Science.gov (United States)

    Athanasiou, Sotirios; Legrand, Charles-Alexandre; Cristoloveanu, Sorin; Galy, Philippe

    2017-02-01

    We propose a novel ESD protection device (GDNMOS: Gated Diode merged NMOS) fabricated with 28 nm UTBB FD-SOI high-k metal gate technology. By modifying the combination of the diode and transistor gate stacks, the robustness of the device is optimized, achieving a maximum breakdown voltage (VBR) of 4.9 V. In addition, modifications of the gate length modulate the trigger voltage (Vt1) with a minimum value of 3.5 V. Variable electrostatic doping (gate-induced) in diode and transistor body enables reconfigurable operation. A lower doping of the base enhances the bipolar gain, leading to thyristor behavior. This innovative architecture demonstrates excellent capability for high-voltage protection while maintaining a latch-up free behavior.

  2. Abundance and diversity of dockerin-containing proteins in the fiber-degrading rumen bacterium, Ruminococcus flavefaciens FD-1.

    Directory of Open Access Journals (Sweden)

    Marco T Rincon

    Full Text Available BACKGROUND: The cellulosome is a multi-enzyme machine, which plays a key role in the breakdown of plant cell walls in many anaerobic cellulose-degrading microorganisms. Ruminococcus flavefaciens FD-1, a major fiber-degrading bacterium present in the gut of herbivores, has the most intricate cellulosomal organization thus far described. Cellulosome complexes are assembled through high-affinity cohesin-dockerin interactions. More than two-hundred dockerin-containing proteins have been identified in the R. flavefaciens genome, yet the reason for the expansion of these crucial cellulosomal components is yet unknown. METHODOLOGY/PRINCIPAL FINDINGS: We have explored the full spectrum of 222 dockerin-containing proteins potentially involved in the assembly of cellulosome-like complexes of R. flavefaciens. Bioinformatic analysis of the various dockerin modules showed distinctive conservation patterns within their two Ca(2+-binding repeats and their flanking regions. Thus, we established the conceptual framework for six major groups of dockerin types, according to their unique sequence features. Within this framework, the modular architecture of the parent proteins, some of which are multi-functional proteins, was evaluated together with their gene expression levels. Specific dockerin types were found to be associated with selected groups of functional components, such as carbohydrate-binding modules, numerous peptidases, and/or carbohydrate-active enzymes. In addition, members of other dockerin groups were linked to structural proteins, e.g., cohesin-containing proteins, belonging to the scaffoldins. CONCLUSIONS/SIGNIFICANCE: This report profiles the abundance and sequence diversity of the R. flavefaciens FD-1 dockerins, and provides the molecular basis for future understanding of the potential for a wide array of cohesin-dockerin specificities. Conserved differences between dockerins may be reflected in their stability, function or expression within

  3. Micromechanical tunable Fabry-Perot filter for IR gas analysis; Mikromechanisches durchstimmbares Fabry-Perot-Filter fuer die IR-Gasanalytik

    Energy Technology Data Exchange (ETDEWEB)

    Neumann, N.; Heinze, M.; Stegbauer, H.J. [InfraTec GmbH, Dresden (Germany); Hiller, K. [Technische Univ. Chemnitz (Germany). Zentrum fuer Mikrotechnologien; Kurth, S. [FhG-Inst. fuer Zuverlaessigkeit und Mikrointegration, Chemnitz (Germany). Aussenstelle Micro Devices and Equipment

    2005-01-01

    This contribution deals with design, fabrication and test of a micro-machined first order Fabry-Perot interferometer (FPI) intended for use as a tunable infrared (IR) filter in the (3-5) {mu}m band. The technical approach minimizes mirror curvature by using 300 {mu}m thick silicon (Si) mirror carriers for the coplanar fixed and movable mirrors of the FPI. Dielectric quarter-wave reflectance stacks consisting of alternating layers of silicon dioxide (SiO{sub 2}) and polycrystalline silicon are used as mirrors. The moveable mirror consists of two parts to form a parallel spring suspension which provides the necessary vertical movement and minimizes any tilting of the movable mirror carrier. The cavity spacing is electrostatically driven and controlled. Tunable filters with a centre wavelength (CWL) of (4.5..3.0) {mu}m and a spectral bandwith (FWHM) of about 60 nm were tested and showed a peak transmittance of about 50%. (orig.)

  4. 光纤Fabry-Perot干涉仪在传感应用中的数学模型%MATHEMATICAL MODELS FOR FIBER-OPTICAL FABRY-PEROT INTERFEROMETRIC CAVITY

    Institute of Scientific and Technical Information of China (English)

    毕卫红

    1999-01-01

    本文应用光学和数学理论导出光纤Fabry-Perot干涉仪在两反射面反射率不同时的反射光与透射光的数学模型、低反射率Fabry-Perot干涉腔长度的变化与干涉光光强的数学模型及传感中的应用;为这种光纤干涉仪的准确使用提供基础.

  5. 用LED作光源的Fabry-Perot光纤甲烷气体传感器的研究%Study on Fabry-Perot CH4 Optical Fiber Sensor with LED

    Institute of Scientific and Technical Information of China (English)

    王玉田; 郭增军; 王莉田

    2002-01-01

    利用Fabry-Perot腔的选频特性,考虑与光纤的低损耗窗口相一致和价格等因素,采用价廉的1.3 μm波段的LED作为光源,实现了PZT对Fabry-Perot腔的正弦调制.对甲烷气体浓度进行谐波检测,检测灵敏度可达10×10-6,大大提高了灵敏度和稳定性.

  6. Gain-guided index-antiguided fiber with a Fabry-Perot layer for large mode area laser amplifiers.

    Science.gov (United States)

    Lai, Chih-Hsien; Chen, Hsuan-Yu; Du, Cheng-Han; Chiou, Yih-Peng

    2015-02-23

    We propose a modified gain-guided index-antiguided (GGIAG) fiber structure for large mode area laser amplifiers, in which a thin dielectric layer is placed between the low-index core and the high-index cladding. The introduced dielectric layer functions as a Fabry-Perot etalon. By letting the resonant wavelength of the Fabry-Perot layer coincide with the signal wavelength, the signal is gain-guided in the fiber core. Moreover, the pump is confined in the low-index core owing to the antiresonant reflection originated from the Fabry-Perot layer. Numerical results indicate that the leakage loss of the pump can be minified over two orders of magnitude in the proposed structure, and thus the end-pumping efficiency could be enhanced significantly.

  7. Double Michelson/Fabry-Perot interferometer for laser- and displacement-noise-free gravitational-wave detection

    CERN Document Server

    Tarabrin, Sergey P

    2009-01-01

    In this paper we demonstrate that a double Michelson interferometer with Fabry-Perot cavities in its arms is able to perform laser- and displacement-noise-free gravitational-wave (GW) detection if certain model assumptions are met. Assuming the input mirrors of a single Michelson/Fabry-Perot interferometer can be rigidly attached to beamsplitter on a central platform one can manipulate with interferometer's response signals in a way to cancel laser noise and displacement noise of all test masses except the cental platform. A pair of symmetrically positioned Michelson/Fabry-Perot interferometers with common central platform can be made insusceptible to the later then, thus allowing complete laser- and displacement-noise-free interferometry (DFI). It is demonstrated that the DFI response to GWs of the proposed interferometer is proportional to $f^2_{\\textrm{gw}}/\\gamma$, where $\\gamma$ is the cavity half-bandwidth, that is the strongest DFI response allowed by general relativity.

  8. Fabry-perot modes enhanced pump-probe coupling in gold micro-disk patterned ruby thin film

    Science.gov (United States)

    Kumari, Satchi; Khare, Alika; Gupta, Reema; Tomar, Monika; Gupta, Vinay

    2017-10-01

    Enhanced pump-probe coupling has been experimentally observed in epitaxial Ruby thin film patterned with equidistant gold micro-disks (∼100 μm), as compared to pure Ruby film. This has been attributed to Fabry-Perot and surface plasmon modes in Ruby/gold film. In case of S polarized pump and probe beam, Fabry-Perot modes leads to a two-wave mixing gain of ∼1.35. Moreover gain was ∼1.62 in P-polarization case, it has been attributed to coupling of Fabry-Perot and surface plasmon modes. Enhanced coupling for P-polarization can lead to improved nonlinear response in the thin film geometry. It can find applications in thin film based compact photonic devices.

  9. Dynamic model and stability analysis of a laser using a nonlinear Fabry-Perot etalon as a cavity mirror

    Energy Technology Data Exchange (ETDEWEB)

    Li, S.; Pons, R. (Autonoma de Barcelona (Spain). Dept. of Fisica); Zhang, Y. (Chongqing Inst. of Posts and Telecommunications, Sichuan (China). Telecommunications Engineering Dept.)

    1994-08-01

    In this paper, the authors study a laser using a nonlinear Fabry-Perot etalon as a cavity mirror. First, using the semiclassical laser theory and the differential equation for the lossy nonlinear Fabry-Perot etalon, they develop dynamic equations describing this system for single-mode operation. In this model, the frequency-pulling effect, a finite response time of the nonlinear medium, and a finite-cavity round-trip time of the Fabry-Perot etalon are included. Second, based on this model, they analyze the stability of this laser and give some numerical results. The results show that (1) this system can exist in the stable state and in the unstable state; (2) there are not only saddle-node bifurcations but also Hopf bifurcations; (3) the detuning parameter will effect the characteristics of the bistability and the number and distribution of Hopf bifurcation points.

  10. Guía para el diagnóstico, seguimiento y tratamiento de la enfermedad de Fabry

    Directory of Open Access Journals (Sweden)

    Pablo Neumann

    2013-10-01

    Full Text Available La enfermedad de Fabry es un trastorno de almacenamiento lisosomal hereditario ligado al cromosoma X, ocasionado por el déficit de la enzima alfa galactosidasa A. El conocimiento sobre esta patología, y en particular su manejo médico, ha progresado notablemente en la última década, incluyendo el desarrollo de su tratamiento específico. La presente guía fue desarrollada por profesionales médicos de diversas especialidades involucrados en la atención de pacientes con enfermedad de Fabry. La discusión y análisis de las evidencias científicas disponibles, sumado a la experiencia de cada uno de los participantes, ha permitido desarrollar los conceptos vertidos en esta guía con el objetivo de brindar una herramienta útil para todos los profesionales que asisten a pacientes con enfermedad de Fabry.

  11. An arc tangent function demodulation method of fiber-optic Fabry-Perot high-temperature pressure sensor

    Science.gov (United States)

    Ren, Qianyu; Li, Junhong; Hong, Yingping; Jia, Pinggang; Xiong, Jijun

    2017-09-01

    A new demodulation algorithm of the fiber-optic Fabry-Perot cavity length based on the phase generated carrier (PGC) is proposed in this paper, which can be applied in the high-temperature pressure sensor. This new algorithm based on arc tangent function outputs two orthogonal signals by utilizing an optical system, which is designed based on the field-programmable gate array (FPGA) to overcome the range limit of the original PGC arc tangent function demodulation algorithm. The simulation and analysis are also carried on. According to the analysis of demodulation speed and precision, the simulation of different numbers of sampling points, and measurement results of the pressure sensor, the arc tangent function demodulation method has good demodulation results: 1 MHz processing speed of single data and less than 1% error showing practical feasibility in the fiber-optic Fabry-Perot cavity length demodulation of the Fabry-Perot high-temperature pressure sensor.

  12. Determination of refractive index dispersion using fiber-optic low-coherence Fabry-Perot interferometer: implementation and validation

    Science.gov (United States)

    Karpienko, Katarzyna; Wróbel, Maciej S.; Jędrzejewska-Szczerska, Małgorzata

    2014-07-01

    We present the implementation and validation of low-coherence Fabry-Perot interferometer for refractive index dispersion measurements of liquids. A measurement system has been created with the use of four superluminescent diodes with different optical parameters, a fiber-optic coupler and an optical spectrum analyzer. The Fabry-Perot interferometer cavity has been formed by the fiber-optic end and mirror surfaces mounted on a micromechanical stage. The positive result of the validation procedure has been determined through statistical analysis. All obtained results were 99.999% statistically significant and were characterized by a strong positive correlation (r>0.98). The accuracy of the measured result of implemented low-coherence Fabry-Perot interferometer sensor is from 83% to 94%, which proves that the sensor can be used in the measurement of refractive index dispersion of liquids.

  13. Superluminescent diode versus Fabry-Perot laser diode seeding in pulsed MOPA fiber laser systems for SBS suppression

    Science.gov (United States)

    Melo, M.; Sousa, J. M.; Salcedo, J. R.

