Maniac Talk - Charles Ichoku

NASA Image and Video Library

2016-07-25

Charles Ichoku Maniac Lecture, July 25, 2016 NASA climate scientist Charles Ichoku presented a Maniac lecture entitled, "Reminiscences of a scientist's journey from Nawfia to NASA." Born in a small town in Nigeria, Charles traced his captivating journey to NASA, which was full of surprises, and related his experiences with the great people he met and interacted with along the way, as well as some of his work.

Non-specific gastrointestinal features: Could it be Fabry disease?

PubMed

Hilz, Max J; Arbustini, Eloisa; Dagna, Lorenzo; Gasbarrini, Antonio; Goizet, Cyril; Lacombe, Didier; Liguori, Rocco; Manna, Raffaele; Politei, Juan; Spada, Marco; Burlina, Alessandro

2018-05-01

Non-specific gastrointestinal symptoms, including pain, diarrhoea, nausea, and vomiting, can be the first symptoms of Fabry disease. They may suggest more common disorders, e.g. irritable bowel syndrome or inflammatory bowel disease. The confounding clinical presentation and rarity of Fabry disease often cause long diagnostic delays and multiple misdiagnoses. Therefore, specialists involved in the clinical evaluation of non-specific upper and lower gastrointestinal symptoms should recognize Fabry disease as a possible cause of the symptoms, and should consider Fabry disease as a possible differential diagnosis. When symptoms or family history suggest Fabry disease, in men, low alpha-galactosidase A enzyme levels, and in women, specific Fabry mutations confirm the diagnosis. In addition to symptomatic treatments, disease-specific enzyme replacement therapy with recombinant human alpha-galactosidase A enzyme or chaperone therapy (migalastat) in patients with amenable mutations can improve the disease, including gastrointestinal symptoms, and should be initiated as early as possible after Fabry disease has been confirmed; starting enzyme replacement therapy at as young an age as possible after diagnosis improves long-term clinical outcomes. Improved diagnostic tools, such as a modified gastrointestinal symptom rating scale, may facilitate diagnosing Fabry disease in patients with gastrointestinal symptoms of unknown cause and thus assure timely initiation of disease-specific treatment. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

Echocardiographic Assessment of Patients with Fabry Disease.

PubMed

Yeung, Darwin F; Sirrs, Sandra; Tsang, Michael Y C; Gin, Kenneth; Luong, Christina; Jue, John; Nair, Parvathy; Lee, Pui K; Tsang, Teresa S M

2018-06-01

Fabry disease is an X-linked lysosomal storage disorder that results from a deficiency of α-galactosidase A. Increased left ventricular wall thickness has been the most commonly described cardiovascular manifestation of the disease. However, a variety of other structural and functional abnormalities have also been reported. Echocardiography is an effective noninvasive method of assessing the cardiac involvement of Fabry disease. A more precise and comprehensive characterization of Fabry cardiomyopathy using conventional and novel echocardiographic techniques may lead to earlier diagnosis, more accurate prognostication, and timely treatment. The aim of this review is to provide a comprehensive overview of the structural and functional abnormalities on echocardiography that have thus far been described in patients with Fabry disease and to highlight potential areas that would benefit from further research. Copyright © 2018 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

Strained layer Fabry-Perot device

DOEpatents

Brennan, Thomas M.; Fritz, Ian J.; Hammons, Burrell E.

1994-01-01

An asymmetric Fabry-Perot reflectance modulator (AFPM) consists of an active region between top and bottom mirrors, the bottom mirror being affixed to a substrate by a buffer layer. The active region comprises a strained-layer region having a bandgap and thickness chosen for resonance at the Fabry-Perot frequency. The mirrors are lattice matched to the active region, and the buffer layer is lattice matched to the mirror at the interface. The device operates at wavelengths of commercially available semiconductor lasers.

Fabry disease in children: a federal screening programme in Russia.

PubMed

Namazova-Baranova, Leyla Seymurovna; Baranov, Alexander Alexandrovich; Pushkov, Aleksander Alekseevich; Savostyanov, Kirill Victorovich

2017-10-01

Our objective was to examine the prevalence of Fabry disease in Russian children with chronic pain in the distal limbs. This non-interventional, multi-centre study included children 2-18 years of age with chronic recurrent unilateral or bilateral pain, burning, or acroparesthesia in the hands or feet. The presence of Fabry disease was defined by abnormal alpha-galactosidase A activity in males or alpha-galactosidase gene (GLA) mutation in females. Among 214 patients (110 males), 84.1% had bilateral limb pain and 31.8% had unilateral limb pain recorded at some time point; 61 (28.5%) patients had a positive family history possibly associated with Fabry disease. Alpha-galactosidase A activity was within the normal range in all 109 of the male patients tested. One female patient had a GLA mutation (C937G > T) and alpha-galactosidase A activity within the normal range. We did not find definitive evidence of Fabry disease in these children with a history of chronic recurrent unilateral or bilateral limb pain or acroparesthesia. The presence of chronic limb pain does not appear to be highly predictive of a diagnosis of Fabry disease in Russian children and adolescents, suggesting that key early signs and symptoms of Fabry disease are not specific to the disease. What is Known: • Signs and symptoms of Fabry disease are seen in children < 10 years of age; pain in the distal limbs is a common early symptom. What is New: • Fabry disease was not diagnosed in this population of Russian children with a history of chronic limb pain. • The presence of acroparesthesia or chronic limb pain does not appear to be highly predictive of a diagnosis of Fabry disease in Russian children and adolescents, suggesting that these early symptoms of Fabry disease are not specific to the disease.

Long-Term Outcomes of Kidney Transplantation in Fabry Disease.

PubMed

Ersözlü, Sara; Desnick, Robert J; Huynh-Do, Uyen; Canaan-Kühl, Sima; Barbey, Frédéric; Genitsch, Vera; Müller, Thomas; Cheetham, Marcus; Flammer, Andreas; Schaub, Stefan; Nowak, Albina

2018-04-24

Fabry disease is a rare X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene that obliterate or markedly reduce α-galactosidase A activity. This results in the systemic accumulation of its glycosphingolipid substrates in body fluids and organs, including the kidney. Fabry nephropathy can lead to end-stage renal disease requiring kidney transplantation. Little is known about its long-term outcomes and the overall patient survival after kidney transplantation. Here, we report 17 Fabry patients (15 males, 2 females) who received kidney transplants and their long-term treatment and follow-up at 4 specialized Fabry centers. The posttransplant follow-up ranged to 25 years, with a median of 11.5 [range 0.8-25.5] years. Graft survival was similar and death-censored graft survival was superior to matched controls. Fabry patients died with functioning kidneys, mostly from cardiac causes. In 2 males 14 and 23 years posttransplant, the grafts had a few typical FD lamellar inclusions, presumably originating from invading host macrophages and vascular endothelial cells. We conclude that kidney transplantation has an excellent long-term outcome in Fabry disease.

Charles' Law of Gases.

ERIC Educational Resources Information Center

Petty, John T.

1995-01-01

Describes an experiment that uses air to test Charles' law. Reinforces the student's intuitive feel for Charles' law with quantitative numbers they can see, introduces the idea of extrapolating experimental data to obtain a theoretical value, and gives a physical quantitative meaning to the concept of absolute zero. (JRH)

Characterization of Classical and Nonclassical Fabry Disease: A Multicenter Study

PubMed Central

Wanner, Christoph; Hughes, Derralynn; Mehta, Atul; Oder, Daniel; Watkinson, Oliver T.; Elliott, Perry M.; Linthorst, Gabor E.; Wijburg, Frits A.; Biegstraaten, Marieke; Hollak, Carla E.

2017-01-01

Fabry disease leads to renal, cardiac, and cerebrovascular manifestations. Phenotypic differences between classically and nonclassically affected patients are evident, but there are few data on the natural course of classical and nonclassical disease in men and women. To describe the natural course of Fabry disease stratified by sex and phenotype, we retrospectively assessed event-free survival from birth to the first clinical visit (before enzyme replacement therapy) in 499 adult patients (mean age 43 years old; 41% men; 57% with the classical phenotype) from three international centers of excellence. We classified patients by phenotype on the basis of characteristic symptoms and enzyme activity. Men and women with classical Fabry disease had higher event rate than did those with nonclassical disease (hazard ratio for men, 5.63, 95% confidence interval, 3.17 to 10.00; P<0.001; hazard ratio for women, 2.88, 95% confidence interval, 1.54 to 5.40; P<0.001). Furthermore, men with classical Fabry disease had lower eGFR, higher left ventricular mass, and higher plasma globotriaosylsphingosine concentrations than men with nonclassical Fabry disease or women with either phenotype (P<0.001). In conclusion, before treatment with enzyme replacement therapy, men with classical Fabry disease had a history of more events than men with nonclassical disease or women with either phenotype; women with classical Fabry disease were more likely to develop complications than women with nonclassical disease. These data may support the development of new guidelines for the monitoring and treatment of Fabry disease and studies on the effects of intervention in subgroups of patients. PMID:27979989

Genealogy of John and Charles Bell: their relationship with the children of Charles Shaw of Ayr.

PubMed

Kaufman, M

2005-11-01

The Reverend William Bell had six children who survived infancy. Two of his sons entered the legal profession and two other sons became distinguished anatomists and surgeons--John Bell, said for 20 years to have been the leading operating surgeon in Britain and throughout the world--and Sir Charles Bell, possibly the most distinguished anatomist and physiologist of his day. Information is not known about the fifth son or their sister. Charles Shaw, a lawyer of Ayr, had four sons and two daughters who survived infancy. Two of his sons, John and Alexander, became anatomists and later surgeons at the Middlesex Hospital, and both worked closely with Charles Bell at the Great Windmill Street School of Anatomy. His third son entered the law and his fourth son became a distinguished soldier. The two daughters of Charles Shaw married into the Bell family: Barbara married George Joseph Bell and Marion married Mr (later Sir) Charles Bell.

Charles Bonnet syndrome: a review.

PubMed

Schadlu, Anita P; Schadlu, Ramin; Shepherd, J Banks

2009-05-01

The aging of the population and the resultant increase in the number of patients with low vision due to age-related macular degeneration and other ocular diseases necessitate an increase in awareness of the Charles Bonnet syndrome among ophthalmic care providers. The clinical features of Charles Bonnet syndrome have been described by several different authors as formed visual hallucinations due to disturbances of the visual system in patients who are otherwise mentally normal. Theories regarding the causes underlying the Charles Bonnet syndrome are multifaceted and offer insight into the function of the visual system. The incidence of the Charles Bonnet syndrome varies among different population groups, but is underdiagnosed in most settings. Recent case reports of treatment options involve varied pharmacologic interventions, but visual improvement and patient reassurance remain the mainstays of treatment. As Charles Bonnet syndrome becomes more prevalent as the population ages, all physicians who care for low vision or elderly patients should be aware of its clinical characteristics and treatment options. Understanding of this syndrome by caregivers will lead to decreased anxiety among the patients who experience it. Further exploration of treatment options will be necessary in the future.

Effects of enzyme replacement therapy for cardiac-type Fabry patients with a Chinese hotspot late-onset Fabry mutation (IVS4+919G>A)

PubMed Central

Lin, Hsiang-Yu; Liu, Hao-Chuan; Huang, Yu-Hsiu; Liao, Hsuan-Chieh; Hsu, Ting-Rong; Shen, Chia-I; Li, Shao-Tzu; Li, Cheng-Fang; Lee, Li-Hong; Lee, Pi-Chang; Huang, Chun-Kai; Chiang, Chuan-Chi; Lin, Ching-Yuang; Lin, Shuan-Pei; Niu, Dau-Ming

2013-01-01

Objective Current studies of newborn screening for Fabry disease in Taiwan have revealed a remarkably high prevalence of cardiac-type Fabry disease with a Chinese hotspot late-onset Fabry mutation (IVS4+919G>A). Design Retrospective cohort study. Setting Tertiary medical centre. Participants 21 patients with cardiac-type Fabry disease (15 men and 6 women) as well as 15 patients with classic Fabry disease (4 men and 11 women) treated with biweekly intravenous infusions of agalsidase β (1 mg/kg) or agalsidase α (0.2 mg/kg) for at least 6 months. Outcome measures These data were collected at the time before enzyme replacement therapy (ERT) began and followed up after ERT for at least 6 months, including patient demographics, medical history, parameter changes of cardiac status and renal functions, plasma globotriaosylsphingosine (lyso-Gb3) and Mainz Severity Score Index. Results After 6–39 months of ERT, plasma lyso-Gb3 was found to be reduced in 89% (17/19) and 93% (14/15) of patients with cardiac-type and classic Fabry disease, respectively, which indicated an improvement of disease severity. For patients with cardiac-type Fabry disease, echocardiography revealed the reduction or stabilisation of left ventricular mass index (LVMI), the thicknesses of intraventricular septum (IVS) and left posterior wall (LPW) in 83% (15/18), 83% (15/18) and 67% (12/18) of patients, respectively, as well as 77% (10/13), 73% (11/15) and 60% (9/15) for those with classic type. Most patients showed stable renal function after ERT. There were statistically significant improvements (p<0.05) between the data at baseline and those after ERT for values of plasma lyso-Gb3, LVMI, IVS, LPW and Mainz Severity Score Index. No severe clinical events were reported during the treatment. Conclusions ERT is beneficial and appears to be safe for Taiwanese patients with cardiac-type Fabry disease, as well as for those with the classic type. PMID:23864212

Fabry disease in children: agalsidase-beta enzyme replacement therapy.

PubMed

Borgwardt, L; Feldt-Rasmussen, U; Rasmussen, A K; Ballegaard, M; Meldgaard Lund, A

2013-05-01

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 increased. Treatment with agalsidase-beta was associated with a reduction of neuropathic and abdominal pain and headache. Although all aspects of the Fabry pain phenotype cannot be treated with ERT, the observed effects were clinically significant in the lives of the majority of Fabry children and together with the absence of serious Fabry manifestations at last follow-up, we argue that early initiation of ERT may be considered. © 2012 John Wiley & Sons A/S.

The first Charles Darwin (1758-78).

PubMed

Harris, Stuart

2009-11-01

The paper places the first Charles Darwin in his family context: the eldest son of Dr Erasmus Darwin and Mary Howard. Mention is made of Charles's upbringing and education, with illustrative material taken from his father's writings and from Anna Seward's Memoirs of the Life of Dr Darwin (1804). The part played by Dr Andrew Duncan of the Edinburgh Medical School is established. The award to Charles in March 1778 of the first medal by the Aesculapian Society of Edinburgh is described. The involvement of Dr William Cullen and Dr Joseph Black in the treatment of Charles's fatal infection is evidenced from Erasmus' letters. Attention is given to 'An Elegy on the much-lamented death of a most ingenious young gentleman who lately died in the College at Edinburgh where he was a student' which was written jointly by Duncan and Erasmus in 1778. The Elegy's curious publishing history will be glanced at. The paper concludes with a statement of Charles's great promise as a medical student and of Erasmus' efforts to ensure that his son's achievements were memorialised.

Coexistence of Fabry disease and IgA nephropathy: a report of two cases.

PubMed

Yin, G; Wu, Y; Zeng, C-H; Chen, H-P; Liu, Z-H

2014-12-01

Coexistence of Fabry disease and IgA nephropathy is rare. Moreover, the coexisting Fabry disease may be unrecognized due to unapparent clinical manifestations. We described two cases with coexisting Fabry disease and IgA nephropathy. The clinicopathological features of these two patients were studied. A 54-year-old male presented with proteinuria, hematuria, and hypertension, and a 33-year-old male presented with proteinuria without clinical signs or family history of Fabry disease. Both of them were diagnosed with IgA nephropathy at admission, whereas Fabry disease was not suspected. Subsequent immunofluorescent study confirmed the diagnosis of IgA nephropathy by showing positive staining for IgA and complement C3 in the mesangium. Meanwhile, light microscopy showed remarkable vacuolation of podocytes with mild mesangial expansion, which was characteristic of Fabry nephropathy. Further examination of toluidine blue-stained semi-thin sections and electron microscopy demonstrated blue bodies and myelin figures in the cytoplasm of podocytes, respectively. The diagnosis of coexisting Fabry disease was finally established based on deficient α-galactosidase A activity in both patients. This case study is an important reminder of the role of kidney biopsy as an indicator of Fabry disease and its rare coexistence with IgA nephropathy.

Treatment with agalsidase beta during pregnancy in Fabry disease.

PubMed

Politei, Juan M

2010-04-01

Fabry disease is an X-linked lysosomal storage disease caused by a deficiency of alpha-galactosidase A, which leads to excessive accumulation of glycosphingolipids in most tissues in the body, with life-threatening clinical consequences in the kidney, heart, and cerebrovascular system. Enzyme replacement therapy using exogenously produced alpha-galactosidase has been available for treatment of this multisystem progressive disease since 2001. Two different preparations of enzyme replacement therapy for Fabry disease are available outside of the USA: agalsidase alfa and agalsidase beta. Despite being X-linked, Fabry disease affects many female patients, and this report presents a successful pregnancy of a female patient receiving agalsidase beta.

[Clinical and histological findings in Fabry nephropathy].

PubMed

Pieruzzi, Federico; Salerno, Fabio; Di Giacomo, Antonella; Torti, Giacomo; Ferrario, Franco; Pagni, Fabio; Stella, Andrea

2013-01-01

Fabry disease is a complex pathology, requiring a multidisciplinar approach both in the diagnostic workout and in the management of therapy. Clinical criteria able to predict its morbidity have not yet been found. The wide variability of clinical signs and symptoms requires an individual approach based on the single patient, in order to achieve an optimal management. Enzyme replacement therapy (ERT) has been introduced in the clinical setting for over ten years, but its ability to change the course of the disease has not yet been clearly proved. Recently the hypothesis that ERT may be ineffective in patients with severe organ involvement has emerged. The clinical course of Fabry disease is usually slower in eterozygous women than emizygous men, but can be frequently associated to severe organ failure and premature death in both cases. In this review we discuss the histological aspects of Fabry nephropathy in relation to diagnosis, prognosis, therapy and its effectiveness.

Hallucinations Experienced by Visually Impaired: Charles Bonnet Syndrome.

PubMed

Pang, Linda

2016-12-01

: Charles Bonnet Syndrome is a condition where visual hallucinations occur as a result of damage along the visual pathway. Patients with Charles Bonnet Syndrome maintain partial or full insight that the hallucinations are not real, absence of psychological conditions, and absence of hallucinations affecting other sensory modalities, while maintaining intact intellectual functioning. Charles Bonnet Syndrome has been well documented in neurologic, geriatric medicine, and psychiatric literature, but there is lack of information in optometric and ophthalmologic literature. Therefore, increased awareness of signs and symptoms associated with Charles Bonnet Syndrome is required among practicing clinicians. This review of the literature will also identify other etiologies of visual hallucinations, pathophysiology of Charles Bonnet Syndrome, and effective management strategies.

Hallucinations Experienced by Visually Impaired: Charles Bonnet Syndrome

PubMed Central

Pang, Linda

2016-01-01

ABSTRACT Charles Bonnet Syndrome is a condition where visual hallucinations occur as a result of damage along the visual pathway. Patients with Charles Bonnet Syndrome maintain partial or full insight that the hallucinations are not real, absence of psychological conditions, and absence of hallucinations affecting other sensory modalities, while maintaining intact intellectual functioning. Charles Bonnet Syndrome has been well documented in neurologic, geriatric medicine, and psychiatric literature, but there is lack of information in optometric and ophthalmologic literature. Therefore, increased awareness of signs and symptoms associated with Charles Bonnet Syndrome is required among practicing clinicians. This review of the literature will also identify other etiologies of visual hallucinations, pathophysiology of Charles Bonnet Syndrome, and effective management strategies. PMID:27529611

Increased Arterial Diameters in the Posterior Cerebral Circulation in Men with Fabry Disease

PubMed Central

Üçeyler, Nurcan; Homola, György A.; Guerrero González, Hans; Kramer, Daniela; Wanner, Christoph; Weidemann, Frank; Solymosi, László; Sommer, Claudia

2014-01-01

A high load of white matter lesions and enlarged basilar arteries have been shown in selected patients with Fabry disease, a disorder associated with an increased stroke risk. We studied a large cohort of patients with Fabry disease to differentially investigate white matter lesion load and cerebral artery diameters. We retrospectively analyzed cranial magnetic resonance imaging scans of 87 consecutive Fabry patients, 20 patients with ischemic stroke, and 36 controls. We determined the white matter lesion load applying the Fazekas score on fluid-attenuated inversion recovery sequences and measured the diameters of cerebral arteries on 3D-reconstructions of the time-of-flight-MR-angiography scans. Data of different Fabry patient subgroups (males – females; normal – impaired renal function) were compared with data of patients with stroke and controls. A history of stroke or transient ischemic attacks was present in 4/30 males (13%) and 5/57 (9%) females with Fabry disease, all in the anterior circulation. Only one man with Fabry disease showed confluent cerebral white matter lesions in the Fazekas score assessment (1%). Male Fabry patients had a larger basilar artery (p<0.01) and posterior cerebral artery diameter (p<0.05) compared to male controls. This was independent of disease severity as measured by renal function and did not lead to changes in arterial blood flow properties. A basilar artery diameter of >3.2 mm distinguished between men with Fabry disease and controls (sensitivity: 87%, specificity: 86%, p<0.001), but not from stroke patients. Enlarged arterial diameters of the posterior circulation are present only in men with Fabry disease independent of disease severity. PMID:24475221

Atypical patterns of cardiac involvement in Fabry disease.

PubMed

Coughlan, J J; Elkholy, K; O'Brien, J; Kiernan, T

2016-03-17

A 58-year-old woman was referred to our cardiology service with chest pain, exertional dyspnoea and palpitations on a background of known Fabry disease diagnosed with genetic testing in 1994. ECG showed sinus rhythm, shortened PR interval, widespread t wave inversion, q waves in the lateral leads and left ventricular hypertrophy (LVH). Coronary angiogram showed only mild atheroma. Transthoracic echocardiogram showed anterolateral LVH and reduced left ventricular cavity size in keeping with Fabry cardiomyopathy. Cardiac MRI demonstrated asymmetric hypertrophy with evidence of diffuse myocardial fibrosis in the maximally hypertrophied segments from base to apex with late gadolinium enhancement in the anterior and anteroseptal walls. This was quite an atypical appearance for Fabry cardiomyopathy. This case highlights the heterogeneity of patterns of cardiac involvement that may be associated with this rare X-linked lysosomal disorder. 2016 BMJ Publishing Group Ltd.

Delayed-enhanced cardiac MRI for differentiation of Fabry's disease from symmetric hypertrophic cardiomyopathy.

PubMed

De Cobelli, Francesco; Esposito, Antonio; Belloni, Elena; Pieroni, Maurizio; Perseghin, Gianluca; Chimenti, Cristina; Frustaci, Andrea; Del Maschio, Alessandro

2009-03-01

Fabry's disease may be difficult to differentiate from symmetric hypertrophic cardiomyopathy. Our aim was to compare the myocardial location and distribution patterns of delayed enhancement between patients with Fabry's disease who are affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy in order to identify a specific sign to best differentiate the two diseases. Patients with Fabry's disease-related hypertrophy showed left ventricular (LV) delayed enhancement with a typical and consistently found pattern characterized by the involvement of the inferolateral basal or mid basal segments and a mesocardial distribution that spared the subendocardium. This pattern seems to be specific to Fabry's disease; in fact, patients with symmetric hypertrophic cardiomyopathy had variable locations and distributions of delayed enhancement. These observations may contribute to identifying Fabry's disease as a specific cause of symmetric hypertrophy.

Miniature fiber Fabry-Perot sensors based on fusion splicing

NASA Astrophysics Data System (ADS)

Zhu, Jia-li; Wang, Ming; Yang, Chun-di; Wang, Ting-ting

2013-03-01

Fiber-optic Fabry-Perot (F-P) sensors are widely investigated because they have several advantages over conventional sensors, such as immunity to electromagnetic interference, ability to operate under bad environments, high sensitivity and the potential for multiplexing. A new method to fabricate micro-cavity Fabry-Perot interferometer is introduced, which is fusion splicing a section of conventional single-mode fiber (SMF) and a section of hollow core or solid core photonic crystal fiber (PCF) together to form a micro-cavity at the splice joint. The technology of fusion splicing is discussed, and two miniature optical fiber sensors based on Fabry-Perot interference using fusion splicing are presented. The two sensors are completely made of fused silica, and have good high-temperature capability.

Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype

PubMed Central

Namer, Barbara; Ørstavik, Kirstin; Schmidt, Roland; Mair, Norbert; Kleggetveit, Inge Petter; Zeidler, Maximillian; Martha, Theresa; Jorum, Ellen; Schmelz, Martin; Kalpachidou, Theodora; Kress, Michaela; Langeslag, Michiel

2017-01-01

The first symptom arising in many Fabry patients is neuropathic pain due to changes in small myelinated and unmyelinated fibers in the periphery, which is subsequently followed by a loss of sensory perception. Here we studied changes in the peripheral nervous system of Fabry patients and a Fabry mouse model induced by deletion of α-galactosidase A (Gla−/0). The skin innervation of Gla−/0 mice resembles that of the human Fabry patients. In Fabry diseased humans and Gla−/0 mice, we observed similar sensory abnormalities, which were also observed in nerve fiber recordings in both patients and mice. Electrophysiological recordings of cultured Gla−/0 nociceptors revealed that the conductance of voltage-gated Na+ and Ca2+ currents was decreased in Gla−/0 nociceptors, whereas the activation of voltage-gated K+ currents was at more depolarized potentials. Conclusively, we have observed that reduced sensory perception due to small-fiber degeneration coincides with altered electrophysiological properties of sensory neurons. PMID:28769867

Fibrosis: a key feature of Fabry disease with potential therapeutic implications

PubMed Central

2013-01-01

Fabry disease is a rare X-linked hereditary disease caused by mutations in the AGAL gene encoding the lysosomal enzyme alpha-galactosidase A. Enzyme replacement therapy (ERT) is the current cornerstone of Fabry disease management. Involvement of kidney, heart and the central nervous system shortens life span, and fibrosis of these organs is a hallmark of the disease. Fibrosis was initially thought to result from tissue ischemia secondary to endothelial accumulation of glycosphingolipids in the microvasculature. However, despite ready clearance of endothelial deposits, ERT is less effective in patients who have already developed fibrosis. Several potential explanations of this clinical observation may impact on the future management of Fabry disease. Alternative molecular pathways linking glycosphingolipids and fibrosis may be operative; tissue injury may recruit secondary molecular mediators of fibrosis that are unresponsive to ERT, or fibrosis may represent irreversible tissue injury that limits the therapeutic response to ERT. We provide an overview of Fabry disease, with a focus on the assessment of fibrosis, the clinical consequences of fibrosis, and recent advances in understanding the cellular and molecular mechanisms of fibrosis that may suggest novel therapeutic approaches to Fabry disease. PMID:23915644

Simultaneous measurement of temperature and pressure with cascaded extrinsic Fabry-Perot interferometer and intrinsic Fabry-Perot interferometer sensors

NASA Astrophysics Data System (ADS)

Zhang, Yinan; Huang, Jie; Lan, Xinwei; Yuan, Lei; Xiao, Hai

2014-06-01

This paper presents an approach for simultaneous measurement of temperature and pressure using miniaturized fiber inline sensors. The approach utilizes the cascaded optical fiber inline intrinsic Fabry-Perot interferometer and extrinsic Fabry-Perot interferometer as temperature and pressure sensing elements, respectively. A CO2 laser was used to create a loss between them to balance their reflection power levels. The multiplexed signals were demodulated using a Fast Fourier transform-based wavelength tracking method. Experimental results showed that the sensing system could measure temperature and pressure unambiguously in a pressure range of 0 to 6.895×105 Pa and a temperature range from 20°C to 700°C.

Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.

PubMed

Wilcox, William R; Feldt-Rasmussen, Ulla; Martins, Ana Maria; Ortiz, Alberto; Lemay, Roberta M; Jovanovic, Ana; Germain, Dominique P; Varas, Carmen; Nicholls, Katherine; Weidemann, Frank; Hopkin, Robert J

2018-01-01

Fabry disease, an X-linked inherited lysosomal storage disorder, is caused by mutations in the gene encoding α-galactosidase, GLA. In patients with Fabry disease, glycosphingolipids accumulate in various cell types, triggering a range of cellular and tissue responses that result in a wide spectrum of organ involvement. Although variable, gastrointestinal symptoms are among the most common and significant early clinical manifestations; they tend to persist into adulthood if left untreated. To further understand the effects of sustained enzyme replacement therapy (ERT) with agalsidase beta on gastrointestinal symptoms in heterozygotes, a data analysis of female patients enrolled in the Fabry Registry was conducted. To be included, females of any age must have received agalsidase beta (average dose 1.0 mg/kg every 2 weeks) for at least 2.5 years. Measured outcomes were self-reported gastrointestinal symptoms (abdominal pain, diarrhea). Outcomes at baseline and last follow-up, and their change from baseline to last follow-up, were assessed. Relevant data were available for 168 female patients. Mean age at the start of ERT was 43 years and mean treatment duration 5.7 years. Baseline pre-treatment abdominal pain was reported by 45% of females and diarrhea by 39%. At last follow-up, 31% reported abdominal pain (p < 0.01) and 27% diarrhea (p < 0.01). The results of this Fabry Registry analysis suggest that while on sustained treatment with agalsidase beta (1.0 mg/kg every 2 weeks), both abdominal pain and diarrhea improved in many female patients with Fabry disease.

Micromachined Tunable Fabry-Perot Filters for Infrared Astronomy

NASA Technical Reports Server (NTRS)

Barclay, Richard; Bier, Alexander; Chen, Tina; DiCamillo, Barbara; Deming, Drake; Greenhouse, Matthew; Henry, Ross; Hewagama, Tilak; Jacobson, Mindy; Loughlin, James;

Musculoskeletal manifestations of Fabry disease: A retrospective study.

PubMed

Lidove, Olivier; Zeller, Valérie; Chicheportiche, Valérie; Meyssonnier, Vanina; Sené, Thomas; Godot, Sophie; Ziza, Jean-Marc

2016-07-01

Fabry disease is a rare X-linked metabolic disorder characterized by a deficiency in the enzyme alpha-galactosidase A. Both males and females can be affected. The main presenting symptom is pain in the extremities, whereas at a more advanced stage, the manifestations include hypertrophic cardiomyopathy, cardiac dysrhythmia, proteinuria, chronic kidney dysfunction, stroke, and hearing loss. When not diagnosed and treated, Fabry disease causes early death. No studies specifically designed to describe the musculoskeletal manifestations of Fabry disease are available. We conducted a single-center retrospective study of patients receiving follow-up at a Fabry disease referral center. We described the musculoskeletal manifestations and analyzed the differential diagnoses. Our study included 40 patients belonging to 20 families, including 25 females with a mean age of 44.2 years (range, 20-76 years) and 15 males with a mean age of 40.1 years (range, 16-61 years). Mean age at the diagnosis of Fabry disease was 37.2 years (range, 7-71 years) in the females and 26.9 years (range, 9-51 years) in the males. Specific enzyme replacement therapy was given to 10 (40%) females and 12 (80%) males. Musculoskeletal manifestations were as follows: past or present pain in the extremities (13 females and 10 males), combined in some patients with vasomotor disorders in the extremities and telangiectasia; exercise intolerance (12 females and 12 males); osteoporotic fractures (2 brothers aged 45 and 44 years, respectively); osteoporosis (3 females, aged 57, 63, and 75 years, respectively), which contributed to death in the oldest patient; osteopenia (2 females aged 38 and 47 years, respectively; and 1 male aged 43 years); Charcot foot and lymphedema with serious infectious complications (4 males older than 40 years), with avascular osteonecrosis of the lower limbs in 2 cases; toe amputations (3 cases); bilateral lower-limb amputation (1 case); abnormally slender lower limbs (5 females and

Hemizygous Fabry disease associated with membranous nephropathy: A rare case report
.

PubMed

Zhou, Wenyan; Ni, Zhaohui; Zhang, Minfang

2018-05-24

Fabry disease may coexist with various glomerular diseases, including IgA nephropathy, focal segmental glomerulosclerosis, etc. In this study, we report a rare case of Fabry disease associated with membranous nephropathy (MN). A 30-year-old man with nephrotic proteinuria, normal renal function, and no other extrarenal manifestations underwent a renal biopsy in February 2017. Light microscopy and immunofluorescence indicated MN (stage 1). Under an electron microscope, there were subepithelial electron-dense deposits and abundant zebra bodies in podocytes. Both the findings of low-activity α-galactosidase A (α-Gal A, GLA) and base deletion in exon 7 of the GLA gene (GLA-E07.1286_*7 del, a newly reported mutation) confirmed that this patient was simultaneously afflicted with Fabry disease. This case report is an important reminder of the role of kidney biopsy, especially electron microscopy, as an indicator of Fabry disease and its rare coexistence with MN.
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Confocal Fabry-Perot interferometer for frequency stabilization of laser

NASA Astrophysics Data System (ADS)

Pan, H.-J.; Ruan, P.; Wang, H.-W.; Li, F.

2011-02-01

The frequency shift of laser source of Doppler lidar is required in the range of a few megahertzs. To satisfy this demand, a confocal Fabry-Perot (F-P) interferometer was manufactured as the frequency standard for frequency stabilization. After analyzing and contrasting the center frequency shift of confocal Fabry-Perot interferometers that are made of three different types of material with the change of temperature, the zerodur material was selected to fabricate the interferometer, and the cavity mirrors were optically contacted onto the end of spacer. The confocal Fabry-Perot interferometer was situated within a double-walled chamber, and the change of temperature in the chamber was less than 0.01 K. The experimental results indicate that the free spectral range is 500 MHz, the full-width at half maximum is 3.33 MHz, and the finesse is 150.

Safety and efficacy of enzyme replacement therapy in the nephropathy of Fabry disease

PubMed Central

Fervenza, Fernando C; Torra, Roser; Warnock, David G

2008-01-01

Kidney involvement with progressive loss of kidney function (Fabry nephropathy) is an important complication of Fabry disease, an X-linked lysosomal storage disorder arising from deficiency of α-galactosidase activity. Clinical trials have shown that enzyme replacement therapy (ERT) with recombinant human α-galactosidase clears globotriaosylceramide from kidney cells, and can stabilize kidney function in patients with mild to moderate Fabry nephropathy. Recent trials show that patients with more advanced Fabry nephropathy and overt proteinuria do not respond as well to ERT alone, but can benefit from anti-proteinuric therapy given in conjunction with ERT. This review focuses on the use of enzyme replacement therapy with agalsidase-alfa and agalsidase-beta in adults with Fabry nephropathy. The current results are reviewed and evaluated. The issues of dosing of enzyme replacement therapy, the use of adjunctive agents to control urinary protein excretion, and the individual factors that affect disease severity are reviewed. PMID:19707461

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

PubMed

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.

High-temperature fiber-optic Fabry-Perot interferometric sensors

NASA Astrophysics Data System (ADS)

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.

Coronographie interferéntielle pour la mission spatuale DARWIN: expérience de validation en laboratoire

NASA Astrophysics Data System (ADS)

Ollivier, Marc; Mariotti, Jean-Marie; Brunaud, Jacqueline; Michel, Guy; Bouchareine, Patrick; Léger, Alain; Artzner, Guy; Malbet, Fabien; Puget, Pascal; Coudé du Foresto, Vincent; Mennesson, Bertrand

2018-04-01

This paper, "Coronographie interferéntielle pour la mission spatuale DARWIN: expérience de validation en laboratoire," was presented as part of International Conference on Space Optics—ICSO 1997, held in Toulouse, France.

Lake Charles CCS Project

DOE Office of Scientific and Technical Information (OSTI.GOV)

Leib, Thomas; Cole, Dan

In late September 2014 development of the Lake Charles Clean Energy (LCCE) Plant was abandoned resulting in termination of Lake Charles Carbon Capture and Sequestration (CCS) Project which was a subset the LCCE Plant. As a result, the project was only funded through Phase 2A (Design) and did not enter Phase 2B (Construction) or Phase 2C (Operations). This report was prepared relying on information prepared and provided by engineering companies which were engaged by Leucadia Energy, LLC to prepare or review Front End Engineering and Design (FEED) for the Lake Charles Clean Energy Project, which includes the Carbon Capture andmore » Sequestration (CCS) Project in Lake Charles, Louisiana. The Lake Charles Carbon Capture and Sequestration (CCS) Project was to be a large-scale industrial CCS project intended to demonstrate advanced technologies that capture and sequester carbon dioxide (CO 2) emissions from industrial sources into underground formations. The Scope of work was divided into two discrete sections; 1) Capture and Compression prepared by the Recipient Leucadia Energy, LLC, and 2) Transport and Sequestration prepared by sub-Recipient Denbury Onshore, LLC. Capture and Compression-The Lake Charles CCS Project Final Technical Report describes the systems and equipment that would be necessary to capture CO 2 generated in a large industrial gasification process and sequester the CO 2 into underground formations. The purpose of each system is defined along with a description of its equipment and operation. Criteria for selection of major equipment are provided and ancillary utilities necessary for safe and reliable operation in compliance with environmental regulations are described. Construction considerations are described including a general arrangement of the CCS process units within the overall gasification project. A cost estimate is provided, delineated by system area with cost breakdown showing equipment, piping and materials, construction labor

A Novel Fabry-Perot Cavity Fiber Sensor

NASA Astrophysics Data System (ADS)

Lin, Chun; Huang, Yuan Qing; Lei, Wang; Ye, Xiao Juan

Fabry-Perot (F-P) cavity fiber sensors are often used in acceleration, vibration and pressure measurement. When the structure of sensors are similar, there are the same disadvantages exist. A novel design of Fabry-Perot (F-P) cavity fiber sensor is described in this paper, which is composed by a non-coating end-face and a holophote. Triple beams interference is formed in the sensor and shows higher sensitivity. In order to demodulate interference signal in great background noise, two photodiodes are connected in series to form short circuit current which delimits the common mode signal. Experimental results are described for the sensor signal responding to the vibration excited by PZT.^p

Lower Charles River Bathymetry: 108 Years of Fresh Water

NASA Astrophysics Data System (ADS)

Yoder, M.; Sacarny, M.

2017-12-01

The Lower Charles River is a heavily utilized urban river that runs between Cambridge and Boston in Massachusetts. The recreational usage of the river is dependent on adequate water depths, but there have been no definitive prior studies on the sedimentation rate of the Lower Charles River. The river transitioned from tidal to a freshwater basin in 1908 due to the construction of the (old) Charles River Dam. Water surface height on the Lower Charles River is maintained within ±1 foot through controlled discharge at the new Charles River Dam. The current study area for historical comparisons is from the old Charles River Dam to the Boston University Bridge. This study conducted a bathymetric survey of the Lower Charles River, digitized three prior surveys in the study area, calculated volumes and depth distributions for each survey, and estimated sedimentation rates from fits to the volumes over time. The oldest chart digitized was produced in 1902 during dam construction deliberations. The average sedimentation rate is estimated as 5-10 mm/year, which implies 1.8-3.5 feet sedimentation since 1908. Sedimentation rates and distributions are necessary to develop comprehensive management plans for the river and there is evidence to suggest that sedimentation rates in the shallow upstream areas are higher than the inferred rates in the study area.

Agalsidase Beta: a review of its use in the management of Fabry disease.

PubMed

Keating, Gillian M; Simpson, Dene

2007-01-01

Agalsidase beta (Fabrazyme) is a recombinant human alpha-galactosidase A enzyme approved for intravenous use in the treatment of Fabry disease. Fabry disease is a progressive, multisystemic, potentially life threatening disorder caused by a deficiency of alpha-galactosidase A. This deficiency results in accumulation of glycosphingolipids, particularly globotriaosylceramide (GL-3), in the lysosomes of various tissues. This accumulation is the underlying driver of disease progression. Agalsidase beta provides an exogenous source of alpha-galactosidase A.Intravenous agalsidase beta is effective and well tolerated in patients with Fabry disease. In a phase III trial, agalsidase beta was shown to clear GL-3 from various target cells and, in a subsequent extension of this trial, prevent GL-3 reaccumulation. In a post-approval trial, agalsidase beta was shown to provide significant clinical benefit by reducing the risk of a major clinical event. Thus, agalsidase beta represents an important advance in the treatment of Fabry disease, and agalsidase beta therapy should be strongly considered in patients with Fabry disease who are suitable candidates.

Value of the CHA2DS2-VASc score and Fabry-specific score for predicting new-onset or recurrent stroke/TIA in Fabry disease patients without atrial fibrillation.

PubMed

Liu, Dan; Hu, Kai; Schmidt, Marie; Müntze, Jonas; Maniuc, Octavian; Gensler, Daniel; Oder, Daniel; Salinger, Tim; Weidemann, Frank; Ertl, Georg; Frantz, Stefan; Wanner, Christoph; Nordbeck, Peter

2018-05-24

To evaluate potential risk factors for stroke or transient ischemic attacks (TIA) and to test the feasibility and efficacy of a Fabry-specific stroke risk score in Fabry disease (FD) patients without atrial fibrillation (AF). FD patients often experience cerebrovascular events (stroke/TIA) at young age. 159 genetically confirmed FD patients without AF (aged 40 ± 14 years, 42.1% male) were included, and risk factors for stroke/TIA events were determined. All patients were followed up over a median period of 60 (quartiles 35-90) months. The pre-defined primary outcomes included new-onset or recurrent stroke/TIA and all-cause death. Prior stroke/TIA (HR 19.97, P < .001), angiokeratoma (HR 4.06, P = .010), elevated creatinine (HR 3.74, P = .011), significant left ventricular hypertrophy (HR 4.07, P = .017), and reduced global systolic strain (GLS, HR 5.19, P = .002) remained as independent risk predictors of new-onset or recurrent stroke/TIA in FD patients without AF. A Fabry-specific score was established based on above defined risk factors, proving somehow superior to the CHA 2 DS 2 -VASc score in predicting new-onset or recurrent stroke/TIA in this cohort (AUC 0.87 vs. 0.75, P = .199). Prior stroke/TIA, angiokeratoma, renal dysfunction, left ventricular hypertrophy, and global systolic dysfunction are independent risk factors for new-onset or recurrent stroke/TIA in FD patients without AF. It is feasible to predict new or recurrent cerebral events with the Fabry-specific score based on the above defined risk factors. Future studies are warranted to test if FD patients with high risk for new-onset or recurrent stroke/TIA, as defined by the Fabry-specific score (≥ 2 points), might benefit from antithrombotic therapy. Clinical trial registration HEAL-FABRY (evaluation of HEArt invoLvement in patients with FABRY disease, NCT03362164).

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

NASA Astrophysics Data System (ADS)

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.

Cardiac involvement in genotype-positive Fabry disease patients assessed by cardiovascular MR.

PubMed

Kozor, Rebecca; Grieve, Stuart M; Tchan, Michel C; Callaghan, Fraser; Hamilton-Craig, Christian; Denaro, Charles; Moon, James C; Figtree, Gemma A

2016-02-15

Cardiac magnetic resonance (CMR) has the potential to provide early detection of cardiac involvement in Fabry disease. We aimed to gain further insight into this by assessing a cohort of Fabry patients using CMR. Fifty genotype-positive Fabry subjects (age 45±2 years; 50% male) referred for CMR and 39 matched controls (age 40±2 years; 59% male) were recruited. Patients had a mean Mainz severity score index of 15±2 (range 0-46), reflecting an overall mild degree of disease severity. Compared with controls, Fabry subjects had a 34% greater left ventricular mass (LVM) index (82±5 vs 61±2 g/m(2), p=0.001) and had a significantly greater papillary muscle contribution to total LVM (13±1 vs 6±0.5%, p<0.001), even in the absence of left ventricular hypertrophy (LVH). Late gadolinium enhancement (LGE) was present in 15 Fabry subjects (9/21 males and 6/23 females). The most common site for LGE was the basal inferolateral wall (93%, 14/15). There was a positive association between LVM index and LGE. Despite this, there were two males and three females with no LVH that displayed LGE. Of Fabry subjects who were not on enzyme replacement therapy at enrolment (n=28), six were reclassified as having cardiac involvement (four LVH-negative/LGE-positive, one LVH-positive/LGE-positive and one LVH-positive/LGE-negative). CMR was able to detect cardiac involvement in 48% of this Fabry cohort, despite the overall mild disease phenotype of the cohort. Of those not on ERT, 21% were reclassified as having cardiac involvement allowing improved risk stratification and targeting of therapy. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Fiber optic, Fabry-Perot high temperature sensor

NASA Technical Reports Server (NTRS)

James, K.; Quick, B.