    2015-03-01

    We demonstrate the use of a pulsed superluminescent diode (SLD) through direct current injection modulation as seeding source in a master oscillator power amplifier (MOPA) configuration when compared to a Fabry-Perot (FP) laser diode in the same system. The performance limitations imposed by the use of the Fabry-Perot lasers, caused by the backward high peak power pulses triggered due to stimulated Brillouin scattering (SBS) are not observed in the case of the SLD. Compared to conventional Fabry-Perot laser diodes, the SLD provides a smooth and broad output spectrum which is independent of the input pulse parameters. Moreover, the spectrum can be sliced and tailored to the application. Thus, free SBS operation is shown when using the SLD seeder in the same system, allowing for a significant increase on the extractable power and energy.

  14. Optimization of parodontium tissue irradiation method for fluorescent diagnostic (FD) and photodynamic therapy (PDT)

    Science.gov (United States)

    Sinyaeva, Maria L.; Mamedov, Ad. A.; Lervkin, V. V.; Kharnas, Sergey S.; Volkova, Anna I.; Loschenov, Victor B.; Berezin, Anatoly N.; Kiselev, Gennady L.

    2004-06-01

    During the last several years research activities have been conducted to study PDT availability in stomatology for pathogenic microflora suppression, which presence causes different kinds of oral cavity inflammatory disease. Studies indicate that PDT can be alternative to traditional antibiotic therapy. We carried out research in order to determine the optimal method of tooth irradiation to provide necessary therapeutic radiation dose for affected parodontium tissue in case of inflammatory disease. These data can be used for fiber-optic catheter positioning for further influence on oral cavity nidus of inflammation during PDT and PD. 20 teeth ex vivo were examined. Teeth were irradiated with diode laser (680 nm). In different teeth regions laser radiation intensity was measured by LESA-01-Biospec spectrometer. Optimal radiation method for PDT and PD was found.

  15. Correlations between Endomyocardial Biopsies and Cardiac Manifestations in Taiwanese Patients with the Chinese Hotspot IVS4+919G>A Mutation: Data from the Fabry Outcome Survey

    Directory of Open Access Journals (Sweden)

    Ting-Rong Hsu

    2017-01-01

    Full Text Available We retrospectively evaluated correlations between cardiac manifestations and globotriaosylceramide (Gb3 accumulation in cardiomyocytes from Taiwanese patients with Fabry disease and the IVS4+919G>A (IVS4 mutation who underwent endomyocardial biopsy (Shire; Fabry Outcome Survey data; extracted January 2015. Of 24 males and six females (median age [Q1; Q3] at biopsy 60.4 [57.4; 64.1] and 61.3 [60.4; 65.1] years, respectively, 13 males (54.2% and five females (83.3% received agalsidase alfa enzyme replacement therapy (ERT before biopsy. Median left ventricular mass indexed to height (LVMI within ±6 months of biopsy was 65.3 (52.7; 93.1 in males and 53.2 (42.0; 55.0 g/m2.7 in females. A moderate, positive, statistically significant correlation was found between the percentage area Gb3 accumulation in cardiomyocytes and LVMI (Spearman’s ρ, 0.45; p = 0.014; a smaller, positive, non-statistically significant correlation was observed between cardiomyocyte diameter and LVMI (Spearman’s ρ 0.16, p = 0.394. Moderate, statistically significant, negative correlations were found between Gb3 accumulation and ERT duration (Spearman’s ρ, −0.49, p = 0.007 and between cardiomyocyte size and ERT duration (Spearman’s ρ, −0.37, p = 0.048. Longer ERT duration was associated with smaller amounts of Gb3 accumulation and smaller cardiomyocyte size. Further follow-up is recommended to confirm these trends in a larger sample size.

  16. Fabry-Perot CCD annular-summing spectroscopy: study and implementation for aeronomy applications.

    Science.gov (United States)

    Coakley, M M; Roesler, F L; Reynolds, R J; Nossal, S

    1996-11-20

    The technique of Fabry-Perot CCD annular-summing spectroscopy, with particular emphasis on applications in aeronomy, is discussed. Parameter choices for optimizing performance by the use of a standard format CCD array are detailed. Spectral calibration methods, techniques for determining the ring pattern center, and effects imposed by limited radial resolution caused by superpixel size, variable by on-chip binning, are demonstrated. The technique is carefully evaluated experimentally relative to the conventional scanning Fabry-Perot that uses a photomultiplier detector. We evaluate three extreme examples typical of aeronomical spectroscopy using calculated signal-to-noise ratios. Predicted sensitivity gains of 10-30 are typical. Of the cases considered, the largest savings in integration time are estimated for the day sky thermospheric O(1)D case, in which the bright sky background dominates the CCD read noise. For profile measurements of faint night sky emission lines, such as exospheric hydrogen Balmer-α, long integration times are required to achieve useful signal-to-noise ratios. In such cases, CCD read noise is largely overcome. Predictions of a factor of 10-15 savings in integration time for night sky Balmer-α observations are supported by field tests. Bright, isolated night sky lines such as thermospheric O(1)D require shorter integration times, and more modest gains dependent on signal level are predicted. For such cases it appears from estimate results that the Fabry-Perot CCD annular-summing technique with a conventional rectangular format may be outperformed by a factor of 2-5 by special CCD formats or by unusual optical coupling configurations that reduce the importance of read noise, based on the ideal transmission for any additional optics used in these configurations.

  17. Extraction and Quantitation of FD&C Red Dye #40 from Beverages Containing Cranberry Juice: A College-Level Analytical Chemistry Experiment

    Science.gov (United States)

    Rossi, Henry F., III; Rizzo, Jacqueline; Zimmerman, Devon C.; Usher, Karyn M.

    2012-01-01

    A chemical separation experiment can be an interesting addition to an introductory analytical chemistry laboratory course. We have developed an experiment to extract FD&C Red Dye #40 from beverages containing cranberry juice. After extraction, the dye is quantified using colorimetry. The experiment gives students hands-on experience in using solid…

  18. Extraction and Quantitation of FD&C Red Dye #40 from Beverages Containing Cranberry Juice: A College-Level Analytical Chemistry Experiment

    Science.gov (United States)

    Rossi, Henry F., III; Rizzo, Jacqueline; Zimmerman, Devon C.; Usher, Karyn M.

    2012-01-01

    A chemical separation experiment can be an interesting addition to an introductory analytical chemistry laboratory course. We have developed an experiment to extract FD&C Red Dye #40 from beverages containing cranberry juice. After extraction, the dye is quantified using colorimetry. The experiment gives students hands-on experience in using solid…

  19. SPL3/4/5 Integrate Developmental Aging and Photoperiodic Signals into the FT-FD Module in Arabidopsis Flowering.

    Science.gov (United States)

    Jung, Jae-Hoon; Lee, Hyo-Jun; Ryu, Jae Yong; Park, Chung-Mo

    2016-12-05

    Environmental sensitivity varies across developmental phases in flowering plants. In the juvenile phase, microRNA156 (miR156)-mediated repression of SQUAMOSA PROMOTER-BINDING PROTEIN-LIKE (SPL) transcription factors renders Arabidopsis plants incompetent to floral inductive signals, including long-day (LD) photoperiod. During the vegetative phase transition, which accompanies a reduction of miR156 and a concomitant elevation of its targets, plants acquire reproductive competence such that LD signals promote flowering. However, it remains largely unknown how developmental signals are associated with photoperiodic flowering. Here, we show that SPL3, SPL4, and SPL5 (SPL3/4/5) potentiate the FLOWERING LOCUS T (FT)-FD module in photoperiodic flowering. SPL3/4/5 function as transcriptional activators through the interaction with FD, a basic leucine zipper transcription factor which plays a critical role in photoperiodic flowering. SPL3/4/5 can directly bind to the promoters of APETALA1, LEAFY, and FRUITFULL, thus mediating their activation by the FT-FD complex. Our findings demonstrate that SPL3/4/5 act synergistically with the FT-FD module to induce flowering under LDs, providing a long-sought molecular knob that links developmental aging and photoperiodic flowering.

  20. Miniaturized Fiber-Optic Fabry-Perot Interferometer for Highly Sensitive Refractive Index Measurement

    Institute of Scientific and Technical Information of China (English)

    Ming Deng; Tao Zhu; Yun-Jiang Rao; Hong Li

    2008-01-01

    This paper presents a novel miniaturized fiber-optic Fabry-Peort interferometer (FPI) for highly sensitive refractive index measurement. This device was tested for the refractive indices of various liquids including acetone and ethanol at room temperature. The sensitivity for measurement of refractive index change of ethanol is 1138 nm/RIU at the wavelength of 1550 nm. In addition, the sensor fabrication is simple including only cleaving, splicing, and etching. The signal is stable with high visibility. Therefore, it provides a valuable tool in biological and chemical applications.

  1. The GREGOR Fabry Perot Interferometer (GFPI), Technical Innovations and Results achieved in 2013

    CERN Document Server

    Puschmann, Klaus Gerhard

    2016-01-01

    This paper shall provide a summary of not yet published technical innovations to the GREGOR Fabry-Perot Interferometer (GFPI) at the 1.5m GREGOR Solar Telescope (Europe's largest solar telescope) that I implemented in 2013 as the Instrument Scientist of the GFPI. It also represents an overview of important and not yet published observational results that I achieved with the GFPI in 2013. The results and achievements can be considered a milestone in the further development, scientific verification and final acceptance of this instrument. The instrument is now in operation and employed by the international scientific community.

  2. Emulation of Fabry-Perot and Bragg resonators with temporal optical solitons

    CERN Document Server

    Voytova, Tanya; Yulin, Alexey; Driben, Rodislav

    2016-01-01

    The scattering of weak dispersive waves (DW) on several equally spaced temporal solitons is studied. It is shown by systematic numerical simulations that the reflection of the DWs from the soliton trains strongly depends on the distance between the solitons. The dependence of the reflection and transmission coefficients on the inter-soliton distance and the frequency of the incident waves is studied in detail, revealing fascinating quasi-periodic behavior. The analogy between the observed nonlinear phenomena in temporal domain and usual Fabry-Perot and Bragg resonators is discussed.

  3. In-fiber Fabry-Perot interferometer for strain and magnetic field sensing.

    Science.gov (United States)

    Costa, Greice K B; Gouvêa, Paula M P; Soares, Larissa M B; Pereira, João M B; Favero, Fernando; Braga, Arthur M B; Palffy-Muhoray, Peter; Bruno, Antonio C; Carvalho, Isabel C S

    2016-06-27

    In this paper we discuss the results obtained with an in-fiber Fabry-Perot interferometer (FPI) used in strain and magnetic field (or force) sensing. The intrinsic FPI was constructed by splicing a small section of a capillary optical fiber between two pieces of standard telecommunication fiber. The sensor was built by attaching the FPI to a magnetostrictive alloy in one configuration and also by attaching the FPI to a small magnet in another. Our sensors were found to be over 4 times more sensitive to magnetic fields and around 10 times less sensitive to temperature when compared to sensors constructed with Fiber Bragg Grating (FBG).

  4. An All Fiber Intrinsic Fabry-Perot Interferometer Based on an Air-Microcavity

    Directory of Open Access Journals (Sweden)

    Ruth I. Mata-Chávez

    2013-05-01

    Full Text Available In this work an Intrinsic Fabry-Perot Interferometer (IFPI based on an air-microcavity is presented. Here the air microcavity, with silica walls, is formed at a segment of a hollow core photonic crystal fiber (HCPCF, which is fusion spliced with a single mode fiber (SMF. Moreover, the spectral response of the IFPI is experimentally characterized and some results are provided. Finally, the viability to use the IFPI to implement a simple, compact size, and low cost refractive index sensor is briefly analyzed.

  5. Linear FBG Temperature Sensor Interrogation with Fabry-Perot ITU Multi-wavelength Reference

    Directory of Open Access Journals (Sweden)

    Minho Song

    2008-10-01

    Full Text Available The equidistantly spaced multi-passbands of a Fabry-Perot ITU filter are used as an efficient multi-wavelength reference for fiber Bragg grating sensor demodulation. To compensate for the nonlinear wavelength tuning effect in the FBG sensor demodulator, a polynomial fitting algorithm was applied to the temporal peaks of the wavelength-scanned ITU filter. The fitted wavelength values are assigned to the peak locations of the FBG sensor reflections, obtaining constant accuracy, regardless of the wavelength scan range and frequency. A linearity error of about 0.18% against a reference thermocouple thermometer was obtained with the suggested method.

  6. Emulation of Fabry-Perot and Bragg resonators with temporal optical solitons.

    Science.gov (United States)

    Voytova, T; Oreshnikov, I; Yulin, A V; Driben, R

    2016-06-01

    The scattering of weak dispersive waves (DWs) on several equally spaced temporal solitons is studied. It is shown by systematic numerical simulations that the reflection of the DWs from the soliton trains strongly depends on the distance between the solitons. The dependence of the reflection and transmission coefficients on the inter-soliton distance and the frequency of the incident waves are studied in detail, revealing fascinating quasi-periodic behavior. The analogy between the observed nonlinear phenomena in the temporal domain and the usual Fabry-Perot and Bragg resonators is discussed.

  7. Characterization of miniature fiber-optic Fabry-Perot interferometric sensors based on hollow silica tube

    Science.gov (United States)

    Jia, Pinggang; Fang, Guocheng; Wang, Daihua

    2016-09-01

    A miniature fiber-optic Fabry-Perot interferometer (MOFPI) fabricated by splicing a hollow silica tube (HST) with inner diameter of 4 µm to the end of a single-mode fiber is investigated and experimentally demonstrated. The theoretical relationship between the free spectrum range and the length of HST is verified by fabricating several MOFPIs with different lengths. We characterize the MOFPIs for temperature, liquid refractive index, and strain. Experimental results show that the sensitivities of the temperature, liquid refractive index, and strain are 16.42 pm/°C,-118.56 dB/RIU, and 1.21 pm/µɛ, respectively.

  8. Mode-resolved Fabry-Perot experiment in low-loss Bragg-reflection waveguides.

    Science.gov (United States)

    Pressl, B; Günthner, T; Laiho, K; Geßler, J; Kamp, M; Höfling, S; Schneider, C; Weihs, G

    2015-12-28

    Based on the interaction between different spatial modes, semiconductor Bragg-reflection waveguides (BRWs) provide a highly functional platform for non-linear optics. For achieving any desired quantum optical functionality, we must control and engineer the properties of each spatial mode. To reach this purpose we extend the Fabry-Perot technique and achieve a detailed linear optical characterization of dispersive multimode semiconductor waveguides. With this efficient broadband spectral method we gain direct experimental access to the relevant modes of our BRWs and determine their group velocities. Furthermore, we show that our waveguides have lower than expected loss coefficients. This renders them suitable for integrated quantum optics applications.

  9. Intrinsic Fabry-Perot Interferometeric Sensor Based on Microfiber Created by Chemical Etching

    Directory of Open Access Journals (Sweden)

    Ruohui Wang

    2014-09-01

    Full Text Available An intrinsic Fabry-Perot interferometeric sensor based on a microfiber has been demonstrated. The micro-size suspended core is created by chemical etching a photonics crystal fiber, of which the cladding has a micrometer-spaced, hexagonal array of air holes. The sensing head is fabricated by chemical etching a short section of photonics crystal fiber spliced with a single mode fiber. The temperature sensing characteristic of the interferometer has also been demonstrated and a sensitivity 14.3 pm/°C is obtained.

  10. A Fabry-Perot interferometer with quantum mirrors: nonlinear light transport and rectification

    CERN Document Server

    Fratini, F; Safari, L; Poizat, J-Ph; Valente, D; Auffèves, A; Gerace, D; Santos, M F

    2014-01-01

    Optical transport represents a natural route towards fast communications, and it is currently used in large scale data transfer. The progressive miniaturization of devices for information processing calls for the microscopic tailoring of light transport and confinement at length scales appropriate for the upcoming technologies. With this goal in mind, we present a theoretical analysis of a one-dimensional Fabry-Perot interferometer built with two highly saturable nonlinear mirrors: a pair of two-level systems. Our approach captures non-linear and non-reciprocal effects of light transport that were not reported previously. Remarkably, we show that such an elementary device can operate as a microscopic integrated optical rectifier.

  11. Dynamic Gain-Clamped Amplifier with Backward-Injection of a Fabry-Perot Laser

    Institute of Scientific and Technical Information of China (English)

    Chien-Hung Yeh; Chih-Yang Chen; Chien-Chung Lee; Sien Chi

    2003-01-01

    We have been experimentally demonstrated an active control technique of dynamic gain-clamped spectra for the erbium-doped fiber amplifiers (EDFA's) by a backward-injected Fabry-Perot (F-P) laser into this EDFA. In addition, employing a short length erbium-doped fiber (EDF) that not cause any gain saturation for preamplification in front of this amplifier module, it can reduce the noise figure degradation and simultaneously achieve gain variation from 11.1 dB to 0.5 dB for 10 dB input power level change.

  12. Dynamic Gain-Clamped Amplifier with Backward-Injection of a Fabry-Perot Laser

    Institute of Scientific and Technical Information of China (English)

    Chien-Hung; Yeh; Chih-Yang; Chen; Chien-Chung; Lee; Sien; Chi

    2003-01-01

    We have been experimentally demonstrated an active control technique of dynamic gain-clamped spectra for theerbium-doped fiber amplifiers (EDFA's) by a backward-injected Fabry-Perot (F-P) laser into this EDFA. In addition, employing a short length erbium-doped fiber (EDF) that not cause any gain saturation for preamplification in front of this amplifier module, it can reduce the noise figure degradation and simultaneously achieve gain variation from 11.1 dB to 0.5 dB for 10 dB input power level change.

  13. Nanofiber Fabry-Perot microresonator for non-linear optics and cavity quantum electrodynamics

    CERN Document Server

    Wuttke, C; Brückner, S; Rothhardt, M; Rauschenbeutel, A

    2012-01-01

    We experimentally realize a Fabry-Perot-type optical microresonator near the cesium D2 line wavelength based on a tapered optical fiber, equipped with two fiber Bragg gratings which enclose a sub-wavelength diameter waist. Owing to the very low taper losses, the finesse of the resonator reaches F = 86 while the on-resonance transmission is T = 11 %. The characteristics of our resonator fulfill the requirements of non-linear optics and cavity quantum electrodynamics in the strong coupling regime. In combination with its demonstrated ease of use and its advantageous mode geometry, it thus opens a realm of applications.

  14. Electrically tunable Fabry-Péerot resonator based on microstructured Si containing liquid crystal

    KAUST Repository

    Tolmachev, Vladimir A.

    2012-01-01

    We have built Fabry-Perot resonators based on microstructured silicon and a liquid crystal. The devices exhibit tuning of the resonance peaks over a wide range, with relative spectral shifts of up to Delta lambda/lambda = 10%. In order to achieve this substantial spectral shift, cavity peaks of high order were used. Under applied voltages of up to 15 V, a variation in the refractive index of the nematic liquid crystal E7 from Delta n(LC) = 0.12 to Delta n(LC) = 0.17 was observed. These results may have practical applications in the near-, mid and far-infrared range.

  15. An optical fiber Fabry-Perot pressure sensor using corrugated diaphragm and angle polished fiber

    Science.gov (United States)

    Zhu, Jiali; Wang, Ming; Chen, Lu; Ni, Xiaoqi; Ni, Haibin

    2017-03-01

    In this paper, a Fabry-Perot pressure sensor using a corrugated diaphragm and angle polished fiber is proposed. A SU-8 structure using two step of lithography is formed to fix the polished fiber, which helps control the cavity length precisely. The fabrication process is described. The characteristics of both pressure and temperature are tested. Also the temperature compensation is realized. Experimental results show that the sensor has high sensitivity and good linearity over the pressure range of 0-0.1 MPa. The sensitivity (change in cavity/loaded pressure) is 705.64 μm/MPa.

  16. Scanning Gate Microscopy of Kondo Dots: Fabry-P\\'erot Interferences and Thermally Induced Rings

    OpenAIRE

    Kleshchonok, Andrii; Fleury, Geneviève; Pichard, Jean-Louis

    2013-01-01

    We study the conductance of an electron interferometer formed in a two dimensional electron gas between a nanostructured quantum contact and the charged tip of a scanning gate microscope. Measuring the conductance as a function of the tip position, thermally induced rings may be observed in addition to Fabry-P\\'erot interference fringes spaced by half the Fermi wavelength. If the contact is made of a quantum dot opened in the middle of a Kondo valley, we show how the location of the rings all...

  17. Scanning Gate Microscopy of Kondo Dots: Fabry-Pérot Interferences and Thermally Induced Rings

    OpenAIRE

    Kleshchonok, Andrii; Fleury, Geneviève; Pichard, Jean-Louis

    2013-01-01

    5 pages, 4 figures; We study the conductance of an electron interferometer formed in a two dimensional electron gas between a nanostructured quantum contact and the charged tip of a scanning gate microscope. Measuring the conductance as a function of the tip position, thermally induced rings may be observed in addition to Fabry-Pérot interference fringes spaced by half the Fermi wavelength. If the contact is made of a quantum dot opened in the middle of a Kondo valley, we show how the locatio...

  18. Cooperative effects of two optical dipole antennas coupled to plasmonic Fabry-Pérot cavity.

    Science.gov (United States)

    Yang, Zhong-Jian; Wang, Qu-Quan; Lin, Hai-Qing

    2012-09-07

    We investigate the cooperative effects of two optical dipole antennas that are coupled to a finite Au nanowire acting as plasmonic Fabry-Pérot (F-P) cavity. The coherent coupling between one single antenna and the F-P cavity can result in Fano resonance, and the coupling strength is antenna position dependent. For two antennas coupled to the F-P cavity, constructive or destructive interference between antennas could be achieved by adjusting their positions along the F-P cavity. Consequently, the Fano resonance will become stronger or weaker correspondingly.

  19. High precision optical fiber Fabry-Perot sensor for gas pressure detection

    Science.gov (United States)

    Mao, Yan; Tong, Xing-lin

    2013-09-01

    An optical fiber Fabry-Perot (F-P) sensor with quartz diaphragm for gas pressure testing was designed and fabricated. It consisted of single-mode fiber, hollow glass tube and quartz diaphragm. It uses the double peak demodulation to obtain the initialized cavity length. The variety of cavity length can be calcultated by the single peak demodulation after changing the gas pressure. The results show that the sensor is small in size, whose sensitivity is 19 pm/kPa in the range of the 10 ~ 260 kPa gas pressure. And it has good linearity and repeatability.

  20. Simulation experiments to generate broadband chaos using dual-wavelength optically injected Fabry-Perot laser

    Science.gov (United States)

    Obaid, Hafiz Muhammad; Khawar Islam, Muhammad; Obaid Ullah, Muhammad

    2016-08-01

    Broadband chaos can be generated by beating two wavelengths in a hybrid arrangement of Fabry-Perot (FP) Laser and Fiber ring cavity by injecting dual wavelengths. The bandwidth of generated chaos can be controlled by detuning different modes of FP Laser for beating. The bandwidth of generated chaos increased to many folds depending upon the injected strength and wavelength spacing matched to FP laser modes. The bandwidth enhancement in different simulation experiments conducted is optimized by varying different parameters of FP laser and cavity. The waveforms are analyzed and Lyapunov exponents are calculated in order to validate the existence of high bandwidth non-pulsating chaos.

  1. Mesa Diaphragm-Based Fabry-Perot Optical MEMS Pressure Sensor

    Institute of Scientific and Technical Information of China (English)

    Yi-Xian Ge; Ming Wang; Hai-Tao Yan

    2008-01-01

    An optical micro electron mechanical system (MEMS) pressure sensor with a mesa membrane is presented. The operating principle of the MEMS pressure sensor is expatiated by the Fabry-Perot (F-P) interference and the relation between deflection and pressure is analyzed. Both the mechanical model of the mesa structure diaphragm and the signal averaging effect is validated by simulation, which declares that the mesa structure diaphragm is superior to the planar one on the parallelism and can reduce the signal averaging effect. Experimental results demonstrate that the mesa structure sensor has a reasonable linearity and sensitivity.

  2. Nanofiber Fabry-Perot microresonator for nonlinear optics and cavity quantum electrodynamics.

    Science.gov (United States)

    Wuttke, C; Becker, M; Brückner, S; Rothhardt, M; Rauschenbeutel, A

    2012-06-01

    We experimentally realize a Fabry-Perot-type optical microresonator near the cesium D2 line wavelength based on a tapered optical fiber, equipped with two fiber Bragg gratings that enclose a subwavelength diameter waist. Owing to the very low taper losses, the finesse of the resonator reaches F=86 while the on-resonance transmission is T=11%. The characteristics of our resonator fulfill the requirements of nonlinear optics and cavity quantum electrodynamics in the strong coupling regime. These characteristics, combined with the demonstrated ease of use and advantageous mode geometry, open a realm of applications.

  3. Triple Fabry-Pérot Imaging Interferometer for High Resolution Solar Spectroscopy using the ATST

    Science.gov (United States)

    Robinson, B. M.; Gary, G. A.; Balasubramaniam, K. S.