1984-01-01

A digital, fiber optic temperature sensor using a variable Fabry-Perot cavity as the sensor element was analyzed, designed, fabricated, and tested. The fiber transmitted cavity reflection spectra is dispersed then converted from an optical signal to electrical information by a charged coupled device (CCD). A microprocessor-based color demodulation system converts the wavelength information to temperature. This general sensor concept not only utilizes an all-optical means of parameter sensing and transmitting, but also exploits microprocessor technology for automated control, calibration, and enhanced performance. The complete temperature sensor system was evaluated in the laboratory. Results show that the Fabry-Perot temperature sensor has good resolution (0.5% of full seale), high accuracy, and potential high temperature ( 1000 C) applications.

Reduction of elevated plasma globotriaosylsphingosine in patients with classic Fabry disease following enzyme replacement therapy.

PubMed

van Breemen, Mariëlle J; Rombach, Saskia M; Dekker, Nick; Poorthuis, Ben J; Linthorst, Gabor E; Zwinderman, Aeilko H; Breunig, Frank; Wanner, Christoph; Aerts, Johannes M; Hollak, Carla E

2011-01-01

Fabry disease is treated by two-weekly infusions with α-galactosidase A, which is deficient in this X-linked globotriaosylceramide (Gb3) storage disorder. Elevated plasma globotriaosylsphingosine (lysoGb3) is a hallmark of classical Fabry disease. We investigated effects of enzyme replacement therapy (ERT) on plasma levels of lysoGb3 and Gb3 in patients with classical Fabry disease treated with agalsidase alfa at 0.2mg/kg, agalsidase beta at 0.2mg/kg or at 1.0mg/kg bodyweight. Each treatment regimen led to prominent reductions of plasma lysoGb3 in Fabry males within 3 months (P=0.0313), followed by relative stability later on. Many males developed antibodies against α-galactosidase A, particularly those treated with agalsidase beta. Patients with antibodies tended towards smaller correction in plasma lysoGb3 concentration, whereas treatment with high dose agalsidase beta allowed a reduction comparable to patients without antibodies. Pre-treatment plasma lysoGb3 concentrations of Fabry females were relatively low. In all females and with each treatment regimen, ERT gave reduction or stabilisation of plasma lysoGb3. Our investigation revealed that ERT of Fabry patients reduces plasma lysoGb3, regardless of the recombinant enzyme used. This finding shows that ERT can correct a characteristic biochemical abnormality in Fabry patients. Copyright © 2010 Elsevier B.V. All rights reserved.

High-Sensitivity Troponin: A Clinical Blood Biomarker for Staging Cardiomyopathy in Fabry Disease.

PubMed

Seydelmann, Nora; Liu, Dan; Krämer, Johannes; Drechsler, Christiane; Hu, Kai; Nordbeck, Peter; Schneider, Andreas; Störk, Stefan; Bijnens, Bart; Ertl, Georg; Wanner, Christoph; Weidemann, Frank

2016-05-31

High-sensitivity troponin (hs-TNT), a biomarker of myocardial damage, might be useful for assessing fibrosis in Fabry cardiomyopathy. We performed a prospective analysis of hs-TNT as a biomarker for myocardial changes in Fabry patients and a retrospective longitudinal follow-up study to assess longitudinal hs-TNT changes relative to fibrosis and cardiomyopathy progression. For the prospective analysis, hs-TNT from 75 consecutive patients with genetically confirmed Fabry disease was analyzed relative to typical Fabry-associated echocardiographic findings and total myocardial fibrosis as measured by late gadolinium enhancement (LE) on magnetic resonance imaging. Longitudinal data (3.9±2.0 years), including hs-TNT, LE, and echocardiographic findings from 58 Fabry patients, were retrospectively collected. Hs-TNT level positively correlated with LE (linear correlation coefficient, 0.72; odds ratio, 32.81 [95% CI, 3.56-302.59]; P=0.002); patients with elevated baseline hs-TNT (>14 ng/L) showed significantly increased LE (median: baseline, 1.9 [1.1-3.3] %; follow-up, 3.2 [2.3-4.9] %; P<0.001) and slightly elevated hs-TNT (baseline, 44.7 [30.1-65.3] ng/L; follow-up, 49.1 [27.6-69.5] ng/L; P=0.116) during follow-up. Left ventricular wall thickness and EF of patients with elevated hs-TNT were decreased during follow-up, indicating potential cardiomyopathy progression. hs-TNT is an accurate, easily accessible clinical blood biomarker for detecting replacement fibrosis in patients with Fabry disease and a qualified predictor of cardiomyopathy progression. Thus, hs-TNT could be helpful for staging and follow-up of Fabry patients. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

Risk factors for severe clinical events in male and female patients with Fabry disease treated with agalsidase beta enzyme replacement therapy: Data from the Fabry Registry.

PubMed

Hopkin, Robert J; Cabrera, Gustavo; Charrow, Joel; Lemay, Roberta; Martins, Ana Maria; Mauer, Michael; Ortiz, Alberto; Patel, Manesh R; Sims, Katherine; Waldek, Stephen; Warnock, David G; Wilcox, William R

2016-09-01

Fabry disease, an X-linked lysosomal storage disorder, causes intracellular accumulation of glycosphingolipids leading to progressive renal, cardiovascular, and cerebrovascular disease, and premature death. This longitudinal Fabry Registry study analyzed data from patients with Fabry disease to determine the incidence and type of severe clinical events following initiation of enzyme replacement therapy (ERT) with agalsidase beta, as well as risk factors associated with occurrence of these events. Severe events assessed included chronic dialysis, renal transplantation, cardiac events, stroke, and death. The analyses included 969 male and 442 female Fabry patients. The mean age at first agalsidase beta infusion was 35 and 44, and median treatment follow-up 4.3years and 3.2years, respectively. Among males, cardiac events were the most common on-ERT events, followed by renal, stroke, and non-cardiac death. Among females, cardiac events were also most common followed by stroke and renal events. Patients with on-ERT events had significantly more advanced cardiac and renal disease at baseline as compared with patients without on-ERT events. Severe events were also associated with older age at ERT initiation (males and females), a history of pre-ERT events (females; approaching statistical significance in males), and a higher urinary protein/creatinine ratio (females). Approximately 65% of patients with pre-ERT events did not experience subsequent on-ERT events. Of patients without pre-ERT events, most (84% of males, 92% of females) remained event-free. Patients with on-ERT severe events had more advanced Fabry organ involvement at baseline than those without such events and patients who initiated ERT at a younger age had less residual risk of on-ERT events. The observed patterns of residual risk may aid clinicians in multidisciplinary monitoring of male and female patients with Fabry disease receiving ERT, and in determining the need for administration of adjunctive

Update on role of agalsidase alfa in management of Fabry disease

PubMed Central

Ramaswami, Uma

2011-01-01

Fabry disease (FD) is an X-linked lysosomal storage disorder that affects both men and women. The manifestations of this heterogeneous disease are multisystemic and progressive. Prior to the development of enzyme replacement therapy, the management and treatment for Fabry disease was largely nonspecific and supportive. Because enzyme replacement therapy became commercially available in 2001, a variety of clinical benefits in Fabry patients have been consistently reported, including improved renal pathology and cardiac function, and reduced severity of neuropathic pain and improved pain-related quality of life. This update focuses on published data on the efficacy and tolerability of enzyme replacement therapy with agalsidase alfa, and gives a brief overview on some of the outstanding management issues in the treatment of this complex disease. PMID:21552486

Identification of mutations in Colombian patients affected with Fabry disease.

PubMed

Uribe, Alfredo; Mateus, Heidi Eliana; Prieto, Juan Carlos; Palacios, Maria Fernanda; Ospina, Sandra Yaneth; Pasqualim, Gabriela; da Silveira Matte, Ursula; Giugliani, Roberto

2015-12-15

Fabry Disease (FD) is an X-linked inborn error of glycosphingolipid catabolism, caused by a deficiency of the lisosomal α-galactosidase A (AGAL). The disorder leads to a vascular disease secondary to the involvement of kidney, heart and the central nervous system. The mutation analysis is a valuable tool for diagnosis and genetic counseling. Although more than 600 mutations have been identified, most mutations are private. Our objective was to describe the analysis of nine Colombian patients with Fabry disease by automated sequencing of the seven exons of the GLA gene. Two novel mutations were identified in two patients affected with the classical subtype of FD, in addition to other 6 mutations previously reported. The present study confirms the heterogeneity of mutations in Fabry disease and the importance of molecular analysis for genetic counseling, female heterozygotes detection as well as therapeutic decisions. Copyright © 2015 Elsevier B.V. All rights reserved.

[Fabry nephropathy in a female with superposed IgA glomerulonephritis].

PubMed

Pisani, A; Sessa, A; Sabbatini, M; Andreucci, M V; Fusco, C; Balletta, M; Cianciaruso, B

2005-01-01

In Anderson-Fabry disease (AFd), the kidney is affected in all hemizygous males and in some heterozygous females. Female carriers can present subtle renal abnormalities due to glycosphingolipid (GSL) accumulation within renal cells. Renal biopsy is rarely performed in female Fabry patients because clinical renal manifestations are usually lacking. However, female carriers can accumulate GSL in their renal cells despite the absence of clinically evident kidney disease. We performed a kidney biopsy in a 52-year-old female patient, a Fabry disease carrier. The patient showed normal glomerular filtration rate, persistent microhematuria and proteinuria (about 1.7 g/24 hr), cornea "verticillata", and evident left ventricular hypertrophy. The molecular study documented a missense mutation R227Q in exon 5 of the alpha-galactosidase A gene. Optical microscopy showed electron-dense mesangial deposits due IgA glomerulonephritis, as confirmed by immunofluorescence. We decided to start therapy with angiotensin-converting enzyme inhibitors (ACE-I). After 8 months of treatment, the patient demonstrated proteinuria of 0.9 g/24 hr. To decide when to start treatment using enzyme replacement therapy (ERT) with human recombinant GAL A (Fabrazyme), we decided to perform an electron microscopy study of the renal biopsy. The renal ultrastructural findings were typical GSL inclusions in all kinds of glomerular cells, in tubular epithelial cells and in endothelial cells of interstitial capillaries, confirming the hypothesis of Fabry nephropathy. Consequently, Fabrazyme was given at a standard dose of 1 mg/kg every 2 weeks. After 24 months of combined treatment (ACE-I-Fabrazyme), proteinuria decreased to 0.2 g/24 hr. The importance of performing the ultrastructural examination of the kidney biopsy is stressed, especially in heterozygous Fabry patients to evaluate the need to treat them with ERT and to evaluate the degree of renal involvement.

Wilhelm Fabry's 1614 report on a giant condyloma of the penis.

PubMed

Marx, F J; Karenberg, A

2012-02-01

For many years it has been the work of Buschke and Löwenstein that has justified calling the exophytic, locally destructive tumour of the anogenital mucosal surface 'giant condyloma of Buschke and Löwenstein' or GCBL. In order to investigate the early history of this rare disease we examined the writings of the barber-surgeon Wilhelm Fabry (1560-1634) who had a serious interest in dermatological disorders and their treatment. We analysed Fabry's 600 Latin case reports and identified the case of a 'monstrous penile tumour'. We then translated this text into English and compared it point by point with later publications. This was followed by a cursory review of surgical treatises from the 16th to the 18th centuries. In 1614 Fabry described and depicted a tumour of the penis; the clinical characteristics (gradual formation of a warty lesion, considerable size, invasive growth, absence of metastases) indicated it was a giant condyloma. His mention of the urethral fistulization enables discrimination from 'common' condylomata acuminata, and the survival period of 10 years after amputation allows exclusion of a 'true' carcinoma. This report is singular among 17th-century case histories. The neoplasias described 300 years later are most probably biologically identical. Thus, Fabry's is the first clinical report; the histological classification, however, belongs to Buschke and Löwenstein. From now on the disease should be designated with the eponym giant condyloma of Fabry-Buschke-Löwenstein or GCFBL. © 2011 The Authors. BJD © 2011 British Association of Dermatologists.

Charles L. Brewer Distinguished Teaching of Psychology Award.

PubMed

2014-01-01

The American Psychological Foundation (APF) Charles L. Brewer Award for Distinguished Teaching of Psychology recognizes an outstanding career contribution to the teaching of psychology. The 2014 recipient of the Charles L. Brewer Distinguished Teaching of Psychology Award is Mary E. Kite. 2014 APA, all rights reserved

Genital angiokeratoma in a woman with Fabry disease: the dermatologist's role.

PubMed

Jesus, Patricia Moraes Resende de; Martins, Ana Maria; Chiacchio, Nilton Di; Aranda, Carolina Sanchez

2018-06-01

Fabry disease is a rare lysosomal storage disorder, inherited in an X-linked manner. It is characterized by the deficiency of the enzyme alpha-galactosidase, leading to a buildup of glycosphingolipids in the cells. Angiokeratoma is one of the cutaneous manifestations of this condition, and it helps making the diagnosis. The typical site involves the genital area in men and lumbosacral, buttocks and trunk region in both sexes. We report a case of genital angiokeratoma in a woman with Fabry disease. The diagnosis is through molecular analysis and, when made early, starting treatment reduces the morbidity and mortality of the disease. Thus, the dermatologist has an important role in the identification of angiokeratoma as a cutaneous marker, and the knowledge of its different presentations is essential for the early diagnosis and management of Fabry disease.

Levitated optomechanics with a fiber Fabry-Perot interferometer

NASA Astrophysics Data System (ADS)

Pontin, A.; Mourounas, L. S.; Geraci, A. A.; Barker, P. F.

2018-02-01

In recent years, quantum phenomena have been experimentally demonstrated on variety of optomechanical systems ranging from micro-oscillators to photonic crystals. Since single photon couplings are quite small, most experimental approaches rely on the realization of high finesse Fabry-Perot cavities in order to enhance the effective coupling. Here we show that by exploiting a, long path, low finesse fiber Fabry-Perot interferometer ground state cooling can be achieved. We model a 100 m long cavity with a finesse of 10 and analyze the impact of additional noise sources arising from the fiber. As a mechanical oscillator we consider a levitated microdisk but the same approach could be applied to other optomechanical systems.

All-fiber, long-active-length Fabry-Perot strain sensor.

PubMed

Pevec, Simon; Donlagic, Denis

2011-08-01

This paper presents a high-sensitivity, all-silica, all-fiber Fabry-Perot strain-sensor. The proposed sensor provides a long active length, arbitrary length of Fabry-Perot cavity, and low intrinsic temperature sensitivity. The sensor was micro-machined from purposely-developed sensor-forming fiber that is etched and directly spliced to the lead-in fiber. This manufacturing process has good potential for cost-effective, high-volume production. Its measurement range of over 3000 µε, and strain-resolution better than 1 µε were demonstrated by the application of a commercial, multimode fiber-based signal processor.

Early Renal Involvement in a Girl with Classic Fabry Disease.

PubMed

Perretta, Fernando; Antongiovanni, Norberto; Jaurretche, Sebastián

2017-01-01

Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency or absence of the enzyme alpha galactosidase A; this defect leads to the systemic accumulation of globotriaosylceramide and its metabolites. Organic involvement in men is well known, but in women it is controversial, mainly due to the random X-chromosome inactivation in each of their cells (Lyon hypothesis). This would explain why women (heterozygotes) present a wide variability in the severity of their phenotype. The manifestations are multisystemic and begin in early childhood, reaching a severe compromise in adulthood. Typical acroparesthesia in hands and feet, gastrointestinal symptoms, angiokeratomas, dyshidrosis, hearing loss, arrhythmias, hypertrophic cardiomyopathy, cerebrovascular accidents, and renal failure can be observed. Nephropathy is one of the major complications of Fabry disease. Glomerular and vascular changes are present before progression to overt proteinuria and decreased glomerular filtration rate, even in pediatric patients. A case of incipient renal involvement in a girl with classic Fabry disease is reported.

Charles Village Apartments Information Sheet

EPA Pesticide Factsheets

Charles Village Apartments (the Company) is located in Baltimore, Maryland. The settlement involves renovation activities conducted at property constructed prior to 1978, located in Baltimore, Maryland.

Impaired small fiber conduction in patients with Fabry disease: a neurophysiological case–control study

PubMed Central

2013-01-01

Background Fabry disease is an inborn lysosomal storage disorder which is associated with small fiber neuropathy. We set out to investigate small fiber conduction in Fabry patients using pain-related evoked potentials (PREP). Methods In this case–control study we prospectively studied 76 consecutive Fabry patients for electrical small fiber conduction in correlation with small fiber function and morphology. Data were compared with healthy controls using non-parametric statistical tests. All patients underwent neurological examination and were investigated with pain and depression questionnaires. Small fiber function (quantitative sensory testing, QST), morphology (skin punch biopsy), and electrical conduction (PREP) were assessed and correlated. Patients were stratified for gender and disease severity as reflected by renal function. Results All Fabry patients (31 men, 45 women) had small fiber neuropathy. Men with Fabry disease showed impaired cold (p < 0.01) and warm perception (p < 0.05), while women did not differ from controls. Intraepidermal nerve fiber density (IENFD) was reduced at the lower leg (p < 0.001) and the back (p < 0.05) mainly of men with impaired renal function. When investigating A-delta fiber conduction with PREP, men but not women with Fabry disease had lower amplitudes upon stimulation at face (p < 0.01), hands (p < 0.05), and feet (p < 0.01) compared to controls. PREP amplitudes further decreased with advance in disease severity. PREP amplitudes and warm (p < 0.05) and cold detection thresholds (p < 0.01) at the feet correlated positively in male patients. Conclusion Small fiber conduction is impaired in men with Fabry disease and worsens with advanced disease severity. PREP are well-suited to measure A-delta fiber conduction. PMID:23705943

Fabry disease, enzyme replacement therapy and the significance of antibody responses.

PubMed

Deegan, Patrick B

2012-03-01

Fabry disease is an X-linked disorder caused by a deficiency of α-galactosidase A. This leads to a progressive accumulation of globotriaosylceramide in tissues throughout the body. Cardiac, renal and neurological manifestations are common and life expectancy is significantly reduced relative to the general population. Management of Fabry disease involves the administration of intravenous enzyme replacement therapy (ERT). Two forms - agalsidase alfa and agalsidase beta - have been licensed in certain jurisdictions and are generally well tolerated; however, some patients develop antibodies to the infused enzyme, which may impair the efficacy and safety of treatment. Agalsidase alfa and agalsidase beta are produced in different systems; this leads to certain differences in post-translational modification that may affect immunogenicity. Immunoglobulin (Ig) G antibodies have frequently been reported in patients with Fabry disease receiving ERT; IgG responses are reported in a greater proportion of patients receiving agalsidase beta than in patients receiving agalsidase alfa. IgE antibodies are less common than IgG antibodies, and have not been observed in patients receiving agalsidase alfa. However, these data are difficult to interpret due to methodological differences in the assessment of seropositivity, and in the doses of enzyme used. The clinical impact of the development of IgG antibodies to ERT in patients with Fabry disease remains unclear, due to lack of data and to the marked heterogeneity of patients both in terms of disease manifestations and response to therapy. Further studies that examine the development of antibodies in patients with Fabry disease and the potential impact of such antibodies on the outcome of ERT are necessary.

Crescent shaped Fabry-Perot fiber cavity for ultra-sensitive strain measurement.

PubMed

Liu, Ye; Wang, D N; Chen, W P

2016-12-02

Optical Fabry-Perot interferometer sensors based on inner air-cavity is featured with compact size, good robustness and high strain sensitivity, especially when an ultra-thin air-cavity is adopted. The typical shape of Fabry-Perot inner air-cavity with reflection mode of operation is elliptic, with minor axis along with and major axis perpendicular to the fiber length. The first reflection surface is diverging whereas the second one is converging. To increase the visibility of the output interference pattern, the length of major axis should be large for a given cavity length. However, the largest value of the major axis is limited by the optical fiber diameter. If the major axis length reaches the fiber diameter, the robustness of the Fabry-Perot cavity device would be decreased. Here we demonstrate an ultra-thin crescent shaped Fabry-Perot cavity for strain sensing with ultra-high sensitivity and low temperature cross-sensitivity. The crescent-shape cavity consists of two converging reflection surfaces, which provide the advantages of enhanced strain sensitivity when compared with elliptic or D-shaped FP cavity. The device is fabricated by fusion splicing an etched multimode fiber with a single mode fiber, and hence is simple in structure and economic in cost.

Crescent shaped Fabry-Perot fiber cavity for ultra-sensitive strain measurement

NASA Astrophysics Data System (ADS)

Liu, Ye; Wang, D. N.; Chen, W. P.

2016-12-01

Optical Fabry-Perot interferometer sensors based on inner air-cavity is featured with compact size, good robustness and high strain sensitivity, especially when an ultra-thin air-cavity is adopted. The typical shape of Fabry-Perot inner air-cavity with reflection mode of operation is elliptic, with minor axis along with and major axis perpendicular to the fiber length. The first reflection surface is diverging whereas the second one is converging. To increase the visibility of the output interference pattern, the length of major axis should be large for a given cavity length. However, the largest value of the major axis is limited by the optical fiber diameter. If the major axis length reaches the fiber diameter, the robustness of the Fabry-Perot cavity device would be decreased. Here we demonstrate an ultra-thin crescent shaped Fabry-Perot cavity for strain sensing with ultra-high sensitivity and low temperature cross-sensitivity. The crescent-shape cavity consists of two converging reflection surfaces, which provide the advantages of enhanced strain sensitivity when compared with elliptic or D-shaped FP cavity. The device is fabricated by fusion splicing an etched multimode fiber with a single mode fiber, and hence is simple in structure and economic in cost.

Charles Bolden Confirmation Hearing

NASA Image and Video Library

2009-07-07

Charles Bolden, nominee for Administrator of NASA, testifies at his confirmation hearing before the Senate Commerce, Science and Transportation Committee in the Russell Senate Office Building on Capitol Hill in Washington, Wednesday, July 8, 2009. Photo Credit: (NASA/Bill Ingalls)

Sapphire Fabry-Perot high-temperature sensor study

NASA Astrophysics Data System (ADS)

Yao, Yi-qiang; Liang, Wei-long; Gui, Xinwang; Fan, Dian

2017-04-01

A new structure sapphire fiber Fabry-Perot (F-P) high-temperature sensor based on sapphire wafer was proposed and fabricated. The sensor uses the sapphire fiber as a transmission waveguide, the sapphire wafer as an Fabry-Perot (F-P) interferometer and the new structure of "Zirconia ferrule-Zirconia tube" as the sensor fixing structure of the sensor. The reflection spectrum of the interferometer was demodulated by a serial of data processing including FIR bandpass filter, FFT (Fast Fourier Transformation) estimation and LSE (least squares estimation) compensation to obtain more precise OPD. Temperature measurement range is from 20 to 1000°C in experiment. The experimental results show that the sensor has the advantages of small size, low cost, simple fabrication and high repeatability. It can be applied for longterm, stable and high-precision high temperature measurement in harsh environments.

Solid, 3-Mirror Fabry-Perot Etalon

NASA Technical Reports Server (NTRS)

Stephen, Mark; Fahey, Molly; Miller, Ian

2017-01-01

We present modeling and performance of a solid, fused silica, 3-mirror Fabry-Perot-type etalon. We show the optical cavity design and construction of the new etalon and show >95% peak transmission, improved passband shape and 20 dB better out of band rejection than a similar 2-mirror etalon.

Three-stage Fabry-Perot liquid crystal tunable filter with extended spectral range.

PubMed

Zheng, Zhenrong; Yang, Guowei; Li, Haifeng; Liu, Xu

2011-01-31

A method to extend spectral range of tunable optical filter is proposed in this paper. Two same tunable Fabry-Perot filters and an additional tunable filter with different free spectral range are cascaded to extend spectral range and reduce sidelobes. Over 400 nm of free spectral range and 4 nm of full width at half maximum of the filter were achieved. Design procedure and simulation are described in detail. An experimental 3-stage tunable Fabry-Perot filter with visible and infrared spectra is demonstrated. The experimental results and the theoretical analysis are presented in detail to verify this method. The results revealed that a compact and extended tunable spectral range of Fabry-Perot filter can be easily attainable by this method.

Use of PZT's for adaptive control of Fabry-Perot etalon plate figure

NASA Technical Reports Server (NTRS)

Skinner, WIlbert; Niciejewski, R.

2005-01-01

A Fabry Perot etalon, consisting of two spaced and reflective glass flats, provides the mechanism by which high resolution spectroscopy may be performed over narrow spectral regions. Space based applications include direct measurements of Doppler shifts of airglow absorption and emission features and the Doppler broadening of spectral lines. The technique requires a high degree of parallelism between the two flats to be maintained through harsh launch conditions. Monitoring and adjusting the plate figure by illuminating the Fabry Perot interferometer with a suitable monochromatic source may be performed on orbit to actively control of the parallelism of the flats. This report describes the use of such a technique in a laboratory environment applied to a piezo-electric stack attached to the center of a Fabry Perot etalon.

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

PubMed Central

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

Enhancing the diagnosis of fabry disease in cardiology with a targeted information: a before–after control–impact study

PubMed Central

Savary, Anne-Louise; Morello, Remy; Brasse-Lagnel, Carole; Milliez, Paul; Bekri, Soumeya; Labombarda, Fabien

2017-01-01

Background Cardiac complications in Fabry disease are frequent and dominated by a high frequency of left ventricular hypertrophy; therefore, cardiologists may have an essential role in screening for this disease. Providing cardiologists with targeted information on Fabry disease would be valuable and could reduce both diagnostic and therapeutic delays. The aim of this study was to evaluate the efficiency of such strategy for Fabry screening. Methods We conducted a before–after control–impact study by comparing observations made before and after targeted information on Fabry disease among cardiologists. The information on Fabry disease consisted of (1) an educational booklet, (2) oral information and (3) screening kits. The programme was evaluated at the end of a 12-month study period. Results Forty-two cardiologists participated to this study. None of them had conducted screening test and new diagnostic for Fabry disease in the 3 years prior the information. After the information, screening with dried blood spots was performed in 55 patients (ranged 18–77 years, men: 39) with cardiac monitoring for supposed sarcomeric hypertrophic cardiomyopathy (n=41) or unexplained left ventricular hypertrophy (n=14) from January 2015 to January 2016. Two new cases of Fabry disease were diagnosed (3.4%) in two men (ages 58 and 51 years). The information was deemed relevant in both content and structure and was deemed useful for everyday practice. Conclusion Cardiologists valued the targeted information on Fabry disease. This information had a direct clinical impact by allowing the diagnosis of two new families with Fabry disease. PMID:28409012

76 FR 20033 - Robert Charles Ley, D.O. ; Dismissal of Proceeding

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-11

... DEPARTMENT OF JUSTICE Drug Enforcement Administration [Docket No. 10-8] Robert Charles Ley, D.O... Charles Ley, D.O. (Respondent), of Kihei, Hawaii. Order to Show Cause at 1. The Order, which also sought... Suspension of Registration issued to Robert Charles Ley, D.O, be, and it hereby is, dismissed. Dated: April 1...

CIV Polarization Measurements using a Vacuum Ultraviolet Fabry-Perot Interferometer

NASA Technical Reports Server (NTRS)

West, Edward; Gary, G. Allen; Cirtain, Jonathan; David, John; Kobayashi, Ken; Pietraszewski, Chris

2009-01-01

Marshall Space Flight Center's (MSFC) is developing a Vacuum Ultraviolet (VUV) Fabry-P rot Interferometer that will be launched on a sounding rocket for high throughput, high-cadence, extended field of view CIV (155nm) measurements. These measurements will provide (i) Dopplergrams for studies of waves, oscillations, explosive events, and mass motions through the transition region, and, (ii), polarization measurements to study the magnetic field in the transition region. This paper will describe the scientific goals of the instrument, a brief description of the optics and the polarization characteristics of the VUV Fabry P rot.

Affective and cognitive behavior in the alpha-galactosidase A deficient mouse model of Fabry disease

PubMed Central

Karl, Franziska; Sommer, Claudia; Üçeyler, Nurcan

2017-01-01

Fabry disease is an X-linked inherited lysosomal storage disorder with intracellular accumulation of globotriaosylceramide (Gb3) due to α-galactosidase A (α-Gal A) deficiency. Fabry patients frequently report of anxiety, depression, and impaired cognitive function. We characterized affective and cognitive phenotype of male mice with α-Gal A deficiency (Fabry KO) and compared results with those of age-matched male wildtype (WT) littermates. Young (3 months) and old (≥ 18 months) mice were tested in the naïve state and after i.pl. injection of complete Freund`s adjuvant (CFA) as an inflammatory pain model. We used the elevated plus maze (EPM), the light-dark box (LDB) and the open field test (OF) to investigate anxiety-like behavior. The forced swim test (FST) and Morris water maze (MWM) were applied to assess depressive-like and learning behavior. The EPM test revealed no intergroup difference for anxiety-like behavior in naïve young and old Fabry KO mice compared to WT littermates, except for longer time spent in open arms of the EPM for young WT mice compared to young Fabry KO mice (p<0.05). After CFA injection, young Fabry KO mice showed increased anxiety-like behavior compared to young WT littermates (p<0.05) and naïve young Fabry KO mice (p<0.05) in the EPM as reflected by shorter time spent in EPM open arms. There were no relevant differences in the LDB and the OF test, except for longer time spent in the center zone of the OF by young WT mice compared to young Fabry KO mice (p<0.05). Complementary to this, depression-like and learning behavior were not different between genotypes and age-groups, except for the expectedly lower memory performance in older age-groups compared to young mice. Our results indicate that genetic influences on affective and cognitive symptoms in FD may be of subordinate relevance, drawing attention to potential influences of environmental and epigenetic factors. PMID:28662189

Nematic Fabry-Perot etalons for ground- and space-based atmospheric remote sensing

NASA Astrophysics Data System (ADS)

Noto, John; Schneller, Kristin E.; Schneller, William J.; Kerr, Robert B.; Doe, R. A.

1997-10-01

Birefringent, nematic liquid crystals (LC) have been laminated between the substrates of several Fabry-Perot etalons. The application of an electric field allows the effective index of refraction of the LC to be varied. A polymer alignment layer is used to align the crystals perpendicular to the optical axis of the Fabry-Perot etalon. An oscillating electric field is used to rotate the crystal around the optical axis of the etalon, effectively changing the index of refraction. This change in index is used to tune the Fabry-Perot etalon in a manner similar to traditional pressure and mechanical tuning systems. However, the approach described here has the advantage of producing a solid-state etalon that is tunable without needing a bulky pressure system or environmentally sensitive piezo-electric stacks. A two etalon spectrometer consisting of two Fabry- Perot etalons coupled to a CID detector has been developed. A suppression etalon with a gap of 10 micrometers , and a LC wit a refractive index of 1.63 are used in conjunction with a high resolution etalon to produce an instrument ideal for observing the atomic spectra of hot, light neutral species and the molecular bands in the atmosphere. Several other etalons have been constructed to further develop this technology. Clear apertures greater than 2 inches have been achieved, and a hybrid spacer technique has been developed to allow for etalons with spacings of up to 1 cm. Fabry- Perot partial reflective coatings capable of operation from the visible to the NIR will also be discussed.

Charles Bolden Confirmation Hearing

NASA Image and Video Library

2009-07-07

Charles Bolden, nominee for Administrator of NASA, waits for his turn to testify at his confirmation hearing before the Senate Commerce, Science and Transportation Committee in the Russell Senate Office Building on Capitol Hill in Washington, Wednesday, July 8, 2009. Photo Credit: (NASA/Bill Ingalls)

Glycosphingolipid storage in Fabry mice extends beyond globotriaosylceramide and is affected by ABCB1 depletion

PubMed Central

Kamani, Mustafa A; Provençal, Philippe; Boutin, Michel; Pacienza, Natalia; Fan, Xin; Novak, Anton; Huang, Tonny C; Binnington, Beth; Au, Bryan C; Auray-Blais, Christiane; Lingwood, Clifford A; Medin, Jeffrey A

2016-01-01

Aim: Fabry disease is caused by α-galactosidase A deficiency leading to accumulation of globotriaosylceramide (Gb3) in tissues. Clinical manifestations do not appear to correlate with total Gb3 levels. Studies examining tissue distribution of specific acyl chain species of Gb3 and upstream glycosphingolipids are lacking. Material & methods/Results: Thorough characterization of the Fabry mouse sphingolipid profile by LC-MS revealed unique Gb3 acyl chain storage profiles. Storage extended beyond Gb3; all Fabry tissues also accumulated monohexosylceramides. Depletion of ABCB1 had a complex effect on glycosphingolipid storage. Conclusion: These data provide insights into how specific sphingolipid species correlate with one another and how these correlations change in the α-galactosidase A-deficient state, potentially leading to the identification of more specific biomarkers of Fabry disease. PMID:28116130

A survey of the pain experienced by males and females with Fabry disease

PubMed Central

Gibas, Andrea L; Klatt, Regan; Johnson, Jack; Clarke, Joe TR; Katz, Joel

2006-01-01

BACKGROUND The clinical onset of Fabry disease, a rare, X-linked, multisystemic disorder, is marked by neuropathic pain. Males suffer extensively from this disease. Females, as genetic ‘carriers’, have traditionally been viewed as either asymptomatic or mildly afflicted with this disease. OBJECTIVES To describe Fabry-related pain and compare experiences between the sexes. Patients’ perceptions of physician pain assessments were also examined. METHODS A disease-specific questionnaire was accessible on-line (www.fabry.org) and mailed to 552 members of a Fabry disease support group. RESULTS The response rate was 14.3% for the support group-based mail questionnaire. Females (58.0%) were significantly older (mean ± SD 45.9±13.5 years) than males (mean ± SD 40.0±12.1; t [86]=−2.11, P<0.05). Females were diagnosed with Fabry disease later (31.1±14.0 years) than males (24.2±11.9 years; t [86]=−2.43, P<0.05). Females (mean score for pain disability rating 3.0±1.4) suffered more extensive disability from migraine pain (mean score 2.2±1.3; F [1, 74]=45.0, P<0.005), and, unlike males, did not exhibit a decline in pain intensity with disease duration. Satisfaction with physician pain assessments was moderate. CONCLUSIONS Contrary to the traditional view of females as carriers, females with Fabry disease experienced intense disease-related pain; pain produced comparable distress and impairment in both sexes. The diagnostic delay and absence of a decline in pain symptoms over time in females suggest additional disease burden. Females may be triply disadvantaged in the health care system due to disease rarity, devalued carrier status and sex. PMID:16960635

Enzyme replacement therapy of Fabry disease.

PubMed

Clarke, Joe T R; Iwanochko, R Mark

2005-08-01

Fabry disease is an X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A and results in pain, progressive renal impairment, cardiomyopathy, and cerebrovascular disease. The results of two major randomized, double-blind, placebo-controlled clinical trials and open-label extensions have shown that replacement of the deficient enzyme with either of two preparations of recombinant human alpha-galactosidase A, agalsidase-alfa, and agalsidase-beta is safe. Biweekly i.v. infusions of 0.2 mg/kg of agalsidase-alfa were associated with a significant decrease in pain and stabilization of renal function. Biweekly infusions of 1 mg/kg of agalsidase-beta were associated with virtually complete clearing of accumulated glycolipid substrate from renal and cutaneous capillary endothelial cells. Several smaller, open-label studies, along with observations made in the course of monitoring large numbers of patients on enzyme replacement therapy, indicated that treatment stabilizes renal function and produces significant improvements in myocardial mass and function. Treatment of Fabry disease by enzyme replacement has a significant impact on at least some serious complications of the disease.

Anderson-Fabry disease in heart failure.

PubMed

Akhtar, M M; Elliott, P M

2018-06-16

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene that result in deficiency of the enzyme alpha-galactosidase A. The worldwide incidence of Fabry's disease is reported to be in the range of 1 in 40,000-117,000, although this value may be a significant underestimate given under recognition of symptoms and delayed or missed diagnosis. Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system. Gb3 accumulation induces pathology via the release of pro-inflammatory cytokines, growth-promoting factors and by oxidative stress, resulting in myocardial extracellular matrix remodelling, left ventricular hypertrophy (LVH), vascular dysfunction and interstitial fibrosis. Cardiac involvement manifesting as ventricular hypertrophy, systolic and diastolic dysfunction, valvular abnormalities and conduction tissue disease is common in AFD and is associated with considerable cardiovascular morbidity and mortality from heart failure, sudden cardiac death and stroke-related death.

The Charles Bonnet syndrome: a review of recent research.

PubMed

Rovner, Barry W

2006-06-01

The Charles Bonnet syndrome is a disorder of visual hallucinations typically occurring in older persons with vision impairment or deafferentation of the visual cortex. This review cites recent studies on Charles Bonnet syndrome and discusses treatment options. The numbers of affected persons will increase with aging of the population, making recognition and treatment important components of ophthalmologic care. The etiology of the Charles Bonnet syndrome is varied; most often it involves direct damage to the visual system (e.g. age-related macular degeneration, glaucoma) but it may also result from cerebral pathology interrupting connections between the eye and the occipital cortex. Case reports of different management approaches demonstrate the range of treatment options. This review suggests that the Charles Bonnet syndrome will affect an increasingly large number of older persons as the population ages and the occurrence of vision and cerebral disorders increases. Clinical trials of antipsychotic and other medications, as well as low-vision rehabilitation, are necessary to establish valid treatments for this disorder.

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

PubMed

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

2016-07-01

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. 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. 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. 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. NCT00196742. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Nano-LC-MS/MS for Quantification of Lyso-Gb3 and Its Analogues Reveals a Useful Biomarker for Fabry Disease

PubMed Central

Sueoka, Hideaki; Ichihara, Junji; Tsukimura, Takahiro; Togawa, Tadayasu; Sakuraba, Hitoshi

2015-01-01

Biomarkers useful for diagnosis and evaluation of treatment for patients with Fabry disease are urgently needed. Recently, plasma globotriaosylsphingosine (lyso-Gb3) and lyso-Gb3-related analogues have attracted attention as promising biomarkers of Fabry disease. However, the plasma concentrations of lyso-Gb3 and its analogues are extremely low or below the detection limits in some Fabry patients as well as in healthy subjects. In this paper, we introduce the novel application of a nano-liquid chromatography-tandem mass spectrometry (nano-LC-MS/MS) system to the measurement of lyso-Gb3 and its analogues in plasma. Nano-LC-MS/MS requires smaller amounts of samples and is more sensitive than conventional techniques. Using this method, we measured the plasma concentrations of lyso-Gb3 and its analogues in 40 healthy subjects, 5 functional variants (males with E66Q), and various Fabry patients (9 classic Fabry males/9 mutations; 7 later-onset Fabry males/5 mutations; and 10 Fabry females/9 mutations). The results revealed that the mean lyso-Gb3 and lyso-Gb3(-2) concentrations in all the Fabry patient subgroups were statistically higher, especially in the classic Fabry males, than those in the functional variants and healthy subjects. The plasma concentrations of lyso-Gb3 and its analogues in healthy subjects, functional variants, and some Fabry patients with specific mutations (R112H and M296I) that cannot be established by conventional techniques were successfully determined by means of nano-LC-MS/MS. The lyso-Gb3 and lyso-Gb3(-2) concentrations in male patients with these mutations were lower than those in most Fabry patients having other mutations, but higher than those in the functional variants and healthy subjects. This new method is expected to be useful for sensitive determination of the plasma concentrations of lyso-Gb3 and its analogues. This study also revealed that not only lyso-Gb3 but also lyso-Gb3(-2) in plasma is a useful biomarker for the diagnosis of

Q&A: Charles Michener

NASA Astrophysics Data System (ADS)

Gould, Julie

2015-05-01

Charles Michener has been studying bees for more than 80 years, and, although he has seen many changes in the field, his interest in these insects has not diminished. Now aged 96, he contributes to bee research as a Watkins distinguished professor emeritus at Kansas University in Lawrence.

Charles Darwin's Botanical Investigations

ERIC Educational Resources Information Center

Harley, Suzanne M.

2010-01-01

Charles Darwin's botanical studies provide a way to expose students to his work that followed the publication of "On the Origin of Species." We can use stories from his plant investigations to illustrate key concepts in the life sciences and model how questions are asked and answered in science.

Charles Darwin 1809-2009.

PubMed

van Wyhe, John

2009-02-01

The year 2009 is the bicentenary of the birth of Charles Darwin and the 150th anniversary of the publication of On the Origin of Species. This article briefly surveys his life and work, dispelling some common myths and summarizes Darwin's achievement and legacy at his death in 1882.

Rayleigh Scattering Measurements Using a Tunable Liquid Crystal Fabry-Perot Interferometer

NASA Technical Reports Server (NTRS)

Mielke-Fagan, Amy F.; Clem, Michelle M.; Elam, Kristie A.

2010-01-01

Spectroscopic Rayleigh scattering is an established flow diagnostic that has the ability to provide simultaneous density, velocity, and temperature measurements. The Fabry-Perot interferometer or etalon is a commonly employed instrument for resolving the spectrum of molecular Rayleigh scattered light for the purpose of evaluating these flow properties. This paper investigates the use of a tunable liquid crystal (LC) Fabry-Perot etalon in Rayleigh scattering experiments at NASA Glenn Research Center. The LC etalon provides a robust interferometry system that can be tuned rapidly by adjusting the voltage applied to the liquid crystal interface. Tuning the interferometer is often necessary to control the physical locations of the concentric interference fringes when Rayleigh light is imaged through the LC etalon. The LC etalon diagnostic system was tested in a 1-cm diameter nozzle flow in two different scattering configurations to evaluate its usefulness for Rayleigh measurements compared to a traditional non-tunable fused silica Fabry-Perot etalon.

76 FR 21889 - Lasky, Charles D.; Notice of Filing

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-19

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ID-4962-007] Lasky, Charles D.; Notice of Filing Take notice that on April 12, 2011, Charles D. Lasky submitted for filing an... the Commission's Rules of Practice and Procedure (18 CFR 385.211, 385.214). Protests will be...

Enzyme replacement therapy with agalsidase beta improves cardiac involvement in Fabry's disease.

PubMed

Spinelli, L; Pisani, A; Sabbatini, M; Petretta, M; Andreucci, M V; Procaccini, D; Lo Surdo, N; Federico, S; Cianciaruso, B

2004-08-01

Fabry's disease is an X-linked lysosomal storage disease caused by a deficiency of alpha-galactosidase that results in an accumulation of neutral glycosphingolipids throughout the body, including the cardiovascular system. Fabry cardiomyopathy, characterized by progressive severe concentric left ventricular (LV) hypertrophy, is very frequent and is the most important cause of death in affected patients. Enzyme replacement therapy (ERT) allows a specific treatment for this disease, however, there are very few data on the effectiveness of therapy on cardiac involvement. Nine patients with Fabry cardiac disease were studied on basal condition and after 6 and 12 months of treatment with algasidase beta (Fabrazyme). A complete clinical, electrocardiographic and echocardiographic evaluation was performed in all patients. Interpretable Doppler recordings of transmitral flow and pulmonary flow velocity curves were also acquired. At baseline, the patients with Fabry's disease had increased LV septum and posterior wall thickness, normal LV fractional shortening, LV ejection fraction, normal Doppler parameters of mitral inflow but a duration of pulmonary vein flow velocity wave exceeding that of the mitral wave at atrial systole. ERT did not affect heart rate and arterial pressure. LV internal diameters did not change, there was a slight but not significant decrease in the LV posterior wall thickening and a progressive decrease in the interventricular septum thickening (p < 0.025) and in LV mass (p < 0.001) The difference in duration between pulmonary vein flow velocity wave and mitral wave at atrial systole significantly decreased (p < 0.001). These results suggest that ERT in patients with Fabry cardiomyopathy is able to reduce the LV mass and ameliorate the LV stiffness. Copyright 2004 Blackwell Munksgaard

A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse.

PubMed

Shin, Seok-Ho; Park, Min-Ho; Byeon, Jin-Ju; Lee, Byeong Ill; Park, Yuri; Ko, Ah-Ra; Seong, Mi-Ran; Lee, Soyeon; Kim, Mi Ra; Seo, Jinwook; Jung, Myung Eun; Jin, Dong-Kyu; Shin, Young G

2018-06-07

Fabry disease is a rare lysosomal storage disorder resulting from the lack of α-Gal A gene activity. Globotriaosylceramide (GB3, ceramide trihexoside) is a novel endogenous biomarker which predicts the incidence of Fabry disease. At the early stage efficacy/biomarker study, a rapid method to determine this biomarker in plasma and in all relevant tissues related to this disease simultaneously is required. However, the limited sample volume, as well as the various levels of GB3 in different matrices makes the GB3 quantitation very challenging. Hereby we developed a rapid method to identify GB3 in mouse plasma and various tissues. Preliminary stability tests were also performed in three different conditions: short-term, freeze-thaw, long-term. The calibration curve was well fitted over the concentration range of 0.042⁻10 μg/mL for GB3 in plasma and 0.082⁻20 μg/g for GB3 in various tissues. This method was successfully applied for the comparison of GB3 levels in Fabry model mice (B6;129-Gla tm1Kul /J), which has not been performed previously to the best of our knowledge.