    2005-05-01

    We present a telecenrically mounted triple Fabry-Pérot imaging interferometer for the NSOs Advanced Technology Solar Telescope (ATST). It consists of three Fabry-Pérot etalons and the feed and imaging optics. This system provides high throughput, flexibility and breadth of operation when compared to other spectroscopic imaging systems. It can operate in four distinct modes: as a spectro-polarimeter, a filter-vector magnetograph, an intermediate-band imager, and broadband high-resolution imager. In the proposed telecentric mount configuration, the transmittance of the etalon system is not a function of position in the field, so that instantaneous spectroscopic measurements can be performed across the entire field of view; however, the transmission peak of the interferometer is broadened. Mitigation of this broadening requires a low F# image at the etalons. Together with the requirement that the field of view be large enough to observe large-scale processes in the solar atmosphere, this limitation dictates that the diameter of the etalons have a large aperture. Specifically, for a spectrographic passband full-width at half-maximum (FWHM) of around 2 pm, and entrance pupil diameter of 4 m, and a field of view of 35", the required etalon diameter is around 200 mm. This is beyond the size of current Fabry-Pérot etalons and near the current projected limit of manufacturability. The development of this instrument will bring these large etalons to realization and take Fabry-Pérot imaging interferometry to the next level of operational capability within telescopes of large aperture. This instrument will provide spectral, spatial, and temporal resolution which is not currently available to large aperture solar astronomy, but which is necessary, in conjunction with the new class telescopes, to the continuing discovery of laws that govern the dynamics of the sun and the earth-sun connection. The resolution afforded by higher aperture telescopes and instrumentation will

  4. A switchable fiber laser based on an all-fiber Fabry-Perot filter

    Science.gov (United States)

    Lopez-Dieguez, Y.; Jauregui-Vázquez, D.; Estudillo-Ayala, J. M.; Herrera-Piad, L. A.; Rojas-Laguna, R.; Sierra-Hernandez, J. M.; Hernandez-Garcia, J. C.; Harush-Negari, A. B.

    2017-02-01

    In this experimental manuscript, a switchable Erbium-doped fiber ring laser based on an all-fiber Fabry-Perot filter was demonstrated. The filter is composed by several air micro-cavities formed into a section of a single-mode fiber splice joints with special hollow-core photonic crystal fiber. These micro-cavities are formed by air and silica, which produces several reflections generated at each silica-air-silica interfaces. Using this experimental setup we obtain a very high stable triple-laser emission at 1529.450nm, 1549.100nm and 1555.350nm with a linewidth of 0.2nm and a side-mode suppression ratio of 32dB, 37dB and 29dB respectively. These laser emission show a maximal peak power fluctuation around 0.4dB, 1.5dB and 2.6dB, with 0.025nm of wavelength oscillations. These results were observed after monitoring the laser cavity during an hour by recording the data each three minutes. By appropriately adjusting of transversal load applied over the Fabry-Perot filter between 0g and 550g, the ring laser cavity can be operated in double- wavelength, triple- wavelength, or quadruple- wavelength states. For this analysis, the all-fiber Fabry-Perot filter was set between a metal layer (below) and a thin glass layer (above) where transversal load was applied, here uniform load distribution over all the Fabry-Perot filter structure is achieved, as a result, the air intra-cavities that conform the filter are affected and the gain-losses profile is modified into the laser arrangement. The lasing emissions obtained in this work have a side-mode suppression ratio greater than 30dB. This ring laser cavity design offers a compact, simple and low-cost implementation and can be used in different applications where a very stable double, triple or quadruple laser lines are required.

  5. Hα kinematics of nearby galaxies using Fabry-Perot and IFU data

    Science.gov (United States)

    Erroz-Ferrer, Santiago; S4G Team; MUSE-GTO Consortium

    2017-03-01

    I present here analysis of the shapes of the rotation curves of a large sample of nearby spiral galaxies with high angular and spectral resolution Hα (Fabry-Perot GHαFaS) kinematics, and the resulting constraints on their total mass distributions. In particular I discuss how their rotation curve shapes relate to key galaxy properties. Finally I present related results from the MUSE Atlas of Disks (MAD) program, which is dissecting the nearby disk population with IFU spectroscopy at ~100pc resolution.

  6. Asymmetric Fabry-Pérot interferometric cavity for fiber optical sensors

    Institute of Scientific and Technical Information of China (English)

    Shaoji Jiang; Youcheng Liang; Xi Zhu; Hezhou Wang

    2006-01-01

    Good linearity and wide dynamic range are the advantages of asymmetric Fabry-Pérot (F-P) interferometric cavity, whose realization has been long for. Based on optical thin film characteristic matrix theory, an asymmetric F-P interferometric cavity with good linearity and wide dynamic range is designed. And by choosing the material of two different thin metallic layers, the asymmetric F-P interferometric cavity is successfully fabricated. The design theory and method of this asymmetric F-P interferometric cavity have been described in detailed. In this paper an asymmetric F-P interferometric cavity used in fiber optical sensor is reported.

  7. Electron polarization measurement using a Fabry-Perot cavity at HERA

    CERN Document Server

    Zhang, Z

    2001-01-01

    A new Compton longitudinal polarimeter currently under construction for HERA is presented. The key component of the polarimeter is a Fabry-Perot cavity located around the electron beam pipe. With such an optical cavity, a continuous laser power equivalent to 5 kW, much higher than those commercially available, can be achieved, leading to one backscattered photon per bunch crossing. This ``few-photon mode'' will allow a very precise determination of the calorimeter response with little systematic uncertainty. The electron polarization measurement at the per mill level is expected.

  8. VTT's Fabry-Perot interferometer technologies for hyperspectral imaging and mobile sensing applications

    Science.gov (United States)

    Rissanen, Anna; Guo, Bin; Saari, Heikki; Näsilä, Antti; Mannila, Rami; Akujärvi, Altti; Ojanen, Harri

    2017-02-01

    VTT's Fabry-Perot interferometers (FPI) technology enables creation of small and cost-efficient microspectrometers and hyperspectral imagers - these robust and light-weight sensors are currently finding their way into a variety of novel applications, including emerging medical products, automotive sensors, space instruments and mobile sensing devices. This presentation gives an overview of our core FPI technologies with current advances in generation of novel sensing applications including recent mobile technology demonstrators of a hyperspectral iPhone and a mobile phone CO2 sensor, which aim to advance mobile spectroscopic sensing.

  9. Compact large-aperture Fabry-Perot interferometer modules for gas spectroscopy at mid-IR

    Science.gov (United States)

    Kantojärvi, Uula; Varpula, Aapo; Antila, Tapani; Holmlund, Christer; Mäkynen, Jussi; Näsilä, Antti; Mannila, Rami; Rissanen, Anna; Antila, Jarkko; Disch, Rolf J.; Waldmann, Torsten A.

    2014-03-01

    VTT has developed Fabry-Pérot Interferometers (FPI) for visible and infrared wavelengths since 90's. Here we present two new platforms for mid-infrared gas spectroscopy having a large optical aperture to provide high optical throughput but still enabling miniaturized instrument size. First platform is a tunable filter that replaces a traditional filter wheel, which operates between wavelengths of 4-5 um. Second platform is for correlation spectroscopy where the interferometer provides a comb-like transmission pattern mimicking absorption of diatomic molecules at the wavelength range of 4.7-4.8 um. The Bragg mirrors have 2-4 thin layers of polysilicon and silicon oxide.

  10. Wind tunnel unsteady pressure measurements using a differential optical fiber Fabry-Perot pressure sensor

    Science.gov (United States)

    Correia, Ricardo; Staines, Stephen E.; James, Stephen W.; Lawson, Nicholas; Garry, Kevin; Tatam, Ralph P.

    2014-05-01

    A differential extrinsic optical fiber Fabry-Perot based pressure sensor has been developed and benchmarked against a conventional piezoresistive Kulite pressure sensor. The sensors were placed on the fuselage of a 1:10/3 sub-scale model of a Scottish aviation Bulldog, which was placed in a wind-tunnel. Pressure tappings that surrounded the sensors aided the mapping of pressure distribution around this section of the fuselage. The results obtained from the fibre optic pressure sensor are in good agreement with those obtained from the Kulite and from the pressure tappings.

  11. Estudio de la fisiopatología del sistema inmune en la enfermedad de Fabry

    OpenAIRE

    2013-01-01

    La hipótesis que proponemos y evaluamos a lo largo de este trabajo de tesis está centrada en la existencia de un estado proinflamatorio en la Enfermedad de Fabry, que involucra células del sistema inmune y es causado por la acumulación de globotriaosilceramida (Gb3) a nivel lisosomal. Esos depósitos de Gb3 generarían el desencadenamiento de mecanismos patogénicos que producen un aumento de apoptosis y una respuesta inmune inflamatoria. Estas respuestas serían las responsables de alteraciones ...

  12. Generation of millimeter-wave sub-carrier optical pulse by using a Fabry-Perot interferometer

    Institute of Scientific and Technical Information of China (English)

    Qing Ye; Ronghui Qu; Zujie Fang

    2007-01-01

    A novel scheme is proposed to transform a Gaussian optical pulse to a millimeter-wave (mm-wave) frequency modulation pulse by using a Fabry-Perot interferometer (FPI) for radio-over-fiber (ROF) system.It is shown that modulation frequency of mm-wave is determined by the optical path of the Fabry-Perot (F-P) cavity, and amplitude decay time and energy transfer efficiency are related to the reflectivity of the F-P cavity mirror. The effect of pulse train extension on inter-symbol interference is also discussed.

  13. Correction of Thermal Deviations of Fabry-Perot Resonator Based Measurements of Specific Gases in Millimeter Wave Bands

    Directory of Open Access Journals (Sweden)

    J. Libich

    2012-04-01

    Full Text Available Due to the thermal expansivity of the material used in the Fabry-Perot resonator mirrors, the resonator cavity length can change and this might therefore have an impact on the resonant frequency during high-resolution spectroscopy measurements. Based on measurements and simulations, this paper discusses the influences of temperature on the precise determination of gas attenuation measured in a Fabry-Perot resonator. Several measures to mitigate such influence and to correct the measured results were tested. A correction method for the measured data was proposed.

  14. External Electro-optic Sampling Utilizing an Asymmetric Fabry-Pérot Film of Poled Electro-optical Polymer

    Institute of Scientific and Technical Information of China (English)

    陈开鑫; 杨罕; 张大明; 张红波; 衣茂斌

    2001-01-01

    External electro-optic sampling has been first demonstrated using a poled electro-optical polymer asymmetricFabry-Pérot film, placed freely on the indium-tin oxide coplanar waveguide transmission line and used as anelectro-optic probe tip. Only one laser beam is required due to the fact that the asymmetric Fabry-Pérot film isutilized to convert the phase modulation to amplitude modulation. A 1.2 GHz microwave signal is sampled, andthe voltage sensitivity of about 2 m V/Hz is obtained. The results are promising for technical applications inhigh-speed electronic devices and monolithic microwave integrated circuits research.

  15. Development FD-SOI MOSFET Amplifiers for Integrated Read-Out Circuit of Superconducting-Tunnel-Junction Single-Photon-Detectors

    Energy Technology Data Exchange (ETDEWEB)

    Kiuchi, Kenji; et al.

    2015-07-27

    We proposed a new high-resolution single-photon infrared spectrometer for search for radiative decay of cosmic neutrino background (CνB). The superconducting-tunnel-junctions(STJs) are used as a single-photon counting device. Each STJ consists of Nb/Al/AlxOy/Al/Nb layers, and their thicknesses are optimized for the operation temperature at 370 mK cooled by a 3He sorption refrigerator. Our STJs achieved the leak current 250 pA, and the measured data implies that a smaller area STJ fulfills our requirement. FD-SOI MOSFETs are employed to amplify the STJ signal current in order to increase signal-to-noise ratio (S/N). FD-SOI MOSFETs can be operated at cryogenic temperature of 370 mK, which reduces the noise of the signal amplification system. FD-SOI MOSFET characteristics are measured at cryogenic temperature. The Id-Vgs curve shows a sharper turn on with a higher threshold voltage and the Id-Vds curve shows a nonlinear shape in linear region at cryogenic temperature. Taking into account these effects, FD-SOI MOSFETs are available for read-out circuit of STJ detectors. The bias voltage for STJ detectors is 0.4 mV, and it must be well stabilized to deliver high performance. We proposed an FD-SOI MOSFET-based charge integrated amplifier design as a read-out circuit of STJ detectors. The requirements for an operational amplifier used in the amplifier is estimated using SPICE simulation. The op-amp is required to have a fast response (GBW ≥ 100 MHz), and it must have low power dissipation as compared to the cooling power of refrigerator.

  16. A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease.

    Science.gov (United States)

    Shirahama, M; Akiyoshi, J; Ishitobi, Y; Tanaka, Y; Tsuru, J; Matsushita, H; Hanada, H; Kodama, K

    2010-01-01

    Fahr disease (FD) is a rare neurological and psychiatric disorder. The disease is classified by intracranial calcification of the basal ganglia with the globus pallidus region being particularly affected. We examined a young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible third-generation FD. Case report of third-generation FD. A 23-year-old woman was arrested for two arsons: i) The patient exhibited progressive psychotic symptoms, including visual hallucinations, delusion of injury, irritability, lability of mood, mental retardation and visual disorders and ii) Computed tomography (CT) imaging demonstrated bilateral calcifications of the basal ganglia (globus pallidus) in the patient, her mother and her grandmother. We found a family with a three-generation history of FD who exhibited calcification in the brain and mental retardation. Compared to her mother, the patient described here displayed anticipation of disease onset.