Fiber optic microphone with large dynamic range based on bi-fiber Fabry-Perot cavity

NASA Astrophysics Data System (ADS)

Cheng, Jin; Lu, Dan-feng; Gao, Ran; Qi, Zhi-mei

2017-10-01

In this paper, we report a fiber optic microphone with a large dynamic range. The probe of microphone consists of bi-fiber Fabry-Perot cavity architecture. The wavelength of the working laser is about 1552.05nm. At this wavelength, the interference spectroscopies of these two fiber Fabry-Perot cavities have a quadrature shift. So the outputs of these two fiber Fabry-Perot sensors are orthogonal signal. By using orthogonal signal demodulation method, this microphone can output a signal of acoustic wave. Due to no relationship between output signal and the linear region on interference spectroscopy, the microphones have a large maximum acoustic pressure above 125dB.

Charles Bolden Confirmation Hearing

NASA Image and Video Library

2009-07-07

Charles Bolden, nominee for Administrator of NASA, left, and Lori Garver, nominee for Deputy Administrator of NASA, listen to Senators questions at their confirmation hearing before the Senate Commerce, Science and Transportation Committee in the Russell Senate Office Building on Capitol Hill in Washington, Wednesday, July 8, 2009. Photo Credit: (NASA/Bill Ingalls)

In-fiber Fabry-Perot refractometer assisted by a long-period grating.

PubMed

Mosquera, L; Sáez-Rodriguez, D; Cruz, J L; Andrés, M V

2010-02-15

We present an optical fiber refractometer based on a Fabry-Perot interferometer defined by two fiber Bragg gratings and an intracavity long-period grating that makes the light confined in the resonator interact with the surrounding medium. The external refractive index is monitored by the resonant frequencies of the Fabry-Perot interferometer, which can be measured either in transmission or in reflection. In this first experiment, wavelength shifts measured with a resolution of 0.1 pm have allowed one to establish a refractive index detection limit of 2.1x10(-5).

Phenotypic characteristics of the p.Asn215Ser (p.N215S) GLA mutation in male and female patients with Fabry disease: A multicenter Fabry Registry study.

PubMed

Germain, Dominique P; Brand, Eva; Burlina, Alessandro; Cecchi, Franco; Garman, Scott C; Kempf, Judy; Laney, Dawn A; Linhart, Aleš; Maródi, László; Nicholls, Kathy; Ortiz, Alberto; Pieruzzi, Federico; Shankar, Suma P; Waldek, Stephen; Wanner, Christoph; Jovanovic, Ana

2018-04-12

The p.Asn215Ser or p.N215S GLA variant has been associated with late-onset cardiac variant of Fabry disease. To expand on the scarce phenotype data, we analyzed natural history data from 125 p.N215S patients (66 females, 59 males) enrolled in the Fabry Registry (NCT00196742) and compared it with data from 401 patients (237 females, 164 males) harboring mutations associated with classic Fabry disease. We evaluated interventricular septum thickness (IVST), left ventricular posterior wall thickness (LVPWT), estimated glomerular filtration rate and severe clinical events. In p.N215S males, mildly abnormal mean IVST and LVPWT values were observed in patients aged 25-34 years, and values gradually increased with advancing age. Mean values were similar to those of classic males. In p.N215S females, these abnormalities occurred primarily in patients aged 55-64 years. Severe clinical events in p.N215S patients were mainly cardiac (males 31%, females 8%) while renal and cerebrovascular events were rare. Renal impairment occurred in 17% of p.N215S males (mostly in patients aged 65-74 years), and rarely in females (3%). p.N215S is a disease-causing mutation with severe clinical manifestations found primarily in the heart. Cardiac involvement may become as severe as in classic Fabry patients, especially in males. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

E-Learning for Rare Diseases: An Example Using Fabry Disease.

PubMed

Cimmaruta, Chiara; Liguori, Ludovica; Monticelli, Maria; Andreotti, Giuseppina; Citro, Valentina

2017-09-24

Rare diseases represent a challenge for physicians because patients are rarely seen, and they can manifest with symptoms similar to those of common diseases. In this work, genetic confirmation of diagnosis is derived from DNA sequencing. We present a tutorial for the molecular analysis of a rare disease using Fabry disease as an example. An exonic sequence derived from a hypothetical male patient was matched against human reference data using a genome browser. The missense mutation was identified by running BlastX, and information on the affected protein was retrieved from the database UniProt. The pathogenic nature of the mutation was assessed with PolyPhen-2. Disease-specific databases were used to assess whether the missense mutation led to a severe phenotype, and whether pharmacological therapy was an option. An inexpensive bioinformatics approach is presented to get the reader acquainted with the diagnosis of Fabry disease. The reader is introduced to the field of pharmacological chaperones, a therapeutic approach that can be applied only to certain Fabry genotypes. The principle underlying the analysis of exome sequencing can be explained in simple terms using web applications and databases which facilitate diagnosis and therapeutic choices.

Modelling the resource implications of managing adults with Fabry disease in Italy.

PubMed

Guest, Julian F; Concolino, Daniela; Di Vito, Raffaele; Feliciani, Claudio; Parini, Rossella; Zampetti, Anna

2011-07-01

This study estimated the resource implications and budget impact of managing adults with Fabry disease in Italy, from the perspective of the Servizio Sanitario Nazionale (SSN). A decision model was constructed using published clinical outcomes and clinician-derived resource utilisation estimates depicting the management of adults with Fabry disease in Italy. The expected annual cost of managing 220 existing and 20 new Fabry patients in Italy was estimated to be €28·3 million. In an average year, patients receiving enzyme replacement therapy (ERT) with 0·2 mg kg(-1) agalsidase alfa (Replagal; Shire Human Genetic Therapies, Basingstoke, Hampshire, UK) or 1·0 mg kg(-1) agalsidase beta (Fabrazyme; Genzyme Europe BV, Naarden, The Netherlands) are collectively expected to make 4500 hospital attendances to a day ward for infusions, which equates to 2000 eight-h days on the day ward associated with ERT. If all ERT-treated patients received their infusions at home, there would be a marginal reduction in the annual health care cost to manage these patients, and the total annual number of days on the day ward associated with ERT in the second year could potentially be reduced from a mean 2000 to zero, thereby releasing substantial hospital resources for use by non-Fabry patients. Currently, only agalsidase alfa is licensed for home treatment in Italy; hence, only patients receiving this enzyme could be offered home treatment. Use of agalsidase alfa (0·2 mg kg(-1) ) instead of agalsidase beta (1·0 mg kg(-1)) has the potential to reduce health care costs and release hospital resources in different specialities for alternative use by non-Fabry patients, thereby improving the efficiency of the public health care system in Italy. © 2011 The Authors. European Journal of Clinical Investigation © 2011 Stichting European Society for Clinical Investigation Journal Foundation.

How well does urinary lyso-Gb3 function as a biomarker in Fabry disease?

PubMed

Auray-Blais, Christiane; Ntwari, Aimé; Clarke, Joe T R; Warnock, David G; Oliveira, João Paulo; Young, Sarah P; Millington, David S; Bichet, Daniel G; Sirrs, Sandra; West, Michael L; Casey, Robin; Hwu, Wuh-Liang; Keutzer, Joan M; Zhang, X Kate; Gagnon, René

2010-12-14

Fabry disease is characterized by accumulation of glycosphingolipids, such as globotriaosylceramide (Gb(3)), in many tissues and body fluids. A novel plasma biomarker, globotriaosylsphingosine (lyso-Gb(3)), is increased in patients with the disease. Until now, lyso-Gb(3) was not detectable in urine, possibly because of the presence of interfering compounds. We undertook to: 1) characterize lyso-Gb(3) in urine; 2) develop a method to quantitate urinary lyso-Gb(3) by mass spectrometry; 3) evaluate urinary lyso-Gb(3) as a potential biomarker for Fabry disease; and 4) determine whether lyso-Gb(3) is an inhibitor of α-galactosidase A activity. We analyzed urinary lyso-Gb(3) from 83 Fabry patients and 77 healthy age-matched controls. The intraday and interday bias and precision of the method were <15%. Increases in lyso-Gb(3)/creatinine correlated with the concentrations of Gb(3) (r(2)=0.43), type of mutations (p=0.0006), gender (p<0.0001) and enzyme replacement therapy status (p=0.0012). Urine from healthy controls contained no detectable lyso-Gb(3). Lyso-Gb(3) did not inhibit GLA activity in dried blood spots. Increased urinary excretion of lyso-Gb(3) of Fabry patients correlated well with a number of indicators of disease severity. Lyso-Gb(3) is a reliable independent biomarker for clinically important characteristics of Fabry disease. Copyright © 2010 Elsevier B.V. All rights reserved.

Cardiomyopathy and response to enzyme replacement therapy in a male mouse model for Fabry disease.

PubMed

Nguyen Dinh Cat, Aurelie; Escoubet, Brigitte; Agrapart, Vincent; Griol-Charhbili, Violaine; Schoeb, Trenton; Feng, Wenguang; Jaimes, Edgar; Warnock, David G; Jaisser, Frederic

2012-01-01

Fabry disease is an X-linked disorder of glycosphingolipid metabolism that results in progressive accumulation of neutral glycosphingolipids, (predominately globotriaosylceramide; GL-3) in lysosomes, as well as other cellular compartments and the extracellular space. Our aim was to characterize the cardiac phenotype of male knock-out mice that are deficient in alpha-galactosidase A activity, as a model for Fabry disease and test the efficacy of Enzyme Replacement Therapy with agalsidase-beta. Male mice (3-4 months of age) were characterized with awake blood pressure and heart rate measurements, cardiac echocardiography and electrocardiography measurements under light anesthesia, histological studies and molecular studies with real-time polymerase chain reaction. The Fabry knock-out mouse has bradycardia and lower blood pressure than control wild type (CB7BL/6J) mice. In Fabry knock-out mice, the cardiomyopathy associated mild hypertrophy at echography with normal systolic LV function and mild diastolic dysfunction. Premature atrial contractions were more frequent in without conduction defect. Heart weight normalized to tibial length was increased in Fabry knock-out mice. Ascending aorta dilatation was observed. Molecular studies were consistent with early stages of cardiac remodeling. A single dose of agalsidase-beta (3 mg/kg) did not affect the LV hypertrophy, function or heart rate, but did improve the mRNA signals of early cardiac remodeling. In conclusion, the alpha-galactosidase A deficient mice at 3 to 4 months of age have cardiac and vascular alterations similar to that described in early clinical stage of Fabry disease in children and adolescents. Enzyme replacement therapy affects cardiac molecular remodeling after a single dose.

Cardiomyopathy and Response to Enzyme Replacement Therapy in a Male Mouse Model for Fabry Disease

PubMed Central

Nguyen Dinh Cat, Aurelie; Escoubet, Brigitte; Agrapart, Vincent; Griol-Charhbili, Violaine; Schoeb, Trenton; Feng, Wenguang; Jaimes, Edgar; Warnock, David G.; Jaisser, Frederic

2012-01-01

Fabry disease is an X-linked disorder of glycosphingolipid metabolism that results in progressive accumulation of neutral glycosphingolipids, (predominately globotriaosylceramide; GL-3) in lysosomes, as well as other cellular compartments and the extracellular space. Our aim was to characterize the cardiac phenotype of male knock-out mice that are deficient in alpha-galactosidase A activity, as a model for Fabry disease and test the efficacy of Enzyme Replacement Therapy with agalsidase-beta. Male mice (3–4 months of age) were characterized with awake blood pressure and heart rate measurements, cardiac echocardiography and electrocardiography measurements under light anesthesia, histological studies and molecular studies with real-time polymerase chain reaction. The Fabry knock-out mouse has bradycardia and lower blood pressure than control wild type (CB7BL/6J) mice. In Fabry knock-out mice, the cardiomyopathy associated mild hypertrophy at echography with normal systolic LV function and mild diastolic dysfunction. Premature atrial contractions were more frequent in without conduction defect. Heart weight normalized to tibial length was increased in Fabry knock-out mice. Ascending aorta dilatation was observed. Molecular studies were consistent with early stages of cardiac remodeling. A single dose of agalsidase-beta (3 mg/kg) did not affect the LV hypertrophy, function or heart rate, but did improve the mRNA signals of early cardiac remodeling. In conclusion, the alpha-galactosidase A deficient mice at 3 to 4 months of age have cardiac and vascular alterations similar to that described in early clinical stage of Fabry disease in children and adolescents. Enzyme replacement therapy affects cardiac molecular remodeling after a single dose. PMID:22574107

Band-pass Fabry-Pèrot magnetic tunnel junctions

NASA Astrophysics Data System (ADS)

Sharma, Abhishek; Tulapurkar, Ashwin. A.; Muralidharan, Bhaskaran

2018-05-01

We propose a high-performance magnetic tunnel junction by making electronic analogs of optical phenomena such as anti-reflections and Fabry-Pèrot resonances. The devices we propose feature anti-reflection enabled superlattice heterostructures sandwiched between the fixed and the free ferromagnets of the magnetic tunnel junction structure. Our predictions are based on non-equilibrium Green's function spin transport formalism coupled self-consistently with the Landau-Lifshitz-Gilbert-Slonczewski equation. Owing to the physics of bandpass spin filtering in the bandpass Fabry-Pèrot magnetic tunnel junction device, we demonstrate an ultra-high boost in the tunnel magneto-resistance (≈5 × 104%) and nearly 1200% suppression of spin transfer torque switching bias in comparison to a traditional trilayer magnetic tunnel junction device. The proof of concepts presented here can lead to next-generation spintronic device design harvesting the rich physics of superlattice heterostructures and exploiting spintronic analogs of optical phenomena.

Patients with Fabry disease after enzyme replacement therapy dose reduction versus treatment switch.

PubMed

Weidemann, Frank; Krämer, Johannes; Duning, Thomas; Lenders, Malte; Canaan-Kühl, Sima; Krebs, Alice; Guerrero González, Hans; Sommer, Claudia; Üçeyler, Nurcan; Niemann, Markus; Störk, Stefan; Schelleckes, Michael; Reiermann, Stefanie; Stypmann, Jörg; Brand, Stefan-Martin; Wanner, Christoph; Brand, Eva

2014-04-01

Because of the shortage of agalsidase-beta in 2009, many patients with Fabry disease were treated with lower doses or were switched to agalsidase-alfa. This observational study assessed end-organ damage and clinical symptoms during dose reduction or switch to agalsidase-alfa. A total of 105 adult patients with Fabry disease who had received agalsidase-beta (1.0 mg/kg body weight) for ≥1 year were nonrandomly assigned to continue this treatment regimen (regular-dose group, n=38), receive a reduced dose of 0.3-0.5 mg/kg (dose-reduction group, n=29), or switch to 0.2 mg/kg agalsidase-alfa (switch group) and were followed prospectively for 1 year. We assessed clinical events (death, myocardial infarction, severe arrhythmia, stroke, progression to ESRD); changes in cardiac, renal, and neurologic function; and Fabry-related symptoms (neuropathic pain, hypohidrosis, diarrhea, and disease severity scores). Organ function and Fabry-related symptoms remained stable in the regular-dose group. In contrast, estimated GFR decreased by about 3 ml/min per 1.73 m(2) (P=0.01) in the dose-reduction group, and the median albumin-to-creatinine ratio increased from 114 (0-606) mg/g to 216 (0-2062) mg/g (P=0.03) in the switch group. Furthermore, mean Mainz Severity Score Index scores and frequencies of pain attacks, chronic pain, gastrointestinal pain, and diarrhea increased significantly in the dose-reduction and switch groups. In conclusion, patients receiving regular agalsidase-beta dose had a stable disease course, but dose reduction led to worsening of renal function and symptoms. Switching to agalsidase-alfa is safe, but microalbuminuria may progress and Fabry-related symptoms may deteriorate.

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

NASA Astrophysics Data System (ADS)

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.

Miniaturized fiber inline Fabry-Perot interferometer for chemical sensing.

DOT National Transportation Integrated Search

2010-01-01

This paper demonstrates the chemical sensing capability of a miniaturized fiber inline Fabry-Prot sensor fabricated by femtosecond : laser. Its accessible cavity enables the device to measure the refractive index within the cavity. The refractive i...

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

ERIC Educational Resources Information Center

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)

Power-ratio tunable dual-wavelength laser using linearly variable Fabry-Perot filter as output coupler.

PubMed

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.

Fabry-Perot Interferometer-Based Electrooptic Modulator using LiNbO3 and Organic Thin Films

NASA Technical Reports Server (NTRS)

Banks, C.; Frazier, D.; Penn, B.; Abdeldayem, H.; Sharma, A.; Yelleswarapu, C.; Leyderman, Alexander; Correa, Margarita; Curreri, Peter A. (Technical Monitor)

2002-01-01

We report the study of a Fabry-Perot electro-optical modulator using thin crystalline film NPP, and Crystalline LiNbO3. We are able to observe 14, and 60 percent degree of modulation. Measurements were carried using a standard lock-in amplifier with a silicon detector. The proposal to design a Fabry-Perot electro-optic modulator with an intracavity electro-optically active organic material was based on the initial results using poled polymer thin films. The main feature of the proposed device is the observation that in traditional electrooptic modulators like a Packets cell, it requires few kilovolts of driving voltage to cause a 3 dB modulation even in high figure-of-merit electrooptic materials like LiNbO3. The driving voltage for the modulator can be reduced to as low as 10 volts by introducing the electrooptic material inside die resonant cavity of a Fabry-Perot modulator. This is because the transmission of the Fabry-Perot cavity varies nonlinearly with the change of refractive index or phase of light due to applied electric field.

Teenager male with burning pain in extremities--suspect Fabry disease, 2 case reports.

PubMed

Patil, Rajesh B; Joglekar, V K

2014-01-01

We present 2 cases of teenager males presented with burning pain in extremities and turned out to be cases of Fabry disease.The purpose of presenting this case is to highlight the fact that suspicion of Fabry disease in patients presenting with these symptoms will lead to early diagnosis and treatment of this condition before occurrences of complications. A 14-year-old male presented with severe burning pain in both hands and feet since last 4 yrs which persisted despite consumption of painkillers and becoming more disabling and without having any family history for such condition. On general examination patient had small reddish coloured lesions around the umbilicus, appearing like angiokeratomas. Skin biopsy confirmed the lesion. On enzyme assay his alpha galactosidase activity found to be '0' nmol/hr/mg of protein, confirming his diagnosis. Patient's creatinine and 2 D ECHO were normal and urine had 1+ proteinuria. Patient started on carbamazepine tablets for pain and referred to higher centre for genetic diagnosis and enzyme replacement therapy. CASE REPORT 2: An 18-year-old male referred to our hospital by general practitioner for fatigue and pedal oedema with deranged renal function tests. On history taking patient gave history of severe burning pain in both hands and feet since age of 9 yrs. Patient's general examination revealed hypertension with pallor, pedal oedema along with angiokeratomas in bathing suit distribution. Patient's ultrasonography of kidney revealed bilaterally normal sized kidneys with altered echotexture and urine examination showed fine granular foamy cells with sub nephrotic range proteinuria. 2 D ECHO revealed concentric left ventricular hypertrophy. Skin biopsy report supported the diagnosis of Fabry disease. Patient advised to undergo renal biopsy to confirm Fabry nephropathy but patient denied any further diagnostic workup for nephropathy or Fabry disease. Patient started on conservative treatment and carbamazepine in renal dose

Patients with Fabry Disease after Enzyme Replacement Therapy Dose Reduction Versus Treatment Switch

PubMed Central

Krämer, Johannes; Duning, Thomas; Lenders, Malte; Canaan-Kühl, Sima; Krebs, Alice; González, Hans Guerrero; Sommer, Claudia; Üçeyler, Nurcan; Niemann, Markus; Störk, Stefan; Schelleckes, Michael; Reiermann, Stefanie; Stypmann, Jörg; Brand, Stefan-Martin; Wanner, Christoph; Brand, Eva

2014-01-01

Because of the shortage of agalsidase-beta in 2009, many patients with Fabry disease were treated with lower doses or were switched to agalsidase-alfa. This observational study assessed end-organ damage and clinical symptoms during dose reduction or switch to agalsidase-alfa. A total of 105 adult patients with Fabry disease who had received agalsidase-beta (1.0 mg/kg body weight) for ≥1 year were nonrandomly assigned to continue this treatment regimen (regular-dose group, n=38), receive a reduced dose of 0.3–0.5 mg/kg (dose-reduction group, n=29), or switch to 0.2 mg/kg agalsidase-alfa (switch group) and were followed prospectively for 1 year. We assessed clinical events (death, myocardial infarction, severe arrhythmia, stroke, progression to ESRD); changes in cardiac, renal, and neurologic function; and Fabry-related symptoms (neuropathic pain, hypohidrosis, diarrhea, and disease severity scores). Organ function and Fabry-related symptoms remained stable in the regular-dose group. In contrast, estimated GFR decreased by about 3 ml/min per 1.73 m2 (P=0.01) in the dose-reduction group, and the median albumin-to-creatinine ratio increased from 114 (0–606) mg/g to 216 (0–2062) mg/g (P=0.03) in the switch group. Furthermore, mean Mainz Severity Score Index scores and frequencies of pain attacks, chronic pain, gastrointestinal pain, and diarrhea increased significantly in the dose-reduction and switch groups. In conclusion, patients receiving regular agalsidase-beta dose had a stable disease course, but dose reduction led to worsening of renal function and symptoms. Switching to agalsidase-alfa is safe, but microalbuminuria may progress and Fabry-related symptoms may deteriorate. PMID:24556354

Charles River Residual Designation: Executive Summary

EPA Pesticide Factsheets

Read an executive summary of the Record of Decision's preliminary decision by the Regional Administrator of EPA Region 1 that storm water permits are needed to address serious water quality problems in the Charles River.

Like grandfather, like grandson: Erasmus and Charles Darwin on evolution.

PubMed

Smith, C U M

2010-01-01

Last year (2009) marked the bicentenary of Charles Darwin's birth and the sesquicentenary of The Origin of Species. This article examines the influence of Erasmus Darwin on Charles's evolutionary thought and shows how, in many ways, Erasmus anticipated his much better-known grandson. It discusses the similarity in the mindsets of the two Darwins, asks how far the younger Darwin was exposed to the elder's evolutionary thought, examines the similarities and differences in their theories of evolution, and ends by showing the surprising similarity between their theories of inheritance. Erasmus's influence on Charles is greater than customarily acknowledged, and now is an opportune time to bring the grandfather out from behind the glare of his stellar grandson.

76 FR 70704 - Foreign-Trade Zone 87-Lake Charles, LA; Application for Reorganization/Expansion

Federal Register 2010, 2011, 2012, 2013, 2014

2011-11-15

... Charles, LA; Application for Reorganization/Expansion An application has been submitted to the Foreign-Trade Zones Board (the Board) by the Lake Charles Harbor & Terminal District, grantee of FTZ 87, requesting authority to reorganize and expand the zone in Lake Charles. The application was submitted...

Contribution of inflammatory pathways to Fabry disease pathogenesis.

PubMed

Rozenfeld, Paula; Feriozzi, Sandro

2017-11-01

Lysosomal storage diseases are usually considered to be pathologies in which the passive deposition of unwanted materials leads to functional changes in lysosomes. Lysosomal deposition of unmetabolized glycolipid substrates stimulates the activation of pathogenic cascades, including immunological processes, and particularly the activation of inflammation. In lysosomal storage diseases, the inflammatory response is continuously being activated because the stimulus cannot be eliminated. Consequently, inflammation becomes a chronic process. Lysosomes play a role in many steps of the immune response. Leukocyte perturbation and over-expression of immune molecules have been reported in Fabry disease. Innate immunity is activated by signals originating from dendritic cells via interactions between toll-like receptors and globotriaosylceramide (Gb3) and/or globotriaosylsphingosine (lyso-Gb3). Evidence indicates that these glycolipids can activate toll-like receptors, thus triggering inflammation and fibrosis cascades. In the kidney, Gb3 deposition is associated with the increased release of transforming growth factor beta and with epithelial-to-mesenchymal cell transition, leading to the over-expression of pro-fibrotic molecules and to renal fibrosis. Interstitial fibrosis is also a typical feature of heart involvement in Fabry disease. Endomyocardial biopsies show infiltration of lymphocytes and macrophages, suggesting a role for inflammation in causing tissue damage. Inflammation is present in all tissues and may be associated with other potentially pathologic processes such as apoptosis, impaired autophagy, and increases in pro-oxidative molecules, which could all contribute synergistically to tissue damage. In Fabry disease, the activation of chronic inflammation over time leads to organ damage. Therefore, enzyme replacement therapy must be started early, before this process becomes irreversible. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights

Charles L. Brewer award for distinguished teaching of psychology.

PubMed

2012-01-01

The American Psychological Foundation (APF) Charles L. Brewer Award for Distinguished Teaching of Psychology recognizes an outstanding career contribution to the teaching of psychology. The 2012 recipient of the Charles L. Brewer Award for Distinguished Teaching of Psychology is Richard L. Miller. Dorothy W. Cantor, president of the APF, will present the APF Gold Medal Awards at the 120th Annual Convention of the American Psychological Association on August 3, 2012, at 4:00 p.m. Members of the 2012 APF Board of Trustees are Dorothy W. Cantor, president; Charles L. Brewer, vice president/secretary; Gerald Koocher, treasurer; Elisabeth R. Straus, executive vice president/executive director; Norman Anderson; Brian N. Baird; David H. Barlow; Camilla Benbow; Sharon Stephens Brehm; Connie Chan; William Howell; Anthony Jackson; Ronald F. Levant; Aurelio Prifitera; Sandra Shullman; Archie L. Turner; and Kurt Geisinger, APA Board of Directors liaison.

Development of the Fabry-Perot Spectrometer Application

NASA Technical Reports Server (NTRS)

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.

Multimode excitation-induced phase shifts in intrinsic Fabry-Perot interferometric fiber sensor spectra.

PubMed

Ma, Cheng; Wang, Anbo

2010-09-01

We report the modal analysis of optical fiber single-mode-multimode-single-mode intrinsic Fabry-Perot interferometer sensors. The multimode nature of the Fabry-Perot cavity gives rise to an additional phase term in the spectrogram due to intermodal dispersion-induced wavefront distortion, which could significantly affect the cavity length demodulation accuracy. By using an exact model to analyze the modal behavior, this phase term is explained by employing a rotating vector approach. Comparison of the theoretical analysis with experimental results is presented.

In Memoriam: Charles Shive | Poster

Cancer.gov

Earlier this summer, NCI at Frederick lost a friend and colleague, Charles Shive, to cancer. Mr. Shive, better known to most as Charlie, was a systems architect and information technology manager for the Data Science and Information Technology Program, focused on the re-engineering initiative of the Clinical Trials Reporting System.

Charles Bolden First Day As Administrator

NASA Image and Video Library

2009-07-16

Charles F. Bolden, Jr. enters the NASA Administrator's office to start his first day as the 12th administrator of the National Aeronautics and Space Administration, Friday, July 17, 2009 in Washington. Photo Credit: (NASA/Bill Ingalls)

Charles F. Richter: A personal tribute

USGS Publications Warehouse

Allen, Clarence R.

1987-01-01

With the death of Charles Richter in 1985, the seismological community lost a renowned colleague, and many of us lost a close friend and advisor. Charles was born on a farm in Ohio in 1900, received his A.B. from Stanford in 1920, and his Ph.D.  from Caltech in 1928. Virtually his entire professional career was spent at the Seismological Laboratory in Pasadena, first as an employee of the Carnegie Institution of Washington and later as a Caltech faculty member. Following his retirement from Caltech in 1970, he was active for several years in the consulting firm of Lindvall, Richter, and Associates. His wife, Lillian, died in 1972, and they had no children. Richter served as President of the Seismological Society of America from 1959 to 1960 and was the second recipient of its medal in 1977.

The use of high resolution melting analysis to detect Fabry mutations in heterozygous females via dry bloodspots.

PubMed

Tai, Chang-Long; Liu, Mei-Ying; Yu, Hsiao-Chi; Chiang, Chiang-Chuan; Chiang, Hung; Suen, Jeng-Hung; Kao, Shu-Min; Huang, Yu-Hsiu; Wu, Tina Jui-Ting; Yang, Chia-Feng; Tsai, Fang-Chih; Lin, Ching-Yuang; Chang, Jan-Gowth; Chen, Hong-Duo; Niu, Dau-Ming

2012-02-18

As an X-linked genetic disorder, Fabry disease was first thought to affect males only, and females were generally considered to be asymptomatic carriers. However, recent research suggests that female carriers of Fabry disease may still develop vital organ damage causing severe morbidity and mortality. In the previous newborn screening, from 299,007 newborns, we identified a total of 20 different Fabry mutations and 121 newborns with Fabry mutations. However, we found that most female carriers are not detected by enzyme assays. A streamlined method for high resolution melting (HRM) analysis was designed to screen for GLA gene mutations using a same PCR and melting program. Primer sets were designed to cover the 7 exons and the Chinese common intronic mutation, IVS4+919G>A of GLA gene. The HRM analysis was successful in identifying heterozygous and hemizygous patients with the 20 surveyed mutations. We were also successful in using this method to test dry blood spots of newborns afflicted with Fabry mutations without having to determine DNA concentration before PCR amplification. The results of this study show that HRM could be a reliable and sensitive method for use in the rapid screening of females for GLA mutations. Copyright © 2011 Elsevier B.V. All rights reserved.

Migalastat improves diarrhea in patients with Fabry disease: clinical-biomarker correlations from the phase 3 FACETS trial.

PubMed

Schiffmann, Raphael; Bichet, Daniel G; Jovanovic, Ana; Hughes, Derralynn A; Giugliani, Roberto; Feldt-Rasmussen, Ulla; Shankar, Suma P; Barisoni, Laura; Colvin, Robert B; Jennette, J Charles; Holdbrook, Fred; Mulberg, Andrew; Castelli, Jeffrey P; Skuban, Nina; Barth, Jay A; Nicholls, Kathleen

2018-04-27

Fabry disease is frequently characterized by gastrointestinal symptoms, including diarrhea. Migalastat is an orally-administered small molecule approved to treat the symptoms of Fabry disease in patients with amenable mutations. We evaluated minimal clinically important differences (MCID) in diarrhea based on the corresponding domain of the patient-reported Gastrointestinal Symptom Rating Scale (GSRS) in patients with Fabry disease and amenable mutations (N = 50) treated with migalastat 150 mg every other day or placebo during the phase 3 FACETS trial (NCT00925301). After 6 months, significantly more patients receiving migalastat versus placebo experienced improvement in diarrhea based on a MCID of 0.33 (43% vs 11%; p = .02), including the subset with baseline diarrhea (71% vs 20%; p = .02). A decline in kidney peritubular capillary globotriaosylceramide inclusions correlated with diarrhea improvement; patients with a reduction > 0.1 were 5.6 times more likely to have an improvement in diarrhea than those without (p = .031). Migalastat was associated with a clinically meaningful improvement in diarrhea in patients with Fabry disease and amenable mutations. Reductions in kidney globotriaosylceramide may be a useful surrogate endpoint to predict clinical benefit with migalastat in patients with Fabry disease. NCT00925301 ; June 19, 2009.

78 FR 74004 - Amendment of Class D and Class E Airspace; Lake Charles, LA

Federal Register 2010, 2011, 2012, 2013, 2014

2013-12-10

...-0948; Airspace Docket No. 13-ASW-25] Amendment of Class D and Class E Airspace; Lake Charles, LA AGENCY... amends Class D and Class E airspace within the Lake Charles, LA, area by updating the geographic coordinates for Lake Charles Regional Airport, and the airport name and geographic coordinates for Chennault...

Nitrogen-doped diamond thin films: potential application in Fabry-Pérot interferometer

NASA Astrophysics Data System (ADS)

Kosowska, M.; Majchrowicz, D.; Sankaran, K. J.; Ficek, M.; Jedrzejewska-Szczerska, M.; Haenen, M. K.

2018-04-01

In this paper we present results of preliminary research of using nitrogen-doped diamond (NDD) films as reflective layer in Fabry-Pérot interferometer. NDD films were deposited on Si substrates by Microwave Plasma Enhanced Chemical Vapor Deposition (MPECVD) with the use of CH4, H2 and N2 gas mixtures. During deposition process methane flow rate varied while nitrogen flow was constant. We performed series of measurements which showed that NDD can be used as a mirror in Fabry-Pérot interferometer. The best signal visibility and repeatability of measurements were obtained for sample made with 3 sccm methane flow rate.

Metabolomic Discovery of Novel Urinary Galabiosylceramide Analogs as Fabry Disease Biomarkers

NASA Astrophysics Data System (ADS)

Boutin, Michel; Auray-Blais, Christiane

2015-03-01

Fabry disease is an X-linked, complex, multisystemic lysosomal storage disorder presenting marked phenotypic and genotypic variability among affected male and female patients. Glycosphingolipids, mainly globotriaosylceramide (Gb3) isoforms/analogs, globotriaosylsphingosine (lyso-Gb3) and analogs, as well as galabiosylceramide (Ga2) isoforms/analogs accumulate in the vascular endothelium, nerves, cardiomyocytes, renal glomerular and tubular epithelial cells, and biological fluids. The search for biomarkers reflecting disease severity and progression is still on-going. A metabolomic study using quadrupole time-of-flight mass spectrometry has revealed 22 galabiosylceramide isoforms/analogs in urine of untreated Fabry patients classified in seven groups according to their chemical structure: (1) Saturated fatty acid; (2) one extra double bond; (3) two extra double bonds; (4) hydroxylated saturated fatty acid; (5) hydroxylated fatty acid and one extra double bond; (6) hydrated sphingosine and hydroxylated fatty acid; (7) methylated amide linkage. Relative quantification of both Ga2 and Gb3 isoforms/analogs was performed. All these biomarkers are significantly more abundant in urine samples from untreated Fabry males compared with healthy male controls. A significant amount of Ga2 isoforms/analogs, accounting for 18% of all glycosphingolipids analyzed (Ga2 + Gb3 and respective isoforms/analogs), were present in urine of Fabry patients. Gb3 isoforms containing saturated fatty acids are the most abundant (60.9%) compared with 26.3% for Ga2. A comparison between Ga2 isoforms/analogs and their Gb3 counterparts also showed that the proportion of analogs with hydroxylated fatty acids is significantly greater for Ga2 (35.8%) compared with Gb3 (1.9%). These results suggest different biological pathways involved in the synthesis and/or degradation of Gb3 and Ga2 metabolites.

77 FR 38085 - Importer of Controlled Substances; Notice of Application; Cambrex Charles City, Inc.

Federal Register 2010, 2011, 2012, 2013, 2014

2012-06-26

... Application; Cambrex Charles City, Inc. Pursuant to Title 21 Code of Federal Regulations 1301.34 (a), this is notice that on May 4, 2011, Cambrex Charles City, Inc., 1205 11th Street, Charles City, Iowa 50616-3466... II are, and will continue to be, required to demonstrate to the Deputy Assistant Administrator...

Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development

PubMed Central

Biko, Lydia; Hose, Dorothea; Hofmann, Lukas; Sommer, Claudia

2016-01-01

Background Fabry disease is an X-linked lysosomal storage disorder due to impaired activity of alpha-galactosidase A with intracellular accumulation of globotriaosylceramide. Associated small fiber pathology leads to characteristic pain in Fabry disease. We systematically assessed sensory system, physical activity, metabolic parameters, and morphology of male and female mice with alpha-galactosidase A deficiency (Fabry ko) from 2 to 27 months of age and compared results with those of age- and gender-matched wild-type littermates of C57Bl/6J background. Results From the age of two months, male and female Fabry mice showed mechanical hypersensitivity (p < 0.001 each) compared to wild-type littermates. Young Fabry ko mice of both genders were hypersensitive to heat stimulation (p < 0.01) and developed heat hyposensitivity with aging (p < 0.05), while cold hyposensitivity was present constantly in young (p < 0.01) and old (p < 0.05) Fabry ko mice compared to wild-type littermates. Stride angle increased only in male Fabry ko mice with aging (p < 0.01) in comparison to wild-type littermates. Except for young female mice, male (p < 0.05) and female (p < 0.01) Fabry ko mice had a higher body weight than wild-type littermates. Old male Fabry ko mice were physically less active than their wild-type littermates (p < 0.05), had lower chow intake (p < 0.001), and lost more weight (p < 0.001) in a one-week treadmill experiment than wild-type littermates. Also, Fabry ko mice showed spontaneous pain protective behavior and developed orofacial dysmorphism resembling patients with Fabry disease. Conclusions Mice with alpha-galactosidase A deficiency show age-dependent and distinct deficits of the sensory system. alpha-galactosidase A-deficient mice seem to model human Fabry disease and may be helpful when studying the pathophysiology of Fabry-associated pain. PMID:27145802

Compressible Fabry-Perot refractometer.

PubMed

Andersson, M; Eliasson, L; Pendrill, L R

1987-11-15

The use of a long, thermally stable Fabry-Perot etalon as a refractometer is considered in detail in this study of the refractive index of air. The etalon consists of two flat plates of fused silica 60 mm in diameter, with a cylindrical spacer made of Zerodur (a polycrystalline glass ceramic of extremely low thermal expansion) 200 mm long. The interferogram of light from a frequency-stabilized He-Ne laser is imaged with large-diameter mirror optics. The principal result is a demonstration of the effects of changes in atmospheric pressure on the etalon. The measured refractive-index values deviate by 2 parts in 10(7) from calculated values. Possible causes of error are considered in detail.

Simplified design of diaphragm-based fiber optic extrinsic Fabry-Perot accelerometer

NASA Astrophysics Data System (ADS)

Wang, Zhaogang; Zhang, Wentao; Han, Jing; Huang, Wenzhu; Li, Fang

2014-11-01

A fiber optic Fabry-Perot accelerometer (FOFPA) with diaphragm-mass-collimator (DMC) gathered structure is presented. This design makes the structure more compacts and the manufacturing process more controllable. The operation principle based on Fabry-Perot interference is described. Several tests using intensity demodulation scheme which can control the working point of FOFPA were carried out. Experimental results show that: axis sensitivity of the proposed FOFPA is 36.07 dB (re: 0 dB=1 V/g) with a fluctuation less than 0.9 dB in a frequency bandwidth of 10-125 Hz, the resonant frequency is about 350 Hz, measurement range is about 70 dB@100 Hz. which are much close to theoretical values

Anxiety and Charles Bonnet Syndrome

ERIC Educational Resources Information Center

Geueke, Anna; Morley, Michael G.; Morley, Katharine; Lorch, Alice; Jackson, MaryLou; Lambrou, Angeliki; Wenberg, June; Oteng-Amoako, Afua

2012-01-01

Introduction: Some persons with Charles Bonnet syndrome (CBS) suffer significant anxiety because of their visual hallucinations, while others do not. The aim of the study presented here was to compare levels of anxiety in persons with low vision with and without CBS. Methods: This retrospective study compared the level of anxiety in 31 persons…

Charles Darwin in the Andes

ERIC Educational Resources Information Center

Bizzo, Nelio; Bizzo, Luis Eduardo Maestrelli

2006-01-01

Considering geological time as an important epistemological obstacle to the construction of ideas on biological evolution, a study was carried out on the so-called "Darwin Papers". The conclusion was that Charles Darwin's excursion in the Andes during March-April 1835 was a crucial step in this regard. An expedition was carried out in…

78 FR 64013 - Importer of Controlled Substances; Notice of Application; Cambrex Charles City, Inc.

Federal Register 2010, 2011, 2012, 2013, 2014

2013-10-25

... Application; Cambrex Charles City, Inc. Pursuant to Title 21 Code of Federal Regulations 1301.34(a), this is notice that on July 24, 2013, Cambrex Charles City, Inc., 1205 11th Street, Charles City, Iowa 50616-3466... class of any controlled substance in schedules I or II are, and will continue to be, required to...

Stable CW Single-Frequency Operation of Fabry-Perot Laser Diodes by Self-Injection Phase Locking

NASA Technical Reports Server (NTRS)

Duerksen, Gary L.; Krainak, Michael A.

1999-01-01

Previously, single-frequency semiconductor laser operation using fiber Bragg gratings has been achieved by two methods: 1) use of the FBG as the output coupler for an anti-reflection-coated semiconductor gain element'; 2) pulsed operation of a gain-switched Fabry-Perot laser diode with FBG-optical and RF-electrical feedback. Here, we demonstrate CW single frequency operation from a non-AR coated Fabry-Perot laser diode using only FBG optical feedback. We coupled a nominal 935 run-wavelength Fabry-Perot laser diode to an ultra narrow band (18 pm) FBG. When tuned by varying its temperature, the laser wavelength is pulled toward the centerline of the Bragg grating, and the spectrum of the laser output is seen to fall into three discrete stability regimes as measured by the side-mode suppression ratio.

Myocardial lipid content in Fabry disease: a combined 1H-MR spectroscopy and MR imaging study at 3 Tesla.

PubMed

Petritsch, B; Köstler, H; Weng, A M; Horn, M; Gassenmaier, T; Kunz, A S; Weidemann, F; Wanner, C; Bley, T A; Beer, M

2016-10-28

Fabry disease is characterized by a progressive deposition of sphingolipids in different organ systems, whereby cardiac involvement leads to death. We hypothesize that lysosomal storage of sphingolipids in the heart as occurring in Fabry disease does not reflect in higher cardiac lipid concentrations detectable by 1 H magnetic resonance spectroscopy (MRS) at 3 Tesla. Myocardial lipid content was quantified in vivo by 1 H-MRS in 30 patients (12 male, 18 female; 18 patients treated with enzyme replacement therapy) with genetically proven Fabry disease and in 30 healthy controls. The study protocol combined 1 H-MRS with cardiac cine imaging and LGE MRI in a single examination. Myocardial lipid content was not significantly elevated in Fabry disease (p = 0.225). Left ventricular (LV) mass was significantly higher in patients suffering from Fabry disease compared to controls (p = 0.019). Comparison of patients without signs of myocardial fibrosis in MRI (LGE negative; n = 12) to patients with signs of fibrosis (LGE positive; n = 18) revealed similar myocardial lipid content in both groups (p > 0.05), while the latter showed a trend towards elevated LV mass (p = 0.076). This study demonstrates the potential of lipid metabolic investigation embedded in a comprehensive examination of cardiac morphology and function in Fabry disease. There was no evidence that lysosomal storage of sphingolipids influences cardiac lipid content as measured by 1 H-MRS. Finally, the authors share the opinion that a comprehensive cardiac examination including three subsections (LGE; 1 H-MRS; T 1 mapping), could hold the highest potential for the final assessment of early and late myocardial changes in Fabry disease.

Cardiac energy metabolism is disturbed in Fabry disease and improves with enzyme replacement therapy using recombinant human galactosidase A.

PubMed

Machann, Wolfram; Breunig, Frank; Weidemann, Frank; Sandstede, Jörn; Hahn, Dietbert; Köstler, Herbert; Neubauer, Stefan; Wanner, Christoph; Beer, Meinrad

2011-03-01

In vitro studies have shown impairment of energy metabolism in cardiac fibroblasts from Fabry patients. A recent in vivo study reported an association between cardiac energy metabolism and increased myocardial mass in Fabry patients. We therefore assessed possible disturbances of cardiac energy metabolism in Fabry patients by in vivo (31)P-MR-spectroscopy. Additionally, the effect of enzyme replacement therapy (ERT) on cardiac energetics was tested. Twenty-three patients (41 ± 9 years; 10 females) with genetically proven Fabry disease were examined with a 1.5 T Scanner, and compared with an age-matched healthy control group. Eight patients underwent ERT and had follow-up examinations after 3 and 14 months. The high-energy phosphate molecules phosphocreatine (PCr) and adenosine triphosphate (ATP) were quantified in localized 31P-spectra by SLOOP (spectral localization with optimum point spread function). Cine- and late gadolinium enhancement (LGE) studies were also performed. When compared with healthy controls, Fabry patients demonstrated reduced PCr- (6.1 ± 1.9 vs. 8.8 ± 2.6 mmol/kg; P = 0.003) and ATP concentrations (3.9 ± 1.5 vs. 4.6 ± 1.0 mmol/kg; P = 0.048). During ERT, PCr concentrations increased (7.1 ± 1.5 mmol/kg vs. 6.1 ± 1.9; P < 0.05) and left ventricular mass decreased (215 ± 55 vs. 185 ± 45 g; P = 0.012). Disturbances in cardiac energetics were not correlated to the presence or absence of cardiac fibrosis on LGE. Cardiac energy metabolism is disturbed in Fabry disease; this may play an important role in the pathogenesis of Fabry cardiomyopathy. Enzyme replacement therapy ameliorates energetic depression.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

PubMed

Yeniçerioğlu, Yavuz; Akdam, Hakan; Dursun, Belda; Alp, Alper; Sağlam Eyiler, Funda; Akın, Davut; Gün, Yelda; Hüddam, Bülent; Batmazoğlu, Mehmet; Gibyeli Genek, Dilek; Pirinççi, Serhat; Ersoy, İsmail Rıfkı; Üzüm, Atilla; Soypaçacı, Zeki; Tanrısev, Mehmet; Çolak, Hülya; Demiral Sezer, Sibel; Bozkurt, Gökay; Akyıldız, Utku Oğan; Akyüz Ünsal, Ayşe İpek; Ünübol, Mustafa; Uslu, Meltem; Eryılmaz, Ufuk; Günel, Ceren; Meteoğlu, İbrahim; Yavaşoğlu, İrfan; Ünsal, Alparslan; Akar, Harun; Okyay, Pınar

2017-11-01

Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 μmol/L/h. A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m 2 , 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 μmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 μmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

The IRAF Fabry-Perot analysis package: Ring fitting

NASA Technical Reports Server (NTRS)

Shopbell, P. L.; Bland-Hawthorn, J.; Cecil, G.

1992-01-01

As introduced at ADASSI, a Fabry-Perot analysis package for IRAF is currently under development as a joint effort of ourselves and Frank Valdes of the IRAF group. Although additional portions of the package were also implemented, we report primarily on the development of a robust ring fitting task, useful for fitting the calibration rings obtained in Fabry-Perot observations. The general equation of an ellipse is fit to the shape of the rings, providing information on ring center, ellipticity, and position angle. Such parameters provide valuable information on the wavelength response of the etalon and the geometric stability of the system. Appropriate statistical weighting is applied to the pixels to account for increasing numbers with radius, the Lorentzian cross-section, and uneven illumination. The major problems of incomplete, non-uniform, and multiple rings are addressed with the final task capable of fitting rings regardless of center, cross-section, or completion. The task requires only minimal user intervention, allowing large numbers of rings to be fit in an extremely automated manner.

Intrinsic Fabry-Perot Sensors for Magnetic Field Detection

NASA Astrophysics Data System (ADS)

Broadway, Christian; Descamps, Frédéric; Kinet, Damien; Caucheteur, Christophe; Mégret, Patrice

2018-01-01

Within the context of ensuring stable nuclear fusion, it is important to monitor and control a number of parametersincluding the magnetic field associated with plasma circulation. Optical fibre sensing techniques have seen a surge in promulgation and research advances in recent years, due to their immunity to electromagnetic radiation and compact dimensions. Prior work has shown that fibre Bragg gratings are one method of recovering the induced magnetic field, with the main point of interest being their use as distributed point sensors. However, Bragg grating inscription leads to the creation of linear birefringence that increases detector noise and could obscure a given signal. We have hypothesised that by using an intrinsic Fabry-Perot cavity comprised of two identical Bragg gratings, we could obtain a more accurate detector with the removal of photo-induced birefringence in the detection region. We present a proof of concept optical fibre sensor based on an intrinsic Fabry-Perot cavity that shows spectrally visible amplitude modulation. Finally, we demonstrate faster data processing that allows real time monitoring of a given scenario.

All-optical logic gates and wavelength conversion via the injection locking of a Fabry-Perot semiconductor laser

NASA Astrophysics Data System (ADS)

Harvey, E.; Pochet, M.; Schmidt, J.; Locke, T.; Naderi, N.; Usechak, N. G.

2013-03-01

This work investigates the implementation of all-optical logic gates based on optical injection locking (OIL). All-optical inverting, NOR, and NAND gates are experimentally demonstrated using two distributed feedback (DFB) lasers, a multi-mode Fabry-Perot laser diode, and an optical band-pass filter. The DFB lasers are externally modulated to represent logic inputs into the cavity of the multi-mode Fabry-Perot slave laser. The input DFB (master) lasers' wavelengths are aligned with the longitudinal modes of the Fabry-Perot slave laser and their optical power is used to modulate the injection conditions in the Fabry-Perot slave laser. The optical band-pass filter is used to select a Fabry- Perot mode that is either suppressed or transmitted given the logic state of the injecting master laser signals. When the input signal(s) is (are) in the on state, injection locking, and thus the suppression of the non-injected Fabry-Perot modes, is induced, yielding a dynamic system that can be used to implement photonic logic functions. Additionally, all-optical photonic processing is achieved using the cavity-mode shift produced in the injected slave laser under external optical injection. The inverting logic case can also be used as a wavelength converter — a key component in advanced wavelength-division multiplexing networks. As a result of this experimental investigation, a more comprehensive understanding of the locking parameters involved in injecting multiple lasers into a multi-mode cavity and the logic transition time is achieved. The performance of optical logic computations and wavelength conversion has the potential for ultrafast operation, limited primarily by the photon decay rate in the slave laser.

All sky imaging Fabry-Perot spectrometer for optical investigation of the upper atmosphere

NASA Astrophysics Data System (ADS)

Sekar, R.; Gurubaran, S.; Sridharan, R.

1993-06-01

A simple optical design, keeping in view of the available components, has been worked out to develop the 'all sky imaging Fabry-Perot spectrometer' to study the spatial structures in thermospheric winds and temperature. This system comprises three subsystems, namely, (1) field widening front-end optics, (2) high resolution Fabry-Perot spectrometer and (3) a two-dimensional detector. The design details of the above imaging spectrometer that has been commissioned for routine observations from Mt. Abu along with the first results on OI 6300 A airglow emission are presented and discussed.

Measured and simulated runoff to the lower Charles River, Massachusetts, October 1999-September 2000

USGS Publications Warehouse

Zarriello, Phillip J.; Barlow, Lora K.

2002-01-01

The lower Charles River, the water body between the Watertown Dam and the New Charles River Dam, is an important recreational resource for the Boston, Massachusetts, metropolitan area, but impaired water quality has affected its use. The goal of making this resource fishable and swimmable requires a better understanding of combined-sewer-overflow discharges, non-combined-sewer-overflow stormwater runoff, and constituent loads. This report documents the modeling effort used to calculate non-combined-sewer-overflow runoff to the lower Charles River. During the 2000 water year, October 1, 1999?September 30, 2000, the U.S. Geological Survey collected precipitation data at Watertown Dam and compiled data from five other precipitation gages in or near the watershed. In addition, surface-water discharge data were collected at eight sites?three relatively homogenous land-use sites, four major tributary sites, and the Charles River at Watertown Dam, which is the divide between the upper and lower watersheds. The precipitation and discharge data were used to run and calibrate Stormwater Management Models developed for the three land-use subbasins (single-family, multi-family, and commercial), and the two tributary subbasins (Laundry and Faneuil Brooks). These calibrated models were used to develop a sixth model to simulate 54 ungaged outfalls to the lower Charles River. Models developed by the U.S. Geological Survey at gaged sites were calibrated with up to 24 storms. Each model was evaluated by comparing simulated discharge against measured discharge for all storms with appreciable precipitation and reliable discharge data. The model-fit statistics indicated that the models generally were well calibrated to peak discharge and runoff volumes. The model fit of the commercial land-use subbasin was not as well calibrated compared to the other models because the measured flows appear to be affected by variable conditions not represented in the model. A separate Stormwater

46 CFR 7.45 - Cape Henlopen, DE to Cape Charles, VA.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 46 Shipping 1 2011-10-01 2011-10-01 false Cape Henlopen, DE to Cape Charles, VA. 7.45 Section 7.45 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY PROCEDURES APPLICABLE TO THE PUBLIC BOUNDARY LINES Atlantic Coast § 7.45 Cape Henlopen, DE to Cape Charles, VA. (a) A line drawn from the easternmost...

Safe and Successful Treatment With Agalsidase Beta During Pregnancy in Fabry Disease.

PubMed

Senocak Tasci, Elif; Bicik, Zerrin

2015-09-01

Fabry disease, an X-linked lysosomal storage disorder, is caused by α-galactosidase A deficiency and leads to accumulation of glycospinhgolipids in most tissues, with life-theratening consequences in the kidney, heart, and cerebrovascular system. Enzyme replacement therapy is available as 2 different preparations: agalsidase alfa and agalsidase beta. Enzyme replacement therapy is started as soon as the diagnosis is confirmed, but there is no data available in the literature about its safety during preganacy. Herein, we described 2 patients with Fabry disease who received agalsidase beta during their pregnancy. This report is important as the data about enzyme replacement therapy during pregnancy is restricted with case reports.

Modelling the resource implications of managing adults with Fabry disease in Norway favours home infusion.

PubMed

Guest, Julian F; Jenssen, Trond; Houge, Gunnar; Aaseboe, Willy; Tøndel, Camilla; Svarstad, Einar

2010-12-01

The aim of this study was to estimate the resource implications and budget impact of managing adults with Fabry disease in Norway, from the perspective of the publicly funded healthcare system. A decision model was constructed using published clinical outcomes and clinician-derived resource utilization estimates. The model was used to estimate the annual healthcare cost of managing a cohort of 64 adult Fabry patients in an average year. The expected annual cost of managing 60 existing Fabry patients and four new patients in Norway each year was estimated to be NOK 55·8 million (€6·7 million). In an average year, patients receiving enzyme replacement therapy (ERT) with agalsidase alfa (Replagal(®)) at 0·2 mg kg⁻¹ or agalsidase beta (Fabrazyme(®)) at 1·0 mg kg⁻¹ are collectively expected to make 586 attendances to their family practitioner's office for their infusions, which equates to 128 eight-hour days associated with ERT. Encouraging more patients to undergo home-based infusions has substantial potential to free-up community-based resources. In comparison, the community-related benefit that can be obtained by switching from agalsidase beta (1·0 mg kg⁻¹) to agalsidase alpha (0·2 mg kg⁻¹) is marginal, and dependent on the two doses being clinically equivalent. Maximizing the proportion of adults with Fabry disease undergoing home-based infusions has the potential to release community-based resources for alternative use by non-Fabry patients, thereby improving the efficiency of the publicly funded healthcare system in Norway. © 2010 The Authors. European Journal of Clinical Investigation © 2010 Stichting European Society for Clinical Investigation Journal Foundation.

Antiproteinuric therapy and fabry nephropathy: sustained reduction of proteinuria in patients receiving enzyme replacement therapy with agalsidase-beta.

PubMed

Tahir, Hindia; Jackson, Leslie L; Warnock, David G

2007-09-01

This report describes an open-label, nonrandomized, prospective evaluation of the effects of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker therapy on patients who have Fabry disease and also received enzyme replacement therapy with agalsidase-beta, given at 1 mg/kg body wt every 2 wk. Previous placebo-controlled phase III and phase IV trials with agalsidase-beta demonstrated clearing of globotriaosylceramide from vascular endothelia but little effect on proteinuria or progressive loss of kidney function in patients with Fabry disease and severe chronic kidney disease marked by overt proteinuria and/or estimated GFR <60 ml/min per 1.73 m2. Angiotensin-converting enzyme inhibitor and/or angiotensin receptor blocker therapy is the standard of care for patients with proteinuric kidney diseases, but their use is challenging in patients with Fabry disease and low or low-normal baseline systemic BP. A group of patients with Fabry disease were treated with antiproteinuric therapy, in conjunction with agalsidase-beta; sustained reductions in proteinuria with stabilization of kidney function were achieved in a group of six patients who had severe Fabry nephropathy; the progression rate was -0.23 +/- 1.12 ml/min per 1.73 m2 per yr with 30 mo of follow-up.

Surges in proteinuria are associated with plasma GL-3 elevations in a young patient with classic Fabry disease.

PubMed

Kanai, Takahiro; Ito, Takane; Odaka, Jun; Saito, Takashi; Aoyagi, Jun; Betsui, Hiroyuki; Yamagata, Takanori

2016-03-01

Fabry disease is an X-linked glycosphingolipidosis caused by deficient synthesis of the enzyme α-galactosidase A, which results in accumulations of globotriaosylceramide (GL-3) in systemic tissues. Nephropathy is a dominant feature of Fabry disease. It still remains unclear how the nephropathy progresses. Recombinant agalsidase replacement therapy is currently the only approved, specific therapy for Fabry disease. The optimal dose of replacement enzyme also still remains unclear. The worldwide shortage of agalsidase-β in 2009 forced dose reduction of administration. It showed that the proteinuria emerged like surges, followed by temporary plasma GL-3 elevations in the early stages of classic Fabry disease. Additionally, it also showed that 1 mg/kg of agalsidase-β every other week could clear the GL-3 accumulations from podocytes and was required to maintain negative proteinuria and normal plasma GL-3 levels. This observation of a young patient with classic Fabry disease about 5 years reveals that the long-term, low-dose agalsidase-β caused proteinuria surges, but not persistent proteinuria, followed by temporary plasma GL-3 elevations, and agalsidase-β at 1 mg/kg every other week could clear accumulated GL-3 from podocytes and was required to maintain normal urinalysis and plasma GL-3 levels.

A Charles' Law Experiment for Beginning Students.

ERIC Educational Resources Information Center

Rockley, Mark G.; Rockley, Natalie L.

1995-01-01

Describes an experiment designed to measure absolute zero using Charles' Law without the use of mercury. Provides the procedure and details of the apparatus and includes experimental results obtained using the apparatus. (DDR)

Charles F. Richter; an interview

USGS Publications Warehouse

Spall, H.

1980-01-01

Charles F. Richter, renowned seismologist, is a professor emeritus at the California Institue of techonology (Caltech). He is best known to the public for the Richter magnitude scale; but he is equally recognized in the scientific community for many other contributions to seismology including his books Elementary Seismology (1958) and Seismicity of the Earth (coauthored in 1954 with Beno Gutenberg). 

A coaxial cable Fabry-Perot interferometer for sensing applications.

PubMed

Huang, Jie; Wang, Tao; Hua, Lei; Fan, Jun; Xiao, Hai; Luo, Ming

2013-11-07

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.

Stable CW Single Frequency Operation of Fabry-Perot Laser Diodes by Self-Injection Phase Locking

NASA Technical Reports Server (NTRS)

Duerksen, Gary L.; Krainak, Michael A.

1999-01-01

Previously, single-frequency semiconductor laser operation using fiber Bragg gratings has been achieved by tWo methods: 1) use of the FBG as the output coupler for an anti-reflection-coated semiconductor gain element'; 2) pulsed operation of a gain-switched Fabry-Perot laser diode with FBG-optical and RF-electrical feedback'. Here, we demonstrate CW single frequency operation from a non-AR coated Fabry-Perot laser diode using only FBG optical feedback.

Stable CW Single-Frequency Operation of Fabry-Perot Laser Diodes by Self-Injection Phase Locking

NASA Technical Reports Server (NTRS)

Duerksen, Gary L.; Krainak, Michael A.

1998-01-01

Previously, single-frequency semiconductor laser operation using fiber Bragg gratings (FBG) has been achieved by two methods: (1) use of the FBG as the output coupler for an anti-reflection-coated semiconductor gain element; (2) pulsed operation of a gain-switched Fabry-Perot laser diode with FBG-optical and RF-electrical feedback. Here, we demonstrate CW single frequency operation from a non-AR coated Fabry-Perot laser diode using only FBG optical feedback.

76 FR 24007 - Notice of Intent To Prepare an Environmental Impact Statement for the Lake Charles Carbon Capture...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-29

... Charles Carbon Capture and Sequestration Project, Lake Charles, LA AGENCY: Department of Energy. ACTION... competitive process under the Industrial Carbon Capture and Sequestration (ICCS) Program. The Lake Charles Carbon Capture and Sequestration Project (Lake Charles CCS Project) would demonstrate: (1) advanced...

Myocardial fibrosis as the first sign of cardiac involvement in a male patient with Fabry disease: report of a clinical case and discussion on the utility of the magnetic resonance in Fabry pathology.

PubMed

Sechi, Annalisa; Nucifora, Gaetano; Piccoli, Gianluca; Dardis, Andrea; Bembi, Bruno

2014-07-16

Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging is increasingly used to assess myocardial involvement in patients with Fabry disease, an X linked lipid storage disorder. However, it is often proposed as an optional tool. A different cardiomyopathic disease progression between male and female patients was hypothesised in previous studies, as in female myocardial fibrosis was found without left ventricular (LV) hypertrophy, while myocardial fibrosis was always detected in association to LV hypertrophy in men. A male Caucasian patient, 19 years old, diagnosed through a family-based molecular screening, presented with LGE of the LV inferolateral wall evidenced at the CMR, without LV hypertrophy, or other clinical signs of the disease. This is the first report of cardiac fibrosis as the first sign of organ involvement in a male patient with Fabry disease. This finding stresses the importance of performing CMR with LGE imaging for the initial staging and monitoring of Fabry patients of both genders.

Myocardial fibrosis as the first sign of cardiac involvement in a male patient with Fabry disease: report of a clinical case and discussion on the utility of the magnetic resonance in Fabry pathology

PubMed Central

2014-01-01

Background Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging is increasingly used to assess myocardial involvement in patients with Fabry disease, an X linked lipid storage disorder. However, it is often proposed as an optional tool. A different cardiomyopathic disease progression between male and female patients was hypothesised in previous studies, as in female myocardial fibrosis was found without left ventricular (LV) hypertrophy, while myocardial fibrosis was always detected in association to LV hypertrophy in men. Case presentation A male Caucasian patient, 19 years old, diagnosed through a family-based molecular screening, presented with LGE of the LV inferolateral wall evidenced at the CMR, without LV hypertrophy, or other clinical signs of the disease. Conclusion This is the first report of cardiac fibrosis as the first sign of organ involvement in a male patient with Fabry disease. This finding stresses the importance of performing CMR with LGE imaging for the initial staging and monitoring of Fabry patients of both genders. PMID:25026990

A biochemical and pharmacological comparison of enzyme replacement therapies for the glycolipid storage disorder Fabry disease.

PubMed

Lee, Karen; Jin, Xiaoying; Zhang, Kate; Copertino, Lorraine; Andrews, Laura; Baker-Malcolm, Jennifer; Geagan, Laura; Qiu, Huawei; Seiger, Keirsten; Barngrover, Debra; McPherson, John M; Edmunds, Tim

2003-04-01

Fabry disease is a lysosomal storage disease arising from deficiency of the enzyme alpha-galactosidase A. Two recombinant protein therapeutics, Fabrazyme (agalsidase beta) and Replagal (agalsidase alfa), have been approved in Europe as enzyme replacement therapies for Fabry disease. Both contain the same human enzyme, alpha-galactosidase A, but they are produced using different protein expression systems and have been approved for administration at different doses. To determine if there is recognizable biochemical basis for the different doses, we performed a comparison of the two drugs, focusing on factors that are likely to influence biological activity and availability. The two drugs have similar glycosylation, both in the type and location of the oligosaccharide structures present. Differences in glycosylation were mainly limited to the levels of sialic acid and mannose-6-phosphate present, with Fabrazyme having a higher percentage of fully sialylated oligosaccharides and a higher level of phosphorylation. The higher levels of phosphorylated oligomannose residues correlated with increased binding to mannose-6-phosphate receptors and uptake into Fabry fibroblasts in vitro. Biodistribution studies in a mouse model of Fabry disease showed similar organ uptake. Likewise, antigenicity studies using antisera from Fabry patients demonstrated that both drugs were indistinguishable in terms of antibody cross-reactivity. Based on these studies and present knowledge regarding the influence of glycosylation on protein biodistribution and cellular uptake, the two protein preparations appear to be functionally indistinguishable. Therefore, the data from these studies provide no rationale for the use of these proteins at different therapeutic doses.

Enzyme replacement therapy in Japanese Fabry disease patients: the results of a phase 2 bridging study.

PubMed

Eto, Y; Ohashi, T; Utsunomiya, Y; Fujiwara, M; Mizuno, A; Inui, K; Sakai, N; Kitagawa, T; Suzuki, Y; Mochizuki, S; Kawakami, M; Hosoya, T; Owada, M; Sakuraba, H; Saito, H

2005-01-01

Fabry Disease (alpha-galactosidase A deficiency) is an X-linked hereditary disorder leading to the pathological accumulation of globotriaosylceramide (GL-3) in lysosomes, particularly in the vascular endothelium of the kidney, heart and brain. We report the results of an open-label phase 2 study that was undertaken to evaluate whether ethnic differences exist that would affect agalsidase beta (Fabrazyme) treatment of Fabry patients in the Japanese population, relative to safety and efficacy. The study design mirrored the design of the completed phase 3 clinical trial that led to approval of the product agalsidase beta. The 13 Japanese, male Fabry patients enrolled in the study received the enzyme replacement therapy over a period of 20 weeks as biweekly infusions. All selected efficacy end points showed improvements that were comparable with findings from the phase 3 study. These improvements included reductions of GL-3 accumulation in both kidney and skin capillary endothelial cells to (near) normal levels (92% of patients). Kidney and plasma GL-3 levels decreased by 51.9% and 100%, respectively, by ELISA. Renal function remained normal. Fabry-associated pain, and quality of life, showed improvement over baseline in multiple categories. Related adverse events were mild or moderate in intensity and mostly infusion-associated (fever and rigors). As expected, IgG antibody formation was observed in 85% of the patients, but had no effect on treatment response. These results suggest that treatment with agalsidase beta is safe and effective in Japanese patients with Fabry disease. With regard to safety and efficacy, no differences were observed as compared to the caucasian population.

Composite-cavity-based Fabry-Perot interferometric strain sensors.

PubMed

Zhang, Jianzhong; Peng, G D; Yuan, Libo; Sun, Weimin

2007-07-01

A composite-cavity-based Fabry-Perot interferometric strain sensor system is proposed to gain the minimum cross sensitivity to temperature and a high multiplexing capability at the same time. The interrogation of the sensor system is based on a white-light interferometric technology, and the demodulation is achieved by analyzing the coherence spectra. A demonstration system with two sensors is presented and tested.

Fiber-Optic Temperature Sensor Using a Thin-Film Fabry-Perot Interferometer

NASA Technical Reports Server (NTRS)

Beheim, Glenn

1997-01-01

A fiber-optic temperature sensor was developed that is rugged, compact, stable, and can be inexpensively fabricated. This thin-film interferometric temperature sensor was shown to be capable of providing a +/- 2 C accuracy over the range of -55 to 275 C, throughout a 5000 hr operating life. A temperature-sensitive thin-film Fabry-Perot interferometer can be deposited directly onto the end of a multimode optical fiber. This batch-fabricatable sensor can be manufactured at a much lower cost than can a presently available sensor, which requires the mechanical attachment of a Fabry-Perot interferometer to a fiber. The principal disadvantage of the thin-film sensor is its inherent instability, due to the low processing temperatures that must be used to prevent degradation of the optical fiber's buffer coating. The design of the stable thin-film temperature sensor considered the potential sources of both short and long term drifts. The temperature- sensitive Fabry-Perot interferometer was a silicon film with a thickness of approx. 2 microns. A laser-annealing process was developed which crystallized the silicon film without damaging the optical fiber. The silicon film was encapsulated with a thin layer of Si3N4 over coated with aluminum. Crystallization of the silicon and its encapsulation with a highly stable, impermeable thin-film structure were essential steps in producing a sensor with the required long-term stability.

A Coaxial Cable Fabry-Perot Interferometer for Sensing Applications

PubMed Central

Huang, Jie; Wang, Tao; Hua, Lei; Fan, Jun; Xiao, Hai; Luo, Ming

2013-01-01

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. PMID:24212121

ASTRONAUT CONRAD, CHARLES (PETE), JR. - X-RAYS - MEDICAL TEAM MEMBERS - CAPE

NASA Image and Video Library

1965-08-17

S65-28699 (17 Aug. 1965) --- Astronaut Charles Conrad Jr. (dark shirt), pilot for the Gemini-5 spaceflight, discusses x-rays with members of the medical team at Cape Kennedy. Left to right are Dr. Eugene Tubbs; astronaut Conrad; Dr. Charles A. Berry, chief, Center Medical Programs, Manned Spacecraft Center; and Dr. Robert Moser (seated), Medical Monitor with the U.S. Army.

Optical fiber Fabry-Perot interferometry

NASA Astrophysics Data System (ADS)

Wang, Anbo

2014-06-01

Fiber Fabry-Perot (FP) interferometry is one of the most important tools for harsh environment sensing because of its great flexibility of sensor material selection, superior long-­-term stability, and nature of remote passive operation. Virginia Tech's Center for Photonics Technology has been involved in the research of this field for many years. After a quick review of the typical methods for the construction of F-P sensors, emphasis will be placed on the whitelight interferometry, which is perhaps the most robust interferometric sensor demodulation technique today. The recent discovery of an additional phase will be presented and its significance to the sensor demodulation will be discussed.

Environmental Education in the Galapagos: 2007 Report to the Charles Darwin Foundation

ERIC Educational Resources Information Center

Stepath, Carl M.

2007-01-01

Background: "Environmental education in the Galapagos: 2007 report to the Charles Darwin Foundation" is a report to the Charles Darwin Foundation (CDF) about the researchers observations about the status of environmental education in the Galapagos in 2006 and 2007. Purpose: This paper reports on environmental education in the Galapagos…

An arc tangent function demodulation method of fiber-optic Fabry-Perot high-temperature pressure sensor

NASA Astrophysics Data System (ADS)

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.

FIFI: The MPE Garching/UC Berkeley Far-Infrared Imaging Fabry-Perot Interferometer

NASA Technical Reports Server (NTRS)

Geis, Norbert; Genzel, Reinhard; Haggerty, M.; Herrmann, F.; Jackson, J.; Madden, Suzanne C.; Nikola, T.; Poglitsch, Albrecht; Rumitz, M.; Stacey, G. J.

1995-01-01

We describe the performance characteristics of the MPE Garching/UC Berkeley Far-Infrared Imaging Fabry-Perot Interferometer (FIFI) for the Kuiper Airborne Observatory (KAO). The spectrometer features two or three cryogenic tunable Fabry-Perot filters in series giving spectral resolution R of up to 10(exp 5) in the range of 40 microns less than lambda less than 200 microns, and an imaging 5x5 array of photoconductive detectors with variable focal plane plate scale. The instrument works at background limited sensitivity of up to 2 x 10(exp -19) W cm(exp -2) Hz(exp -1/2) per pixel per resolution element at R = 10(exp 5) on the KAO.

Thin-film-based optical fiber Fabry-Perot interferometer used for humidity sensing.

PubMed

Peng, Jiankun; Qu, Yapeng; Wang, Weijia; Sun, Tengpeng; Yang, Minghong

2018-04-20

A thin-film-based optical fiber Fabry-Perot interferometer that consists of ZrO 2 and SiO 2 porous thin films is designed and fabricated by electron beam physical vapor deposition. Since the SiO 2 porous thin film has the capability of water adsorption, the proposed Fabry-Perot interferometer is appropriate to detect humidity. Experimental results show that the prepared sensor has a humidity detection range from 0.06% RH to 70% RH. A cycling test shows that the humidity sensor has a responding or recover time of 4 s and good repeatability among different humidity environments. Especially, the proposed humidity sensor is insensitive to temperature variation and suitable for the detection of low relative humidity.

Charles Darwin and John Herschel

NASA Astrophysics Data System (ADS)

Warner, B.

2009-11-01

The influence of John Herschel on the philosophical thoughts of Charles Darwin, both through the former's book, Natural Philosophy, and through their meeting in 1836 at the Cape of Good Hope, is discussed. With Herschel having himself speculated on evolution just a few months before he met Darwin, it is probable that he stimulated at least the beginnings of the latter's lifelong work on the subject.

Temperature-independent refractometer based on fiber-optic Fabry-Perot interferometer

NASA Astrophysics Data System (ADS)

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.

MALDI-TOF and cluster-TOF-SIMS imaging of Fabry disease biomarkers

NASA Astrophysics Data System (ADS)

Touboul, David; Roy, Sandrine; Germain, Dominique P.; Chaminade, Pierre; Brunelle, Alain; Laprevote, Olivier

2007-02-01

Fabry disease is an X-linked disorder of glycosphingolipid metabolism, in which a partial or total deficiency of [alpha]-galactosidase A, a lysosomal enzyme, results in the progressive accumulation of neutral glycosphingolipids (globotriaosylceramide and digalactosylceramide) in most fluids and tissues of the body. Few information is available about the composition and distribution in tissues of the accumulated glycosphingolipids species. Mass spectrometry imaging is an innovative technique, which can provide pieces of information about the distribution of numerous biological compounds, such as lipids, directly on the tissue sections. MALDI-TOF and cluster-TOF-SIMS imaging approaches were used to study the localization of lipids (cholesterol, cholesterol sulfate, vitamin E, glycosphingolipids ...) on skin and kidney sections of patients affected by the Fabry disease. Numerous information on pathophysiology were enlightened by both techniques.

Significant improvement in Fabry disease podocytopathy after 3 years of treatment with agalsidase beta.

PubMed

Ito, Shuichi; Ogura, Masao; Kamei, Koichi; Matsuoka, Kentaro; Warnock, David G

2016-08-01

Fabry disease is an X-linked lysosomal disorder caused by decreased activity of α-galactosidase A (GLA). Consequent accumulation of globotriaosylceramide (GL-3) in lysosomes results in damage to a variety of organs, including the kidneys. Enzyme replacement therapy (ERT) is an effective treatment, but whether it should be started before organ damage is evident is a matter of debate. A 10-year-old boy who complained of severe sole pain for 3 years had been misdiagnosed with juvenile idiopathic arthritis. Further investigations revealed decreased GLA activity and a M1T mutation in the GLA gene causing protein truncation, suggestive of Fabry disease. Despite normal renal function and urinalysis, renal biopsy showed abnormal structure, with marked accumulation of GL-3 in podocytes, partial effacement of foot processes and irregularly reduced expression of nephrin in the slit diaphragm. After 1 year of ERT with 1 mg/kg agalsidase beta once every 2 weeks, his pain had resolved with ERT combined with carbamazepine and pregabalin. After 3 years of the ERT, repeat biopsy showed little renal GL-3 deposition, resolution of foot process effacement, and a dramatic improvement in nephrin expression. There may be a window of opportunity in which pain and renal injury can be addressed in the early stages of Fabry disease. Early initiation of ERT should therefore be considered for children with Fabry disease.

Agalsidase alfa: a review of its use in the management of Fabry disease.

PubMed

Keating, Gillian M

2012-10-01

The enzyme replacement therapy agalsidase alfa (Replagal®) has an amino acid sequence identical to that of native α-galactosidase A; intravenous agalsidase alfa 0.2 mg/kg every other week is indicated for the long-term treatment of patients with confirmed Fabry disease. This article reviews the efficacy and tolerability of agalsidase alfa in patients with Fabry disease, as well as summarizing its pharmacologic properties. Agalsidase alfa had beneficial effects in adult men with Fabry disease, according to the results of two randomized, double-blind, placebo-controlled, 6-month trials (n = 15 and 26). For example, left ventricular mass index was reduced to a significantly greater extent with agalsidase alfa than with placebo. Although the change in myocardial globotriaosylceramide content (primary endpoint in one study) did not significantly differ between agalsidase alfa and placebo recipients, the change in the Brief Pain Inventory (BPI) 'pain at its worst' score (reflecting neuropathic pain while without pain medications; primary endpoint in the second study) was improved to a significantly greater extent with agalsidase alfa than with placebo. In addition, the change in creatinine clearance, but not inulin clearance, significantly favored agalsidase alfa versus placebo recipients. Abnormalities in functional cerebral blood flow and cerebrovascular responses were also reversed with agalsidase alfa therapy. In extensions of these placebo-controlled trials, the reduction in left ventricular mass and improvements in BPI pain scores were maintained after longer-term agalsidase alfa therapy. The significant decline in estimated glomerular filtration rate (eGFR) seen after 48 months' agalsidase alfa treatment was mainly driven by a marked decline in eGFR seen in four patients with stage 3 chronic kidney disease at baseline (although the progression of decline appeared slower than that seen in historic controls); renal function appeared stable in patients with

Long-term systemic therapy of Fabry disease in a knockout mouse by adeno-associated virus-mediated muscle-directed gene transfer

PubMed Central

Takahashi, Hiroshi; Hirai, Yukihiko; Migita, Makoto; Seino, Yoshihiko; Fukuda, Yuh; Sakuraba, Hitoshi; Kase, Ryoichi; Kobayashi, Toshihide; Hashimoto, Yasuhiro; Shimada, Takashi

2002-01-01

Fabry disease is a systemic disease caused by genetic deficiency of a lysosomal enzyme, α-galactosidase A (α-gal A), and is thought to be an important target for enzyme replacement therapy. We studied the feasibility of gene-mediated enzyme replacement for Fabry disease. The adeno-associated virus (AAV) vector containing the α-gal A gene was injected into the right quadriceps muscles of Fabry knockout mice. A time course study showed that α-gal A activity in plasma was increased to ≈25% of normal mice and that this elevated activity persisted for up to at least 30 weeks without development of anti-α-gal A antibodies. The α-gal A activity in various organs of treated Fabry mice remained 5–20% of those observed in normal mice. Accumulated globotriaosylceramide in these organs was completely cleared by 25 weeks after vector injection. Reduction of globotriaosylceramide levels was also confirmed by immunohistochemical and electronmicroscopic analyses. Echocardiographic examination of treated mice demonstrated structural improvement of cardiac hypertrophy 25 weeks after the treatment. AAV vector-mediated muscle-directed gene transfer provides an efficient and practical therapeutic approach for Fabry disease. PMID:12370426

Screening for Fabry disease in left ventricular hypertrophy: documentation of a novel mutation.

PubMed

Baptista, Ana; Magalhães, Pedro; Leão, Sílvia; Carvalho, Sofia; Mateus, Pedro; Moreira, Ilídio

2015-08-01

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/m2 for women or ≥ 116 g/m2 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/m2 (± 28.5; 99.2 to 228.5 g/m2] 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).

Official portrait of astronaut Charles J. Precourt

NASA Technical Reports Server (NTRS)

1991-01-01

Official portrait of astronaut Charles J. Precourt. Precourt, a member of Astronaut Class 13 and United States Air Force (USAF), wears blue flight suit and poses with space shuttle orbiter model with a United States flag creating the backdrop.

Charles Darwin: What Else Did He Write?

ERIC Educational Resources Information Center

Berra, Tim M.

1980-01-01

Lists a number of books written by Charles Darwin, selected to indicate the depth and breadth of Darwin's biological interests. Each entry is described with a short annotation. Also provides a reading list of references about Darwin's life. (CS)

75 FR 33999 - Safety Zone; Fourth of July Fireworks Event, Cape Charles City Harbor, Cape Charles, VA

Federal Register 2010, 2011, 2012, 2013, 2014

2010-06-16

...: Coast Guard, DHS. ACTION: Temporary final rule. SUMMARY: The Coast Guard is establishing a 420-foot..., and falling hot embers or other debris, vessel traffic will be temporarily restricted within 420 feet... navigable waters of the Cape Charles City Harbor within the area bounded by a 420-foot radius circle...

Differential Radiometers Using Fabry-Perot Interferometric Technique for Remote Sensing of Greenhouse Gases

NASA Technical Reports Server (NTRS)

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.

Streamflow, water quality, and contaminant loads in the lower Charles River Watershed, Massachusetts, 1999-2000

USGS Publications Warehouse

Breault, Robert F.; Sorenson, Jason R.; Weiskel, Peter K.

2002-01-01

Streamflow data and dry-weather and stormwater water-quality samples were collected from the main stem of the Charles River upstream of the lower Charles River (or the Basin) and from four partially culverted urban streams that drain tributary subbasins in the lower Charles River Watershed. Samples were collected between June 1999 and September 2000 and analyzed for a number of potential contaminants including nitrate (plus nitrite), ammonia, total Kjeldahl nitrogen, phosphorus, cadmium, chromium, copper, lead, and zinc; and water-quality properties including specific conductance, turbidity, biochemical oxygen demand, fecal coliform bacteria, Entero-coccus bacteria, total dissolved solids, and total suspended sediment. These data were used to identify the major pathways and to determine the magnitudes of contaminants loads that contribute to the poor water quality of the lower Charles River. Water-quality and streamflow data, for one small urban stream and two storm drains that drain subbasins with uniform (greater than 73 percent) land use (including single-family residential, multifamily residential, and commercial), also were collected. These data were used to elucidate relations among streamflow, water quality, and subbasin characteristics. Streamflow in the lower Charles River Watershed can be characterized as being unsettled and flashy. These characteristics result from the impervious character of the land and the complex infrastructure of pipes, pumps, diversionary canals, and detention ponds throughout the watershed. The water quality of the lower Charles River can be considered good?meeting water-quality standards and guidelines?during dry weather. After rainstorms, however, the water quality of the river becomes impaired, as in other urban areas. The poor quality of stormwater and its large quantity, delivered over short periods (hours and days), together with illicit sanitary cross connections, and combined sewer overflows, results in large contaminant

Novel Fabry-Perot fiber optic sensor with multiple applications

NASA Astrophysics Data System (ADS)

Chen, Xiaopei; Shen, Fabin; Wang, Anbo; Wang, Zhuang; Zhang, Yan

2004-12-01

A novel Intrinsic Fabry-Perot fiber-optic sensor is presented in this paper. The sensors were made through two simple steps: wet chemical etch and fusion splice. Micro air-gaps were generated inside the fibers and functioned as reflective mirrors. This procedure not only provides a simple and cost effective technology for fabricating intrinsic Fabry-Perot Interferometric (IFPI) fiber sensors, but also provides two possible IFPI structures. Both of the fiber cavity between the air-gaps or the air-gap and cleaved fiber end can be used as sensing elements. With these two structures, this sensor can be used to measure the temperature, strain, pressure, refractive index of chemicals and the thin film thickness by itself. Multi-point measurements can also be achieved by multiplexing. Furthermore, it also can be multiplexed with other sensors such as Long Period Gratings (LPG) to provide compensations for other perturbation sensing. Theoretical and experimental studies of two sensor structures are described. Experimental results show that high resolution and high sensitivity can be obtained with appropriate signal processing.

The use of pressure controlled Fabry-Pérot interferometer with linear scanning of data for Brillouin-type experiments

NASA Astrophysics Data System (ADS)

Błachowicz, Tomasz

2000-08-01

The article presents results from work with Fabry-Pérot interferometers in Brillouin laser light scattering experiments, where optical signals of very low level intensity are observed. The information presented here can be useful in other types of optical experiments where scanning in the Fabry-Pérot interferometer spectral range has to be used. In such situations the shape of spectral lines as well as their relative distances can be detected. The key to the solution presented here is the use of a silicon-membrane pressure sensor coupled to a pressure chamber. It makes it possible to view spectral lines equally spaced after nonlinear flow of air from a chamber where the Fabry-Pérot interferometer is placed. Linear scanning in the spectral range equal to a frequency of about 150 GHz is possible. The method can be applied to Fabry-Pérot's etalons, very frequently produced some years ago. Now it should find new fields of application, in a simple and cost effective way, in student laboratories as well as in other research institutions.

Identification of a Novel GLA Mutation (L206 P) in a Patient with Fabry Disease.

PubMed

Kim, Ji-Hoon; Kim, Gee-Hee; Park, Hoon-Suk; Choi, Jin-A; Bae, Jung-Min; Cho, Uiju

2017-03-01

We report a new α-Galactosidase A (αGal-A) mutation in a 39-year-old Korean born, male Fabry disease patient. Fabry disease is a devastating, progressive inborn error of metabolism caused by X-linked genetic mutations. In this case, the first clinical symptom to occur was in childhood consisting of a burning pain originating in the extremities then radiating inwards to the limbs. This patient also stated to have ringing in his ears, angiokeratomas on his trunk, and cornea verticillata. He visited an outpatient cardiologist due to intermittent and atypical chest discomfort at the age of 39. Electrocardiographic and echocardiographic examination showed left ventricular hypertrophy. A physical examination revealed proteinuria without hematuria. The patient's plasma αGal-A activity was markedly lower than the mean value of the controls. After genetic counseling and obtaining written informed consent, we identified one hemizygous mutation in exon 4 of galactosidase alpha, c.617T>C (p.Leu206 Pro). He was eventually diagnosed as having Fabry disease.

The Scientific Legacy of Charles Wheatstone

ERIC Educational Resources Information Center

Greenslade, Thomas B., Jr.

2017-01-01

Charles Wheatstone's name was once familiar to students because they used his eponymous bridge to measure resistances. That usage seems to be disappearing--we all have access to digital ohmmeters--but the techniques that he developed for making electrical measurements can still be used with profit. Also, his work with measuring very short time…

78 FR 51803 - Culturally Significant Objects Imported for Exhibition Determinations: “Charles Marville...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-08-21

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77 FR 67829 - Charles M. Russell National Wildlife Refuge and UL Bend National Wildlife Refuge, MT...

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Deep Fabry-Perot imaging of NGC 6240: Kinematic evidence for merging galaxies

NASA Technical Reports Server (NTRS)

Hawthorn, J. Bland; Wilson, A. S.; Tully, R. B.

1990-01-01

The authors have observed the superluminous, infrared galaxy NGC 6240 (z = 0.025) at H alpha with the Hawaii Imaging Fabry-Perot Interferometer (HIFI - Bland and Tully 1989). During the past decade, observational evidence from all wavebands indicates that the unusual appearance of NGC 6240 has resulted from a collision between two gas-rich systems, a view which is supported by our spectrophotometric data. However, the origin of the enormous infrared luminosity (4 times 10(exp 11) solar luminosity) detected by the Infrared Astronomy Satellite (IRAS) remains highly controversial, where opinions differ on the relative roles of large-scale shocks, massive star formation or a buried 'active' nucleus. These mechanisms are discussed in the light of the author's Fabry-Perot observations.

Charles A. Beard at Mid-Career: "Reflections".

ERIC Educational Resources Information Center

Braeman, John

1987-01-01

Examines Charles A. Beard's contribution to how scholars interpret the framing of the Constitution and other events of U.S. history. Describes his work in some detail and includes a transcript of a 1926 conference at which Beard spoke in 1926. (RKM)

Cilioretinal artery occlusion and anterior ischemic optic neuropathy as the initial presentation in a child female carrier of Fabry disease.

PubMed

Ersoz, M Giray; Ture, Gamze

2018-04-01

To report the youngest female carrier of Fabry disease, complicated by cilioretinal artery occlusion and anterior ischemic optic neuropathy (AION). Case report. An 11-year-old girl was referred to our clinic with painless, acute loss of vision in her right eye. Posterior segment examination and fluorescein angiography revealed cilioretinal artery occlusion and AION. Systemic evaluations were unremarkable, except for a low blood α-galactosidase A enzyme level of 242.27 pmol/spot*20 h (reference range: 450-2000 pmol/spot*20 h). The patient was diagnosed with female carrier of Fabry disease. Retinal vascular occlusions are rare in childhood, and Fabry disease may present with retinal vascular occlusion. Ophthalmological examinations may be contributing for early detection of the disease. To the best of our knowledge, this is the first report of a child female carrier of Fabry disease, complicated by cilioretinal artery occlusion and AION.

Fabry disease presenting as apical left ventricular hypertrophy in a patient carrying the missense mutation R118C.

PubMed

Caetano, Francisca; Botelho, Ana; Mota, Paula; Silva, Joana; Leitão Marques, António

2014-03-01

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by abnormalities of the GLA gene, which encodes the enzyme α-galactosidase A. A deficiency of this enzyme leads to the lysosomal accumulation of glycosphingolipids, which may cause left ventricular hypertrophy that is typically concentric and symmetric. We present the case of a 60-year-old woman with symptoms of dyspnea, atypical chest pain and palpitations, in whom a transthoracic echocardiogram revealed an apical variant of hypertrophic cardiomyopathy. Analysis of specific sarcomeric genetic mutations was negative. The patient underwent a screening protocol for Anderson-Fabry disease, using a dried blood spot test, which was standard at our institution for patients with left ventricular hypertrophy. The enzymatic activity assay revealed reduced α-galactosidase A enzymatic activity. Molecular analysis identified a missense point mutation in the GLA gene (p.R118C). This case report shows that Anderson-Fabry disease may cause an apical form of left ventricular hypertrophy. The diagnosis was only achieved because of systematic screening, which highlights the importance of screening for Anderson-Fabry disease in patients with unexplained left ventricular hypertrophy, including those presenting with more unusual patterns, such as apical variants of left ventricular hypertrophy. This case also supports the idea that the missense mutation R118C is indeed a true pathogenic mutation of Anderson-Fabry disease. Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

A theoretical multi-reflection method for analysis of optomechanical behavior of the Fabry-Perot cavity with moving boundary condition

NASA Astrophysics Data System (ADS)

Bahrampour, A. R.; Vahedi, M.; Abdi, M.; Ghobadi, R.; Golshani, M.; Tofighi, S.; Parvin, B.