  17. Fiber-optic Fabry-Pérot strain sensor based on graded-index multimode fiber

    Institute of Scientific and Technical Information of China (English)

    Tian Zhao; Yuan Gong; Yunjiang Rao; Yu Wu; Zengling Ran; Huijuan Wu

    2011-01-01

    By using a graded-index multimode fiber (GI-MMF) with a relatively flat index profile and high refractive index of the fiber core, a microextrinsic fiber-optic Fabry-Pérot interferometric (MEFPI) strain sensor is fabricated through chemical etching and fusion splicing. Higher reflectance of the microcavity is obtained due to the less-curved inner wall in the center of the fiber core after etching and higher index contrast between the GI-MMF core and air. The maximum reflection of the sensor is enhanced 12 dB than that obtained by etching of the Er- or B-doped fibers. High fringe contrast of 22 dB is obtained. The strain and temperature responses of the MEFPI sensors are investigated in this experiment. Good linearity and high sensitivity axe achieved, with wavelength-strain and wavelength-temperature sensitivities of 7.82 pm/μεand 5.01 pm/℃, respectively.%@@ By using a graded-index multimode fiber (GI-MMF) with a relatively flat index profile and high refractive index of the fiber core, a microextrinsic fiber-optic Fabry-Pérot interferometric (MEFPI) strain sensor is fabricated through chemical etching and fusion splicing.Higher reflectance of the microcavity is obtained due to the less-curved inner wall in the center of the fiber core after etching and higher index contrast between the GI-MMF core and air.

  18. Nonlinear regression method for estimating neutral wind and temperature from Fabry-Perot interferometer data.

    Science.gov (United States)

    Harding, Brian J; Gehrels, Thomas W; Makela, Jonathan J

    2014-02-01

    The Earth's thermosphere plays a critical role in driving electrodynamic processes in the ionosphere and in transferring solar energy to the atmosphere, yet measurements of thermospheric state parameters, such as wind and temperature, are sparse. One of the most popular techniques for measuring these parameters is to use a Fabry-Perot interferometer to monitor the Doppler width and breadth of naturally occurring airglow emissions in the thermosphere. In this work, we present a technique for estimating upper-atmospheric winds and temperatures from images of Fabry-Perot fringes captured by a CCD detector. We estimate instrument parameters from fringe patterns of a frequency-stabilized laser, and we use these parameters to estimate winds and temperatures from airglow fringe patterns. A unique feature of this technique is the model used for the laser and airglow fringe patterns, which fits all fringes simultaneously and attempts to model the effects of optical defects. This technique yields accurate estimates for winds, temperatures, and the associated uncertainties in these parameters, as we show with a Monte Carlo simulation.

  19. [Quartz-enhanced photoacoustic spectroscopy trace gas detection system based on the Fabry-Perot demodulation].

    Science.gov (United States)

    Lin, Cheng; Zhu, Yong; Wei, Wei; Zhang, Jie; Tian, Li; Xu, Zu-Wen

    2013-05-01

    An all-optical quartz-enhanced photoacoustic spectroscopy system, based on the F-P demodulation, for trace gas detection in the open environment was proposed. In quartz-enhanced photoacoustic spectroscopy (QEPAS), an optical fiber Fabry-Perot method was used to replace the conventional electronic demodulation method. The photoacoustic signal was obtained by demodulating the variation of the Fabry-Perot cavity between the quartz tuning fork side and the fiber face. An experimental system was setup. The experiment for detection of water vapour in the open environment was carried on. A normalized noise equivalent absorption coefficient of 2.80 x 10(-7) cm(-1) x W x Hz(-1/2) was achieved. The result demonstrated that the sensitivity of the all-optical quartz-enhanced photoacoustic spectroscopy system is about 2.6 times higher than that of the conventional QEPAS system. The all-optical quartz-enhanced photoacoustic spectroscopy system is immune to electromagnetic interference, safe in flammable and explosive gas detection, suitable for high temperature and high humidity environments and realizable for long distance, multi-point and network sensing.

  20. Contact grating device with Fabry-Perot resonator for effective terahertz light generation.

    Science.gov (United States)

    Tsubouchi, Masaaki; Nagashima, Keisuke; Yoshida, Fumiko; Ochi, Yoshihiro; Maruyama, Momoko

    2014-09-15

    A novel design for a contact grating device with an incorporated Fabry-Perot resonator is proposed for high-power terahertz (THz) light generation. We deposited a multilayer consisting of Ta(2)O(5) and Al(2)O(3) on a magnesium-doped stoichiometric LiNbO(3) substrate and fabricated grating grooves on the outermost layer. The multilayer was designed such that conditions for a Fabry-Perot resonator were satisfied for light diffracted by the grating. Consequently, the fraction of light transmitted into the LiNbO(3) substrate, i.e., the diffraction efficiency, was enhanced by the resonator. The diffraction efficiency of the fabricated device was 71%, which is close to the calculated value of 78% from the optimized design. THz light generation was also demonstrated with the contact grating device. The THz output of 0.41 μJ was obtained using near-infrared pump light of 2.7 mJ.

  1. Modified Fabry-Perot interferometer for displacement measurement in ultra large measuring range.

    Science.gov (United States)

    Chang, Chung-Ping; Tung, Pi-Cheng; Shyu, Lih-Horng; Wang, Yung-Cheng; Manske, Eberhard

    2013-05-01

    Laser interferometers have demonstrated outstanding measuring performances for high precision positioning or dimensional measurements in the precision industry, especially in the length measurement. Due to the non-common-optical-path structure, appreciable measurement errors can be easily induced under ordinary measurement conditions. That will lead to the limitation and inconvenience for in situ industrial applications. To minimize the environmental and mechanical effects, a new interferometric displacement measuring system with the common-optical-path structure and the resistance to tilt-angle is proposed. With the integration of optomechatronic modules in the novel interferometric system, the resolution up to picometer order, high precision, and ultra large measuring range have been realized. For the signal stabilization of displacement measurement, an automatic gain control module has been proposed. A self-developed interpolation model has been employed for enhancing the resolution. The novel interferometer can hold the advantage of high resolution and large measuring range simultaneously. By the experimental verifications, it has been proven that the actual resolution of 2.5 nm can be achieved in the measuring range of 500 mm. According to the comparison experiments, the maximal standard deviation of the difference between the self-developed Fabry-Perot interferometer and the reference commercial Michelson interferometer is 0.146 μm in the traveling range of 500 mm. With the prominent measuring characteristics, this should be the largest dynamic measurement range of a Fabry-Perot interferometer up till now.

  2. Highly sensitive force sensor based on optical microfiber asymmetrical Fabry-Perot interferometer.

    Science.gov (United States)

    Gong, Yuan; Yu, Cai-Bin; Wang, Ting-Ting; Liu, Xiu-Ping; Wu, Yu; Rao, Yun-Jiang; Zhang, Ming-Lei; Wu, Hui-Juan; Chen, Xiao-Xiao; Peng, Gang-Ding

    2014-02-10

    An asymmetrical Fabry-Perot interferometric (AFPI) force sensor is fabricated based on a narrowband reflection of low-reflectivity fiber Bragg grating (LR-FBG) and a broadband Fresnel reflection of the cleaved fiber end. The AFPI sensor includes a section of microfiber made by tapering and it achieves a force sensitivity of 0.221 pm/μN with a tapered microfiber of 40 mm length and 6.1 μm waist diameter. Compared with similar AFPI structure in 125 μm-diameter single mode fiber, the force sensitivity of the microfiber AFPI structure is greatly enhanced due to its smaller diameter and can be optimized for different force scales by controlling the diameter. The fabrication process of the AFPI sensor is simple and cost-effective. The AFPI sensor has better multiplexing capacity than conventional extrinsic fiber-optic Fabry-Perot sensors, while it also release the requirement on the wavelength matching of the FBG-pair-based FPI.

  3. From the "Göttingen" Fabry-Perot Interferometer to the GREGOR FPI

    Science.gov (United States)

    Puschmann, K. G.; Kneer, F.; Nicklas, H.; Wittmann, A. D.

    Fabry-Perot Interferometers (FPIs) have advantages over slit spectrographs, allowing fast two-dimensional, narrowband imaging and post factum image reconstruction of the spectropolarimetric data obtained. The resulting intensity, velocity and magnetic field maps are a fundamental base for the understanding of the dynamics of the solar atmosphere and its magnetic fields at smallest spatial scales. Efforts are undertaken to provide, with the Göttingen Fabry-Perot interferometer, an up-todate post-focus instrument for the German 1.5 m GREGOR solar telescope. Therefore a renewal of the spectrometer has been achieved during the first half of 2005. First observations at the German Vacuum Tower Telescope (VTT) reveal new scientific aspects and a very promising outlook for the future at GREGOR. In this contribution a general description of the upgraded spectrometer is given. Its final optical design at GREGOR is described and an optical analysis of the GREGOR FPI is outlined. Latest results with the new instrument obtained at the VTT are presented.

  4. Fabry-Perot Based Radiometers for Precise Measurement of Greenhouse Gases

    Science.gov (United States)

    Heaps, William S.; Wilson, Emily L.; Georgieva, Elena

    2007-01-01

    Differential radiometers based upon the Fabry-Perot interferometer have been developed and demonstrated that exhibit very great sensitivity to changes in the atmospheric column of carbon dioxide, oxygen, and water vapor. These instruments employ a solid Fabry-Perot etalon that is tuned to the proper wavelength by changing the temperature. By choosing the thickness of the etalon its multiple pass bands can be made to align with regularly space absorption features of the molecule under investigation. Use of multiple absorption features improves the optical throughput of the instrument and improves the stability of the instrument response with respect to environmental changes. Efforts are underway at Goddard to extend this technique to the carbon 13 isotope of carbon dioxide and to methane. These instruments are intrinsically rugged and can be made rather small and inexpensively. They therefore hold promise for widespread use in ground based networks for calibration of satellite instruments such as OCO and GOSAT. Results will be presented for ground based and airborne operations for these systems. The effects of atmospheric scattering, pointing errors, pressure broadening and temperature effects will be discussed with regard to achieving precision better than .5% required for validation of carbon dioxide column measured from space. Designs permitting the extension of the technique to an even larger number of atmospheric species will be discussed along with theoretical analysis of potential system performance.

  5. Dynamics of a noncontacting, white light Fabry-Perot interferometric displacement sensor.

    Science.gov (United States)

    Moro, Erik A; Todd, Michael D; Puckett, Anthony D

    2012-07-01

    A white light extrinsic Fabry-Perot interferometer is implemented as a noncontacting displacement sensor, providing robust, absolute displacement measurements with micrometer accuracy at a sampling rate of 10 Hz. This paper presents a dynamic model of the sensing cavity between the sensor probe and the nearby target surface using a Fabry-Perot etalon approach obtained from straightforward electromagnetic field formulations. Such a model is important for system characterization, as the dynamically changing cavity length imparts a Doppler shift on any signals circulating within the sensing cavity. Contrary to previously published results, Doppler-induced shifting within the low-finesse sensing cavity is shown to significantly distort the measurement signal as recorded by the sensor. Experimental and simulation results are compared, and the direct effects of cavity dynamics on the measurement signal are analyzed along with their indirect impact on sensor performance. This document has been approved by Los Alamos National Laboratory for unlimited public release (LA-UR 12-00301).

  6. Fabry-Perot Temperature Sensor for Quasi-Distributed Measurement Utilizing OTDR

    Institute of Scientific and Technical Information of China (English)

    Ping Xu; Fu-Fei Pang; Na Chen; Zhen-Yi Chen; Ting-Yun Wang

    2008-01-01

    A quasi-distributed Fabry-Perot fiber optic temperature sensor array using optical time domain reflectometry (OTDR) technique is presented. The F-P sensor is made by two face to face single-mode optical fibers and their surfaces have been polished. Due to the low reflectivity of the fiber surfaces, the sensor is described as low Fresnel Fabry-Perot interferometer (FPI). The working principle is analyzed using two-beam optical interference approximation. To measure the temperature, a certain temperature sensitive material is filled in the cavity. The slight changes of the reflective intensity which is induced by the refractive index of the material was eaught by OTDR. The length of the cavity is obtained by monitoring the interference spectrum which is used for the setting of the sensor static characteristics within the quasi-linear range. Based on our design, a three point sensor array are fabricated and characterized. The experimental results show that with the temperature increasing from -30℃ to 80℃, the reflectivity increase in a good linear manner. The sensitivity was approximate 0.074 dB℃. For the low transmission loss, more sensors can be integrated.

  7. Formation of super-resolution spot through nonlinear Fabry-Perot cavity structures: theory and simulation.

    Science.gov (United States)

    Wei, Jingsong; Wang, Rui; Yan, Hui; Fan, Yongtao

    2014-04-07

    This study explores how interference manipulation breaks through the diffraction limit and induces super-resolution nano-optical hot spots through the nonlinear Fabry-Perot cavity structure. The theoretical analytical model is established, and the numerical simulation results show that when the thickness of the nonlinear thin film inside the nonlinear Fabry-Perot cavity structure is adjusted to centain value, the constructive interference effect can be formed in the central point of the spot, which causes the nanoscale optical hot spot in the central region to be produced. The simulation results also tell us that the hot spot size is sensitive to nonlinear thin film thickness, and the accuracy is required to be up to nanometer or even subnanometer scale, which is very large challenging for thin film deposition technique, however, slightly changing the incident laser power can compensate for drawbacks of low thickness accuracy of nonlinear thin films. Taking As(2)S(3) as the nonlinear thin film, the central hot spot with a size of 40nm is obtained at suitable nonlinear thin film thickness and incident laser power. The central hot spot size is only about λ/16, which is very useful in super-high density optical recording, nanolithography, and high-resolving optical surface imaging.

  8. Vanadium dioxide based Fabry-Perot emitter for dynamic radiative cooling applications

    Science.gov (United States)

    Taylor, Sydney; Yang, Yue; Wang, Liping

    2017-08-01

    An asymmetric Fabry-Perot emitter is proposed with a lossless dielectric spacer inserted between a vanadium dioxide (VO2) thin film and an opaque aluminum substrate. Switchable mid-infrared emittance has been achieved due to the insulator-to-metal transition of VO2. When VO2 is dielectric below 341 K, the structure is highly reflective, thereby minimizing thermal radiation loss. Above 345 K, the VO2 becomes metallic and forms a Fabry-Perot resonance cavity with high broadband emissivity around 10 μm wavelength, providing a radiative cooling effect due to enhanced thermal emission. The radiative properties are calculated via a uniaxial transfer matrix method and Bruggeman effective medium theory. The physical mechanisms that provide the observed absorption enhancements are elucidated by examining the total phase shift in the multilayer structure and the phonon modes of VO2. When experiencing the VO2 phase transition, the radiative power of the proposed coating achieves a 6.5 fold enhancement for extraterrestrial spacecraft systems, and 7.3 fold enhancement for terrestrial systems such as buildings, making it a promising choice for dynamic radiative cooling applications in a variable environment. The findings here will facilitate research and development of novel coating materials for radiative cooling applications.

  9. Randomly Distributed Fabry-Pérot-type Metal Nanowire Resonators and Their Lasing Action

    Science.gov (United States)

    Kwon, Kyungmok; Jung, Youngho; Kim, Minkyung; Shim, Jaeho; Yu, Kyoungsik

    2016-04-01

    Optical feedback mechanisms are often obtained from well-defined resonator structures fabricated by top-down processes. Here, we demonstrate that two-dimensional networks of metallic nanowires dispersed on the semiconductor slab can provide strong in-plane optical feedback and, thus, form randomly-distributed Fabry-Pérot-type resonators that can achieve multi- or single-mode lasing action in the near infrared wavelengths. Albeit with their subwavelength-scale cross-sections and uncontrolled inter-nanowire distances, a cluster of nearly parallel metal nanowires acts as an effective in-situ reflector for the semiconductor-metal slab waveguide modes for coherent optical feedback in the lateral direction. Fabry-Pérot type resonance can be readily developed by a pair of such clusters coincidentally formed in the solution-processed random nanowire network. Our low-cost and large-area approach for opportunistic random cavity formation would open a new pathway for integrated planar light sources for low-coherence imaging and sensing applications.

  10. Voltage adjusting characteristics in terahertz transmission through Fabry-Pérot-based metamaterials

    Directory of Open Access Journals (Sweden)

    Jun Luo

    2015-10-01

    Full Text Available Metallic electric split-ring resonators (SRRs with featured size in micrometer scale, which are connected by thin metal wires, are patterned to form a periodically distributed planar array. The arrayed metallic SRRs are fabricated on an n-doped gallium arsenide (n-GaAs layer grown directly over a semi-insulating gallium arsenide (SI-GaAs wafer. The patterned metal microstructures and n-GaAs layer construct a Schottky diode, which can support an external voltage applied to modify the device properties. The developed architectures present typical functional metamaterial characters, and thus is proposed to reveal voltage adjusting characteristics in the transmission of terahertz waves at normal incidence. We also demonstrate the terahertz transmission characteristics of the voltage controlled Fabry-Pérot-based metamaterial device, which is composed of arrayed metallic SRRs. To date, many metamaterials developed in earlier works have been used to regulate the transmission amplitude or phase at specific frequencies in terahertz wavelength range, which are mainly dominated by the inductance-capacitance (LC resonance mechanism. However, in our work, the external voltage controlled metamaterial device is developed, and the extraordinary transmission regulation characteristics based on both the Fabry-Pérot (FP resonance and relatively weak surface plasmon polariton (SPP resonance in 0.025-1.5 THz range, are presented. Our research therefore shows a potential application of the dual-mode-resonance-based metamaterial for improving terahertz transmission regulation.

  11. 初级馈源位置对Fabry-Perot谐振天线性能的影响%Effect of Location of Primary Source on Fabry-Perot Resonator Antenna

    Institute of Scientific and Technical Information of China (English)

    刘震国

    2011-01-01

    通过平面波叠加法,分析了初级馈源位置对PEC和PMC作为接地板的2类Fabry-Perot谐振天线的最大方向性系数及其带宽的影响.分析表明初级馈源位置在满足特定条件取值时,Fabry-Perot谐振天线将获得最大的定向性,但其定向性带宽并不随馈源位置有明显改变.同时,首先指出定向性与其带宽的乘积仅是Fabry-Perot谐振天线的反射盖板其反射系数模的函数,并随着反射系数模的增加而趋于某一定值.最后进行了仿真验证,仿真结果与理论计算具有很好的一致性,对Fabry-Perot谐振天线的设计具有很好的理论指导意义.%The directivity and its bandwidth properties of Fabry-Perot resonator antennas made of two different ground pWe covered with a pautially-reflecting surface are studied theoretically, respectively. The relationship between the directivity and the primary source location is also obtained. It is shown that the directivity of Fabry-Perot resonator will obtain its maximum when primary source is located吐 certain of position for different type of ground plate. But the directivity bandwidth will not very obviously with the source location. At the same time the normalized product of maxium of directivity and its handwidth is a function of reflection coefficient of partially reflective 8Urface, which trends to certain constant with increase of reflection coefficient. Finally the simulated results of planar Fabry-Perot resonator antenna are validated to have good agreement with the calculated theoretically.

  12. Clinical, biochemical and genetic heterogeneity in lysosomal storage diseases

    NARCIS (Netherlands)

    A.J.J. Reuser (Arnold)

    1977-01-01

    textabstractThe history of lysosomal storage diseases dates back to the end of the last century when the first clinical reports appeared of patients suffering from these genetic, metabolic disorders (Tay, 1881; Gaucher, 1882; Sachs, 1887; Fabry, 1898). About seventy years wouid pass before the term

  13. Long-term instrumental parameter investigation of a Fabry-Perot spectrometer at an isolated field station.

    Science.gov (United States)

    Hernandez, G; McCarthy, M P

    2011-05-01

    To insure that long-term determinations of Doppler width and shift--derived from observations of atmospheric emissions--are internally consistent and reliable, we have developed a method to both continuously and nonintrusively determine and monitor the instrumental constants of the Fabry-Perot spectrometer making the observations. We have used this method at our isolated field experiment at South Pole, Antarctica, because the instrument is only accessible to us for a few days every year. Here we report both the method and the Fabry-Perot stability results for the past 22 years of operation. The method involves the description of real Fabry-Perot instrumental constants as a small departure from those of an ideal Fabry-Perot. In general, this model is applicable for most observations. However, experimentally, there are times when the small-departure model is not applicable, thus indicating how to best reduce the observations into physical quantities for the utmost consistency in the geophysical results.

  14. Power-ratio tunable dual-wavelength laser using linearly variable Fabry-Perot filter as output coupler.

    Science.gov (United States)

    Wang, Xiaozhong; Wang, Zhongfa; Bu, Yikun; Chen, Lujian; Cai, Guoxiong; Huang, Wencai; Cai, Zhiping; Chen, Nan

    2016-02-01

    For a linearly variable Fabry-Perot filter, the peak transmission wavelengths change linearly with the transverse position shift of the substrate. Such a Fabry-Perot filter is designed and fabricated and used as an output coupler of a c-cut Nd:YVO4 laser experimentally in this paper to obtain a 1062 and 1083 nm dual-wavelength laser. The peak transmission wavelengths are gradually shifted from 1040.8 to 1070.8 nm. The peak transmission wavelength of the Fabry-Perot filter used as the output coupler for the dual-wavelength laser is 1068 nm and resides between 1062 and 1083 nm, which makes the transmissions of the desired dual wavelengths change in opposite slopes with the transverse shift of the filter. Consequently, powers of the two wavelengths change in opposite directions. A branch power, oppositely tunable 1062 and 1083 nm dual-wavelength laser is successfully demonstrated. Design principles of the linear variable Fabry-Perot filter used as an output coupler are discussed. Advantages of the method are summarized.

  15. Amelioration of serum 8-OHdG level by enzyme replacement therapy in patients with Fabry cardiomyopathy.

    Science.gov (United States)

    Chen, Kuan-Hsuan; Chou, Yueh-Ching; Hsiao, Chen-Yuan; Chien, Yueh; Wang, Kang-Ling; Lai, Ying-Hsiu; Chang, Yuh-Lih; Niu, Dau-Ming; Yu, Wen-Chung

    2017-04-29

    The level of 8-hydroxy-2-deoxyguanosise (8-OHdG) is a marker of oxidative stress. The objective of this study was to evaluate the effect of enzyme replacement therapy (ERT) on the level of 8-OHdG in patients with Fabry cardiomyopathy and the clinical evolution of Fabry cardiomyopathy. We measured the serum levels of 8-OHdG in 20 healthy control and 22 patients with Fabry cardiomyopathy before and after ERT. The mean lysoGb3 and 8-OHdG levels was significantly increased in patients with Fabry cardiomyopathy compared with that of control subjects (lysoGb3, 3.6 ± 1.1 nM vs. 0.4 ± 0.1 nM, p cardiomyopathy (FC) and that successful management of FC with ERT is associated with a decrease in this oxidative stress marker. Serum 8-OHdG levels can be used not only as a noninvasive biomarker of oxidative stress in patients with FC but also an objective and quantitative parameter in the follow-up of patients during ERT. Copyright © 2017. Published by Elsevier Inc.

  16. Reduce of the Linewidth of a Diode Laser by Locking to a High-Finesse Fabry-Perot Cavity

    Institute of Scientific and Technical Information of China (English)

    HUANG Kai-Kai; ZHANG Jian-Wei; CHEN Jing-Biao; YANG Dong-Hai

    2006-01-01

    @@ We report frequency locking of a commercial 657nm diode laser to a high finesse Fabry-Perot cavity by the Pound-Drever-Hall method. The laser linewidth relative to the cavity is estimated to be about 6 kHz.

  17. Lineshape Engineering in an All-Pass Ring Resonator with Backreflection Coupled to a Symmetrical Fabry-Perot Resonator

    KAUST Repository

    Melnikov, Vasily

    2012-11-10

    We derive transfer functions for an all-pass ring resonator with internal backreflection coupled to a symmetrical Fabry-Perot resonator and demonstrate electromagnetically induced transparency-like and Fano-like lineshapes tunable by backreflection in the ring resonator.

  18. The comparison of environmental effects on michelson and fabry-perot interferometers utilized for the displacement measurement.

    Science.gov (United States)

    Wang, Yung-Cheng; Shyu, Lih-Horng; Chang, Chung-Ping

    2010-01-01

    The optical structure of general commercial interferometers, e.g., the Michelson interferometers, is based on a non-common optical path. Such interferometers suffer from environmental effects because of the different phase changes induced in different optical paths and consequently the measurement precision will be significantly influenced by tiny variations of the environmental conditions. Fabry-Perot interferometers, which feature common optical paths, are insensitive to environmental disturbances. That would be advantageous for precision displacement measurements under ordinary environmental conditions. To verify and analyze this influence, displacement measurements with the two types of interferometers, i.e., a self-fabricated Fabry-Perot interferometer and a commercial Michelson interferometer, have been performed and compared under various environmental disturbance scenarios. Under several test conditions, the self-fabricated Fabry-Perot interferometer was obviously less sensitive to environmental disturbances than a commercial Michelson interferometer. Experimental results have shown that induced errors from environmental disturbances in a Fabry-Perot interferometer are one fifth of those in a Michelson interferometer. This has proved that an interferometer with the common optical path structure will be much more independent of environmental disturbances than those with a non-common optical path structure. It would be beneficial for the solution of interferometers utilized for precision displacement measurements in ordinary measurement environments.