2011-09-01

The opto-mechanical coupling and the generation of Stokes and anti-Stokes frequencies in the in-band and intra-band regimes of operation of the Fabry-Perot cavity with a moving mirror on the basis of multi-reflection method (MRM) are described by a unique theory. The frequency characteristic function of the Fabry-Perot filter is modified. By increasing the amplitude of mirror oscillation the Fabry-Perot bandwidth increases and normal mode splitting occurred. The conversion efficiencies of the Stokes and anti-Stokes frequencies versus the mechanical amplitude of oscillation have an optimum value. Also, the delay function corresponding to the radiation pressure is obtained.

Solid, 3-mirror Fabry-Perot etalon.

PubMed

Stephen, Mark; Fahey, Molly; Miller, Ian

2017-04-01

We present modeling and performance of a solid, fused silica, 3-mirror Fabry-Perot-type etalon. 3-mirror etalons have been known for decades to have superior theoretical performance but for the first time we demonstrate an etalon with sufficient quality to realize the benefits of the more complex design. 3-mirror etalons have better passband shape and higher contrast ratio enabling significantly improved wavelength separation. We show the optical cavity design and construction of the new etalon and show >95% peak transmission, improved passband shape and 20 dB better out-of-band rejection than a similar 2-mirror etalon.

Absolute-length determination of a long-baseline Fabry-Perot cavity by means of resonating modulation sidebands.

PubMed

Araya, A; Telada, S; Tochikubo, K; Taniguchi, S; Takahashi, R; Kawabe, K; Tatsumi, D; Yamazaki, T; Kawamura, S; Miyoki, S; Moriwaki, S; Musha, M; Nagano, S; Fujimoto, M K; Horikoshi, K; Mio, N; Naito, Y; Takamori, A; Yamamoto, K

1999-05-01

A new method has been demonstrated for absolute-length measurements of a long-baseline Fabry-Perot cavity by use of phase-modulated light. This method is based on determination of a free spectral range (FSR) of the cavity from the frequency difference between a carrier and phase-modulation sidebands, both of which resonate in the cavity. Sensitive response of the Fabry-Perot cavity near resonant frequencies ensures accurate determination of the FSR and thus of the absolute length of the cavity. This method was applied to a 300-m Fabry-Perot cavity of the TAMA gravitational wave detector that is being developed at the National Astronomical Observatory, Tokyo. With a modulation frequency of approximately 12 MHz, we successfully determined the absolute cavity length with resolution of 1 microm (3 x 10(-9) in strain) and observed local ground strain variations of 6 x 10(-8).

78 FR 28205 - Notice of Availability of the Draft Environmental Impact Statement for the Lake Charles Carbon...

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... Lake Charles Carbon Capture and Sequestration Project (DOE/EIS-0464D) AGENCY: U.S. Department of Energy...) announces the availability of the Lake Charles Carbon Capture and Sequestration Project Draft [[Page 28206... potential environmental impacts associated with the Lake Charles Carbon Capture and Sequestration Project...

3 CFR - The Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles...

Code of Federal Regulations, 2013 CFR

2013-01-01

... Respect to the Former Liberian Regime of Charles Taylor Presidential Documents Other Presidential... Liberian Regime of Charles Taylor On July 22, 2004, by Executive Order 13348, the President declared a... connected to the former Liberian regime of Charles Taylor, pursuant to the International Emergency Economic...

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

PubMed Central

2011-01-01

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 Fabry disease appears to be

Enhanced transmission in rolled-up hyperlenses utilizing Fabry-Pérot resonances

NASA Astrophysics Data System (ADS)

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

2011-11-01

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

Reaccumulation of globotriaosylceramide in podocytes after agalsidase dose reduction in young Fabry patients.

PubMed

Skrunes, Rannveig; Svarstad, Einar; Kampevold Larsen, Kristin; Leh, Sabine; Tøndel, Camilla

2017-05-01

Agalsidase-α 0.2 mg/kg every other week (eow) and agalsidase-β 1.0 mg/kg/eow are licensed in Europe as equipotent treatment of the α-galactosidase deficiency in Fabry disease. This case series describes the effects of agalsidase dose adjustments in serial kidney biopsies in switch patients. All treatment-naïve patients with classical Fabry disease in our centre started on agalsidase-β 1.0 mg/kg/eow and subsequently switched to agalsidase-α 0.2 mg/kg/eow were included ( n = 3). The median age at enzyme replacement therapy start was 11 (range 7-18) years. Kidney biopsies were performed at baseline, after 5 years of agalsidase-β 1.0 mg/kg/eow and after 3 subsequent years of agalsidase-α 0.2 mg/kg/eow. One patient was re-biopsied 2 years after reswitch to agalsidase-β 1.0 mg/kg/eow. The scoring system of the International Scoring Group of Fabry Nephropathy was used. The patients completely cleared globotriaosylceramide (GL3) from mesangial and endothelial cells and partly cleared podocytes on agalsidase-β 1.0 mg/kg/eow. Reaccumulation of GL3 in podocytes, but not in the mesangium or endothelium, occurred after 3 years of agalsidase-α 0.2 mg/kg/eow. Subsequent reduction of podocyte GL3 was observed in the single patient rebiopsied 2 years after reswitch to agalsidase-β 1.0 mg/kg/eow. Partial clearance, reaccumulation and renewed partial clearance of podocyte GL3 deposits in serial kidney biopsies over 8-10 years were seen in parallel with agalsidase dose adjustments. Repeated kidney biopsies may impact therapeutic choices in Fabry disease. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Energy utilization of induced pluripotent stem cell-derived cardiomyocyte in Fabry disease.

PubMed

Chou, Shih-Jie; Yu, Wen-Chung; Chang, Yuh-Lih; Chen, Wen-Yeh; Chang, Wei-Chao; Chien, Yueh; Yen, Jiin-Cherng; Liu, Yung-Yang; Chen, Shih-Jen; Wang, Chien-Ying; Chen, Yu-Han; Niu, Dau-Ming; Lin, Shing-Jong; Chen, Jaw-Wen; Chiou, Shih-Hwa; Leu, Hsin-Bang

2017-04-01

Fabry disease (FD) is a lysosomal storage disease in which glycosphingolipids (GB3) accumulate in organs of the human body, leading to idiopathic hypertrophic cardiomyopathy and target organ damage. Its pathophysiology is still poorly understood. We aimed to generate patient-specific induced pluripotent stem cells (iPSC) from FD patients presenting cardiomyopathy to determine whether the model could recapitulate key features of the disease phenotype and to investigate the energy metabolism in Fabry disease. Peripheral blood mononuclear cells from a 30-year-old Chinese man with a diagnosis of Fabry disease, GLA gene (IVS4+919G>A) mutation were reprogrammed into iPSCs and differentiated into iPSC-CMs and energy metabolism was analyzed in iPSC-CMs. The FD-iPSC-CMs recapitulated numerous aspects of the FD phenotype including reduced GLA activity, cellular hypertrophy, GB3 accumulation and impaired contractility. Decreased energy metabolism with energy utilization shift to glycolysis was observed, but the decreased energy metabolism was not modified by enzyme rescue replacement (ERT) in FD-iPSCs-CMs. This model provided a promising in vitro model for the investigation of the underlying disease mechanism and development of novel therapeutic strategies for FD. This potential remedy for enhancing the energetic network and utility efficiency warrants further study to identify novel therapies for the disease. Copyright © 2017 Elsevier B.V. All rights reserved.

77 FR 42179 - Safety Zone; Fireworks Display, Potomac River, Charles County, Newburg, MD

Federal Register 2010, 2011, 2012, 2013, 2014

2012-07-18

...]30[sec] W, located at Newburg in Charles County, Maryland (NAD 1983). The temporary safety zone will... 1625-AA00 Safety Zone; Fireworks Display, Potomac River, Charles County, Newburg, MD AGENCY: Coast Guard, DHS. ACTION: Temporary final rule. SUMMARY: The Coast Guard will establish a safety zone upon...

Cardiovascular testing in Fabry disease: exercise capacity reduction, chronotropic incompetence and improved anaerobic threshold after enzyme replacement.

PubMed

Lobo, T; Morgan, J; Bjorksten, A; Nicholls, K; Grigg, L; Centra, E; Becker, G

2008-06-01

The aim of this study was to document exercise capacity and serial electrocardiogram and echocardiograph findings in a cohort of Australian patients with Fabry disease, in relation to their history of enzyme replacement therapy (ERT). Fabry disease has multifactorial effects on the cardiovascular system. Most previous studies have focused on electrocardiographic and echocardiographic parameters. Exercise capacity can be used as an integrated measure of cardiovascular function and allows the effects of treatment to be monitored. A total of 38 patients (30 men and 8 women) with Fabry disease were monitored by 12-lead electrocardiograms every 6-12 months, and by annual standardized-protocol echocardiograms. Bicycle stress tests with VO(2) max measurement and once-only 6 minutes' walk tests were also carried out in subsets of patients whose general health status allowed testing. Seventy per cent of patients met electrocardiogram criteria for left ventricular hypertrophy. Left ventricular hypertrophy on echocardiograph was present in 64% of patients (80% of men). Exercise capacity was reduced in patients with Fabry disease compared with that predicted from normative population data. Mild improvement in anaerobic threshold was seen in the first year of ERT (14.1 +/- 3.0 to 15.8 +/- 3.0, P = 0.02), but no consistent further increase was seen beyond the first year. Most patients had resting bradycardia, with impaired ability to increase heart rate during exercise. Serial testing on ERT showed an improvement in anaerobic threshold but no significant change in VO(2) max. Male patients with Fabry disease were unable to attain predicted maximal heart rate on exercise or to achieve normal exercise levels. ERT was associated with a small improvement in anaerobic threshold over the first year.

Fabry disease in children: correlation between ocular manifestations, genotype and systemic clinical severity.

PubMed

Allen, L E; Cosgrave, E M; Kersey, J P; Ramaswami, U

2010-12-01

Fabry disease is an X linked lysosomal disorder associated with severe multiorgan failure and premature death. This study aims to determine the prevalence of ophthalmic manifestations in children with the condition and investigate the correlation with genotype and systemic disease severity. The records of 26 children from 18 pedigrees with Fabry disease undergoing regular ophthalmic and systemic examination were reviewed. All pedigrees underwent GLA gene sequencing to determine genotype. Correlations between ocular and systemic phenotype and genotype were investigated. Corneal verticillata occurred in 50% of the children in this study (95% CI, 29% to 79%). Children with ophthalmic manifestations were more likely to have loss-of-function GLA mutations (p=0.003). Retinal vascular tortuosity was seen in seven children (27%), all of whom had systemic symptoms suggestive of autonomic neuropathy, such as diarrhoea and syncope. These symptoms seemed less prevalent in children without retinal vascular changes, although this did not reach statistical significance (p=0.134). Ophthalmic manifestations of Fabry disease are common even in young children with loss-of-function GLA gene mutations. Although the limited sample size possibly prevented statistical significance, systemic symptoms of autonomic neuropathy often coexist with retinal vascular changes and may share the same pathogenesis.

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

NASA Technical Reports Server (NTRS)

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.

A miniature extrinsic fiber Fabry-Perot pressure sensor based on fiber etching

NASA Astrophysics Data System (ADS)

Ge, Yixian; Wang, Ming; Yang, Chundi

2009-10-01

This paper presents a miniature fiber optic pressure sensor based on Fabry-Perot interference fabricated on the tip of a single mode (SM) fiber. The sensor measures only 125μm in diameter. A Fabry-Perot cavity and a thin silica diaphragm are fabricated by simple techniques involving only fusion splicing, cleaving, and wet chemical etching. Interference pattern of the sensor is analyzed and issues in sensor design are discussed. The overall chemical reaction of the fiber wet etching is specifically represented. Pressure testing system is carried out. By tracing a peak point in the interference spectrum, the gap length of the sensor can be demodulated. The sensor is made entirely of fused silica, whose structure has good stability, cabinet, simple for fabrication and low cost. It may also find uses in medical applications.

A miniature extrinsic fiber Fabry-Perot pressure sensor based on fiber etching

NASA Astrophysics Data System (ADS)

Ge, Yixian; Zhou, Junping; Wang, Tingting

2011-11-01

A miniature fiber optic pressure sensor based on Fabry-Perot interference fabricated on the tip of a single mode (SM) fiber is presented. The sensor measures only 125μm in diameter. A Fabry-Perot cavity and a thin silica diaphragm are fabricated by simple techniques involving only cleaving, wet chemical etching and fusion splicing. Interference pattern of the sensor is analyzed and issues in sensor design are discussed. The overall chemical reaction of the fiber wet etching is specifically represented. Pressure testing system is carried out. By tracing a peak point in the interference spectrum, the gap length of the sensor can be demodulated. Experimental results show the sensor has a good linearity. The sensor is made entirely of fused silica, whose structure has good stability, cabinet, simple for fabrication and low cost.

Obituary: Charles Latif Hyder, 1930-2004

NASA Astrophysics Data System (ADS)

White, Oran Richard

2004-12-01

My friend and colleague, Charles Hyder, was a true physicist with a sound intuitive grasp of fundamentals in modern physics and the underlying mathematics. I admired his knowledge of the history of modern physics and quantum mechanics when we discussed contemporary problems in interpreting solar observations. He had the ability to present his ideas clearly and persuasively to both students and his colleagues. His insatiable curiosity about life in general led him to consider the effects of nuclear weapons development on the human race. Appreciation of the biological effects of radioactive materials produced in the course of weapons and power reactor development led him to a more public career beyond traditional research. Charles Hyder was born April 18, 1930 in Albuquerque, New Mexico. He graduated from Albuquerque High School and served in the Air Force during the Korean War. He received a BS and MS in physics from the University of New Mexico (1958, 1960) and a PhD in astrogeophysics at the University of Colorado (1964). His positions included the Department of Astronomy and Institute of Geophysics at UCLA (1964-65), Sacramento Peak Solar Observatory (1965-1970) and the Goddard Space Flight Center (1970-1977). He also taught at the University of New Mexico (1970-1977) and was active on the Solar Maximum Mission science team (1970-1977, 1980-1984). He was married twice with both marriages ending in divorce. He and his first wife Ann had three children (Paul, Roxanne and Querida) and he and his second wife Laurie had a son Niels. Charles Hyder's professional career in solar physics began in 1961 during his graduate studies at the Department of AstroGeophysics of the University of Colorado and continued until 1983 when he chose to follow his convictions to expose the threat of nuclear proliferation. His early research was in the study of the quantum mechanics of polarized light produced in the presence of magnetic fields. Application of this work to interpretation

A hybrid demodulation method of fiber-optic Fabry-Perot pressure sensor

NASA Astrophysics Data System (ADS)

Yu, Le; Lang, Jianjun; Pan, Yong; Wu, Di; Zhang, Min

2013-12-01

The fiber-optic Fabry-Perot pressure sensors have been widely applied to measure pressure in oilfield. For multi-well it will take a long time (dozens of seconds) to demodulate downhole pressure values of all wells by using only one demodulation system and it will cost a lot when every well is equipped with one system, which heavily limits the sensor applied in oilfield. In present paper, a new hybrid demodulation method, combining the windowed nonequispaced discrete Fourier Transform (nDFT) method with segment search minimum mean square error estimation (MMSE) method, was developed, by which the demodulation time can be reduced to 200ms, i.e., measuring 10 channels/wells was less than 2s. Besides, experimental results showed the demodulation cavity length of the fiber-optic Fabry-Perot sensor has a maximum error of 0.5 nm and consequently pressure measurement accuracy can reach 0.4% F.S.

Sensing Properties of a Fabry-Perot Dielectric Structure and Dimer Nanoparticles

DOE PAGES

Polemi, A.; Shuford, K. L.

2012-01-01

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 arrangedmore » 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.« less

Enzyme replacement therapy for Anderson-Fabry disease.

PubMed

El Dib, Regina; Gomaa, Huda; Carvalho, Raíssa Pierri; Camargo, Samira E; Bazan, Rodrigo; Barretti, Pasqual; Barreto, Fellype C

2016-07-25

Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. Survival is reduced among affected males and symptomatic female carriers.This is an update of a Cochrane review first published in 2010, and previously updated in 2013. To evaluate the effectiveness and safety of enzyme replacement therapy compared to other interventions, placebo or no interventions, for treating Anderson-Fabry disease. We searched the Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register (date of the most recent search: 08 July 2016). We also searched 'Clinical Trials' on The Cochrane Library, MEDLINE, Embase and LILACS (date of the most recent search: 24 September 2015). Randomized controlled trials of agalsidase alfa or beta in participants diagnosed with Anderson-Fabry disease. Two authors selected relevant trials, assessed methodological quality and extracted data. Nine trials comparing either agalsidase alfa or beta in 351 participants fulfilled the selection criteria.Both trials comparing agalsidase alfa to placebo reported on globotriaosylceramide concentration in plasma and tissue; aggregate results were non-significant. One trial reported pain scores measured by the Brief Pain Inventory severity, there was a statistically significant improvement for participants receiving treatment at up to three months, mean difference -2.10 (95% confidence interval -3.79 to -0.41; at up to five months, mean difference -1.90 (95% confidence interval -3.65 to -0.15); and at up to six months, mean difference -2.00 (95% confidence interval -3.66 to -0.34). There was a significant difference in the Brief Pain Inventory pain-related quality of life at over five months and up to six months, mean difference -2.10 (95% confidence interval -3.92 to -0.28) but not at other time points. Death was not an outcome

Electro-optic Modulation Using a DAST Single-crystal Film in a Fabry-Perot Cavity

NASA Astrophysics Data System (ADS)

Kutty, S. P.

2005-03-01

In this paper, we report a multiple-pass electro-optic modulator using a single- crystal film of 4'-dimethyamino-N-methyl-4-stilbazolium tosylate (DAST) placed inside a Fabry-Perot cavity. The single-crystal film was prepared using the modified shear method. Electro-optic modulation was achieved at 633 nm using field-induced birefringence in the cross polarized geometry including the Fabry-Perot cavity. The modulation due to the electro-optic effect was recorded as a function of phase while the phase was controlled by moving one of the mirrors in the cavity. The observed modulation was high (80 percent) for a low field (0.5V/micron) applied along the charge transfer axis on the film. Similar modulation using the Fabry-Perot cavity with a lower modulation depth was observed involving electroabsorption at 633 nm. Electroabsorption in the DAST film has been recently reported [1]. These are important results considering applications in photonics. [1] ``Electroabsorption in single-crystal film of a second-order optical material,'' R. K. Swamy, S. P. Kutty, J. Titus, S. Khatavkar, and M. Thakur, APL, Vol. 85, 4025, (2004).

Le Laboratoire de Mécanique Appliquée Raymond Chaléat a 40 ans !

NASA Astrophysics Data System (ADS)

Comité D'Organisation Du Colloque Ime 2002, Le

2002-12-01

Le LMARC a été créé par Raymond Chaléat dans les locaux de l'École Nationale d'Horlogerie (a présent Lycée Technique Jules Haag).L'équipe de recherche était alors constituée de 2 enseignants-chercheurs (Raymond Chaléat et Gérard Lallement) et d'un technicien (Bernard Prêtre). Les travaux de recherche se situaient naturellement dans le domaine de la chronométrie et en particulier dans la mécanique non linéaire analytique inhérente à la montre en fonctionnement. En effet, Raymond Chaléat fut l'élève, puis le collaborateur du Professeur Jules Haag, mathématicien et mécanicien, lui-même élève de Poincarré.Le LMARC a étendu ses activités avec l'arrivée de Claude Oudet, puis de Claude Oytana à la rhéologie des matériaux solides qui est devenue plus tard l'Équipe Propriétés Mécaniques des Matériaux. En parallèle, G. Lallement a créé une équipe Vibrations devenue à ce jour Dynamique des Structures. À ce jour, le Laboratoire compte plus de 110 personnes dont 40 doctorants.Il a depuis diversifié ses activités avec 3 nouvelles équipes tournées vers la Modélisation et la Mise en Forme des Matériaux, les Micromachines et la Biomécanique et les Mécanismes. Il a su également prendre toute sa place au sein de l'Institut des Microtechniques de Franche-Comté en contribuant à plusieurs thèmes.Notre Laboratoire a été l'un des premiers laboratoires associé au CNRS (4ème) et a toujours été rattaché à l'Université de Franche-Comté (Présidence par C. Oytana de 1996 a 2001) par l'entremise de I'UFR Sciences et Techniques. Il a toujours maintenu des liens forts avec I'ENSMM (École Nationale Supérieure de Mécanique et des Microtechniques) d'une part par ses enseignants-chercheurs et d'autre part deux de ses directeurs, Raymond Chaléat et actuellement Jean-Claude Gelin.En plus de ses relations constantes avec l'industrie régionale, le LMARC a développé des actions contractuelles avec de grands groupes tels que EDF

[Heart involvement in Anderson-Fabry disease: Italian recommendations for diagnostic, follow-up and therapeutic management].

PubMed

Pieruzzi, Federico; Pieroni, Maurizio; Zachara, Elisabetta; Marziliano, Nicola; Morrone, Amelia; Cecchi, Franco

2015-11-01

Anderson-Fabry disease is a rare X-linked lysosomal storage disorder caused by mutations of the GLA gene that encodes alpha-galactosidase A. It is characterized by a multisystemic involvement: the renal, neurological, heart, cochleovestibular and cutaneous systems are the most damaged. Morbidity and mortality of Anderson-Fabry disease depend on renal insufficiency, heart failure and nervous system involvement. Left ventricular hypertrophy is the most common cardiac manifestation followed by conduction system disease, valve dysfunction, and arrhythmias. Mild to moderate left ventricular hypertrophy may simulate a non-obstructive hypertrophic cardiomyopathy. Management of Anderson-Fabry disease starting from the diagnosis of cardiac involvement, the prevention of complications, the therapeutic aspects, up to appropriate clinical follow-up, requires a multidisciplinary approach. According to recent management guidelines, only few evidence-based data are available to guide the clinical and therapeutic approach to this rare disease. An Italian Board, composed by nephrologists, cardiologists, geneticists, pediatricians and neurologists has been established in order to approve by consensus a diagnostic and therapeutic management protocol. The authors report the results of this cardiologic management consensus.

Gastrointestinal manifestations of Fabry disease: clinical response to enzyme replacement therapy.

PubMed

Banikazemi, Maryam; Ullman, Thomas; Desnick, Robert J

2005-08-01

Gastrointestinal symptoms are often an early and prominent manifestation of Fabry disease, an X-linked inborn error of metabolism caused by the deficient activity of the lysosomal enzyme, alpha-galactosidase A. This enzyme deficiency results in the progressive accumulation of globotriaosylceramide and other glycosphingolipids in tissue lysosomes throughout the body. In classically affected patients, glycosphingolipid accumulation in the vascular endothelium eventually culminates in life-threatening renal, cardiac, and cerebrovascular disease. In addition, over 50% of patients experience post-prandial abdominal pain and diarrhea that interferes with the ability to work and quality of life. Here, we describe four males aged 17-40 years with classic Fabry disease and severe gastrointestinal symptoms who participated in clinical trials of enzyme replacement therapy with agalsidase beta (Fabrazyme, 1 mg/kg every 2 weeks). Before therapy, the three adult patients experienced post-prandial abdominal pain, bloating, and severe diarrhea with 7-10 bowel movements per day every day and the 17-year-old had weekly episodes of diarrhea with six bowel movements per day. Other symptoms included vomiting, food intolerance, and poor weight gain. All patients took medications for these symptoms (diphenoxylate-atropine [Lomotil], ranitidine hydrochloride [Zantac], or sulfasalazine). After 6-7 months of agalsidase beta therapy, all patients reported "no or only occasional" abdominal pain or diarrhea, had discontinued their gastrointestinal medications, and had gained 3-8 kg. These marked improvements in gastrointestinal symptoms have persisted for over 3 years of treatment. In such patients, enzyme replacement at 1 mg/kg effects an early and significant clinical improvement in the gastrointestinal manifestations of Fabry disease.

Use of gamma ray radiation to parallel the plates of a Fabry-Perot interferometer

NASA Technical Reports Server (NTRS)

Skinner, Wilbert R.; Hays, Paul B.; Anderson, Sally M.

1987-01-01

The use of gamma radiation to parallel the plates of a Fabry-Perot etalon is examined. The method for determining the etalon parallelism, and the procedure for irradiating the posts are described. Changes in effective gap for the etalon over the surface are utilized to measure the parallelism of the Fabry-Perot etalon. An example in which this technique is applied to an etalon of fused silica plates, which are 132 mm in diameter and coded with zinc sulfide and cryolite, with Zerodur spaces 2 cm in length. The effect of the irradiation of the posts on the thermal performance of the etalon is investigated.

Creative Work: The Case of Charles Darwin.

ERIC Educational Resources Information Center

Gruber, Howard E.; Wallace, Doris B.

2001-01-01

Describes the evolving systems approach (ESA) to creative work, which emerged from a case study of Charles Darwin. Explains how the ESA differs from other approaches and describes various facets of creative work (networks of enterprise, uniqueness, insight, pluralism, and evolving belief systems and ensembles of metaphor). Emphasizes the…

Agalsidase beta treatment is associated with improved quality of life in patients with Fabry disease: findings from the Fabry Registry.

PubMed

Watt, Torquil; Burlina, Alessandro P; Cazzorla, Chiara; Schönfeld, Dorothee; Banikazemi, Maryam; Hopkin, Robert J; Martins, Ana Maria; Sims, Katherine; Beitner-Johnson, Dana; O'Brien, Fanny; Feldt-Rasmussen, Ulla

2010-11-01

To evaluate the effect of agalsidase beta on longitudinal health-related quality of life in patients with Fabry disease. The SF-36® Health Survey was used to measure health-related quality of life in Fabry Registry patients. Seventy-one men and 59 women who were treated with agalsidase beta (median dose: 1.0 mg/kg/² weeks) and who had baseline and at least 2 yearly posttreatment health-related quality of life measurements were included in these analyses. A repeated measures model was used to analyze change in score from baseline. Men improved in the physical component summary and in all eight scales of the SF-36 after 1 and 2 years and in the mental component summary after 1 year of agalsidase beta treatment (P < 0.05). Women improved in the mental component summary and in six of the eight scales after 1 and/or 2 years of treatment. Patients whose baseline SF-36 scores were below the median showed the greatest improvements. These responses were comparable with or greater than the published effects of various treatments for multiple sclerosis, rheumatoid arthritis, central neuropathic pain, and Gaucher disease. Long-term treatment with agalsidase beta resulted in substantial improvements in health-related quality of life in both men and women; the effect was more pronounced in men.

75 FR 8804 - Safety Zone; NASSCO Launching of USNS Charles Drew, San Diego Bay, San Diego, CA.

Federal Register 2010, 2011, 2012, 2013, 2014

2010-02-26

...-AA00 Safety Zone; NASSCO Launching of USNS Charles Drew, San Diego Bay, San Diego, CA. AGENCY: Coast... United States Naval Ship (USNS) Charles Drew. The safety zone is necessary to provide for the safety of... to the safety of the USNS Charles Drew and surrounding vessels as this ship launches from NASSCO...

Neuropathic symptoms and findings in women with Fabry disease.

PubMed

Laaksonen, Satu M; Röyttä, Matias; Jääskeläinen, Satu K; Kantola, Ilkka; Penttinen, Maila; Falck, Björn

2008-06-01

To examine the neurologic and neurophysiologic findings and neurologic symptoms in 12 women with Fabry disease and to study the relationship between the subjective symptoms and the findings on the various tests done. Neurography, vibratory and thermal quantitative sensory testing (QST), skin biopsy for measuring intraepidermal nerve fiber density (IENFD). Heart rate variability (HRV) and sympathetic skin response (SSR) tests for detecting autonomic dysfunction, pain-, depression- and somatic symptom questionnaires and clinical examination. Only two women had no persistent symptoms or signs of polyneuropathy, 10 had symptoms of small fiber neuropathy. Neurological examination was normal in most patients. Five patients had decreased IENFD or thermal hypoesthesia in QST. In QST, Adelta-fiber function for innocuous cold was more often impaired than C-fiber function. Conventional nerve conduction studies were mostly normal. Carpal tunnel syndrome (CTS) incidence was increased, 25% had symptomatic CTS. Heterozygous women carrying the gene for Fabry disease have symptoms and findings of small-fiber polyneuropathy more often than has previously been considered. The prevalence of CTS is also increased. While the clinical diagnosis of small-fiber neuropathy is difficult, the diagnostic yield can be increased using a combination of thermal QST and IENFD measurements.

Altered Dynamics of a Lipid Raft Associated Protein in a Kidney Model of Fabry Disease

PubMed Central

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

2013-01-01

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. PMID:24215843

Diaphragm based long cavity Fabry-Perot fiber acoustic sensor using phase generated carrier

NASA Astrophysics Data System (ADS)

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.

Application of thin dielectric films in low coherence fiber-optic Fabry-Pérot sensing interferometers: comparative study

NASA Astrophysics Data System (ADS)

Hirsch, Marzena; Wierzba, Paweł; Jedrzejewska-Szczerska, Małgorzata

2016-11-01

We examine the application of selected thin dielectric films, deposited by atomic layer deposition (ALD), in a low coherence fiber-optic Fabry-Pérot interferometer designed for sensing applications. Such films can be deposited on the end-face of a single mode optical fiber (SMF-28) in order to modify the reflectivity of the Fabry-Pérot cavity, to provide protection of the fibers from aggressive environments or to create a multi-cavity interferometric sensor. Spectral reflectance of films made from zinc oxide (ZnO), titanium dioxide (TiO2), aluminum oxide (Al2O3) and boron nitride (BN) was calculated for various thickness of the films and compared. The results show that the most promising materials for use in fiber-optic Fabry-Pérot interferometer are TiO2 and ZnO, although Al2O3 is also suitable for this application.

Cost-effectiveness of enzyme replacement therapy for Fabry disease.

PubMed

Rombach, Saskia M; Hollak, Carla E M; Linthorst, Gabor E; Dijkgraaf, Marcel G W

2013-02-19

The cost-effectiveness of enzyme replacement therapy (ERT) compared to standard medical care was evaluated in the Dutch cohort of patients with Fabry disease. Cost-effectiveness analysis was performed using a life-time state-transition model. Transition probabilities, effectiveness data and costs were derived from retrospective data and prospective follow-up of the Dutch study cohort consisting of males and females aged 5-78 years. Intervention with ERT (either agalsidase alfa or agalsidase beta) was compared to the standard medical care. The main outcome measures were years without end organ damage (renal, cardiac en cerebrovascular complications), quality adjusted life years (QALYs), and costs. Over a 70 year lifetime, an untreated Fabry patient will generate 55.0 years free of end-organ damage (53.5 years in males, 56.9 years in females) and 48.6 QALYs (47.8 in males, 49.7 in females). Starting ERT in a symptomatic patient increases the number of years free of end-organ damage by 1.5 year (1.6 in males, 1.3 in females), while the number of QALYs gained increases by a similar amount (1.7 in males, 1.4 in females). The costs of ERT starting in the symptomatic stage are between €9 - €10 million (£ 7.9 - £ 8.8 million, $13.0- $14.5 million) during a patient's lifetime. Consequently, the extra costs per additional year free of end-organ damage and the extra costs per additional QALY range from €5.5 - €7.5 million (£ 4.8 - £ 6.6 million, $ 8.0 - $ 10.8 million), undiscounted. In symptomatic patients with Fabry disease, ERT has limited effect on quality of life and progression to end organ damage. The pharmaco-economic evaluation shows that this modest effectiveness drives the costs per QALY and the costs per year free of end-organ damage to millions of euros. Differentiation of patients who may benefit from ERT should be improved to enhance cost-effectiveness.

Effect of reduced agalsidase Beta dosage in fabry patients: the Australian experience.

PubMed

Ghali, Joanna; Nicholls, Kathy; Denaro, Charles; Sillence, David; Chapman, Ian; Goldblatt, Jack; Thomas, Mark; Fletcher, Janice

2012-01-01

In Australia, enzyme replacement therapy (ERT) for Fabry Disease (FD), both Agalsidase alfa (Replagal, Shire HGT) and beta (Fabrazyme, Genzyme), is funded and monitored through a specific government program. Agalsidase beta supply has been rationed by Genzyme since 2009 due to manufacturing issues. Consequently, the Australian Fabry Disease Advisory Committee has treated patients on Agalsidase beta at 50% of their usual dose from mid-2009, with a further reduction to 30% for some patients from late 2009. To determine the clinical effect of Agalsidase beta dose reduction in the Australian FD patient cohort. A questionnaire assessing FD symptoms was administered to 40 patients on long-term ERT. Clinical data from The Fabry Registry for patients receiving Agalsidase alfa or beta, for at least 2 years prior to the time of enforced Agalsidase beta dose reduction, were reviewed. Disease burden and quality of life (QOL) were graded using the Disease Severity Scoring System, Mainz Severity Score Index, Brief Pain Inventory and Short Form 36 Health Survey at 2 years before dose reduction, at the time of dose reduction and at the most recent clinical review following dose reduction. Disease severity and QOL scores did not change between the ERT groups. Males on Agalsidase beta reported lower energy levels after dose reduction, while no change was reported by females on either product or by males on a stable dose of Agalsidase alfa. This study suggests that energy levels in male patients worsen after dose reduction of Agalsidase beta.

A tunable Fabry-Perot filter (λ/18) based on all-dielectric metamaterials

NASA Astrophysics Data System (ADS)

Ao, Tianhong; Xu, Xiangdong; Gu, Yu; Jiang, Yadong; Li, Xinrong; Lian, Yuxiang; Wang, Fu

2018-05-01

A tunable Fabry-Perot filter composed of two separated all-dielectric metamaterials is proposed and numerically investigated. Different from metallic metamaterials reflectors, the all-dielectric metamaterials are constructed by high-permittivity TiO2 cylinder arrays and exhibit high reflection in a broadband of 2.49-3.08 THz. The high reflection is attributed to the first and second Mie resonances, by which the all-dielectric metamaterials can serve as reflectors in the Fabry-Perot filter. Both the results from phase analysis method and CST simulations reveal that the resonant frequency of the as-proposed filter appears at 2.78 THz, responding to a cavity with λ/18 wavelength thickness. Particularly, the resonant frequency can be adjusted by changing the cavity thickness. This work provides a feasible approach to design low-loss terahertz filters with a thin air cavity.

The effect of enzyme replacement therapy on clinical outcomes in paediatric patients with Fabry disease - A systematic literature review by a European panel of experts.

PubMed

Spada, Marco; Baron, Ralf; Elliott, Perry M; Falissard, Bruno; Hilz, Max J; Monserrat, Lorenzo; Tøndel, Camilla; Tylki-Szymańska, Anna; Wanner, Christoph; Germain, Dominique P

2018-04-26

Fabry disease is caused by a deficiency of the lysosomal enzyme α-galactosidase, resulting in progressive accumulation of globotriaosylceramide (GL-3). The disease can manifest early during childhood and adolescence. Enzyme replacement therapy (ERT) with recombinant human α-galactosidase is the first specific treatment for Fabry disease and has been available in Europe since 2001. This paper presents the findings of a systematic literature review of clinical outcomes with ERT in paediatric patients with Fabry disease. A comprehensive systematic review of published literature on ERT in Fabry disease was conducted in January 2017. The literature analysis included all original articles reporting outcomes of ERT in paediatric patients. Treatment-related outcomes in the paediatric population were reported in six publications derived from open-label clinical trials and in 10 publications derived from observational or registry-based studies. ERT was shown to significantly reduce plasma and urine GL-3 levels in paediatric patients with Fabry disease. The effect of ERT on GL-3 clearance from renal podocytes appeared to be agalsidase dose-dependent. ERT relieved pain and improved gastrointestinal symptoms and quality of life. Based on the published literature, the use of ERT in paediatric patients can significantly clear GL-3 accumulation, ameliorate the early symptoms of Fabry disease, and improve quality of life. Treatment with ERT in paediatric patients with Fabry disease may be important to prevent further disease progression and overt organ damage. Copyright © 2018. Published by Elsevier Inc.

The MPE/UCB far-infrared imaging Fabry-Perot interferometer (FIFI)

NASA Technical Reports Server (NTRS)

Poglitsch, A.; Geis, N.; Genzel, R.; Haggerty, M.; Beeman, J. W.

1991-01-01

FIFI, an imaging spectrometer with two or three Fabry-Perot interferometers in a series for astronomical observations in the FIR range, is described. Spectral resolutions of 2 km/s can be obtained with FIFI. Design considerations are discussed as well as optics, the detector array, the transimpedance amplifier array, signal demodulation, data acquisition, and instrument control.

A Cultural Resources Survey of the St. Charles Parish Hurricane Protection Levee, St. Charles Parish, Louisiana

DTIC Science & Technology

1988-09-01

Environments, Inc. kDEC 2 1198 8 1260 Main Street Baton Rouge, Louisiana 70802 H Prepared for U.S. Army Corps of Engineers New Orleans District P.O. Box 60267...Geological interpretations were developed by Charles Pearson with the assistance of Sherwood Gagliano. Figures for the report were prepared by...November 1987. Analysis of the collected data and report preparation took place in December 1987 and January 1988. Although the hurricane protection levee

Optimal Design of an Hourglass in-Fiber Air Fabry-Perot Microcavity—Towards Spectral Characteristics and Strain Sensing Technology

PubMed Central

Wang, Qi; Yan, Dongchao; Cui, Binbin; Guo, Zixuan

2017-01-01

An hourglass in-fiber air microcavity Fabry-Perot interferometer is proposed in this paper, and its second reflecting surface of in-fiber microcavity is designed to be a concave reflector with the best curvature radius in order to improve the spectral characteristics. Experimental results proved that the extinction ratio of Fabry-Perot interferometer with cavity length of 60 μm and concave reflector radius of 60 μm is higher than for a rectangular Fabry-Perot interferometer with cavity length of 60 μm (14 dB: 11 dB). Theory and numerical simulation results show that the strain sensitivity of sensor can be improved by reducing the microcavity wall thickness and microcavity diameter, and when the in-fiber microcavity length is 40 μm, the microcavity wall thickness is 10 μm, the microcavity diameter is 20 μm, and the curvature radius of reflective surface II is 50 μm, the interference fringe contrast of is greater than 0.97, an Axial-pull sensitivity of 20.46 nm/N and resolution of 1 mN can be achieved in the range of 0–1 N axial tension. The results show that the performance of hourglass in-fiber microcavity interferometer is far superior to that of the traditional Fabry-Perot interferometer. PMID:28587221

Phase-demodulation error of a fiber-optic Fabry-Perot sensor with complex reflection coefficients.

PubMed

Kilpatrick, J M; MacPherson, W N; Barton, J S; Jones, J D

2000-03-20

The influence of reflector losses attracts little discussion in standard treatments of the Fabry-Perot interferometer yet may be an important factor contributing to errors in phase-stepped demodulation of fiber optic Fabry-Perot (FFP) sensors. We describe a general transfer function for FFP sensors with complex reflection coefficients and estimate systematic phase errors that arise when the asymmetry of the reflected fringe system is neglected, as is common in the literature. The measured asymmetric response of higher-finesse metal-dielectric FFP constructions corroborates a model that predicts systematic phase errors of 0.06 rad in three-step demodulation of a low-finesse FFP sensor (R = 0.05) with internal reflector losses of 25%.

Charles Lyell and scientific thinking in geology

NASA Astrophysics Data System (ADS)

Virgili, Carmina

2007-07-01

Charles Lyell (1797-1875) was born at Kinnordy, Scotland. His father, an amateur botanist, and his grandfather, a navigator, gave him very soon a taste for the observation of the Nature. He went to the Oxford University to study classical literature, but he also followed the geological course of William Buckland. After having been employed as jurist for some years, in 1827 he decided on a career of geologist and held the chair of geology of the King's College of London, from 1831 on. He was a contemporary of Cuvier, Darwin, von Humboldt, Hutton, Lavoisier, and was elected 'membre correspondant' of the 'Académie des sciences, France', in January 1862. Charles Lyell is one of the eminent geologists who initiated the scientific thinking in geology, in which his famous volumes of the Principles of Geology were taken as the authority. These reference volumes are based on multiple observations and field works collected during numerous fieldtrips in western Europe (principally Spain, France, and Italy) and North America. To his name are attached, among others: ( i) the concept of uniformitarism (or actualism), which was opposed to the famous catastrophism, in vogue at that time, and which may be summarized by the expression "The present is the key to the past"; ( ii) the division of the Tertiary in three series denominated Eocene, Miocene, and Pliocene, due to the study of the age of strata by fossil faunas; ( iii) the theory according to which the orogenesis of a mountain chain, as the Pyrenees, results from different pulsations on very long time scales and was not induced by a unique pulsation during a short and intense period. The uniformity of the laws of Nature is undeniably a principle Charles Lyell was the first to state clearly and to apply to the study of the whole Earth's crust, which opened a new era in geology.

The Lost Acting Treatise of Charles Macklin.

ERIC Educational Resources Information Center

Mackey, Barbara

This paper examines the career of Charles Macklin of London, an 18th-century actor/director/teacher, whose treatise on his performative approach and pedagogical techniques, "On the Science of Acting," was lost at sea in a 1772 shipwreck. Citing two letters Macklin received from his actress daughter, Maria, and fragments of his own…

Charles Dickens, Social Worker in His Time

ERIC Educational Resources Information Center

Andrews, Arlene Bowers

2012-01-01

As the world marks the 200th anniversary of Charles Dickens's birth, social workers may take note of the contributions Dickens made to 19th century social reform. Ever the advocate for people who were poor and oppressed, Dickens, in his timeless fictional narratives, continues to have relevance for contemporary social justice advocacy. This…

Charles Bonnet Syndrome: A Review of the Literature

ERIC Educational Resources Information Center

O'Farrell, Lauren; Lewis, Sandra; McKenzie, Amy; Jones, Lynda

2010-01-01

Charles Bonnet syndrome (CBS) commonly occurs in older adults with visual impairments, particularly those with age-related macular degeneration. It is characterized by complex visual hallucinations in individuals without mental disorders. The authors explore diagnostic criteria, demographic characteristics, clinical features, theories of…

77 FR 23599 - Special Local Regulations for Marine Events; Potomac River, Charles County, MD

Federal Register 2010, 2011, 2012, 2013, 2014

2012-04-20

...-AA08 Special Local Regulations for Marine Events; Potomac River, Charles County, MD AGENCY: Coast Guard...; telephone 410-576-2674, email Ronald.L[email protected] . If you have questions on viewing the docket, call... ``Special Local Regulations for Marine Events; Potomac River, Charles County, MD'' in the Federal Register...

[Enzyme replacement therapy in patients with Fabry disease: state of the art and review of the literature].

PubMed

Riccio, Eleonora; Capuano, Ivana; Visciano, Bianca; Marchetiello, Cristina; Petrillo, Fortunato; Pisani, Antonio

2013-01-01

Anderson-Fabry disease is a hereditary X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha galactosidase A. It results in the accumulation of the glycosphingolypid globotrioasoyl ceramide (Gb3 in different cells and organs, resulting in a multi-system pathology including end organ failure. Patients with Fabry disease present clinically with cardiac, renal and neurological involvement; both life expectancy and quality of life are severely compromised. The current causal treatment for Fabry disease is enzyme replacement therapy (ERT), available since 2001. The two recombinant preparations available for ERT are agalsidase alfa (Replagal) and agalsidase beta (Fabrazyme). They have both been showed to have positive effect on kidney and heart, on the symptoms of pain and quality of life. Few data to date are available on comparison of the two preparations of ERT. This article reviews evidence of the literature and shows our personal experience about the safety and efficacy of ERT.

Charles L. Brewer Award for Distinguished Teaching of Psychology

ERIC Educational Resources Information Center

American Psychologist, 2007

2007-01-01

This article announces the 2007 recipient of the Charles L. Brewer Award for Distinguished Teaching of Psychology: Baron Perlman. A brief biography, highlighting areas of special focus in Perlman's work, is provided.

Treatment in Fabry disease.

PubMed

López Rodríguez, M

2018-04-13

Fabry disease is an X-linked inborn disease caused by deficit of alpha-galactosidaseA. This results in accumulation of glycosphingolipids in all cells and tissues. All males should receive enzyme replacement treatment in case of very low or undetectable levels of alpha-galactosidaseA. Female carriers and males with marginally levels of alpha-galactosidaseA should be treated in case of renal, neurologic o cardiac manifestations. There are two intravenous formulations of human recombinant enzyme, agalsidase alpha and agalsidase beta, showing similar efficacy and safety. Patients with amenable mutations of alpha-galactosidase can be treated with oral migalastat hydrochloride. Migalastat hydrochloride is a pharmacological chaperone that facilitates trafficking of alpha-galactosidaseA to lysosomes increasing enzyme activity. Patients treated with migalastat hydrochloride had significant improvements in left ventricular mass and gastrointestinal symptoms. Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

Long-Term Dose-Dependent Agalsidase Effects on Kidney Histology in Fabry Disease.

PubMed

Skrunes, Rannveig; Tøndel, Camilla; Leh, Sabine; Larsen, Kristin Kampevold; Houge, Gunnar; Davidsen, Einar Skulstad; Hollak, Carla; van Kuilenburg, André B P; Vaz, Frédéric M; Svarstad, Einar

2017-09-07

Dose-dependent clearing of podocyte globotriaosylceramide has previously been shown in patients with classic Fabry disease treated with enzyme replacement. Our study evaluates the dose-dependent effects of agalsidase therapy in serial kidney biopsies of patients treated for up to 14 years. Twenty patients with classic Fabry disease (12 men) started enzyme replacement therapy at a median age of 21 (range =7-62) years old. Agalsidase- α or - β was prescribed for a median of 9.4 (range =5-14) years. The lower fixed dose group received agalsidase 0.2 mg/kg every other week throughout the follow-up period. The higher dose group received a range of agalsidase doses (0.2-1.0 mg/kg every other week). Dose changes were made due to disease progression, suboptimal effect, or agalsidase- β shortage. Serial kidney biopsies were performed along with clinical assessment and biomarkers and scored according to recommendations from the International Study Group of Fabry Nephropathy. No statistical differences were found in baseline or final GFR or albuminuria. Kidney biopsies showed significant reduction of podocyte globotriaosylceramide in both the lower fixed dose group (-1.39 [SD=1.04]; P =0.004) and the higher dose group (-3.16 [SD=2.39]; P =0.002). Podocyte globotriaosylceramide (Gb3) reduction correlated with cumulative agalsidase dose ( r =0.69; P =0.001). Arterial/arteriolar intima Gb3 cleared significantly in the higher dose group, all seven patients with baseline intimal Gb3 cleared the intima, one patient gained intimal Gb3 inclusions ( P =0.03), and medial Gb3 did not change statistically in either group. Residual plasma globotriaosylsphingosine levels remained higher in the lower fixed dose group (20.1 nmol/L [SD=11.9]) compared with the higher dose group (10.4 nmol/L [SD=8.4]) and correlated with cumulative agalsidase dose in men ( r =0.71; P =0.01). Reduction of podocyte globotriaosylceramide was found in patients with classic Fabry disease treated with long

Chronology of Fabry-Perot Interferometer Fiber-Optic Sensors and Their Applications: A Review

PubMed Central

Islam, Md. Rajibul; Ali, Muhammad Mahmood; Lai, Man-Hong; Lim, Kok-Sing; Ahmad, Harith

2014-01-01

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. PMID:24763250

Optical power equalization for upstream traffic with injection-locked Fabry-Perot lasers in TDM-PON

NASA Astrophysics Data System (ADS)

Huang, Ting-Tsan; Sheu, Lih-Gen; Chi, Sien

2010-10-01

An optical power equalization of upstream traffic in time-division-multiplexed passive optical network (TDM-PON) based on injection-locked Fabry-Perot lasers has been experimentally investigated. The upstream transmitters with stable spectrum are achieved by using an external injection light source in the optical line terminal (OLT). The different upstream powers can be equalized by injection locking a Fabry-Perot laser diode (FP-LD) biased below threshold current in OLT. The dynamic upstream power range from - 8.5 to - 19.5 db m is reduced to a 1.6 dB maximal power variation, when the uplink signal is directly modulated at 1.25 Gb/s.

The Contentious Social Interactionism of Charles Tilly

ERIC Educational Resources Information Center

Collins, Randall

2010-01-01

Charles Tilly, who died in 2008 just before reaching the age of 79, was one of the great historical sociologists of the last half century. Along with Theda Skocpol, Michael Mann, and a few others, he carried out a revolution in people's understanding of social movements, revolutions, the rise and effects of the modern state, and social conflict or…

Charles H. Winston and Confederate Sulfuric Acid.

ERIC Educational Resources Information Center

Riethmiller, Steven

1995-01-01

Describes the invention and use of a sulfuric acid chamber by Charles Henry Winston during the Civil War. This invention helped supply munitions for the South. Winston, who was President of the Richmond Female Institute in Virginia, constructed the chamber at his farm and was granted a patent by the Confederate Patent Office in 1863. (PVD)

Official portrait of Astronaut Charles F. Bolden, Jr.

NASA Technical Reports Server (NTRS)

1986-01-01

New Official portrait of Astronaut Charles F. Bolden Jr. Bolden is in the blue shuttle flight suit with his helmet under his arm and an American flag behind him. Above and to the right of his head is a view of the shuttle flying.

Long-term outcome of enzyme-replacement therapy in advanced Fabry disease: evidence for disease progression towards serious complications

PubMed Central

Weidemann, F; Niemann, M; Störk, S; Breunig, F; Beer, M; Sommer, C; Herrmann, S; Ertl, G; Wanner, C

2013-01-01

Objective The long-term effects of enzyme-replacement therapy (ERT) in Fabry disease are unknown. Thus, the aim of this study was to determine whether ERT in patients with advanced Fabry disease affects progression towards ‘hard’ clinical end-points in comparison with the natural course of the disease. Methods A total of 40 patients with genetically proven Fabry disease (mean age 40 ± 9 years; n = 9 women) were treated prospectively with ERT for 6 years. In addition, 40 subjects from the Fabry Registry, matched for age, sex, chronic kidney disease stage and previous transient ischaemic attack (TIA), served as a comparison group. The main outcome was a composite of stroke, end-stage renal disease (ESRD) and death. Secondary outcomes included changes in myocardial left ventricular (LV) wall thickness and replacement fibrosis, change in glomerular filtration rate (GFR), new TIA and change in neuropathic pain. Results During a median follow-up of 6.0 years (bottom and top quartiles: 5.1, 7.2), 15 events occurred in 13 patients (n = 7 deaths, n = 4 cases of ESRD and n = 4 strokes). Sudden death occurred (n = 6) only in patients with documented ventricular tachycardia and myocardial replacement fibrosis. The annual progression of myocardial LV fibrosis in the entire cohort was 0.6 ± 0.7%. As a result, posterior end-diastolic wall thinning was observed (baseline, 13.2 ± 2.0 mm; follow-up, 11.4 ± 2.1 mm; P < 0.01). GFR decreased by 2.3 ± 4.6 mL min−1 per year. Three patients experienced a TIA. The major clinical symptom was neuropathic pain (n = 37), and this symptom improved in 25 patients. The event rate was not different between the ERT group and the untreated (natural history) group of the Fabry Registry. Conclusion Despite ERT, clinically meaningful events including sudden cardiac death continue to develop in patients with advanced Fabry disease. PMID:23586858

Enzyme replacement therapy with agalsidase beta in kidney transplant patients with Fabry disease: a pilot study.

PubMed

Mignani, Renzo; Panichi, Vincenzo; Giudicissi, Antonio; Taccola, Daniele; Boscaro, Francesca; Feletti, Carlo; Moneti, Gloriano; Cagnoli, Leonardo

2004-04-01

We sought to assess the safety and efficacy of enzyme replacement therapy (ERT) with recombinant human-alpha-galactosidase A (rh-alpha-Gal A) in kidney transplant recipients with Fabry disease, a previously unstudied population. Three male kidney transplant recipients with biochemically, genetically, and histologically confirmed Fabry disease and documented Fabry myocardiopathy received the rh-alpha-Gal A, agalsidase beta, 1 mg/kg of body weight every 2 weeks by intravenous infusion and were monitored biochemically, clinically, and electrocardiographically and echocardiographically for 18 months. Patients showed biochemical, clinical/functional, and morphologic response to ERT. Plasma globotriaosylceramide decreased 23% to 50%. Extremity pain resolved within 2 months in the patient with this manifestation. On echocardiography, left ventricular mass, end diastolic diameter (EDD), and cardiac contractility, shown by ejection fraction (EF), improved in 2 of the 3 patients receiving essentially all planned infusions. EDD and EF remained basically stable, but cardiac morphologic abnormalities progressed in the other patient, who had a 5-month interruption in ERT after the initial month. Mild mitral insufficiency persisted in all patients, as did atrial fibrillation in the affected individual. After a combined total of 116 infusions, no treatment-related adverse event, intolerance, or seroconversion was seen. Renal function remained stable and the immunosuppression regimen unchanged in all patients. Our pilot study provides preliminary evidence that ERT with agalsidase beta, 1 mg/kg every 2 weeks, is safe and often effective against extra-renal manifestations in kidney transplant patients with Fabry disease. Studies with longer courses of this and higher doses of ERT are merited in this population.

77 FR 52681 - Reorganization and Expansion of Foreign-Trade Zone 87 Lake Charles, LA

Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-30

... Foreign-Trade Zone 87 Lake Charles, LA Pursuant to its authority under the Foreign-Trade Zones Act of June 18, 1934, as amended (19 U.S.C. 81a-81u), the Foreign-Trade Zones Board (the Board) adopts the following Order: Whereas, the Lake Charles Harbor & Terminal District, grantee of Foreign-Trade Zone 87...

ASTRONAUT CONRAD, CHARLES - SKYLAB-II - JSC

NASA Image and Video Library

1973-05-09

S73-25283 (8 May 1973) --- Astronaut Paul J. Weitz, prime crew pilot of the first manned Skylab mission, is suited up in Bldg. 5 at Johnson Space Center during prelaunch training activity. He is assisted by astronaut Charles Conrad Jr., prime crew commander. The man in the left background is wearing a face mask to insure that Conrad, Joseph Kerwin and Weitz are not exposed to disease prior to launch. Photo credit: NASA

Ocean Connections with the Historic Whaling Ship Charles W. Morgan

NASA Astrophysics Data System (ADS)

Whitney, M. M.

2016-02-01

This scientific outreach project involved the Charles W. Morgan, Mystic Seaport's historic whaling ship. We educated K-2 students, trained undergraduate and graduate students, and informed the general public about oceanographic data collection, pathways from coastal to ocean waters, and connections in marine ecosystems. I was aboard the Charles W. Morgan for the Provincetown to Stellwagen Bank leg of the historic 38th voyage in summer 2014. While at sea, our voyager team released several GPS-tracked surface drifters to reveal important flow pathways and how the Stellwagen Bank National Marine Sanctuary is connected to other ocean areas. These drifters were built by graduate and undergraduate students and the drifter artwork was designed by elementary school students. Surface currents dispersed the drifters and carried them much farther offshore than the Charles W. Morgan itself. Many drifters reached Georges Bank, another important biologically productive area. The Charles W. Morgan encountered whales for the first time in decades. Some of the food-chain connections that may explain the abundance of whales at Stellwagen bank that summer are described. This outreach project has been presented in lectures to high school teachers and the general public and also featured in an online interview, a television news story, and a newspaper article. K-2 students at an elementary school math and science day first painted drifters in advance of the voyage, viewed real-time updates in the months following drifter release, and engaged in activities illustrating ocean connectivity and marine habitats at the end of the following academic year. We aimed to convey how sensitive whales are to human activities (on land and water) and to changes in the marine environment. Successes and lessons learned will be discussed. ED003: Creative Ways to Connect Ocean Sciences to the Public

Efficacy of Enzyme and Substrate Reduction Therapy with a Novel Antagonist of Glucosylceramide Synthase for Fabry Disease

PubMed Central

Ashe, Karen M; Budman, Eva; Bangari, Dinesh S; Siegel, Craig S; Nietupski, Jennifer B; Wang, Bing; Desnick, Robert J; Scheule, Ronald K; Leonard, John P; Cheng, Seng H; Marshall, John

2015-01-01

Fabry disease, an X-linked glycosphingolipid storage disorder, is caused by the deficient activity of α-galactosidase A (α-Gal A). This results in the lysosomal accumulation in various cell types of its glycolipid substrates, including globotriaosylceramide (GL-3) and lysoglobotriaosylceramide (globotriaosyl lysosphingolipid, lyso-GL-3), leading to kidney, heart, and cerebrovascular disease. To complement and potentially augment the current standard of care, biweekly infusions of recombinant α-Gal A, the merits of substrate reduction therapy (SRT) by selectively inhibiting glucosylceramide synthase (GCS) were examined. Here, we report the development of a novel, orally available GCS inhibitor (Genz-682452) with pharmacological and safety profiles that have potential for treating Fabry disease. Treating Fabry mice with Genz-682452 resulted in reduced tissue levels of GL-3 and lyso-GL-3 and a delayed loss of the thermal nociceptive response. Greatest improvements were realized when the therapeutic intervention was administered to younger mice before they developed overt pathology. Importantly, as the pharmacologic profiles of α-Gal A and Genz-682452 are different, treating animals with both drugs conferred the greatest efficacy. For example, because Genz-682452, but not α-Gal A, can traverse the blood–brain barrier, levels of accumulated glycosphingolipids were reduced in the brain of Genz-682452–treated but not α-Gal A–treated mice. These results suggest that combining substrate reduction and enzyme replacement may confer both complementary and additive therapeutic benefits in Fabry disease. PMID:25938659

Composite material embedded fiber-optic Fabry-Perot strain rosette

NASA Astrophysics Data System (ADS)

Valis, Thomas; Hogg, Dayle; Measures, Raymond M.

1990-12-01

A fiber-optic strain rosette is embedded in Kevlar/epoxy. The individual arms of the rosette are fiber Fabry-Perot interferometers operated in reflection-mode with gauge (i.e., cavity) lengths of approximately 5 mm. Procedures for manufacturing the cavities, and bending the fibers, to form a strain rosette are described. Experimental results showing 2D interlaminar strain-tensor measurement are presented. The sensor is also tested as a surface adhered device.

Fabry-Perot interferometer development for rocket engine plume spectroscopy

NASA Astrophysics Data System (ADS)

Bickford, R. L.; Madzsar, G.

1990-07-01

This paper describes a new rugged high-resolution Fabry-Perot interferometer (FPI) designed for rocket engine plume spectroscopy, which is capable of detecting spectral signatures of eroding engine components during rocket engine tests and/or flight operations. The FPI system will make it possible to predict and to respond to the incipient rocket engine failures and to indicate the presence of rocket components degradation. The design diagram of the FPI spectrometer is presented.

Fabry-Perot interferometer development for rocket engine plume spectroscopy

NASA Technical Reports Server (NTRS)

Bickford, R. L.; Madzsar, G.

1990-01-01

This paper describes a new rugged high-resolution Fabry-Perot interferometer (FPI) designed for rocket engine plume spectroscopy, which is capable of detecting spectral signatures of eroding engine components during rocket engine tests and/or flight operations. The FPI system will make it possible to predict and to respond to the incipient rocket engine failures and to indicate the presence of rocket components degradation. The design diagram of the FPI spectrometer is presented.

An electronically tunable, first-order Fabry-Perot infrared filter

NASA Astrophysics Data System (ADS)

Knudtson, J. T.; Levy, D. S.; Herr, K. C.

1995-04-01

A tunable infrared filter capable of scanning from 8.2 to 12.8 micrometers has been designed, constructed and tested. It is a first order Fabry Perot interferometer with piezoelectrically driven cavity spacing. Multilayer dielectric coatings for the partially transmitting mirrors were designed to minimize the wavelength dependent phase change produced by reflection. The transmission bandwidth ranged from 2.8 to 4.0% across the tuning range. Continuous scanning at 20 Hz rates was demonstrated.

Geohydrologic data for the St. Charles County well field and public-water supply, 1985-91, and projected public-water supply, 1995 and 2000,for St. Charles County, Missouri

USGS Publications Warehouse

Mugel, D.N.

1993-01-01

The St. Charles County well field consists of 8 wells that penetrate the entire thickness of the Missouri River alluvial aquifer. The wells range from 98 to 116 ft deep. The lower 40 ft of each well is screened and open to the aquifer. The specific capacities of the wells calculated soon after well completion ranged from 115 to 248 gal/min/ft of drawdown. Transmissivities range from 900 to 60,200 sq ft/day. Hydraulic conductivities range from 23 to 602 ft/day. Storage coefficients range from 0.005 to 0.2. A tracer test determined effective porosity ranging from 0.21 to 0.32. A point dilution test determined a groundwater velocity of 0.83 ft/day. From 1985-91, the average daily water supply from the St. Charles County well field and water- treatment plant increased from 5.76 to 10.23 Mgal/day, an increase from 36.2 to 42.2 percent of the total quantity of water supplied by major public-water suppliers in St. Charles County. The average daily water supply from the well field and water-treatment plant is projected to increase to 11.0 Mgal/day during 1995 and 12.2 Mgal/day during 2000. The St. Charles County Water Department's projections of peak daily demands from customers indicate that these demands will exceed the capacity of the water-treatment plant during 1995 and will exceed the capacities of both the well field and water-treatment plant during 2000.

Integration of miniature Fabry-Perot fiber optic sensor with FBG for the measurement of temperature and strain

NASA Astrophysics Data System (ADS)

Li, L.; Tong, X. L.; Zhou, C. M.; Wen, H. Q.; Lv, D. J.; Ling, K.; Wen, C. S.

2011-03-01

A sensor has been fabricated by the integration of a fiber Bragg gating sensor (FBGs) with a fiber Fabry-Perot (F-P) sensor fabricated by etching method. In the integrated sensor, the FBG was used to measure temperature, while the fiber Fabry-Perot interferometer sensor (FFPIs) was used for strain measurement. Wavelength decoding for FBG and peak tracking for FFPI was employed for demodulation, respectively. The result showed that the temperature and strain sensitivity for the integrated sensor is ~ 2.7 pm/ μɛand ~ 9.3 pm/°C, respectively.

Low-Cost, Single-Frequency Sources for Spectroscopy using Conventional Fabry-Perot Diode Lasers

NASA Technical Reports Server (NTRS)

Duerksen, Gary L.; Krainak, Michael A.

1999-01-01

Commercial (uncoated) Fabry-Perot laser diodes are converted to single-frequency spectroscopy sources by passively locking the laser frequency to the band edge of a fiber Bragg grating, which phase-locks the laser oscillations through self-injection seeding.

Low-Cost, Single-Frequency Sources for Spectroscopy Using Conventional Fabry-Perot Diode Lasers

NASA Technical Reports Server (NTRS)

Krainak, Michael A.; Duerksen, Gary L.

1999-01-01

Commercial (uncoated) Fabry-Perot laser diodes are converted to single-frequency spectroscopy sources by passively locking the laser frequency to the band edge of a fiber Bragg grating, which phase-locks the laser oscillations through self-injection seeding.

[Role of cardiac magnetic resonance in cardiac involvement of Fabry disease].

PubMed

Serra, Viviana M; Barba, Miguel Angel; Torrá, Roser; Pérez De Isla, Leopoldo; López, Mónica; Calli, Andrea; Feltes, Gisela; Torras, Joan; Valverde, Victor; Zamorano, José L

2010-09-04

Fabry disease is a hereditary disorder. Clinical manifestations are multisystemic. The majority of the patients remain undiagnosed until late in life, when alterations could be irreversible. Early detection of cardiac symptoms is of major interest in Fabry's disease (FD) in order to gain access to enzyme replacement therapy. Echo-Doppler tissular imaging (TDI) has been used as a cardiologic early marker in FD. This study is intended to determine whether the cardiac magnetic resonance is as useful tool as TDI for the early detection of cardiac affectation in FD. Echocardiography, tissue Doppler and Cardio magnetic resonance was performed in 20 patients with confirmed Fabry Disease. Left ventricular hypertrophy was defined as septum and left ventricular posterior wall thickness ≥12 mm. An abnormal TDI velocity was defined as (Sa), (Ea) and/or (Aa) velocities <8 cm/s at either the septal or lateral corner. Late phase gadolinium-enhanced images sequences were obtained using magnetic resonance. Twenty patients included in the study were divided into three groups: 1. Those without left ventricular hypertrophy nor tissue Doppler impairment 2. Those without left ventricular hypertrophy and tissue Doppler impairment 3. Those with left ventricular hypertrophy and Tissue Doppler impairment. Late gadolinium enhancement was found in only one patient, who has already altered DTI and LVH. Tissue Doppler imaging (TDI) is the only diagnostic tool able to provide early detection of cardiac affectation in patients with FD. Magnetic resonance provides information of the disease severity in patients with LVH, but can not be used as an early marker of cardiac disease in patients with FD. However MRI could be of great value for diagnostic stratification. Copyright © 2009 Elsevier España, S.L. All rights reserved.

Ten-year outcome of enzyme replacement therapy with agalsidase beta in patients with Fabry disease.

PubMed

Germain, Dominique P; Charrow, Joel; Desnick, Robert J; Guffon, Nathalie; Kempf, Judy; Lachmann, Robin H; Lemay, Roberta; Linthorst, Gabor E; Packman, Seymour; Scott, C Ronald; Waldek, Stephen; Warnock, David G; Weinreb, Neal J; Wilcox, William R

2015-05-01

Fabry disease results from deficient α-galactosidase A activity and globotriaosylceramide accumulation causing renal insufficiency, strokes, hypertrophic cardiomyopathy and early demise. We assessed the 10-year outcome of recombinant α-galactosidase A therapy. The outcomes (severe clinical events, renal function, cardiac structure) of 52/58 patients with classic Fabry disease from the phase 3 clinical trial and extension study, and the Fabry Registry were evaluated. Disease progression rates for patients with low renal involvement (LRI, n=32) or high renal involvement (HRI, n=20) at baseline were assessed. 81% of patients (42/52) did not experience any severe clinical event during the treatment interval and 94% (49/52) were alive at the end of the study period. Ten patients reported a total of 16 events. Patients classified as LRI started therapy 13 years younger than HRI (mean 25 years vs 38 years). Mean slopes for estimated glomerular filtration rate for LRI and HRI were -1.89 mL/min/1.73 m(2)/year and -6.82 mL/min/1.73 m(2)/year, respectively. Overall, the mean left ventricular posterior wall thickness and interventricular septum thickness remained unchanged and normal. Patients who initiated treatment at age ≥ 40 years exhibited significant increase in left ventricular posterior wall thickness and interventricular septum thickness. Mean plasma globotriaosylceramide normalised within 6 months. This 10-year study documents the effectiveness of agalsidase beta (1 mg/kg/2 weeks) in patients with Fabry disease. Most patients remained alive and event-free. Patients who initiated treatment at a younger age and with less kidney involvement benefited the most from therapy. Patients who initiated treatment at older ages and/or had advanced renal disease experienced disease progression. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Ten-year outcome of enzyme replacement therapy with agalsidase beta in patients with Fabry disease

PubMed Central

Germain, Dominique P; Charrow, Joel; Desnick, Robert J; Guffon, Nathalie; Kempf, Judy; Lachmann, Robin H; Lemay, Roberta; Linthorst, Gabor E; Packman, Seymour; Scott, C Ronald; Waldek, Stephen; Warnock, David G; Weinreb, Neal J; Wilcox, William R

2015-01-01

Background Fabry disease results from deficient α-galactosidase A activity and globotriaosylceramide accumulation causing renal insufficiency, strokes, hypertrophic cardiomyopathy and early demise. We assessed the 10-year outcome of recombinant α-galactosidase A therapy. Methods The outcomes (severe clinical events, renal function, cardiac structure) of 52/58 patients with classic Fabry disease from the phase 3 clinical trial and extension study, and the Fabry Registry were evaluated. Disease progression rates for patients with low renal involvement (LRI, n=32) or high renal involvement (HRI, n=20) at baseline were assessed. Results 81% of patients (42/52) did not experience any severe clinical event during the treatment interval and 94% (49/52) were alive at the end of the study period. Ten patients reported a total of 16 events. Patients classified as LRI started therapy 13 years younger than HRI (mean 25 years vs 38 years). Mean slopes for estimated glomerular filtration rate for LRI and HRI were −1.89 mL/min/1.73 m2/year and −6.82 mL/min/1.73 m2/year, respectively. Overall, the mean left ventricular posterior wall thickness and interventricular septum thickness remained unchanged and normal. Patients who initiated treatment at age ≥40 years exhibited significant increase in left ventricular posterior wall thickness and interventricular septum thickness. Mean plasma globotriaosylceramide normalised within 6 months. Conclusions This 10-year study documents the effectiveness of agalsidase beta (1 mg/kg/2 weeks) in patients with Fabry disease. Most patients remained alive and event-free. Patients who initiated treatment at a younger age and with less kidney involvement benefited the most from therapy. Patients who initiated treatment at older ages and/or had advanced renal disease experienced disease progression. PMID:25795794

On-Chip High-Finesse Fabry-Perot Microcavities for Optical Sensing and Quantum Information.

PubMed

Bitarafan, Mohammad H; DeCorby, Ray G

2017-07-31

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.

On-Chip High-Finesse Fabry-Perot Microcavities for Optical Sensing and Quantum Information

PubMed Central

Bitarafan, Mohammad H.; DeCorby, Ray G.

2017-01-01

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. PMID:28758967

The Scientific Legacy of Charles Wheatstone

NASA Astrophysics Data System (ADS)

Greenslade, Thomas B.

2017-02-01

Charles Wheatstone's name was once familiar to students because they used his eponymous bridge to measure resistances. That usage seems to be disappearing—we all have access to digital ohmmeters—but the techniques that he developed for making electrical measurements can still be used with profit. Also, his work with measuring very short time intervals and his work with musical acoustics are still with us, if in modified forms. Here are a few examples of his work.

Microwave radiometric aircraft observations of the Fabry-Perot interference fringes of an ice-water system

NASA Technical Reports Server (NTRS)

Harrington, R. F.; Swift, C. T.; Fedors, J. C.

1980-01-01

Airborne stepped-frequency microwave radiometer (SFMR) observations of the Fabry-Perot interference fringes of ice-water systems are discussed. The microwave emissivity at normal incidence of a smooth layered dielectric medium over a semi-infinite dielectric medium is examined for the case of ice over water as a function of ice thickness and attenuation coefficient, and the presence of quarter-wavelength oscillations in emissivity as the ice thickness and frequency are varied is pointed out. Experimental observations of pronounced quarter-wavelength oscillations in radiometric brightness temperature due to the Fabry-Perot interference fringes over smooth sea ice and lake ice varying in roughness as the radiometer frequencies were scanned are then presented.

Did Jean François Barbe Anticipate Charles Darwin?

PubMed

Drouin, Anne-Sophie; Drouin, Emmanuel; Pereon, Yann

2016-12-01

The publication of Charles Darwin's On the Origin of Species in 1859 is widely suppose to have initiated a revolution in science. In 1837, he broke with dogmatic fixism and argued that the adaptation of populations to their local environment was the cause of transmutation. Some contributors helped him start his reasoning: he indeed expressed his indebtedness to Samuel Rowley for having called his attention to Charles Wells' notions of natural selection. Darwin was certainly not the first to suggest the idea of evolution as an alternative to the creation of species by God. We report on a medical thesis published in 1837 being concluded by an unexpected and important statement related to the appearance of mammals on Earth. It remained unknown but it constitutes a link between the transformative thought of Lamarck and Geoffroy Saint Hilaire, and Darwin's work.

Microscopie par rayons X dans la fenêtre de l'eau : faisabilité et intérêt pour la biologie d'un instrument de laboratoire

NASA Astrophysics Data System (ADS)

Adam, J. F.; Moy, J. P.

2005-06-01

La biologie étudie des structures ou des phénomènes sub-cellulaires. Pour cela la microscopie est la technique d'observation privilégiée. La résolution spatiale de la microscopie optique s'avère bien souvent insuffisante pour de telles observations. Les techniques plus résolvantes, comme la microscopie électronique par transmission sont souvent destructrices et d'une complexité peu adaptée aux besoins des biologistes. La microscopie par rayons X dans la fenêtre de l'eau permet l'imagerie rapide de cellules dans leur milieu naturel, nécessite peu de préparation et offre des résolutions de quelques dizaines de nanomètres. De plus, il existe un bon contraste naturel entre les structures carbonées (protéines, lipides) et l'eau. Actuellement cette technique est limitée aux centres de rayonnement synchrotron, ce qui impose une planification et des déplacements incompatibles avec les besoins de la biologie. Un tel microscope fonctionnant avec uns source de laboratoire serait d'une grande utilité. Ce document présente un état de l'art de la microscopie par rayons X dans la fenêtre de l'eau. Un cahier des charges détaillé pour un appareil de laboratoire ayant les performances optiques requises par les biologistes est présenté et confronté aux microscopes X de laboratoire déjà existants. Des solutions concernant la source et les optiques sont également discutées.

Rapid Immunochromatographic Detection of Serum Anti-α-Galactosidase A Antibodies in Fabry Patients after Enzyme Replacement Therapy.

PubMed

Nakano, Sachie; Tsukimura, Takahiro; Togawa, Tadayasu; Ohashi, Toya; Kobayashi, Masahisa; Takayama, Katsuyoshi; Kobayashi, Yukuharu; Abiko, Hiroshi; Satou, Masatsugu; Nakahata, Tohru; Warnock, David G; Sakuraba, Hitoshi; Shibasaki, Futoshi

2015-01-01

We developed an immunochromatography-based assay for detecting antibodies against recombinant α-galactosidase A proteins in serum. The evaluation of 29 serum samples from Fabry patients, who had received enzyme replacement therapy with agalsidase alpha and/or agalsidase beta, was performed by means of this assay method, and the results clearly revealed that the patients exhibited the same level of antibodies against both agalsidase alpha and agalsidase beta, regardless of the species of recombinant α-galactosidase A used for enzyme replacement therapy. A conventional enzyme-linked immunosorbent assay supported the results. Considering these, enzyme replacement therapy with agalsidase alpha or agalsidase beta would generate antibodies against the common epitopes in both agalsidase alpha and agalsidase beta. Most of the patients who showed immunopositive reaction exhibited classic Fabry phenotype and harbored gene mutations affecting biosynthesis of α-galactosidase A. As immunochromatography is a handy and simple assay system which can be available at bedside, this assay method would be extremely useful for quick evaluation or first screening of serum antibodies against agalsidase alpha or agalsidase beta in Fabry disease with enzyme replacement therapy.

Phase-sensitive reflection technique for characterization of a fabry-perot interferometer.

PubMed

Slagmolen, B J; Gray, M B; Baigent, K G; McClelland, D E

2000-07-20

Using a radio frequency coherent modulation and demodulation technique, we explicitly measure both the amplitude and the phase response of Fabry-Perot interferometers in reflection. This allows us to differentiate clearly between overcoupled and undercoupled cavities and allows a detailed measurement of the full width at half-maximum, the free spectral range, and the finesse of the cavities.

Metrology of semiconductor structures using novel Fabry Perot fringe stretching system

NASA Astrophysics Data System (ADS)

Walecki, Wojtek J.; Pravdivtsev, Alexander

2017-08-01

We describe patent pending fiber optic apparatus for measurements of thicknesses and distance employing low resolution spectrometer and etalon. The application of an additional known reference etalon "stretches fringes" and allows us to use Fabry Perot interference to investigate thick samples and large distances which would not be possible when using the low resolution spectrometer alone.

The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat.

PubMed

Benjamin, Elfrida R; Della Valle, Maria Cecilia; Wu, Xiaoyang; Katz, Evan; Pruthi, Farhana; Bond, Sarah; Bronfin, Benjamin; Williams, Hadis; Yu, Julie; Bichet, Daniel G; Germain, Dominique P; Giugliani, Roberto; Hughes, Derralynn; Schiffmann, Raphael; Wilcox, William R; Desnick, Robert J; Kirk, John; Barth, Jay; Barlow, Carrolee; Valenzano, Kenneth J; Castelli, Jeff; Lockhart, David J

2017-04-01

Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene. Migalastat, a pharmacological chaperone, binds to specific mutant forms of α-galactosidase A to restore lysosomal activity. A pharmacogenetic assay was used to identify the α-galactosidase A mutant forms amenable to migalastat. Six hundred Fabry disease-causing mutations were expressed in HEK-293 (HEK) cells; increases in α-galactosidase A activity were measured by a good laboratory practice (GLP)-validated assay (GLP HEK/Migalastat Amenability Assay). The predictive value of the assay was assessed based on pharmacodynamic responses to migalastat in phase II and III clinical studies. Comparison of the GLP HEK assay results in in vivo white blood cell α-galactosidase A responses to migalastat in male patients showed high sensitivity, specificity, and positive and negative predictive values (≥0.875). GLP HEK assay results were also predictive of decreases in kidney globotriaosylceramide in males and plasma globotriaosylsphingosine in males and females. The clinical study subset of amenable mutations (n = 51) was representative of all 268 amenable mutations identified by the GLP HEK assay. The GLP HEK assay is a clinically validated method of identifying male and female Fabry patients for treatment with migalastat.Genet Med 19 4, 430-438.

First results from SAM-FP: Fabry-Perot observations with ground-layer adaptive optics - the structure and kinematics of the core of 30 Doradus

NASA Astrophysics Data System (ADS)

Mendes de Oliveira, C.; Amram, P.; Quint, Bruno C.; Torres-Flores, S.; Barbá, R.; Andrade, D.

2017-08-01

The aim of this paper is to present the first data set obtained with SOAR Adaptive Module-Fabry-Parot (SAM-FP), a Fabry-Perot instrument mounted inside the SOAR telescope Adaptive-Optics Module. This is the only existing imaging Fabry-Perot interferometer using laser-assisted ground-layer adaptive optics. SAM-FP was used to observe the ionized gas, traced by Hα, in the centre of the 30 Doradus starburst (the Tarantula Nebula) in the Large Magellanic Cloud, with high spatial (˜0.6 arcsec, or 0.15 pc) and spectral (R ≃ 11 200) resolution. Radial velocity, velocity dispersion and monochromatic maps were derived. The region displays a mix of narrow, σ ˜ 20 km s-1 profiles and multiple broader profiles with σ ˜ 70-80 km s-1, indicating the complex nature of the nebula kinematics. A comparison with previously obtained VLT/FLAMES spectroscopy demonstrates that the data agree well in the regions of overlap, but the Fabry-Perot data are superior in spatial coverage. A preliminary analysis of the observations finds a new expanding bubble south of R136, with a projected radius of r = 5.6 pc and an expansion velocity of 29 ± 4 km s-1. In addition, the first-time detailed kinematic maps derived here for several complexes and filaments of 30 Doradus allow identification of kinematically independent structures. These data exemplify the power of the combination of a high-order Fabry-Perot with a wide-field imager (3 × 3 arcmin2 GLAO-corrected field of view) for high-resolution spatial and spectral studies. In particular, SAM-FP data cubes are highly advantageous over multifibre or long-slit data sets for nebula structure studies and to search for small-scale bubbles, given their greatly improved spatial coverage. For reference, this paper also presents two appendices with detailed descriptions of the usage of Fabry-Perot devices, including formulae and explanations for understanding Fabry-Perot observations.

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

PubMed

Smid, Bouwien E; Rombach, Saskia M; Aerts, Johannes M F G; Kuiper, Symen; Mirzaian, Mina; Overkleeft, Hermen S; Poorthuis, Ben J H M; Hollak, Carla E M; Groener, Johanna E M; Linthorst, Gabor E

2011-10-31

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. 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. 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. No increase in clinical event incidence was found in the adult Dutch Fabry cohort during the agalsidase beta shortage. Increases in lysoGb3, however, suggest

Fabry-Perot confocal resonator optical associative memory

NASA Astrophysics Data System (ADS)

Burns, Thomas J.; Rogers, Steven K.; Vogel, George A.

1993-03-01

A unique optical associative memory architecture is presented that combines the optical processing environment of a Fabry-Perot confocal resonator with the dynamic storage and recall properties of volume holograms. The confocal resonator reduces the size and complexity of previous associative memory architectures by folding a large number of discrete optical components into an integrated, compact optical processing environment. Experimental results demonstrate the system is capable of recalling a complete object from memory when presented with partial information about the object. A Fourier optics model of the system's operation shows it implements a spatially continuous version of a discrete, binary Hopfield neural network associative memory.

Fabry-Perot enhanced Faraday rotation in graphene.

PubMed

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.

A fiber-Bragg-grating sensor interrogation system using in-fiber Fabry-Pérot interferometer

NASA Astrophysics Data System (ADS)

Wang, Ting-ting; Wang, Ming

2011-11-01

A fiber-Bragg-grating sensor interrogation system using a in-fiber Fabry-Pérot interferometer (IFFPI) is presented. The IFFPI was formed by splicing together a conventional single-mode fiber and a photonic crystal fiber with simple arcdischarge technique. The ellipsoidal air-cavity between the two fibers forms Fabry-Pérot cavity. The diffraction loss can be very low due to the focusing of reentrant and very short cavity length, thus resulting in high visibility and long period. The IFFPI is used as the filter component of the interrogation system. The resolving wavelength can achieve 2pm by using an Er-doped ring FBG laser in the experimental system. The advantages of this system are an all-fiber design, temperature insensitivity, quasistatic and dynamic operation, potential high speed and large range demodulation.

The low coherence Fabry-Pérot interferometer with diamond and ZnO layers

NASA Astrophysics Data System (ADS)

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.

Weakly modulated silicon-dioxide-cladding gratings for silicon waveguide Fabry-Pérot cavities.

PubMed

Grote, Richard R; Driscoll, Jeffrey B; Biris, Claudiu G; Panoiu, Nicolae C; Osgood, Richard M

2011-12-19

We show by theory and experiment that silicon-dioxide-cladding gratings for Fabry-Pérot cavities on silicon-on-insulator channel ("wire") waveguides provide a low-refractive-index perturbation, which is required for several important integrated photonics components. The underlying refractive index perturbation of these gratings is significantly weaker than that of analogous silicon gratings, leading to finer control of the coupling coefficient κ. Our Fabry-Pérot cavities are designed using the transfer-matrix method (TMM) in conjunction with the finite element method (FEM) for calculating the effective index of each waveguide section. Device parameters such as coupling coefficient, κ, Bragg mirror stop band, Bragg mirror reflectivity, and quality factor Q are examined via TMM modeling. Devices are fabricated with representative values of distributed Bragg reflector lengths, cavity lengths, and propagation losses. The measured transmission spectra show excellent agreement with the FEM/TMM calculations.

2. Historic American Buildings Survey Charles E. Peterson, Photographer July ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

2. Historic American Buildings Survey Charles E. Peterson, Photographer July 29, 1940. VIEW FROM SOUTH - Joseph R. Brown House, Sam Brown Memorial Park (moved from Dakota Territory), Browns Valley, Traverse County, MN

4. Historic American Buildings Survey Charles E. Peterson, Photographer July ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

4. Historic American Buildings Survey Charles E. Peterson, Photographer July 29, 1940 VIEW FROM NORTHEAST - Joseph R. Brown House, Sam Brown Memorial Park (moved from Dakota Territory), Browns Valley, Traverse County, MN

Astronaut Charles Conrad during visual acuity experiments over Laredo

NASA Technical Reports Server (NTRS)

1965-01-01

Astronaut Charles Conrad Jr., pilot for the prime crew on the Gemini 5 space flight, takes pictures of predetermined land areas during visual acuity experiments over Laredo, Texas. The experiments will aid in learning to identify known terrestrial features under controlled conditions.

Perfect Undetectable Acoustic Device from Fabry-Pérot Resonances

NASA Astrophysics Data System (ADS)

Chen, Huanyang; Zhou, Yangyang; Zhou, Mengying; Xu, Lin; Liu, Qing Huo

2018-02-01

Transformation acoustics is a method to design novel acoustic devices, while the complexity of the material parameters hinders its progress. In this paper, we analytically present a three-dimensional perfect undetectable acoustic device from Fabry-Pérot resonances and confirm its functionality from Mie theory. Such a mechanism goes beyond the traditional transformation acoustics. In addition, such a reduced version can be realized by holey-structured metamaterials. Our theory paves a way to the implementation of three-dimensional transformation acoustic devices.

Education and Utopia: Robert Owen and Charles Fourier

ERIC Educational Resources Information Center

Leopold, David

2011-01-01

The aims of education, and the appropriate means of realising them, are a recurring preoccupation of utopian authors. The utopian socialists Robert Owen (1771-1858) and Charles Fourier (1772-1837) both place human nature at the core of their educational views, and both see education as central to their wider objective of social and political…

[Medical services at Paris-Charles-de-Gaulle airport].

PubMed

Bargain, Philippe

2015-01-01

Charles-de-Gaulle airport in Roissy, a 3 400 hectare citadel, contains a multitude of airlines, service companies, businesses, retailers and public services, including firefighters, police officers, customs officers, ministers and medical teams. This article presents its missions, notably with regard to health services. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

24. View looking north up corridor from Charles Street Bridge. ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

24. View looking north up corridor from Charles Street Bridge. Providence, Providence Co., RI. Sec. 4116, mp 186.44. - Northeast Railroad Corridor, Amtrak route between CT & MA state lines, Providence, Providence County, RI

3. Historic American Buildings Survey Charles E. Peterson, Photographer July ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

3. Historic American Buildings Survey Charles E. Peterson, Photographer July 29, 1940. VIEW FROM THE WEST - Joseph R. Brown House, Sam Brown Memorial Park (moved from Dakota Territory), Browns Valley, Traverse County, MN

9. Photocopy of Charles H. Parkers Patent (Original in possession ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

9. Photocopy of Charles H. Parkers Patent (Original in possession of United States Patent Office) PATENT DRAWING OF C. H. PARKER TRUSS BRIDGE - Elm Street Bridge, Spanning Ottauquechee River, Woodstock, Windsor County, VT

Soils maps supplement to soil moisture ground truth, Lafayette, Indiana, site St. Charles, Missouri, site

NASA Technical Reports Server (NTRS)

Jones, E. B.; Olt, S. E.

1975-01-01

A compilation of soils information obtained as the result of a library search of data on the Lafayette, Indiana, site and St. Charles, Missouri, site is presented. Soils data for the Lafayette, Indiana, site are shown in Plates 1 and 2; and soils data for the St. Charles, Missouri, site are shown in Plates 3 and 4.

Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation.

PubMed

Abe, A; Gregory, S; Lee, L; Killen, P D; Brady, R O; Kulkarni, A; Shayman, J A

2000-06-01

We used a potent inhibitor of glucosylceramide synthase to test whether substrate deprivation could lower globotriaosylceramide levels in alpha-galactosidase A (alpha-gal A) knockout mice, a model of Fabry disease. C57BL/6 mice treated twice daily for 3 days with D-threo-1-ethylendioxyphenyl-2-palmitoylamino-3-pyrrolidi no-propanol (D-t-EtDO-P4) showed a concentration-dependent decrement in glucosylceramide levels in kidney, liver, and spleen. A single intraperitoneal injection of D-t-EtDO-P4 resulted in a 55% reduction in renal glucosylceramide, consistent with rapid renal glucosylceramide metabolism. A concentration-dependent decrement in renal and hepatic globotriaosylceramide levels was observed in alpha-Gal A(-) males treated for 4 weeks with D-t-EtDO-P4. When 8-week-old alpha-Gal A(-) males were treated for 8 weeks with 10 mg/kg twice daily, renal globotriaosylceramide fell to below starting levels, consistent with an alpha-galactosidase A-independent salvage pathway for globotriaosylceramide degradation. Complications observed with another glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin, including weight loss and acellularity of lymphatic organs, were not observed with D-t-EtDO-P4. These data suggest that Fabry disease may be amenable to substrate deprivation therapy.

Genetic polymorphisms of vitamin D receptor (VDR) in Fabry disease.

PubMed

Teitcher, Michael; Weinerman, Sarah; Whybra, Catharina; Beck, Michael; Sharon, Nir; Elstein, Deborah; Altarescu, Gheona

2008-11-01

Fabry disease, an X-linked inborn error of metabolism, is characterized by multi-organ involvement including cardiac signs of left ventricular hypertrophy and abnormal intima-medial (IMT) thickening of arteries, progressive renal failure, neurological involvement, and more. The vitamin D receptor (VDR) and an enzyme producing vitamin D3 result in an autocrine loop with direct effects on blood vessels. The purpose of this study is to assess VDR polymorphisms (BsmI, FokI, ApaI, and TaqI) relative to clinically important disease parameters using a disease-specific severity score (MSSI) and haplotype analysis. There were statistically significant differences between females (43% of 74 patients) and males in MSSI total scores, and in general and neurologic sub-scores. There appears to be a protective effect of the TaqI tt genotype so that there were significantly lower scores in clinical categories between those with the tt genotype versus those with the TT genotype. Multivariate models of haplotypes with MSSI scores reveal that T-A-f-B and t-a-F-b haplotypes of the VDR gene polymorphisms are significantly associated with variation in the Fabry phenotype. Despite the limitations of using the MSSI score as a clinical correlate, these results are provocative and further studies in larger cohorts with more males are recommended.