  19. Ff-nano, Short Functionalized Nanorods Derived from Ff (f1, fd or M13 Filamentous Bacteriophage

    Directory of Open Access Journals (Sweden)

    Sadia eSattar

    2015-04-01

    Full Text Available F-specific filamentous phage of Escherichia coli (Ff: f1, M13 or fd are long thin filaments (860 nm x 6 nm. They have been a major workhorse in display technologies and bionanotechnology; however, some applications are limited by the high length-to-diameter ratio of Ff. Furthermore, use of functionalized Ff outside of laboratory containment is in part hampered by the fact that they are genetically modified viruses. We have now developed a system for production and purification of very short functionalized Ff-phage-derived nanorods, named Ff-nano, that are only 50 nm in length. In contrast to standard Ff-derived vectors that replicate in E. coli and contain antibiotic-resistance genes, Ff-nano are protein DNA complexes that cannot replicate on their own and do not contain any coding sequences. These nanorods show an increased resistance to heating at 70 °C in 1 % SDS in comparison to the full-length Ff phage of the same coat composition. We demonstrate that functionalized Ff-nano particles are suitable for application as detection particles in sensitive and quantitative dipstick lateral flow diagnostic assay for human plasma fibronectin.

  20. Quantitation of polycyclic aromatic hydrocarbons (PAH4) in cocoa and chocolate samples by an HPLC-FD method.

    Science.gov (United States)

    Raters, Marion; Matissek, Reinhard

    2014-11-05

    As a consequence of the PAH4 (sum of four different polycyclic aromatic hydrocarbons, named benzo[a]anthracene, chrysene, benzo[b]fluoranthene, and benzo[a]pyrene) maximum levels permitted in cocoa beans and derived products as of 2013, an high-performance liquid chromatography with fluorescence detection method (HPLC-FD) was developed and adapted to the complex cocoa butter matrix to enable a simultaneous determination of PAH4. The resulting analysis method was subsequently successfully validated. This method meets the requirements of Regulation (EU) No. 836/2011 regarding analysis methods criteria for determining PAH4 and is hence most suitable for monitoring the observance of the maximum levels applicable under Regulation (EU) No. 835/2011. Within the scope of this work, a total of 218 samples of raw cocoa, cocoa masses, and cocoa butter from several sample years (1999-2012), of various origins and treatments, as well as cocoa and chocolate products were analyzed for the occurrence of PAH4. In summary, it is noted that the current PAH contamination level of cocoa products can be deemed very slight overall.

  1. Ff-nano, short functionalized nanorods derived from Ff (f1, fd, or M13) filamentous bacteriophage.

    Science.gov (United States)

    Sattar, Sadia; Bennett, Nicholas J; Wen, Wesley X; Guthrie, Jenness M; Blackwell, Len F; Conway, James F; Rakonjac, Jasna

    2015-01-01

    F-specific filamentous phage of Escherichia coli (Ff: f1, M13, or fd) are long thin filaments (860 nm × 6 nm). They have been a major workhorse in display technologies and bionanotechnology; however, some applications are limited by the high length-to-diameter ratio of Ff. Furthermore, use of functionalized Ff outside of laboratory containment is in part hampered by the fact that they are genetically modified viruses. We have now developed a system for production and purification of very short functionalized Ff-phage-derived nanorods, named Ff-nano, that are only 50 nm in length. In contrast to standard Ff-derived vectors that replicate in E. coli and contain antibiotic-resistance genes, Ff-nano are protein-DNA complexes that cannot replicate on their own and do not contain any coding sequences. These nanorods show an increased resistance to heating at 70(∘)C in 1% SDS in comparison to the full-length Ff phage of the same coat composition. We demonstrate that functionalized Ff-nano particles are suitable for application as detection particles in sensitive and quantitative "dipstick" lateral flow diagnostic assay for human plasma fibronectin.

  2. Cytostatic activity of the duplex drug linking 2'-deoxy-5-fluorouridine (5FdU) with 3'-C-ethynylcytidine (ECyd) against gastric adenocarcinoma cell lines.

    Science.gov (United States)

    Weinreich, Jürgen; Schott, Sarah; Königsrainer, Ingmar; Zieker, Derek; Königsrainer, Alfred; Schott, Herbert

    2011-12-01

    The cytostatic potential of the new duplex drug 2'-deoxy-5-fluorouridylyl-(5'5')-3'-C-ethynylcytidine (5FdU(5'-5')ECyd) was evaluated in comparison to those of 5-fluorouracil (5FU), 2'-deoxy-5-fluorourindine (5FdU), 3'-C-ethynylycytidine (ECyd), cisplatin, an equimolar mixture of 5FdU + ECyd and a three component-mixture of 0.75 μM epirubicin/0.90 μM cisplatin/3.0 μM 5FU (ECF) by incubation of the two human gastric adenocarcinoma cell lines 23132/87 and MKN-45. The molar composition of ECF was taken from data of a triple combination chemotherapy for human gastric cancer. Time and dose depending inhibition of cell growth was determinated using the CASY technology. A growth decrease of both cell lines from 100% to about 20% was observed by treatment with ECF over a course of 14 days. This result provided basis to estimate the cytostatic potential of all tested drugs and combinations thereof. Corresponding high activities in respect to ECF were achieved by incubation of 23132/87 cells with single drugs 49 μM 5FU, 10 μM cisplatin, 3.4 μM 5FdU, 0.65 μM ECyd, the mixture 0.32 μM 5FdU + 0.32 μM ECyd and 0.32 μM 5FdU(5'-5')ECyd. The less sensitive MKN-45 cells require a 1.5-4 fold higher dose of the standard chemotherapeutics in order to achieve an equivalent cytostatic effect, in respect to the 23132/87 cell line,. However, the effect of the duplex drugs on MKN-45 cells was gained with a 5-fold lower dose than ECF. Due to its high cytostatic potential the duplex drug, which covalently links two active anticancer compounds, could be a new therapeutic alternative for chemotherapy in gastric cancer, currently treated with different combinations.

  3. MIMO-OFDM系统的IFD-SAGE信道估计算法%Improved FD-SAGE channel estimation algorithm for MIMO-OFDM system

    Institute of Scientific and Technical Information of China (English)

    高敬鹏; 赵旦峰; 周相超; 付芳

    2013-01-01

    针对MIMO-OFDM系统中频域的空间交替广义期望最大化(FD-SAGE)算法估计信道性能较差以及收敛速度慢的问题,提出了一种改进的FD-SAGE信道估计算法。该算法在FD-SAGE算法的基础上,通过对SAGE算法的潜在数据和不完全数据进行分解分析推导出一种修正的SAGE算法,同时在SAGE的更新数据信息时引入最大似然算法,进而提高系统的可靠性。理论研究和仿真结果表明,该算法以牺牲少量复杂度为代价,能较好地追踪信道变化且收敛速度较快,其性能优于传统的LS算法,信号检测采用最大似然算法时,在相同误比特率情况下与理想信道估计仅相差0.5 dB。%Aiming at the poor performance of Frequency Domain Space-Alternating Generalized Expectation-Maximization (FD-SAGE)algorithm in channel estimation and the slow convergence speed in the MIMO-OFDM system, this paper presents an improved FD-SAGE channel estimate algorithm. On the basis of FD-SAGE algorithm, a modified SAGE algorithm is derived through decomposing and analyzing the potential and incomplete data of SAGE algorithm. Meanwhile, the maximum likelihood algorithm is introduced in the process of refreshing data information in SAGE algorithm, and then the reliability of system is improved. The theoretical analysis and simulation results show that this algorithm can better track the channel change and acquire a faster convergence speed, just in cost of a slight system complexity. The performance is better than the traditional Least Square (LS)algorithm. Comparing with the ideal channel estimation under the maximum likelihood algorithm in signal detection, the new proposed algorithm has only a loss of 0.5 dB with the same bit error rate.

  4. The technique of detecting the laser-ultrasonic vector displacement with a confocal Fabry-Perot interferometer waves in a plate

    Institute of Scientific and Technical Information of China (English)

    PAN Yongdong; QIAN Menglu

    2003-01-01

    Generally, a confocal Fabry-Perot interferometer is only able to detect the out-of-plane component of a displacement field; while the in-plane component often has the information about the material which cannot be found in this out-of-plane component. In this paper, based on a confocal Fabry-Perot interferometer set-up for detecting the out-of-plane component of a laser generated acoustic field, a technique is developed to detect both the out-of-plane and inplane displacement components simultaneously with a novel two-channel confocal Fabry-Perot interferometer.

  5. An RBF-FD polynomial method based on polyharmonic splines for the Navier-Stokes equations: Comparisons on different node layouts

    CERN Document Server

    Barnett, Gregory A; Wicker, Louis J

    2015-01-01

    Polyharmonic spline (PHS) radial basis functions (RBFs) are used together with polynomials to create local RBF-finite-difference (RBF-FD) weights on different node layouts for spatial discretization of the compressible Navier-Stokes equations at low Mach number, relevant to atmospheric flows. Test cases are taken from the numerical weather prediction community and solved on bounded domains. Thus, attention is given on how to handle boundaries with the RBF-FD method, as well as a novel implementation for the presented approach. Comparisons are done on Cartesian, hexagonal, and quasi-uniformly scattered node layouts. Since RBFs are independent of a coordinate system (and only depend on the distance between nodes), changing the node layout amounts to changing one line of code. In addition, consideration and guidelines are given on PHS order, polynomial degree and stencil size. The main advantages of the present method are: 1) capturing the basic physics of the problem surprisingly well, even at very coarse resol...

  6. Effect of the back bias on the analog performance of standard FD and UTBB transistors-based self-cascode structures

    Science.gov (United States)

    Doria, Rodrigo T.; Flandre, Denis; Trevisoli, Renan; de Souza, Michelly; Pavanello, Marcelo A.

    2017-09-01

    This work demonstrates that active back biasing can improve significantly the analog performance of two-transistors self-cascode structures. The study was performed by applying both standard and UTBB fully depleted (FD) SOI MOSFETs to the structures and has shown that a voltage gain improvement of about 7 dB is obtained when a forward back bias is applied to the drain-sided transistor of standard FD devices-based structure. In the case of UTBB transistors, an improvement larger than 5 dB of the output voltage gain is shown depending on the back bias applied to both n- or p-type devices. Finally, it is shown that the mirroring precision of current mirrors composed by SC structures can be more than 20% better than the one composed by single devices and the improvement is better when adequate back bias is applied.

  7. Homologs of FT, CEN and FD respond to developmental and environmental signals affecting growth and flowering in the perennial vine kiwifruit.

    Science.gov (United States)

    Varkonyi-Gasic, Erika; Moss, Sarah M A; Voogd, Charlotte; Wang, Tianchi; Putterill, Joanna; Hellens, Roger P

    2013-05-01

    FLOWERING LOCUS T (FT) and CENTRORADIALIS (CEN) homologs have been implicated in regulation of growth, determinacy and flowering. The roles of kiwifruit FT and CEN were explored using a combination of expression analysis, protein interactions, response to temperature in high-chill and low-chill kiwifruit cultivars and ectopic expression in Arabidopsis and Actinidia. The expression and activity of FT was opposite from that of CEN and incorporated an interaction with a FLOWERING LOCUS D (FD)-like bZIP transcription factor. Accumulation of FT transcript was associated with plant maturity and particular stages of leaf, flower and fruit development, but could be detected irrespective of the flowering process and failed to induce precocious flowering in transgenic kiwifruit. Instead, transgenic plants demonstrated reduced growth and survival rate. Accumulation of FT transcript was detected in dormant buds and stem in response to winter chilling. In contrast, FD in buds was reduced by exposure to cold. CEN transcript accumulated in developing latent buds, but declined before the onset of dormancy and delayed flowering when ectopically expressed in kiwifruit. Our results suggest roles for FT, CEN and FD in integration of developmental and environmental cues that affect dormancy, budbreak and flowering in kiwifruit.