1. Historic American Buildings Survey Charles E. Peterson, Photographer Copied ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

1. Historic American Buildings Survey Charles E. Peterson, Photographer Copied July 29, 1940 Copied from old photograph - Joseph R. Brown House, Sam Brown Memorial Park (moved from Dakota Territory), Browns Valley, Traverse County, MN

The beneficial effects of long-term enzyme replacement therapy on cardiac involvement in Japanese Fabry patients.

PubMed

Hongo, Kenichi; Ito, Keiichi; Date, Taro; Anan, Ikuko; Inoue, Yasunori; Morimoto, Satoshi; Ogawa, Kazuo; Kawai, Makoto; Kobayashi, Hiroshi; Kobayashi, Masahisa; Ida, Hiroyuki; Ohashi, Toya; Taniguchi, Ikuo; Yoshimura, Michihiro; Eto, Yoshikatsu

2018-06-01

Fabry disease is a hereditary disorder that occurs due to the reduction or absence of alpha-galactosidase A activity, which leads to cardiac involvement including left ventricular hypertrophy (LVH). Enzyme replacement therapy (ERT) provides better patient outcomes by preventing serious complications. However, there have been very few studies on the long-term effects of ERT on the cardiac manifestations in Japanese Fabry patients. We retrospectively analyzed the data from the medical records of 42 Fabry patients (male, n = 17; female, n = 25) who were followed at Jikei University Hospital, and in whom the long-term effects of ERT could be evaluated (median follow-up period: male, 11 years; female, 8 years). The slope of the left ventricular mass (LVM) increase was 3.02 ± 3.41 g/m 2 /year in males and 1.69 ± 2.73 g/m 2 /year in females. In a subgroup analysis, the slopes of males with and without LVH did not differ to a statistically significant extent; however, the slope in female patients without LVH was significantly smaller than that of female patients with LVH. We then compared our data to the natural historical data that have previously been reported. In comparison to the previously reported data, we found a significant reduction in the LVM changes (g/height 2.7 /year) of patients who received long-term ERT (male, 4.07 ± 1.03 to 1.25 ± 1.39; female, 2.31 ± 0.81 to 0.78 ± 1.23). Long-term ERT effectively prevents LVH in Fabry patients. This effect was also observed in the patients with LVH prior to the initiation of ERT. Copyright © 2018 Elsevier Inc. All rights reserved.

Charles A. Triplehorn: an inordinate fondness for Darkling Beetles

USDA-ARS?s Scientific Manuscript database

Charles A. Triplehorn is an American entomologist, former President of the Entomological Society of America and the Coleopterists Society. He is recognized as a foremost world authority on Tenebrionidae and the present monograph on Tenebrionids and related Coleoptera is dedicated to him. In this int...

Multiple Voices in Charles Van Riper's Desensitization Therapy

ERIC Educational Resources Information Center

Leahy, Margaret M.

2008-01-01

Background: Charles Van Riper (1905-94) author, researcher and practitioner, was one of the major contributors to the field of stuttering in the 20th century. His series of Action Therapy videotapes provide a useful model of how therapy was implemented by a master clinician. Aims: Van Riper's session with a client is analysed to reveal how voices…

HEADSTONE OF MEDAL OF HONOR RECIPIENT CHARLES L. RUSSELL, SECTION ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

HEADSTONE OF MEDAL OF HONOR RECIPIENT CHARLES L. RUSSELL, SECTION 3-E, GRAVE R1. VIEW TO NORTH. - Hot Springs National Cemetery, Virginia Medical Center 500 North Fifth Street, Hot Springs, Fall River County, SD

A Fabry-Pérot electro-optic sensing system using a drive-current-tuned wavelength laser diode.

PubMed

Kuo, Wen-Kai; Wu, Pei-Yu; Lee, Chang-Ching

2010-05-01

A Fabry-Pérot enhanced electro-optic sensing system that utilizes a drive-current-tuned wavelength laser diode is presented. An electro-optic prober made of LiNbO(3) crystal with an asymmetric Fabry-Pérot cavity is used in this system. To lock the wavelength of the laser diode at resonant condition, a closed-loop power control scheme is proposed. Experiment results show that the system can keep the electro-optic prober at high sensitivity for a long working time when the closed-loop control function is on. If this function is off, the sensitivity may be fluctuated and only one-third of the best level in the worst case.

A modified cross-correlation method for white-light optical fiber extrinsic Fabry-Perot interferometric hydrogen sensors

NASA Astrophysics Data System (ADS)

Yang, Zhen; Zhang, Min; Liao, Yanbiao; Lai, Shurong; Tian, Qian; Li, Qisheng; Zhang, Yi; Zhuang, Zhi

2009-11-01

An extrinsic Fabry-Perot interferometric (EFPI) optical fiber hydrogen sensor based on palladium silver (Pd-Ag) film is designed for hydrogen leakage detection. A modified cross correlation signal processing method for an optical fiber EFPI hydrogen sensor is presented. As the applying of a special correlating factor which advises the effect on the fringe visibility of the gap length and wavelength, the cross correlation method has a high accuracy which is insensitive to light source power drift or changes in attenuation in the fiber, and the segment search method is employed to reduce computation and demodulating speed is fast. The Fabry-Perot gap length resolution of better than 0.2nm is achieved in a certain concentration of hydrogen.

Frequency of unrecognized Fabry disease among young European-American and African-American men with first ischemic stroke.

PubMed

Wozniak, Marcella A; Kittner, Steven J; Tuhrim, Stanley; Cole, John W; Stern, Barney; Dobbins, Mark; Grace, Marie E; Nazarenko, Irina; Dobrovolny, Robert; McDade, Eric; Desnick, Robert J

2010-01-01

The cause of initial ischemic stroke in up to 30% of young patients remains unclear. Fabry disease, due to deficient alpha-galactosidase A (alpha-Gal A) activity, is a vascular endothelial glycosphingolipid storage disease typically presenting in childhood. With advancing age, patients develop renal, cardiac, and cerebrovascular disease and die prematurely. A European study suggested an increased prevalence of unrecognized Fabry disease in patients with cryptogenic stroke. We hypothesized that alpha-Gal A deficiency is a rare cause of initial early-onset ischemic stroke in men. The Stroke Prevention in Young Men Study enrolled >550 men (15 to 49 years) with first ischemic stroke in the Baltimore-Washington area in 2004 to 2007. Frozen plasma samples were assayed for alpha-Gal A activity, and DNA from patients with consistently low plasma alpha-Gal A activities were sequenced. The study sample consisted of 558 men (42% African-American; median age 44 years). Stroke was cryptogenic in 154 men (40% African-American). In 10 patients with low plasma alpha-Gal A activities, DNA sequencing identified alterations in the alpha-Gal A gene in 2 patients. The polymorphism, D313Y, which results in low plasma enzyme activity, but near normal levels of cellular activity was seen in one European-American male. The Fabry disease-causing A143T mutation was seen in an African-American male with cryptogenic stroke (0.18% of all strokes: upper 95% CI=0.53%; 0.65% of cryptogenic strokes: upper 95% CI=1.92%). In this biracial population, unrecognized Fabry disease is a rare but treatable cause of initial ischemic stroke in young men.

Clinical benefit in Fabry patients given enzyme replacement therapy--a case series.

PubMed

Guffon, N; Fouilhoux, A

2004-01-01

Fabry disease is a rare lysosomal storage disorder resulting from deficient activity of alpha-galactosidase A and subsequent pathological accumulation of glycosphingolipids throughout the body. Traditionally, Fabry disease was managed symptomatically, but the introduction of enzyme replacement therapies (ERTs) (agalsidase beta (Fabrazyme); agalsidase alfa (Replagal)) has transformed treatment of this disorder. Clinical studies of both compounds have demonstrated clearance of glycosphingolipds from key tissues. To explore whether substrate clearance translates into clinical benefit, a retrospective survey of 17 patients (mean age 34.7 years) treated with agalsidase beta (1 mg/kg every 2 weeks) was undertaken, using an eight-item retrospective questionnaire developed specifically to assess the effect of ERT on the symptoms of Fabry disease. Pain severity, heat tolerance, physical activity, fatigue and psychological status were scored using a 10-point visual analogue scale (e.g. for pain severity: 1=none, 10=strong). Answers to all other questions were quantitative. Changes in mean scores were 4.69 to 2.25 (p =0.012) for pain severity; 4.38 to 2.21 (p =0.019) for number of pain crises per month; 8.69 to 2.98 (p =0.097) for duration of pain crises in hours; 2.76 to 5.76 (p =0.002) for heat tolerance; 3.28 to 2.51 (p =0.058) for bowel movements per day; 2.47 to 4.47 (p =0.007) for frequency of physical activity; 5.53 to 3.71 (p =0.046) for fatigue, and 5.82 to 8.12 (p =0.005) for psychological status. All patients improved in at least one aspect, although the degree of improvement across patients and aspects varied widely; reasons for this remain unclear. Despite the inherent bias involved in retrospective questionnaires, we believe that the findings are encouraging. A prospective version of the questionnaire is currently under validation.

An Analysis of Environmental Issues in 19th Century England Using the Writings of Charles Dickens

ERIC Educational Resources Information Center

MacKenzie, Ann Haley

2008-01-01

Charles Dickens lived during the best and worst of times in 19th century England. His writings were greatly influenced by the ongoing industrial revolution. He described abhorrent environmental conditions, inadequate sanitary practices, child abuse, and other social maladies of the times. By bringing Charles Dickens into the biology classroom,…

Rapid Immunochromatographic Detection of Serum Anti-α-Galactosidase A Antibodies in Fabry Patients after Enzyme Replacement Therapy

PubMed Central

Nakano, Sachie; Tsukimura, Takahiro; Togawa, Tadayasu; Ohashi, Toya; Kobayashi, Masahisa; Takayama, Katsuyoshi; Kobayashi, Yukuharu; Abiko, Hiroshi; Satou, Masatsugu; Nakahata, Tohru; Warnock, David G.; Sakuraba, Hitoshi; Shibasaki, Futoshi

2015-01-01

We developed an immunochromatography-based assay for detecting antibodies against recombinant α-galactosidase A proteins in serum. The evaluation of 29 serum samples from Fabry patients, who had received enzyme replacement therapy with agalsidase alpha and/or agalsidase beta, was performed by means of this assay method, and the results clearly revealed that the patients exhibited the same level of antibodies against both agalsidase alpha and agalsidase beta, regardless of the species of recombinant α-galactosidase A used for enzyme replacement therapy. A conventional enzyme-linked immunosorbent assay supported the results. Considering these, enzyme replacement therapy with agalsidase alpha or agalsidase beta would generate antibodies against the common epitopes in both agalsidase alpha and agalsidase beta. Most of the patients who showed immunopositive reaction exhibited classic Fabry phenotype and harbored gene mutations affecting biosynthesis of α-galactosidase A. As immunochromatography is a handy and simple assay system which can be available at bedside, this assay method would be extremely useful for quick evaluation or first screening of serum antibodies against agalsidase alpha or agalsidase beta in Fabry disease with enzyme replacement therapy. PMID:26083343

14. Charles Acey Cobb standing adjacent to the fish screen ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

14. Charles Acey Cobb standing adjacent to the fish screen he designed and installed in the Congdon Canal, facing southeast. Photo dates ca. late 1920's. - Congdon Canal, Fish Screen, Naches River, Yakima, Yakima County, WA

10. A westward view across Charles Street of the eastern ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

10. A westward view across Charles Street of the eastern exterior wall of buildings #6-A, #6, and #6-B.(left to right). - American Chain & Cable Company, East Princess Street (400 Block), York, York County, PA

Fabry-Pérot Interference in Gapped Bilayer Graphene with Broken Anti-Klein Tunneling

NASA Astrophysics Data System (ADS)

Varlet, Anastasia; Liu, Ming-Hao; Krueckl, Viktor; Bischoff, Dominik; Simonet, Pauline; Watanabe, Kenji; Taniguchi, Takashi; Richter, Klaus; Ensslin, Klaus; Ihn, Thomas

2014-09-01

We report the experimental observation of Fabry-Pérot interference in the conductance of a gate-defined cavity in a dual-gated bilayer graphene device. The high quality of the bilayer graphene flake, combined with the device's electrical robustness provided by the encapsulation between two hexagonal boron nitride layers, allows us to observe ballistic phase-coherent transport through a 1-μm-long cavity. We confirm the origin of the observed interference pattern by comparing to tight-binding calculations accounting for the gate-tunable band gap. The good agreement between experiment and theory, free of tuning parameters, further verifies that a gap opens in our device. The gap is shown to destroy the perfect reflection for electrons traversing the barrier with normal incidence (anti-Klein tunneling). The broken anti-Klein tunneling implies that the Berry phase, which is found to vary with the gate voltages, is always involved in the Fabry-Pérot oscillations regardless of the magnetic field, in sharp contrast with single-layer graphene.

A novel mutation of α-galactosidase A gene causes Fabry disease mimicking primary erythromelalgia in a Chinese family.

PubMed

Ge, Wei; Wei, Bin; Zhu, Hao; Miao, Zhigang; Zhang, Weimin; Leng, Cuihua; Li, Jizhen; Zhang, Dan; Sun, Miao; Xu, Xingshun

2017-05-01

Fabry disease is an X-linked genetic disorder caused by the mutations of α-galactosidase A (GLA, MIM 300644) gene presenting with various clinical symptoms including small-fiber peripheral neuropathy and limb burning pain. Here, we reported a Chinese pedigree with the initial diagnosis of primary erythromelalgia in an autosomal dominant (AD)-inherited pattern. Mutation analysis of SCN9A and GLA genes by direct sequencing and functional analysis of a novel mutation of GLA in cells were performed. Our data did not show any pathological mutations in SCN9A gene; however, a novel missense mutation c.139T>C (p.W47R) of GLA was identified in a male proband as well as two female carriers in this family. Enzyme assay of α-galactosidase A activity showed deficient enzyme activity in male patients and female carriers, further confirming the diagnosis of Fabry disease. Finally, a functional analysis indicated that the replacement of the 47th amino acid tryptophan (W47) with arginine (W47R) or glycine (W47G) led to reduced activity of α-galactosidase A in 293T cells. Therefore, these findings demonstrated that the novel mutation p.W47R of GLA is the cause of Fabry disease. Because Fabry disease and primary erythromelalgia share similar symptoms, it is a good strategy for clinical physicians to perform genetic mutation screenings on both SCN9A and GLA genes in those patients with limb burning pain but without a clear inheritant pattern.

Transfer functions of double- and multiple-cavity Fabry-Perot filters driven by Lorentzian sources.

PubMed

Marti, J; Capmany, J

1996-12-20

We derive expressions for the transfer functions of double- and multiple-cavity Fabry-Perot filters driven by laser sources with Lorentzian spectrum. These are of interest because of their applications in sensing and channel filtering in optical frequency-division multiplexing networks.

Transfer functions of double- and multiple-cavity Fabry Perot filters driven by Lorentzian sources

NASA Astrophysics Data System (ADS)

Marti, Javier; Capmany, Jose

1996-12-01

We derive expressions for the transfer functions of double- and multiple-cavity Fabry Perot filters driven by laser sources with Lorentzian spectrum. These are of interest because of their applications in sensing and channel filtering in optical frequency-division multiplexing networks.

The Grand Strategy of Charles de Gaulle

DTIC Science & Technology

1989-09-08

4. TITLE AND SUBTITLE The Grand Startegy of Charles de Gaulle 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S...dependent on French influence within NATO and the Common Market . De Gaulle frequently used these fora to veto British and American initiatives. As Cook...the reserve currency. Gold would be the basis of international finance and a French-dominated European Common Market would provide the framework

Astronaut Charles Conrad uses lunar equipment conveyer at Lunar Module

NASA Image and Video Library

1969-11-19

Astronaut Charles Conrad Jr., commander, uses the lunar equipment conveyer (LEC) at the Lunar Module during the Apollo 12 extravehicular activity on the lunar surface. This photograph was taken by Astronaut Alan L. Bean, lunar module pilot.

Lionel Charles Renwick (Rennick) Emmett (1913-96): physician and Olympian.

PubMed

Biswas, Tamoghna; Datta, Adrija; Chandra, Shivika

2012-08-01

Lionel Charles Renwick Emmett, a physician who trained in pre-independent India as a medical student, participated in the 1936 Berlin Summer Olympics as a part of the Indian field hockey team that won the Gold Medal.

Could Charles Darwin Teach Psychology in the 1980s?

ERIC Educational Resources Information Center

Rigby, Marilyn K.

1988-01-01

Discusses the implications of Charles Darwin's personal and professional history for an academic career in psychology. Relationships between his theoretical position and the content of an introductory psychology course he might teach and how he might fare in a contemporary academic environment are sketched in this fictionalized account.…

Payload Specialist Charles Walker with handheld protein growth experiment

NASA Image and Video Library

1985-11-26

61B-02-014 (26 Nov-3 Dec 1985) --- Payload Specialist Charles D. Walker works with the handheld protein growth experiment -- one of a series of tests being flown to study the possibility of crystallizing biological materials. Walker rests the experiment against the larger continuous flow electrophoresis systems experiment.

9. Charles B. Wheelock, Photographer ca. 1890 'VIEW OF WEST ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

9. Charles B. Wheelock, Photographer ca. 1890 'VIEW OF WEST AUSTIN TAKEN FROM CAPITOL DOME' SHOWING J.H. HOUGHTON HOUSE WITH TAYLOR-HUNNICUTT HOUSE JUST BEHIND - John H. Houghton House, 307 West Twelfth Street, Austin, Travis County, TX

Auditing the frequency and the clinical and economic impact of testing for Fabry disease in patients under the age of 70 with a stroke admitted to Saint Vincent's University Hospital over a 6-month period.

PubMed

Lambe, J; Noone, I; Lonergan, R; Tubridy, N

2018-02-01

Fabry disease is an X-linked recessive lysosomal storage disorder that provokes multi-organ morbidity, including early-onset stroke. Worldwide prevalence may be greater than previously estimated, with many experiencing first stroke prior to diagnosis of Fabry disease. The aim of this study is to screen a cohort of stroke patients under 70 years of age, evaluating the clinical and economic efficacy of such a broad screening programme for Fabry disease. All stroke patients under 70 years of age who were entered into the Saint Vincent's University Hospital stroke database over a 6-month period underwent enzyme analysis and/or genetic testing as appropriate for Fabry disease. Patients' past medical histories were analysed for clinical signs suggestive of Fabry disease. Cost-effectiveness analysis of testing was performed and compared to overall economic impact of young stroke in Ireland. Of 22 patients tested for Fabry disease, no new cases were detected. Few clinical indicators of Fabry disease were identified at the time of testing. Broad screening programmes for Fabry disease are highly unlikely to offset the cost of testing. The efficacy of future screening programmes will depend on careful selection of an appropriate patient cohort of young stroke patients with multi-organ morbidity and a positive family history.

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

PubMed Central

2011-01-01

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 beta shortage

Charles L. Brewer Award for Distinguished Teaching of Psychology

ERIC Educational Resources Information Center

American Psychologist, 2009

2009-01-01

The American Psychological Foundation (APF) Charles L. Brewer Distinguished Teaching of Psychology Award recognizes an outstanding career contribution to the teaching of psychology. The 2009 recipient of the Distinguished Teaching Award is William Buskist. Dorothy W. Cantor, president of the APF, will present the APF Teaching Award at the 117th…

An Effective Leader in Higher Education: Charles William Eliot

ERIC Educational Resources Information Center

Elliott, Robert Wayne; Paton, Valerie Osland

2014-01-01

While responding to increasing calls for accountability, educational leaders are challenged continually by a myriad of complex tasks amidst the need to adapt their postsecondary institutions for responding to the changing needs of society. This review of literature examines comparisons between the leadership style of Charles William Eliot and the…

Micromachined fiber optic Fabry-Perot underwater acoustic probe

NASA Astrophysics Data System (ADS)

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.

Fabry-Perot Based Radiometers for Precise Measurement of Greenhouse Gases

NASA Technical Reports Server (NTRS)

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.

Tunable Fabry-Perot etalon-based long-wavelength infrared imaging spectroradiometer.

PubMed

Marinelli, W J; Gittins, C M; Gelb, A H; Green, B D

1999-04-20

Imaging spectrometry enables passive, stand-off detection and analysis of the chemical composition of gas plumes and surfaces over wide geographic areas. We describe the use of a long-wavelength infrared imaging spectroradiometer, comprised of a low-order tunable Fabry-Perot etalon coupled to a HgCdTe detector array, to perform multispectral detection of chemical vapor plumes. The tunable Fabry-Perot etalon used in this research provides coverage of the 9.5-14-microm spectral region with a resolution of 7-9 cm(-1). The etalon-based imaging system provides the opportunity to image a scene at only those wavelengths needed for chemical species identification and quantification and thereby minimize the data volume necessary for selective species detection. We present initial results using a brassboard imaging system for stand-off detection and quantification of chemical vapor plumes against near-ambient-temperature backgrounds. These data show detection limits of 22 parts per million by volume times meter (ppmv x m) and 0.6 ppmv x m for dimethyl methyphosphonate and SF6, respectively, for a gas/background DeltaT of 6 K. The system noise-equivalent spectral radiance is approximately 2 microW cm(-2) sr(-1) microm(-1). Model calculations are presented comparing the measured sensitivity of the sensor to the anticipated signal levels for two chemical release scenarios.

Ultrasonic imaging of seismic physical models using a fringe visibility enhanced fiber-optic Fabry-Perot interferometric sensor.

PubMed

Zhang, Wenlu; Chen, Fengyi; Ma, Wenwen; Rong, Qiangzhou; Qiao, Xueguang; Wang, Ruohui

2018-04-16

A fringe visibility enhanced fiber-optic Fabry-Perot interferometer based ultrasonic sensor is proposed and experimentally demonstrated for seismic physical model imaging. The sensor consists of a graded index multimode fiber collimator and a PTFE (polytetrafluoroethylene) diaphragm to form a Fabry-Perot interferometer. Owing to the increase of the sensor's spectral sideband slope and the smaller Young's modulus of the PTFE diaphragm, a high response to both continuous and pulsed ultrasound with a high SNR of 42.92 dB in 300 kHz is achieved when the spectral sideband filter technique is used to interrogate the sensor. The ultrasonic reconstructed images can clearly differentiate the shape of models with a high resolution.

STS-71 Pilot Charles J. Precourt suits up

NASA Technical Reports Server (NTRS)

1995-01-01

STS-71 Pilot Charles J. Precourt gets a helping hand from a suit technician as he dons his launch/entry suit in the Operations and Checkout Building. About to embark on his second spaceflight, Precourt and six fellow crew members will shortly depart for Launch Pad 39A, where the Space Shuttle Atlantis is poised for a third liftoff attempt at 3:32 p.m. EDT.

Charles Eastman: The Story of an American Indian.

ERIC Educational Resources Information Center

Lee, Betsy

Dr. Charles Alexander Eastman was a product of two worlds--the Indian and the White. A member of the Santee Sioux Tribe, he was respected and admired in both of these worlds for the work he did on behalf of American Indians, first as a young doctor caring for the sick on the Reservation, and later as a writer and speaker, showing the richness of…

A Theoretical Study and Numerical Simulation of a Quasi-Distributed Sensor Based on the Low-Finesse Fabry-Perot Interferometer: Frequency-Division Multiplexing

PubMed Central

Guillen Bonilla, José Trinidad; Guillen Bonilla, Alex; Rodríguez Betancourtt, Verónica M.; Guillen Bonilla, Héctor; Casillas Zamora, Antonio

2017-01-01

The application of the sensor optical fibers in the areas of scientific instrumentation and industrial instrumentation is very attractive due to its numerous advantages. In the industry of civil engineering for example, quasi-distributed sensors made with optical fiber are used for reliable strain and temperature measurements. Here, a quasi-distributed sensor in the frequency domain is discussed. The sensor consists of a series of low-finesse Fabry-Perot interferometers where each Fabry-Perot interferometer acts as a local sensor. Fabry-Perot interferometers are formed by pairs of identical low reflective Bragg gratings imprinted in a single mode fiber. All interferometer sensors have different cavity length, provoking frequency-domain multiplexing. The optical signal represents the superposition of all interference patterns which can be decomposed using the Fourier transform. The frequency spectrum was analyzed and sensor’s properties were defined. Following that, a quasi-distributed sensor was numerically simulated. Our sensor simulation considers sensor properties, signal processing, noise system, and instrumentation. The numerical results show the behavior of resolution vs. signal-to-noise ratio. From our results, the Fabry-Perot sensor has high resolution and low resolution. Both resolutions are conceivable because the Fourier Domain Phase Analysis (FDPA) algorithm elaborates two evaluations of Bragg wavelength shift. PMID:28420083

A Theoretical Study and Numerical Simulation of a Quasi-Distributed Sensor Based on the Low-Finesse Fabry-Perot Interferometer: Frequency-Division Multiplexing.

PubMed

Guillen Bonilla, José Trinidad; Guillen Bonilla, Alex; Rodríguez Betancourtt, Verónica M; Guillen Bonilla, Héctor; Casillas Zamora, Antonio

2017-04-14

The application of the sensor optical fibers in the areas of scientific instrumentation and industrial instrumentation is very attractive due to its numerous advantages. In the industry of civil engineering for example, quasi-distributed sensors made with optical fiber are used for reliable strain and temperature measurements. Here, a quasi-distributed sensor in the frequency domain is discussed. The sensor consists of a series of low-finesse Fabry-Perot interferometers where each Fabry-Perot interferometer acts as a local sensor. Fabry-Perot interferometers are formed by pairs of identical low reflective Bragg gratings imprinted in a single mode fiber. All interferometer sensors have different cavity length, provoking frequency-domain multiplexing. The optical signal represents the superposition of all interference patterns which can be decomposed using the Fourier transform. The frequency spectrum was analyzed and sensor's properties were defined. Following that, a quasi-distributed sensor was numerically simulated. Our sensor simulation considers sensor properties, signal processing, noise system, and instrumentation. The numerical results show the behavior of resolution vs. signal-to-noise ratio. From our results, the Fabry-Perot sensor has high resolution and low resolution. Both resolutions are conceivable because the Fourier Domain Phase Analysis (FDPA) algorithm elaborates two evaluations of Bragg wavelength shift.

4. Historic American Buildings Survey Charles E. Peterson, Photographer c. ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

4. Historic American Buildings Survey Charles E. Peterson, Photographer c. 1936 Brick Parapet, Earth Traverse, and Columbiad Mounted in Water Battery East of Sally-Port - Fort McHenry National Monument & Historic Shrine, East Fort Avenue at Whetstone Point, Baltimore, Independent City, MD

Watt-level widely tunable single-mode emission by injection-locking of a multimode Fabry-Perot quantum cascade laser

NASA Astrophysics Data System (ADS)

Chevalier, Paul; Piccardo, Marco; Anand, Sajant; Mejia, Enrique A.; Wang, Yongrui; Mansuripur, Tobias S.; Xie, Feng; Lascola, Kevin; Belyanin, Alexey; Capasso, Federico

2018-02-01

Free-running Fabry-Perot lasers normally operate in a single-mode regime until the pumping current is increased beyond the single-mode instability threshold, above which they evolve into a multimode state. As a result of this instability, the single-mode operation of these lasers is typically constrained to few percents of their output power range, this being an undesired limitation in spectroscopy applications. In order to expand the span of single-mode operation, we use an optical injection seed generated by an external-cavity single-mode laser source to force the Fabry-Perot quantum cascade laser into a single-mode state in the high current range, where it would otherwise operate in a multimode regime. Utilizing this approach, we achieve single-mode emission at room temperature with a tuning range of 36 cm-1 and stable continuous-wave output power exceeding 1 W at 4.5 μm. Far-field measurements show that a single transverse mode is emitted up to the highest optical power, indicating that the beam properties of the seeded Fabry-Perot laser remain unchanged as compared to free-running operation.

Molecular dispersion spectroscopy based on Fabry-Perot quantum cascade lasers.

PubMed

Sterczewski, Lukasz A; Westberg, Jonas; Wysocki, Gerard

2017-01-15

Two Fabry-Perot quantum cascade lasers are used in a differential dual comb configuration to perform rapidly swept dispersion spectroscopy of low-pressure nitrous oxide with <1  ms acquisition time. Active feedback control of the laser injection current enables simultaneous wavelength modulation of both lasers at kilohertz rates. The system demonstrates similar performance in both absorption and dispersion spectroscopy modes and achieves a noise-equivalent absorption figure of merit in the low 10^-4/Hz range.

Clinical observations on enzyme replacement therapy in patients with Fabry disease and the switch from agalsidase beta to agalsidase alfa.

PubMed

Lin, Hsiang-Yu; Huang, Yu-Hsiu; Liao, Hsuan-Chieh; Liu, Hao-Chuan; Hsu, Ting-Rong; Shen, Chia-I; Li, Shao-Tzu; Li, Cheng-Fang; Lee, Li-Hong; Lee, Pi-Chang; Huang, Chun-Kai; Chiang, Chuan-Chi; Lin, Shuan-Pei; Niu, Dau-Ming

2014-04-01

Fabry disease is an X-linked inherited lysosomal storage disease that can be treated with the enzymes of agalsidase beta (Fabrazyme) and agalsidase alfa (Replagal). Since June 2009, viral contamination of Genzyme's production facility has resulted in a worldwide shortage of agalsidase beta, leading to the switch to agalsidase alfa for patients with Fabry disease in Taiwan. The medical records were retrospectively reviewed for nine male patients with Fabry disease from the start of agalsidase beta treatment until the switch to agalsidase alfa for at least 1 year. After 12-112 months of enzyme replacement therapy (ERT), decreased plasma globotriaosylsphingosine (lyso-Gb3) was found in five out of seven patients, indicating improvement in disease severity. Among the six patients with available echocardiographic data at baseline and after ERT, all six experienced reductions of left ventricular mass index. Renal function, including microalbuminuria and estimated glomerular filtration rate, showed stability after ERT. Mainz Severity Score Index scores revealed that all nine patients remained stable at 12 months after switching to agalsidase alfa. ERT improved or stabilized cardiac status and stabilized renal function, while reducing plasma lyso-Gb3. ERT was well tolerated, even among the three patients who had hypersensitivity reactions. The switch of ERT from agalsidase beta to agalsidase alfa appears to be safe after 1 year of follow-up for Taiwanese patients with Fabry disease. Copyright © 2013. Published by Elsevier B.V.

A Pharmacogenetic Approach to Identify Mutant Forms of α-Galactosidase A that Respond to a Pharmacological Chaperone for Fabry Disease

PubMed Central

Wu, Xiaoyang; Katz, Evan; Valle, Maria Cecilia Della; Mascioli, Kirsten; Flanagan, John J; Castelli, Jeffrey P; Schiffmann, Raphael; Boudes, Pol; Lockhart, David J; Valenzano, Kenneth J; Benjamin, Elfrida R

2011-01-01

Fabry disease is caused by mutations in the gene (GLA) that encodes α-galactosidase A (α-Gal A). The iminosugar AT1001 (GR181413A, migalastat hydrochloride, 1-deoxygalactonojirimycin) is a pharmacological chaperone that selectively binds and stabilizes α-Gal A, increasing total cellular levels and activity for some mutant forms (defined as “responsive”). In this study, we developed a cell-based assay in cultured HEK-293 cells to identify mutant forms of α-Gal A that are responsive to AT1001. Concentration-dependent increases in α-Gal A activity in response to AT1001 were shown for 49 (60%) of 81 mutant forms. The responses of α-Gal A mutant forms were generally consistent with the responses observed in male Fabry patient-derived lymphoblasts. Importantly, the HEK-293 cell responses of 19 α-Gal A mutant forms to a clinically achievable concentration of AT1001 (10 µM) were generally consistent with observed increases in α-Gal A activity in peripheral blood mononuclear cells from male Fabry patients orally administered AT1001 during Phase 2 clinical studies. This indicates that the cell-based responses can identify mutant forms of α-Gal A that are likely to respond to AT1001 in vivo. Thus, the HEK-293 cell-based assay may be a useful aid in the identification of Fabry patients with AT1001-responsive mutant forms. Hum Mutat 32:1–13, 2011. © 2011 Wiley-Liss, Inc. PMID:21598360

Cavalier King Charles Spaniels with Chiari-like malformation and Syringomyelia have increased variability of spatio-temporal gait characteristics.

PubMed

Olsen, Emil; Suiter, Emma Jane; Pfau, Thilo; McGonnell, Imelda M; Matiasek, Kaspar; Giejda, Anna; Volk, Holger Andreas

2017-06-06

Chiari-like malformation in the Cavalier King Charles Spaniel is a herniation of the cerebellum and brainstem into or through the foramen magnum. This condition predisposes to Syringomyelia; fluid filled syrinxes within the spinal cord. The resulting pathology in spinal cord and cerebellum create neuropathic pain and changes in gait. This study aims to quantify the changes in gait for Cavalier King Charles Spaniel with Chiari-like malformation and Syringomyelia. We compared Cavalier King Charles Spaniel with Chiari-like malformation with (n = 9) and without (n = 8) Syringomyelia to Border Terriers (n = 8). Two video cameras and manual tracking was used to quantify gait parameters. We found a significant increase in coefficient of variation for the spatio-temporal characteristics and ipsilateral distance between paws and a wider base of support in the thoracic limbs but not in the pelvic limbs for Cavalier King Charles Spaniels compared with the border terrier.

Astronaut Charles Conrad Jr. working with control panel in Skylab simulation

NASA Image and Video Library

1973-02-01

S73-16765 (1 Feb. 1973) --- Astronaut Charles Conrad Jr. is seen working with the control panels of the Skylab Orbital Workshop trainer during Skylab training at the Johnson Space Center. Photo credit: NASA

Charles L. Brewer Award for distinguished teaching of psychology: Neil Lutsky.

PubMed

2011-01-01

The American Psychological Foundation (APF) Charles L. Brewer Award for Distinguished Teaching of Psychology recognizes an outstanding career contribution to the teaching of psychology. The 2011 recipient of the Distinguished Teaching Award is Neil Lutsky. Dorothy W. Cantor, president of the APF, will present the APF Distinguished Teaching Award at the 119th Annual Convention of the American Psychological Association on August 5, 2011, at 4:00 p.m. Members of the 2011 APF Board of Trustees are Dorothy W. Cantor, president; William Howell, vice president/secretary; Gerald Koocher, treasurer; Elisabeth R. Straus, executive vice president/executive director; Norman Anderson; David H. Barlow, Camilla Benbow; Sharon Stephens Brehm; Charles L. Brewer; Connie Chan; Anthony Jackson; Ronald F. Levant; Sandra Shullman; Archie L. Turner; and Kurt Geisinger, APA Board of Directors liaison.

Bravo Emma! Music in the life and work of Charles Darwin.

PubMed

Derry, J F

2009-03-01

The long-term marital dance of Emma and Charles Darwin was set to the routine beat of an almost daily piano recital. Emma was a proficient pianist, and so a quality instrument was a welcome and appropriate house-warming present for their first marital home in London. That same piano accompanied the Darwins on their move to Downe before being upgraded for a newer model, which is still there, whilst another, cheaper piano may have played in Charles Darwin's work, particularly on earthworms. Whilst he lamented his own lack of musicality, Darwin revelled in his wife's prowess, a capacity that he recognised could be inherited, not least through observation of his own children. The evolution of musicality, he reasoned, was rooted in sexual attraction as a form of communication that preceded language.

SUNLITE program. Sub-Hertz relative frequency stabilization of two diode laser pumped Nd:YAG lasers locked to a Fabry-Perot interferometer

NASA Technical Reports Server (NTRS)

Byer, R. L.

1990-01-01

Two laser pumped Nd:YAG lasers were frequency stabilized to a commercial 6.327 GHz free spectral range Fabry-Perot interferometer yielding a best case beatnote linewidth of 330 MHz. In addition, a Fabry-Perot interferometer with a free spectral range of 680 MHz, a linewidth of 25 kHz, and a finesse of 27,500 was built, and when it was substituted in place of the commercial interferometer, it produced a robust and easily repeatable beatnote linewidth of 700 MHz.

Development of a Fabry-Perot Interferometer for Ultra-Precise Measurements of Column CO2

NASA Technical Reports Server (NTRS)

Wilson, Emily L.; Georgieva, Elena M.; Heaps, William S.

2005-01-01

A passive Fabry-Perot based instrument is described for detecting column CO2 through absorption measurements at 1.58 microns . In this design, solar flux reaches the instrument platform and is directed through two channels. In the first channel, transmittance fi5nges from a Fabry-Perot interferometer are aligned with CO2 absorption lines so that absorption due to CO2 is primarily detected. The second channel encompasses the same frequency region as the first, but is comparatively more sensitive to changes in the solar flux than absorption due to CO2. The ratio of these channels is sensitive to changes in the total CO2 column, but not to changes in solar flux. This inexpensive instrument will offer high precision measurements (error 4%) in a compact package. Design of this instrument and preliminary ground-based measurements of column CO2 are presented here as well as strategies for deployment on aircraft and satellite platforms.

78 FR 35756 - Drawbridge Operation Regulations; Charles River, Boston, MA

Federal Register 2010, 2011, 2012, 2013, 2014

2013-06-14

... DEPARTMENT OF HOMELAND SECURITY Coast Guard 33 CFR Part 117 [USCG-2013-0464] Drawbridge Operation Regulations; Charles River, Boston, MA AGENCY: Coast Guard, DHS. ACTION: Notice of temporary deviation from regulations. SUMMARY: The Commander, First Coast Guard District, has issued a temporary [[Page 35757...

A Novel, Poly-Etalon, Fabry-Perot for Planetary Research

NASA Technical Reports Server (NTRS)

Kerr, Robert B.; Doe, Richard; Noto, John

1997-01-01

In an effort to develop a mechanically robust, high throughput and solid state spectrometer several liquid crystal Fabry-Perot etalons were constructed. The etalons were tested for spectral response, radiation resistance and optical transmission. The first year of this project was spent developing and understanding the properties of the liquid crystal etalons; in the second year an intensified all-sky imaging system was developed around a pair of LC etalons. The imaging system, developed jointly with SRI International represents a unique brassboard to demonstrate the use of LC etalons as tunable filters. The first set of etalons constructed in year one of this project were tested for spectral response and throughput while etalon surrogates were exposed to proton radiation simulating the exposure of an object in Low Earth Orbit (LEO). The 2" diameter etalons had a measure finesse of approximately 10 and were tunable over five orders. Liquid crystals exposed to proton irradiation showed no signs of damage. In year two two larger diameter (3") etalons were constructed with gaps of 3 and 5 microns. This pair of etalons is for use in a high resolution, all-sky spectral imager. The WATUMI imager system follows the heritage of all sky, narrow band, intensified imagers however it includes two LC Fabry-Perot etalons to provide tunability and the ability to switch wavelengths rapidly, an import consideration in auroral airglow imaging. This work also resulted in two publications and one poster presentation. The instrument will be uniquely capable, with superior throughput and speed, to measure optical airglow of multiple emission lines in harsh conditions.

Hypersonic force measurements using internal balance based on optical micromachined Fabry-Perot interferometry.

PubMed

Qiu, Huacheng; Min, Fu; Zhong, Shaolong; Song, Xin; Yang, Yanguang

2018-03-01

Force measurements using wind tunnel balance are necessary for determining a variety of aerodynamic performance parameters, while the harsh environment in hypersonic flows requires that the measurement instrument should be reliable and robust, in against strong electromagnetic interference, high vacuum, or metal (oxide) dusts. In this paper, we demonstrated a three-component internal balance for hypersonic aerodynamic force measurements, using novel optical micromachined Fabry-Perot interferometric (FPI) strain gauges as sensing elements. The FPI gauges were fabricated using Micro-Opto-Electro-Mechanical Systems (MOEMS) surface and bulk fabrication techniques. High-reflectivity coatings are used to form a high-finesse Fabry-Perot cavity, which benefits a high resolution. Antireflective and passivation coatings are used to reduce unwanted interferences. The FPI strain gauge based balance has been calibrated and evaluated in a Mach 5 hypersonic flow. The results are compared with the traditional technique using the foil resistive strain gauge balance, indicating that the proposed balance based on the MOEMS FPI strain gauge is reliable and robust and is potentially suitable for the hypersonic wind tunnel harsh environment.

Hypersonic force measurements using internal balance based on optical micromachined Fabry-Perot interferometry

NASA Astrophysics Data System (ADS)

Qiu, Huacheng; Min, Fu; Zhong, Shaolong; Song, Xin; Yang, Yanguang

2018-03-01

Force measurements using wind tunnel balance are necessary for determining a variety of aerodynamic performance parameters, while the harsh environment in hypersonic flows requires that the measurement instrument should be reliable and robust, in against strong electromagnetic interference, high vacuum, or metal (oxide) dusts. In this paper, we demonstrated a three-component internal balance for hypersonic aerodynamic force measurements, using novel optical micromachined Fabry-Perot interferometric (FPI) strain gauges as sensing elements. The FPI gauges were fabricated using Micro-Opto-Electro-Mechanical Systems (MOEMS) surface and bulk fabrication techniques. High-reflectivity coatings are used to form a high-finesse Fabry-Perot cavity, which benefits a high resolution. Antireflective and passivation coatings are used to reduce unwanted interferences. The FPI strain gauge based balance has been calibrated and evaluated in a Mach 5 hypersonic flow. The results are compared with the traditional technique using the foil resistive strain gauge balance, indicating that the proposed balance based on the MOEMS FPI strain gauge is reliable and robust and is potentially suitable for the hypersonic wind tunnel harsh environment.

Spatial distribution, temporal variability, and chemistry of the salt wedge in the lower Charles River, Massachusetts, June 1998 to July 1999

USGS Publications Warehouse

Breault, R.F.; Barlow, L.K.; Reisig, K.D.; Parker, G.W.

2000-01-01

The Charles River is of great recreational and ecological value to the Boston metropolitan region and the Commonwealth of Massachusetts. It is also the focus of the U.S. Environmental Protection Agency (USEPA) Region I, Clean Charles 2005 Task Force. The main goal of the Task Force is to make the Charles River 'fishable and swimmable' by the year 2005. Achieving 'fishable and swimmable' conditions will require continued progress in addressing a range of environmental conditions now degrading water quality, including the infiltration of saltwater from Boston Harbor into the freshwater Charles River.To better understand the pattern of saltwater intrusion, the U.S. Geological Survey (USGS), in cooperation with the U.S. Environmental Protection Agency (USEPA), Massachusetts Department of Environmental Management (MADEM), and New England Interstate Water Pollution Control Commission (NEIWPCC), collected data on the spatial distribution, temporal variability, and chemistry of the saltwater that entered the lower Charles River from June 1998 to July 1999. The purpose of this investigation is to extend and complement a regional-scale study of Charles River water quality conducted in 1996 (T. Faber, U.S. Environmental Protection Agency, written commun., 1997), and the ongoing water monitoring activities of the Massachusetts Water Resources Authority (MWRA) and the Charles River Watershed Association (CRWA). The data collected by this investigation supports the Clean Charles 2005 Task Force by providing detailed information concerning a major factor limiting 'fishable and swimmable' conditions in the lower Charles River. Finally, the study will be used to assist current planning efforts of the Metropolitan District Commission (MDC) to restore the historic parklands of the lower Charles River.The 'Basin' is the local term for the reach of the Charles River that begins at the Watertown Dam in Watertown, Mass., and extends about 8 mi through suburban and urban areas to Boston

78 FR 40213 - Charles Schwab Investment Management, Inc., et al.;

Federal Register 2010, 2011, 2012, 2013, 2014

2013-07-03

... Schwab Investment Management, Inc., et al.; Notice of Application June 27, 2013. AGENCY: Securities and... exemption from sections 12(d)(1)(A) and (B) of the Act. Applicants: Charles Schwab Investment Management...) certain registered management investment companies and unit investment trusts outside of the same group of...

Fabry disease: Review and experience during newborn screening.