  8. Automated Phase Mapping with AgileFD and its Application to Light Absorber Discovery in the V-Mn-Nb Oxide System.

    Science.gov (United States)

    Suram, Santosh K; Xue, Yexiang; Bai, Junwen; Le Bras, Ronan; Rappazzo, Brendan; Bernstein, Richard; Bjorck, Johan; Zhou, Lan; van Dover, R Bruce; Gomes, Carla P; Gregoire, John M

    2017-01-09

    Rapid construction of phase diagrams is a central tenet of combinatorial materials science with accelerated materials discovery efforts often hampered by challenges in interpreting combinatorial X-ray diffraction data sets, which we address by developing AgileFD, an artificial intelligence algorithm that enables rapid phase mapping from a combinatorial library of X-ray diffraction patterns. AgileFD models alloying-based peak shifting through a novel expansion of convolutional nonnegative matrix factorization, which not only improves the identification of constituent phases but also maps their concentration and lattice parameter as a function of composition. By incorporating Gibbs' phase rule into the algorithm, physically meaningful phase maps are obtained with unsupervised operation, and more refined solutions are attained by injecting expert knowledge of the system. The algorithm is demonstrated through investigation of the V-Mn-Nb oxide system where decomposition of eight oxide phases, including two with substantial alloying, provides the first phase map for this pseudoternary system. This phase map enables interpretation of high-throughput band gap data, leading to the discovery of new solar light absorbers and the alloying-based tuning of the direct-allowed band gap energy of MnV2O6. The open-source family of AgileFD algorithms can be implemented into a broad range of high throughput workflows to accelerate materials discovery.

  9. Measurement of reflection phase using thick-gap Fabry-Perot etalon.

    Science.gov (United States)

    Yung, Tsz Kit; Gao, Wensheng; Leung, Ho Ming; Zhao, Qiuling; Wang, Xia; Tam, Wing Yim

    2016-09-10

    We report measurement of the reflection phase of a dielectric (glass)/titanium (Ti) surface in the visible wavelength using a thick-gap Fabry-Perot (FP) interferometry technique. Using a two-beam interference model for the reflection peaks and troughs of the FP etalon, we obtain the air-gap spacing of the etalon and, more importantly, the reflection phase of the etalon substrate. We find systematic dependence of the as-measured reflection phase on the air-gap spacing due to the numerical aperture effect of the measuring objective. However, the relative reflection phase of Ti with respect to glass is independent of the air-gap spacing. As a demonstration of our approach in the optical characterization of small metamaterial samples, we also measure the reflection phase of a micron-sized 2D Au sawtooth nanoarray. The experiment is in good agreement with the model simulation.

  10. On-chip optical phase locking of single growth monolithically integrated Slotted Fabry Perot lasers.

    Science.gov (United States)

    Morrissey, P E; Cotter, W; Goulding, D; Kelleher, B; Osborne, S; Yang, H; O'Callaghan, J; Roycroft, B; Corbett, B; Peters, F H

    2013-07-15

    This work investigates the optical phase locking performance of Slotted Fabry Perot (SFP) lasers and develops an integrated variable phase locked system on chip for the first time to our knowledge using these lasers. Stable phase locking is demonstrated between two SFP lasers coupled on chip via a variable gain waveguide section. The two lasers are biased differently, one just above the threshold current of the device with the other at three times this value. The coupling between the lasers can be controlled using the variable gain section which can act as a variable optical attenuator or amplifier depending on bias. Using this, the width of the stable phase locking region on chip is shown to be variable.

  11. Waveguide grating mirror in a fully suspended 10 meter Fabry-Perot cavity

    CERN Document Server

    Friedrich, Daniel; Brückner, Frank; Hild, Stefan; Nelson, John; Mcarthur, John; Plissi, Michael V; Edgar, Matthew P; Huttner, Sabina H; Sorazu, Borja; Kroker, Stefanie; Britzger, Michael; Kley, Ernst-Bernhard; Danzmann, Karsten; Tünnermann, Andreas; Strain, Ken A; Schnabel, Roman

    2011-01-01

    We report on the first demonstration of a fully suspended 10m Fabry-Perot cavity incorporating a waveguide grating as the coupling mirror. The cavity was kept on resonance by reading out the length fluctuations via the Pound-Drever-Hall method and employing feedback to the laser frequency. From the achieved finesse of 790 the grating reflectivity was determined to exceed 99.2% at the laser wavelength of 1064\\,nm, which is in good agreement with rigorous simulations. Our waveguide grating design was based on tantala and fused silica and included a ~20nm thin etch stop layer made of Al2O3 that allowed us to define the grating depth accurately during the fabrication process. Demonstrating stable operation of a waveguide grating featuring high reflectivity in a suspended low-noise cavity, our work paves the way for the potential application of waveguide gratings as mirrors in high-precision interferometry, for instance in future gravitational wave observatories.

  12. Fabry-Perot microcavity sensor for H2-breath-test analysis

    Science.gov (United States)

    Vincenti, Maria Antonietta; De Sario, Marco; Petruzzelli, V.; D'Orazio, Antonella; Prudenzano, Francesco; de Ceglia, Domenico; Scalora, Michael

    2007-10-01

    Leak detection of hydrogen for medical purposes, based on the monitoring of the optical response of a simple Fabry-Perot microcavity, is proposed to investigate either the occurrence of lactose intolerance, or lactose malabsorption condition. Both pathologic conditions result in bacterial overgrowth in the intestine, which causes increased spontaneous emission of H2 in the human breath. Two sensitivity figures of merit are introduced to inspect changes in the sensor response, and to relate the microcavity response to a pathologic condition, which is strictly related to a different level of exhaled hydrogen. Different sensor configurations using a metal-dielectric microcavity are reported and discussed in order to make the most of the well-known ability of palladium to spontaneously absorb hydrogen.

  13. Surface-Plasmon-Polariton Laser based on an Open-Cavity Fabry-Perot Resonator

    CERN Document Server

    Zhu, Wenqi; Agrawal, Amit; Lezec, Henri J

    2016-01-01

    Recent years have witnessed growing interest in the development of small-footprint lasers for potential applications in small-volume sensing and on-chip optical communications. Surface-plasmons, electromagnetic modes evanescently confined to metal-dielectric interfaces, offer an effective route to achieving lasing at nanometer-scale dimensions when resonantly amplified in contact with a gain-medium. Here, we achieve visible frequency ultra-narrow linewidth lasing at room-temperature by leveraging surface plasmons propagating in an open Fabry-Perot cavity formed by a flat metal surface coated with a subwavelength-thick layer of optically-pumped gain medium and orthogonally bound by a pair of flat metal sidewalls. Low perturbation transmission-configuration sampling of the lasing plasmon mode is achieved via an evanescently coupled recessed nanoslit, opening the way to high-figure-of-merit refractive-index sensing of analytes interacting with the open cavity.

  14. Fabry-Perot based metal-dielectric multilayered filters and metamaterials.

    Science.gov (United States)

    Jen, Yi-Jun; Lee, Cheng-Chung; Lu, Kun-Han; Jheng, Ci-Yao; Chen, Yu-Jen

    2015-12-28

    The traditional three-layered metal-dielectric-metal Fabry-Perot filter is developed as a new metal-dielectric multilayered band-pass filter. Our design method allows metal and dielectric films to be alternatively arranged to achieve a narrow and high transmission peak and the peak height remains unchanged for any number of metal films arranged in the multilayer. Furthermore, the equivalent refractive index of a subwavelength metal-dielectric multilayer could be negative real at the passband of the filter and such metamaterial exhibits stronger figure of merit than a previous result. By choosing a material with high refractive index as the dielectric film, such metamaterial exhibits a pass band that depends weakly on the angle of incidence.

  15. Graphene-based electromagnetically induced transparency with coupling Fabry-Perot resonators.

    Science.gov (United States)

    Zhuang, Huawei; Kong, Fanmin; Li, Kang; Sheng, Shiwei

    2015-08-20

    We investigate the plasmonic analog of electromagnetically induced transparency (EIT) using two adjacent graphene-based Fabry-Perot (F-P) resonators side coupling to a nanoribbon waveguide. By the coupling mode theory in time and F-P resonant model, the destructive interference from the coupling of the two F-P resonators results in the EIT-like optical response. The induced peak and width of the transparency window can be dynamically manipulated by varying the coupling distance of the two resonators, and the transparent window is easily shifted by tuning the resonator length or the chemical potential of the graphene nanoribbon. In order to verify the characteristics of slow light, the group index profile is analyzed at different coupling distances. The proposed graphene-based EIT-like system could open up new opportunities for potential applications in plasmonic slow light and optical information buffering devices.

  16. Dual-band Fabry-Perot lasing from single ZnO microbelt

    Science.gov (United States)

    Zhu, Qiuxiang; Qin, Feifei; Lu, Junfeng; Zhu, Zhu; Shi, Zengliang; Xu, Chunxiang

    2016-10-01

    Dual-band semiconductor microbelt lasing are promising for multifunctional applications ranging from optical communication to spectroscopy analysis. Here, we demonstrated a dual-band Fabry-Perot (F-P) lasing from both length and width directions in a single ZnO microbelt. The lasing performance, spectral variation and mode structure significantly depended on the cavity size, which corresponded to the length and width of the ZnO microbelts. The resonant process and mechanism were investigated systematically through the experimental analysis and numerically FDTD simulation. The results of the dual-band F-P lasing modes and wide lasing wavelength are helpful to design the dual-wavelength electronic and optoelectronic devices.

  17. Study of Antenna Superstrates Using Metamaterials for Directivity Enhancement Based on Fabry-Perot Resonant Cavity

    Directory of Open Access Journals (Sweden)

    Haixia Liu

    2013-01-01

    Full Text Available Metamaterial superstrate is a significant method to obtain high directivity of one or a few antennas. In this paper, the characteristics of directivity enhancement using different metamaterial structures as antenna superstrates, such as electromagnetic bandgap (EBG structures, frequency selective surface (FSS, and left-handed material (LHM, are unifiedly studied by applying the theory of Fabry-Perot (F-P resonant cavity. Focusing on the analysis of reflection phase and magnitude of superstrates in presently proposed designs, the essential reason for high-directivity antenna with different superstrates can be revealed in terms of the F-P resonant theory. Furthermore, a new design of the optimum reflection coefficient of superstrates for the maximum antenna directivity is proposed and validated. The optimum location of the LHM superstrate which is based on a refractive lens model can be determined by the F-P resonant distance.

  18. Phase Space of Tristability in Dual Injection-Locked Fabry-Perot Laser Diodes

    Directory of Open Access Journals (Sweden)

    R. V. Pajković

    2015-06-01

    Full Text Available We investigate theoretically the case of dual injection-locking, in which the two light signals are simultaneously externally injected into the cavity of a slave Fabry-Perot laser diode. We show that dual injection-locking leads to formation of new stationary points, and potentially to optical tristability of the slave laser. We show that a region in which the slave laser exhibits three stable steady-states occurs only for sufficiently different frequency detunings of the two injection signals. Moreover, the slave laser tunability depends on the choice of injection modes, and strongly depends on the slave laser bias current, with an optimal value around 2 times threshold current.

  19. The PVLAS experiment: measuring vacuum magnetic birefringence and dichroism with a birefringent Fabry-Perot cavity

    CERN Document Server

    Della Valle, F; Ejlli, A; Gastaldi, U; Messineo, G; Zavattini, G; Pengo, R; Ruoso, G

    2015-01-01

    Vacuum magnetic birefringence was predicted long time ago and is still lacking a direct experimental confirmation. Several experimental efforts are striving to reach this goal, and the sequence of results promises a success in the next few years. This measurement generally is accompanied by the search for hypothetical light particles that couple to two photons. The PVLAS experiment employs a sensitive polarimeter based on a high finesse Fabry-Perot cavity. In this paper we report on the latest experimental results of this experiment. The data are analysed taking into account the intrinsic birefringence of the dielectric mirrors of the cavity. Besides the limit on the vacuum magnetic birefringence, the measurements also allow the model-independent exclusion of new regions in the parameter space of axion-like and milli-charged particles. In particular, these last limits hold also for all types of neutrinos, resulting in a laboratory limit on their charge.

  20. Sapphire hard X-ray Fabry-Perot resonators for synchrotron experiments.

    Science.gov (United States)

    Tsai, Yi Wei; Wu, Yu Hsin; Chang, Ying Yi; Liu, Wen Chung; Liu, Hong Lin; Chu, Chia Hong; Chen, Pei Chi; Lin, Pao Te; Fu, Chien Chung; Chang, Shih Lin

    2016-05-01

    Hard X-ray Fabry-Perot resonators (FPRs) made from sapphire crystals were constructed and characterized. The FPRs consisted of two crystal plates, part of a monolithic crystal structure of Al2O3, acting as a pair of mirrors, for the backward reflection (0 0 0 30) of hard X-rays at 14.3147 keV. The dimensional accuracy during manufacturing and the defect density in the crystal in relation to the resonance efficiency of sapphire FPRs were analyzed from a theoretical standpoint based on X-ray cavity resonance and measurements using scanning electron microscopic and X-ray topographic techniques for crystal defects. Well defined resonance spectra of sapphire FPRs were successfully obtained, and were comparable with the theoretical predictions.