PubMed

Hsu, Ting-Rong; Niu, Dau-Ming

2018-05-01

Fabry disease (FD) is an X-linked lysosomal storage disease and is the result of mutation in the α-Galactosidase A gene; such mutations cause a deficiency in α-Galactosidase A enzyme and an accumulation of glycosphingolipid in tissue. Affected males with classic FD have little or no enzyme activity and have an early onset of symptoms and signs, including acroparesthesias, hypohidrosis, angiokeratomas, gastrointestinal dysfunction and/or a characteristic corneal dystrophy during childhood/adolescence. Males with late-onset FD who have residual enzyme activity develop progressive multi-systemic involvement that leads to renal failure and hypertrophic cardiomyopathy, as well as cerebrovascular disease; these events mostly occur during the fourth to seventh decades of life. Heterozygous females can develop vital organ damage that in turn causes severe morbidity and mortality; these symptoms may be as severe as those in affected males. For the treatable disease, this review aims to raise awareness of early recognition and further management of FD based on newborn screening. As newborn screening for FD has been implemented worldwide, it allows the early detection of individuals with Fabry mutations. Based on screening studies, the prevalence of the later-onset type FD is much higher than that of classical type FD. Newborn screening studies have also revealed that patients with FD may develop insidious but ongoing irreversible organ damage. The timing of enzyme replacement therapy, which is able to stabilize the progression of disease, is important in order to prevent irreversible organ damage. Therapies that may become available in the future include pharmacological chaperones and substrate reduction therapy, both of which are still under investigation as ways of improving the health of individuals with FD. Copyright © 2017 Elsevier Inc. All rights reserved.

Study of Fabry-Perot Etalon Stability and Tuning for Spectroscopic Rayleigh Scattering

NASA Technical Reports Server (NTRS)

Clem, Michelle M.; Mielke-Fagan, Amy F.; Elam, Kristie A.

2010-01-01

The Fabry-Perot interferometer is a commonly employed instrument for resolving the spectrum of molecular Rayleigh scattered light for the purpose of evaluating flow properties such as gas velocity and temperature. Rayleigh scattered light from a focused laser beam can be directly imaged through a solid Fabry-Perot etalon onto a CCD detector to provide the spectral content of the scattered light. The spatial resolution of the measurements is governed by the locations of interference fringes. The location of the fringes can be changed by altering the etalon?s physical characteristics, such as thickness and index of refraction. For a fused silica solid etalon the physical properties can be adjusted by changing the etalon temperature; hence changing the order of the interference pattern and the physical fringe locations. Controlling the temperature of the etalon can provide for a slow time-response spatial scanning method for this type of etalon system. A custom designed liquid crystal Fabry-Perot (LCFP) can provide for a fast time-response method of scanning the etalon system. Voltage applied to the liquid crystal interface sets the etalon?s properties allowing Rayleigh measurements to be acquired at varying spatial locations across the image of the laser beam over a very short time period. A standard fused silica etalon and a tunable LCFP etalon are characterized to select the system that is best suited for Rayleigh scattering measurements in subsonic and supersonic flow regimes. A frequency-stabilized laser is used to investigate the apparent frequency stability and temperature sensitivity of the etalon systems. Frequency stability and temperature sensitivity data of the fused silica and LCFP etalon systems are presented in this paper, along with measurements of the LCFP etalon?s tuning capabilities. Rayleigh scattering velocity measurements with both etalon systems are presented, in an effort to determine which etalon is better suited to provide optical flow

3 CFR - Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor

Code of Federal Regulations, 2012 CFR

2012-01-01

... 3 The President 1 2012-01-01 2012-01-01 false Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor Presidential Documents Other Presidential Documents Notice of July 20, 2011 Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor On July 22, 2004, by...

3 CFR - Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor

Code of Federal Regulations, 2010 CFR

2010-01-01

... 3 The President 1 2010-01-01 2010-01-01 false Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor Presidential Documents Other Presidential Documents Notice of July 16, 2009 Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor On July 22, 2004, by...

3 CFR - Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor

Code of Federal Regulations, 2014 CFR

2014-01-01

... 3 The President 1 2014-01-01 2014-01-01 false Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor Presidential Documents Other Presidential Documents Notice of July 17, 2013 Continuation of the National Emergency With Respect to the Former Liberian Regime of Charles Taylor On July 22, 2004, by...

Observations of comet Levy 1990c in the (OI) 6300-A line with an imaging Fabry-Perot

NASA Technical Reports Server (NTRS)

Prasad, C. Debi; Jockers, Klaus; Rauer, H.; Geyer, E. H.

1992-01-01

We have observed the comet Levy 1990c during 16-25 August 1990 using the MPAE focal reducer system based Fabry-Perot etalon coupled with the 1 meter telescope of the Observatory of Hoher List. The free spectral range and resolution limit of the interferometer was approximately 2.18 A and approximately 0.171 A respectively. Classical Fabry-Perot fringes were recorded on a CCD in the cometary (OI) 6300 A line. They are well resolved from telluric air glow and cometary NH2 emission. Our observations indicate that the (OI) is distributed asymmetrically with respect to the center of the comet. In this paper we report the spatial distribution of (OI) emission and its line width in the coma of comet Levy.

Endomyocardial biopsies in patients with left ventricular hypertrophy and a common Chinese later-onset fabry mutation (IVS4 + 919G > A)

PubMed Central

2014-01-01

Background In Taiwan, DNA-based newborn screening showed a surprisingly high incidence of a cardiac Fabry mutation (IVS4 + 919G > A). The prevalence of this mutation is too high to be believed that it is a real pathogenic mutation. The purpose of this study is to identify the cardiac pathologic characteristics in patients with left ventricular hypertrophy and this mutation Methods and results Endomyocardial biopsies were obtained in 22 patients (Median age: 61, males: 17; females: 5) with left ventricular hypertrophy and the IVS4 + 919G > A mutation; five patients had not received enzyme replacement therapy (ERT) before biopsy, while the other 17 patients had received ERT from 8 months to 51 months. Except for three patients who had received ERT for more than 3 years, all other patients showed significant pathological change and globotriaosylceramide (Gb3) accumulation in their cardiomyocytes. In contrast to classical Fabry patients, no Gb3 accumulation was found in the capillary endothelial cells of any of our patients. Fourteen patients (63.6%) were found to have myofibrillolysis. Conclusions All of the untreated and most of the treated IVS4 + 919G > A patients showed typical pathological changes of Fabry disease in their cardiomyocytes. No endothelial accumulation of Gb3 was found, which is similar to the findings of several previous reports regarding later-onset Fabry disease. This result highly suggests that the IVS4 + 919G > A is a real pathogenic later-onset Fabry mutation. PMID:24980630

Charles L. Brewer Award for Distinguished Teaching of Psychology: Regan A. R. Gurung.

PubMed

2017-01-01

The American Psychological Foundation (APF) Charles L. Brewer Award for Distinguished Teaching of Psychology recognizes exemplary career contributions to the teaching of psychology. The 2017 recipient of the Charles L. Brewer Award for Distinguished Teaching of Psychology is Regan A. R. Gurung, PhD. Terence M. Keane, PhD, president of the APF, will present the APF Charles L. Brewer Award for Distinguished Teaching of Psychology at the 125th Annual Convention of the American Psychological Association on August 4, 2017, at 4:00 p.m. Members of the 2017 APF Board of Trustees are Terence M. Keane, PhD, president; Melba J. T. Vasquez, PhD, vice president/interim secretary; Richard C. McCarty, PhD, treasurer; Elisabeth R. Straus, executive vice president/executive director; Camilla Benbow, EdD; Rosie Phillips Bingham, PhD; Connie S. Chan; Arthur C. Evans, Jr., PhD; Linda M. Forrest, PhD; Douglas C. Haldeman, PhD; Anthony W. Jackson, PhD; Archie L. Turner; W. Bruce Walsh, PhD; and Stewart E. Cooper, PhD, APA Board of Directors liaison. (PsycINFO Database Record (c) 2017 APA, all rights reserved).

Detection of Anderson-Fabry cardiomyopathy with CMR in a patient with chest pain and elevated cardiac biomarkers.

PubMed

Albin, Glenn; Ryan, Michael; Heltne, Carl

2006-01-01

This case illustrates the utility of CMR in evaluating a patient with undiagnosed Anderson-Fabry disease who presented with chest pain, elevated cardiac biomarkers, normal coronary arteries, and an abnormal echocardiogram.

Characteristics of Extrinsic Fabry-Perot Interferometric (EFPI) Fiber-Optic Strain Gages

NASA Technical Reports Server (NTRS)

Hare, David A.; Moore, Thomas C., Sr.

2000-01-01

The focus of this paper is a comparison of the strain-measuring characteristics of one type of commercially available fiber-optic strain sensor with the performance of conventional resistance strain gages. Fabry-Perot type fiber-optic strain sensors were selected for this testing program. Comparative testing is emphasized and includes load testing at room temperature with apparent strain characterization cryogenically and at elevated temperatures. The absolute accuracy of either of these types of strain gages is not addressed.

The Characteristics in the Sensitivity of Microfiber Fabry-Perot Interferometric Transducers

NASA Astrophysics Data System (ADS)

Wang, Xiuxin; Li, Zhangyong; Lin, Jinzhao; Wang, Wei; Tian, Yin; Pang, Yu

2018-01-01

We inscribe a Fabry-Perot (FP) resonator in the microfiber utilizing the 193-nm UV exposure and the phase mask technique. Some new characteristics in contrast to the conventional counterparts are measured, which are attributed to the index change in the grating and the dispersion of the effective grating length, respectively. The FP spectral dependencies on external strain, temperature, and refractive index are investigated. Our fabricated structures can have potential of acting as ultrasonic transducers and photo acoustic imaging.

Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study.

PubMed

Imbriaco, M; Pisani, A; Spinelli, L; Cuocolo, A; Messalli, G; Capuano, E; Marmo, M; Liuzzi, R; Visciano, B; Cianciaruso, B; Salvatore, M

2009-07-01

Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson-Fabry disease. Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson-Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease.

EPA and partners celebrate redevelopment at Charles George Landfill Superfund Site

EPA Pesticide Factsheets

The Charles George Reclamation Trust Landfill Superfund site, a former landfill, is now home to a new solar facility. The USEPA oversaw the cleanup of the 70-acre Superfund site, preventing any exposure to contaminants and reducing leachate generation.

Charles Nordmann and Multicolour Stellar Photometry

NASA Astrophysics Data System (ADS)

Lequeux, James

2010-11-01

Charles Nordmann (1881-1940), an astronomer at the Paris Observatory, was the first to determine the effective temperature of stars with his photometre heterochrome, simultaneously and independently of Rosenberg, Wilsing and Scheiner in Germany. He is also the remote precursor of the multicolour photometry of Johnson and Morgan. In spite of the quality of his temperature determinations, which were as good or better than those made by spectrophotometry, he rapidly fell into oblivion because of some failures in his scientific work. We examine his activity in the international context of the time, and explain why he has been forgotten, to be rediscovered only recently.

[Charles Bonnet syndrome precipitated by brimonidine].

PubMed

García-Catalán, M R; Arriola-Villalobos, P; Santos-Bueso, E; Gil-de-Bernabé, J; Díaz-Valle, D; Benítez-del-Castillo, J M; García-Sánchez, J

2013-09-01

An 81-year-old woman with age-related macular degeneration and pseudoexfoliative glaucoma developed visual hallucinations (faces, flowers and frames) shortly after beginning brimonidine drops. Neurologic and psychiatric examination was normal. Visual hallucinations disappeared within 10 days after discontinuing the drug. The Charles Bonnet syndrome (CBS) is characterised by complex visual hallucinations in elderly patients in the setting of significant visual impairment without any psychiatric symptoms. Awareness of CBS among ophthalmologist is essential. Clinicians should treat visual impairment and be aware of possible visual hallucinations in patients treated with brimonidine. Copyright © 2010 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

[The project and simulation of a compositive miniature spectrum instrument based on the array of Fabry-Perot cavity].

PubMed

Wen, Zhi-yu; Chen, Gang; Wang, Jian-guo

2006-10-01

This paper advances a kind of micro-spectrometer based on Fabry-Perot cavity's character of filtering the waves. The basic structure of the micro-spectrometer is the array of Fabry-Perot cavity which contains many different lengths of cavity on the substrate of silicon, consequently the authors can achieve the detection at several wavelengths simultaneously. The unit of probing is a Fabry-Perot cavity made up of the substrate of silicon-metal film-silicon dioxide layer-metal film. The authors carried out the corresponding simulation. In the basic structure of aluminum film(14 nm)-silicon dioxide layer-silver film(39 nm), the resolution can reach 15 nm. When the area of a unit of probing is 0.14 mm x 0.14 mm only, it can reach the luminous flux of miniature grating spectrum instrument (the minimum volume in the order of cm), but the volume of the part of spectrum detection is only of the order of mm. The design size of the micro-spectrometer is a few millimeters. Furthermore it has no movable parts and could detect several wavelengths at the same time. It is possible to fabricate such micro-spectrometer through existing processing methods of IC technology.

Clinical observation of patients with Fabry disease after switching from agalsidase beta (Fabrazyme) to agalsidase alfa (Replagal).

PubMed

Tsuboi, Kazuya; Yamamoto, Hiroshi

2012-09-01

Fabry disease is a rare, X-linked, inherited lysosomal storage disorder that can be treated with the enzymes agalsidase alfa (Replagal) and agalsidase beta (Fabrazyme). Currently, there is a global shortage of agalsidase beta, and this has increased global demand for agalsidase alfa. We assess the feasibility of switching patients on agalsidase beta treatment to agalsidase alfa instead. This analysis is part of an ongoing observational study involving 11 patients with Fabry disease in whom the treatment was switched from agalsidase beta (1 mg/kg every other week) to agalsidase alfa (0.2 mg/kg every other week). Data were collected for a minimum of 36 months: 24 months before and 12 months after the switch. Serial data were evaluated with respect to renal function, cardiac mass, pain, quality of life, and tolerability/safety. Indexes of renal function (estimated glomerular filtration rate) and cardiac mass (left-ventricular mass index), pain (Brief Pain Inventory), and quality of life (EuroQoL-Dimensions) clearly showed that, in patients switched to agalsidase alfa, Fabry disease stabilized during the 12 months of follow-up. Despite the limitations of this preliminary observational study, it was found that all the patients maintained disease stability when treated with agalsidase alfa, as evidenced by estimated glomerular filtration rate, left-ventricular mass index, pain scores, and quality-of-life indexes, throughout 12 months of follow-up.

Clinical observation of patients with Fabry disease after switching from agalsidase beta (Fabrazyme) to agalsidase alfa (Replagal).

PubMed

Tsuboi, Kazuya; Yamamoto, Hiroshi

2012-09-01

Fabry disease is a rare, X-linked, inherited lysosomal storage disorder that can be treated with the enzymes agalsidasealfa (Replagal) and agalsidase beta (Fabrazyme). Currently, there is a global shortage of agalsidase beta, and this has increased global demand for agalsidase alfa. We assess the feasibility of switching patients on agalsidase beta treatment to agalsidase alfa instead. This analysis is part of an ongoing observational study involving 11 patients with Fabry disease in whom the treatment was switched from agalsidase beta (1 mg/kg every other week) to agalsidase alfa (0.2 mg/kg every other week). Data were collected for a minimum of 36 months: 24 months before and 12 months after the switch. Serial data were evaluated with respect to renal function, cardiac mass, pain, quality of life, and tolerability/safety. Indexes of renal function (estimated glomerular filtration rate) and cardiac mass (left-ventricular mass index), pain (Brief Pain Inventory), and quality of life (EuroQoL-Dimensions) clearly showed that, in patients switched to agalsidase alfa, Fabry disease stabilized during the 12 months of follow-up. Despite the limitations of this preliminary observational study, it was found that all the patients maintained disease stability when treated with agalsidase alfa, as evidenced by estimated glomerular filtration rate, left-ventricular mass index,pain scores, and quality-of-life indexes, throughout 12 months of follow-up.

Sen. Jake Garn and payload specialist Charles Waler in middeck simulation

NASA Technical Reports Server (NTRS)

1985-01-01

Two payload specialists for the STS 51-D mission get in some training time in the crew compartment trainerat JSC. Charles D. Walker, left, rehearses photography of U.S. Senator E.J. (Jake) Garn in the middeck section of the trainer.

Sen. Jake Garn and payload specialist Charles Waler in middeck simulation

NASA Image and Video Library

1985-04-12

Two payload specialists for the STS 51-D mission get in some training time in the crew compartment trainerat JSC. Charles D. Walker, left, rehearses photography of U.S. Senator E.J. (Jake) Garn in the middeck section of the trainer.

[Transient charles bonnet syndrome after excision of a right occipital meningioma: a case report].

PubMed

Arai, Takao; Hasegawa, Yuzuru; Tanaka, Toshihide; Kato, Naoki; Watanabe, Mitsuyoshi; Nakamura, Aya; Murayama, Yuichi

2014-05-01

Charles Bonnet syndrome is a condition characterized by visual hallucinations. These simple or complex visual hallucinations are more common in elderly individuals with impaired peripheral vision. The current report describes a case of transient Charles Bonnet syndrome appearing after the removal of a meningioma. The patient was a 61-year-old man who already had impaired visual acuity due to diabetic retinopathy. Brain MRI revealed a cystic tumor severely compressing the right occipital lobe. Starting on day 2 postoperatively, the patient was troubled by recurring visual hallucinations involving people, flowers, pictures, and familiar settings(the train and a coffee shop). These continued for 3.5 months. This period roughly coincided with the time for the occipital lobe to recover from the compression caused by the tumor, a fact that was confirmed by several MRI scans. ¹²³I-IMP SPECT performed 1 month after the surgical operation showed an area of hypoperfusion in the right parieto-occipital lobe. Based on the patient's clinical course and MRI findings, the mechanism of onset of visual hallucinations in this patient was put forward. The release of pressure in the brain by tumor removal and subsequent recovery changed the blood flow to the brain. This triggered visual hallucinations in the patient, who was already predisposed to developing Charles Bonnet syndrome because of diabetic retinopathy. This case is interesting since it indicates that central neurological factors, as well as visual deficits, may induce the appearance of visual hallucinations in Charles Bonnet syndrome.

Prévalence des dyslipidémies au laboratoire de biochimie du CHU Aristide le Dantec de Dakar, Sénégal

PubMed Central

Cissé, Fatou; Agne, Fatou Diallo; Diatta, Alassane; Mbengue, Abdou Salam; Ndiaye, Arame; Samba, Abdourahmane; Thiam, Souleymane; Doupa, Dominique; Sarr, Gaston Ndéné; Sall, Niama Diop; Touré, Méissa

2016-01-01

Introduction L'objectif de cette étude était d'évaluer la prévalence des dyslipidémies chez les patients reçus au laboratoire de Biochimie de l'Hôpital Aristide Le Dantec pour le dosage d'un paramètre lipidique au cours de l'année 2013. Méthodes Il s'agit d'une étude rétrospective portant sur 1356 patients âgés de 10 à 94 ans reçus au laboratoire de Biochimie du CHU Le Dantec de janvier à décembre 2013. Etaient inclus dans l'étude, tous les patients ayant au moins un paramètre du bilan lipidique dont les résultats étaient enregistrés dans le registre du laboratoire. Le cholestérol total, le cholestérol HDL, le cholestérol LDL ainsi que les triglycérides ont été dosés grâce à des méthodes enzymatiques sur un automate de Biochimie de type Cobas Integra 400 (Roche Diagnostics). Résultats La prévalence des dyslipidémies dans notre population d'étude est de 39,30%. Les prévalences de l'hypercholestérolémie, l'hypoHDLémie, l'hyperLDLémie, l'hypertriglycéridémie et l'hyperlipidémie mixte étaient respectivement : 30,89% ; 7,30% ; 31,19% ; 0,51% ; 7,22%. Les sujets de 40 à 59 ans semblaient être plus exposés et on note une prédominance féminine en ce qui concerne l'hypercholestérolémie (54,17% vs 45,82%), l'hypoHDLémie (54,54% vs45, 45%), et l'hyperlipidémie mixte (51,08% vs 48,97%). Enfin les dyslipidémies étaient fortement corrélées à l'HTA et l'obésité. Conclusion La forte prévalence des dyslipidémies retrouvée dans notre étude démontre l'intérêt d'étudier la prévalence des facteurs de risque cardio-vasculaires en particulier les dyslipidémies dans la population sénégalaise. PMID:28292030

Charles Lucas and medical legislation in eighteenth century Ireland.

PubMed

Mullaney, S

2015-09-01

Charles Lucas, apothecary, physician and MP, was instrumental in facilitating legislation in Ireland in 1765 that established a nationwide network of hospitals in Ireland. This legislation was unique in contemporary Europe, and by the end of the century, there was a hospital in every county in the country. His work as an apothecary provided him with the knowledge to attempt to address the problems in the apothecaries' trade, and his 1741 pamphlet, Pharmacomastix, provided the framework for the 1761 Irish Apothecaries Act, which attempted to address these issues. It was, however, 1791 before nationwide regulation of the Irish apothecaries' trade was implemented following the establishment of the Apothecaries Hall, and this was 24 years before similar regulatory legislation was passed in Britain. Lucas sought enhanced regulation of the apothecaries' trade to provide better quality drugs and medicines for the general public, and he tried to ensure that untrained quacks did not practise as apothecaries, unbeknownst to their patients. He was aware that his proposals would meet with opposition, but he had the courage to pursue these without any element of personal gain. In medical terms, Charles Lucas was man ahead of his time.

Phase-generated carrier demodulation scheme for fiber Fabry-Pérot interferometric sensor with high finesse

NASA Astrophysics Data System (ADS)

Rao, Wei; Niu, Siliang; Zhang, Nan; Cao, Chunyan; Hu, Yongmin

2011-09-01

This paper presents a demodulation scheme using phase-generated carrier (PGC) for a fiber Fabry-Pérot interferometric (FFPI) sensor with high finesse. The FFPI is constructed by a polarization maintaining fiber ring resonator with dual-coupler (PMDC-FRR), which can eliminate the polarization induced fading phenomenon. Compared with the former phase demodulation methods, the PGC scheme in this paper does not assume a two-beam interferometric approximation for the Fabry-Pérot cavity, and can work at arbitrary value of finesse in theory. Two PMDC-FRRs with reflective coefficients of 0.5 and 0.9 are made in experiments for demodulation. Both the single-frequency and the wideband signals are successfully demodulated from the transmission intensities using the PGC demodulation scheme. The experimental results demonstrate that the PGC demodulation scheme is feasible for the FFPI sensor with high finesse. The effects of the reflective coefficient and the intensity loss to the finesse are also discussed.

A double-fibre Fabry-Perot sensor based on modified fringe counting and direct phase demodulation

NASA Astrophysics Data System (ADS)

Li, M.; Tong, B.; Arsad, N.; Guo, J. J.

2013-09-01

A modified double-fibre Fabry-Perot cavity is developed for determination of the fringe moving direction and higher sensitivity in applications of liquid level and displacement sensors. Two fibres are integrated into a silica ferrule where the ends of the two fibres in the ferrule serve as the front surfaces of the Fabry-Perot cavities, and a diaphragm, which is replaced by a moving mirror for measurement of displacement, serves as the rear surface for both cavities in liquid level sensing. Our design has no strict requirements for a specific phase difference between the two optical paths, just a constant difference resulting from the processing error between the two fibre end positions rather than a precise optical path difference of λ/8 to judge the pattern shift direction. Experimental results demonstrate the feasibility of this approach to determining the fringe moving direction, a displacement sensitivity of 3 µm and good linearity for both applications.

Geometric phase and o -mode blueshift in a chiral anisotropic medium inside a Fabry-Pérot cavity

NASA Astrophysics Data System (ADS)

Timofeev, Ivan V.; Gunyakov, Vladimir A.; Sutormin, Vitaly S.; Myslivets, Sergey A.; Arkhipkin, Vasily G.; Vetrov, Stepan Ya.; Lee, Wei; Zyryanov, Victor Ya.

2015-11-01

Anomalous spectral shift of transmission peaks is observed in a Fabry-Pérot cavity filled with a chiral anisotropic medium. The effective refractive index value resides out of the interval between the ordinary and the extraordinary refractive indices. The spectral shift is explained by contribution of a geometric phase. The problem is solved analytically using the approximate Jones matrix method, numerically using the accurate Berreman method, and geometrically using the generalized Mauguin-Poincaré rolling cone method. The o -mode blueshift is measured for a 4-methoxybenzylidene-4 '-n -butylaniline twisted-nematic layer inside the Fabry-Pérot cavity. The twist is electrically induced due to the homeoplanar-twisted configuration transition in an ionic-surfactant-doped liquid crystal layer. Experimental evidence confirms the validity of the theoretical model.

Tuning operating point of extrinsic Fabry-Perot interferometric fiber-optic sensors using microstructured fiber and gas pressure.

PubMed

Tian, Jiajun; Zhang, Qi; Fink, Thomas; Li, Hong; Peng, Wei; Han, Ming

2012-11-15

Intensity-based demodulation of extrinsic Fabry-Perot interferometric (EFPI) fiber-optic sensors requires the light wavelength to be on the quadrature point of the interferometric fringes for maximum sensitivity. In this Letter, we propose a novel and remote operating-point tuning method for EFPI fiber-optic sensors using microstructured fibers (MFs) and gas pressure. We demonstrated the method using a diaphragm-based EFPI sensor with a microstructured lead-in fiber. The holes in the MF were used as gas channels to remotely control the gas pressure inside the Fabry-Perot cavity. Because of the deformation of the diaphragm with gas pressure, the cavity length and consequently the operating point can be remotely tuned for maximum sensitivity. The proposed operating-point tuning method has the advantage of reduced complexity and cost compared to previously reported methods.

Geology-based method of assessing sensitivity of streams to acidic deposition in Charles and Anne Arundel Counties, Maryland

USGS Publications Warehouse

Rice, Karen C.; Bricker, Owen P.

1991-01-01

The report describes the results of a study to assess the sensitivity of streams to acidic deposition in Charles and Anne Arundel Counties, Maryland using a geology-based method. Water samples were collected from streams in July and August 1988 when streams were at base-flow conditions. Eighteen water samples collected from streams in Charles County, and 17 water samples from streams in Anne Arundel County were analyzed in the field for pH, specific conductance, and acid-neutralizing capacity (ANC); 8 water samples from streams in Charles County were analyzed in the laboratory for chloride and sulfate concentrations. The assessment revealed that streams in these counties are sensitive to acidification by acidic deposition.

Identification of novel mutations in the α-galactosidase A gene in patients with Fabry disease: pitfalls of mutation analyses in patients with low α-galactosidase A activity.

PubMed

Yoshimitsu, Makoto; Higuchi, Koji; Miyata, Masaaki; Devine, Sean; Mattman, Andre; Sirrs, Sandra; Medin, Jeffrey A; Tei, Chuwa; Takenaka, Toshihiro

2011-05-01

Fabry disease is an X-linked lysosomal storage disorder caused by mutations of the α-galactosidase A (GLA) gene, and the disease is a relatively prevalent cause of left ventricular hypertrophy followed by conduction abnormalities and arrhythmias. Mutation analysis of the GLA gene is a valuable tool for accurate diagnosis of affected families. In this study, we carried out molecular studies of 10 unrelated families diagnosed with Fabry disease. Genetic analysis of the GLA gene using conventional genomic sequencing was performed in 9 hemizygous males and 6 heterozygous females. In patients with no mutations in coding DNA sequence, multiplex ligation-dependent probe amplification (MLPA) and/or cDNA sequencing were performed. We identified a novel exon 2 deletion (IVS1_IVS2) in a heterozygous female by MLPA, which was undetectable by conventional sequencing methods. In addition, the g.9331G>A mutation that has previously been found only in patients with cardiac Fabry disease was found in 3 unrelated, newly-diagnosed, cardiac Fabry patients by sequencing GLA genomic DNA and cDNA. Two other novel mutations, g.8319A>G and 832delA were also found in addition to 4 previously reported mutations (R112C, C142Y, M296I, and G373D) in 6 other families. We could identify GLA gene mutations in all hemizygotes and heterozygotes from 10 families with Fabry disease. Mutations in 4 out of 10 families could not be identified by classical genomic analysis, which focuses on exons and the flanking region. Instead, these data suggest that MLPA analysis and cDNA sequence should be considered in genetic testing surveys of patients with Fabry disease. Copyright © 2011 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

A miniature electronically tunable Fabry-Perot filter

NASA Astrophysics Data System (ADS)

O'Sullivan, B.; Pietraszewski, K. A. R.

A miniature electronically tunable, servo controlled Fabry-Perot filter for use in fiber optic sensors, spectroscopy, data and telecommunications, and laser tuning has been developed. The servo control system utilizes capacitance micrometry and piezo technology to maintain stable cavity mirror separations with a noise of less than 0.9nm rms while enabling random access tuning to any wavelength in the design range in less than 0.5ms. Free spectral ranges from 75,000GHz to 300GHz (560nm to 1.5nm at 1500nm wavelength) are typical with finesses between 3 and 300. At present the device has been made commercially available in two formats: fiber optically coupled, with single-mode or multimode fiber, or with a 3mm clear aperture. The design and performance of the instrument are presented along with some typical application examples.

Nonlinear regression method for estimating neutral wind and temperature from Fabry-Perot interferometer data.

PubMed

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.

[Quartz-enhanced photoacoustic spectroscopy trace gas detection system based on the Fabry-Perot demodulation].

PubMed

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.

Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

PubMed

Maruyama, Hiroki; Miyata, Kaori; Mikame, Mariko; Taguchi, Atsumi; Guili, Chu; Shimura, Masaru; Murayama, Kei; Inoue, Takeshi; Yamamoto, Saori; Sugimura, Koichiro; Tamita, Koichi; Kawasaki, Toshihiro; Kajihara, Jun; Onishi, Akifumi; Sugiyama, Hitoshi; Sakai, Teiko; Murata, Ichijiro; Oda, Takamasa; Toyoda, Shigeru; Hanawa, Kenichiro; Fujimura, Takeo; Ura, Shigehisa; Matsumura, Mimiko; Takano, Hideki; Yamashita, Satoshi; Matsukura, Gaku; Tazawa, Ryushi; Shiga, Tsuyoshi; Ebato, Mio; Satoh, Hiroshi; Ishii, Satoshi

2018-03-15

PurposePlasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females.MethodsBetween 1 July 2014 and 31 December 2015, we screened 2,360 patients (1,324 males) referred from 169 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively.ResultsOf 8 males with elevated lyso-Gb3 levels (≥2.0 ng ml -1 ) and low α-Gal A activity (≤4.0 nmol h -1  ml -1 ), 7 presented a GLA mutation (2 classic and 5 late-onset). Of 15 females with elevated lyso-Gb3, 7 displayed low α-Gal A activity (5 with GLA mutations; 4 classic and 1 late-onset) and 8 exhibited normal α-Gal A activity (1 with a classic GLA mutation and 3 with genetic variants of uncertain significance).ConclusionPlasma lyso-Gb3 is a potential primary screening biomarker for classic and late-onset Fabry disease probands.Genet Med advance online publication, 15 March 2018; doi:10.1038/gim.2018.31.

Spectral response of fiber-coupled Fabry-Perot etalons.

PubMed

Ionov, Pavel

2014-03-01

In many remote sensing applications one or multiple Fabry-Perot etalons are used as high-spectral-resolution filter elements. These etalons are often coupled to a receiving telescope with a multimode fiber, leading to subtle effects of the fiber mode order on the overall spectral response of the system. A theoretical model is developed to treat the spectral response of the combined system: fiber, collimator, and etalon. The method is based on a closed-form expression of the diffracted mode in terms of a Hankel transform. In this representation, it is shown how the spectral effect of the fiber and collimator can be separated from the details of the etalon and can be viewed as a mode-dependent spectral broadening and shift.

Measurement of the carrier envelope offset frequency of a femtosecond frequency comb using a Fabry-Perot interferometer

DOE Office of Scientific and Technical Information (OSTI.GOV)

Basnak, D V; Bikmukhametov, K A; Dmitrieva, N I

2010-10-15

A method for measuring the carrier envelope offset (CEO) frequency of the femtosecond frequency comb with a bandwidth of less than one octave by using a Fabry-Perot interferometer is proposed and experimentally demonstrated. (laser components)

Passings to note: Paul Michael Packman, MD; S. Charles Schulz, MD.

PubMed

Black, Donald W

2018-02-01

One of the keys to the success of Annals of Clinical Psychiatry has always been the tireless efforts of our dedicated Editorial Board. We recently lost 2 longtime Editorial Board members, Drs. Paul Michael Packman and S. Charles Schulz. Both will be greatly missed.

75 FR 62469 - Drawbridge Operation Regulations; Charles River, Boston, MA, Maintenance

Federal Register 2010, 2011, 2012, 2013, 2014

2010-10-12

... DEPARTMENT OF HOMELAND SECURITY Coast Guard 33 CFR Part 117 [Docket No. USCG-2010-0847] Drawbridge Operation Regulations; Charles River, Boston, MA, Maintenance AGENCY: Coast Guard, DHS. ACTION: Notice of temporary deviation from regulations. SUMMARY: The Commander, First Coast Guard District, has issued a...

The Rice University Press Initiative: An Interview with Charles Henry

ERIC Educational Resources Information Center

Trevitte, Chad; Henry, Charles

2007-01-01

In this interview Charles Henry, publisher of the Rice University Press (RUP), discusses RUP's rebirth as a fully digital university press. Henry addresses the circumstances that led to this decision, and he further outlines the RUP business model whereby the press will publish its own titles--both digitally and in print-on-demand--while…

Occasional Papers in Distance Education: Number 11, Charles Sturt University.

ERIC Educational Resources Information Center

Roberts, David, Ed.; Meacham, David, Ed.

The four papers in this collection focus on distance education and open education. In the first paper, "The Open Learning Institute," D. W. Roberts cites the original recommendation that Charles Sturt University establish a new division to be known as the Open Learning Institute, and presents a definition of the concept. In the second…

Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature.

PubMed

Loeb, Josefine; Feldt-Rasmussen, Ulla; Madsen, Christoffer Valdorff; Vogel, Asmus

2018-04-14

Fabry disease is a rare progressive X-linked lysosomal storage disorder which leads to neuropathic pain, organ dysfunction and cerebral pathology. Few studies have investigated cognitive impairment in Fabry disease and these previous studies are difficult to compare due to heterogeneous methodological designs and small cohorts. The objective was to investigate the frequency of cognitive impairment in the Danish nationwide cohort of Fabry patients. Further, we examined if subjective cognitive complaints were associated with objective cognitive performances in this patient group. Neuropsychological tests (17 measures) and evaluation of subjective complaints with the Perceived Deficits Questionnaire (PDQ) were applied in 41 of 63 patients. According to an a priori definition, 12 patients (29.3%) were cognitively impaired. Tests tapping psychomotor speed, attention and executive functions had the highest frequency of impairment. In general, disease related variables as Mainz Severity Score Index, enzyme activity and years since onset and depression did not have a significant impact on the categorisation of patients as being cognitively impaired or non-impaired. Thus, cognitive impairment in Fabry disease does not seem to occur solely by having symptoms for many years or by having high disease burden. However, impaired neuropsychological test results were significantly more common in patients with cerebrovascular disease. Only three patients had scores in the abnormal range of the PDQ scale and subjective perceptions of cognition were not associated with cognitive performances. The levels of subjective cognitive complaints were generally very low in the studied patients demonstrating that the absence of subjective cognitive complaints does not exclude the presence of objective cognitive problems.

High-precision thermal expansion measurements using small Fabry-Perot etalons

NASA Astrophysics Data System (ADS)

Davis, Mark J.; Hayden, Joseph S.; Farber, Daniel L.

2007-09-01

Coefficient of thermal expansion (CTE) measurements using small Fabry-Perot etalons were conducted on high and low thermal expansion materials differing in CTE by a factor of nearly 400. The smallest detectable change in length was ~10 -12 m. The sample consisted of a mm-sized Fabry-Perot etalon equipped with spherical mirrors; the material-under-test served as the 2.5 mm-thick spacer between the mirrors. A heterodyne optical setup was used with one laser locked to an ~780 nm hyperfine line of Rb gas and the other locked to a resonance of the sample etalon; changes in the beat frequency between the two lasers as a function of temperature directly provided a CTE value. The measurement system was tested using the high-CTE SCHOTT optical glass N-KF9 (CTE = 9.5 ppm/K at 23 °C). Measurements conducted under reproducibility conditions using five identically-prepared N-KF9 etalons demonstrate a precision of 0.1 ppm/K; absolute values (accuracy) are within 2-sigma errors with those made using mechanical dilatometers with 100-mm long sample rods. Etalon-based CTE measurements were also made on a high-CTE (~10.5 ppm/K), proprietary glass-ceramic used for high peak-pressure electrical feedthroughs and revealed statistically significant differences among parts made under what were assumed to be identical conditions. Finally, CTE measurements were made on etalons constructed from SCHOTT's ultra-low CTE Zerodur (R) glass-ceramic (CTE about -20 ppb/K at 50 °C for the material tested herein).

A thermodynamic assay to test pharmacological chaperones for Fabry disease.

PubMed

Andreotti, Giuseppina; Citro, Valentina; Correra, Antonella; Cubellis, Maria Vittoria

2014-03-01

The majority of the disease-causing mutations affect protein stability, but not functional sites and are amenable, in principle, to be treated with pharmacological chaperones. These drugs enhance the thermodynamic stability of their targets. Fabry disease, a disorder caused by mutations in the gene encoding lysosomal alpha-galactosidase, represents an excellent model system to develop experimental protocols to test the efficiency of such drugs. The stability of lysosomal alpha-galactosidase under different conditions was studied by urea-induced unfolding followed by limited proteolysis and Western blotting. We measured the concentration of urea needed to obtain half-maximal unfolding because this parameter represents an objective indicator of protein stability. Urea-induced unfolding is a versatile technique that can be adapted to cell extracts containing tiny amounts of wild-type or mutant proteins. It allows testing of protein stability as a function of pH, in the presence or in the absence of drugs. Results are not influenced by the method used to express the protein in transfected cells. Scarce and dispersed populations pose a problem for the clinical trial of drugs for rare diseases. This is particularly true for pharmacological chaperones that must be tested on each mutation associated with a given disease. Diverse in vitro tests are needed. We used a method based on chemically induced unfolding as a tool to assess whether a particular Fabry mutation is responsive to pharmacological chaperones, but, by no means is our protocol limited to this disease. Copyright © 2013 The Authors. Published by Elsevier B.V. All rights reserved.

Simple interrogator for optical fiber-based white light Fabry-Perot interferometers.

PubMed

Yu, Zhihao; Tian, Zhipeng; Wang, Anbo

2017-02-15

In this Letter, we present the design of a simple signal interrogator for optical fiber-based white light Fabry-Perot (F-P) interferometers. With the hardware being composed of only a flat fused silica wafer and a CCD camera, this interrogator translates the spectral interference into a spatial interference pattern, and then demodulates the F-P cavity length with the use of a relatively simple demodulation algorithm. The concept is demonstrated experimentally in a fiber optic sensor with a sapphire wafer as the F-P cavity.

Clinical observation of patients with Fabry disease after switching from agalsidase beta (Fabrazyme) to agalsidase alfa (Replagal)

PubMed Central

Tsuboi, Kazuya; Yamamoto, Hiroshi

2012-01-01

Purpose: Fabry disease is a rare, X-linked, inherited lysosomal storage disorder that can be treated with the enzymes agalsidase alfa (Replagal) and agalsidase beta (Fabrazyme). Currently, there is a global shortage of agalsidase beta, and this has increased global demand for agalsidase alfa. We assess the feasibility of switching patients on agalsidase beta treatment to agalsidase alfa instead. Methods: This analysis is part of an ongoing observational study involving 11 patients with Fabry disease in whom the treatment was switched from agalsidase beta (1 mg/kg every other week) to agalsidase alfa (0.2 mg/kg every other week). Data were collected for a minimum of 36 months: 24 months before and 12 months after the switch. Serial data were evaluated with respect to renal function, cardiac mass, pain, quality of life, and tolerability/safety. Results: Indexes of renal function (estimated glomerular filtration rate) and cardiac mass (left-ventricular mass index), pain (Brief Pain Inventory), and quality of life (EuroQoL-Dimensions) clearly showed that, in patients switched to agalsidase alfa, Fabry disease stabilized during the 12 months of follow-up. Conclusion: Despite the limitations of this preliminary observational study, it was found that all the patients maintained disease stability when treated with agalsidase alfa, as evidenced by estimated glomerular filtration rate, left-ventricular mass index, pain scores, and quality-of-life indexes, throughout 12 months of follow-up. PMID:22498845

Charles Darwin: Genius or Plodder?

PubMed Central

Wilkins, Adam S.

2009-01-01

There is no doubt about the magnitude of Charles Darwin's contributions to science. There has, however, been a long-running debate about how brilliant he was. His kind of intelligence was clearly different from that of the great physicists who are deemed geniuses. Here, the nature of Darwin's intelligence is examined in the light of Darwin's actual style of working. Surprisingly, the world of literature and the field of neurobiology might supply more clues to resolving the puzzle than conventional scientific history. Those clues suggest that the apparent discrepancy between Darwin's achievements and his seemingly pedestrian way of thinking reveals nothing to Darwin's discredit but rather a too narrow and inappropriate set of criteria for “genius.” The implications of Darwin's particular creative gifts with respect to the development of scientific genius in general are briefly discussed. PMID:19933233

Charles Darwin: genius or plodder?

PubMed

Wilkins, Adam S

2009-11-01

There is no doubt about the magnitude of Charles Darwin's contributions to science. There has, however, been a long-running debate about how brilliant he was. His kind of intelligence was clearly different from that of the great physicists who are deemed geniuses. Here, the nature of Darwin's intelligence is examined in the light of Darwin's actual style of working. Surprisingly, the world of literature and the field of neurobiology might supply more clues to resolving the puzzle than conventional scientific history. Those clues suggest that the apparent discrepancy between Darwin's achievements and his seemingly pedestrian way of thinking reveals nothing to Darwin's discredit but rather a too narrow and inappropriate set of criteria for "genius." The implications of Darwin's particular creative gifts with respect to the development of scientific genius in general are briefly discussed.

Water resources of St. Charles Parish, Louisiana

USGS Publications Warehouse

White, Vincent E.; Prakken, Lawrence B.

2015-01-01

Information concerning the availability, use, and quality of water in St. Charles Parish, Louisiana, is critical for proper water-supply management. The purpose of this fact sheet is to present information that can be used by water managers, parish residents, and others for stewardship of this vital resource. Information on the availability, past and current use, use trends, and water quality from groundwater and surface-water sources in the parish is presented. Previously published reports and data stored in the U.S. Geological Survey’s National Water Information System (http://waterdata.usgs.gov/nwis) are the primary sources of the information presented here.

Scientific Cousins: The Relationship between Charles Darwin and Francis Galton

ERIC Educational Resources Information Center

Fancher, Raymond E.

2009-01-01

This article traces the personal as well as the intellectual and scientific relationship between Charles Darwin and his younger half-cousin Francis Galton. Although they had been on friendly terms as young men, and Darwin had in some ways been a role model for Galton, the two did not share major scientific interests until after the publication of…

Charles Darwin: His Life, Journeys and Discoveries. A Teacher's Guide.

ERIC Educational Resources Information Center

Overy, Caroline

This handbook aims to: (1) introduce teachers and pupils to Charles Darwin, his life and work at Down House, his voyage on the Beagle, and his evolutionary theory; (2) set his ideas within the wider context of the 19th century; (3) link the subject areas to the British National Curriculum, particularly in history, science, and English at various…

A Wireless World: Charles County Public Schools Makes Wireless Universal

ERIC Educational Resources Information Center

Hoffman, Richard

2007-01-01

Wireless connectivity in schools is all the rage, and many school systems have at least gotten their feet wet with a wireless lab or a few portable laptop carts. But Bijaya Devkota, the chief information officer of Charles County Public Schools, has done what many school systems only dream of--implemented universal wireless access throughout his…

Astronaut Charles Conrad trims hair of Astronaut Paul Weitz

NASA Technical Reports Server (NTRS)

1973-01-01

Astronaut Charles Conrad Jr., Skylab 2 commander, trims the hair of Astronaut Paul J. Weitz, Skylab 2 pilot, during the 28-day Skylab 2 mission in Earth orbit. They are in the crew quarters wardroom of the Orbital Workshop of the Skylab 1 and 2 space station. Weitz is holding a vacuum hose in his right hand. This picture was taken by Scientist-Astronaut Joseph P. Kerwin, Skylab 2 science pilot.

Two-wavelength quadrature multipoint detection of partial discharge in power transformers using fiber Fabry-Perot acoustic sensors

NASA Astrophysics Data System (ADS)

Dong, Bo; Han, Ming; Wang, Anbo

2012-06-01

A reliable and low-cost two-wavelength quadrature interrogating method has been developed to demodulate optical signals from diaphragm-based Fabry-Perot interferometric fiber optic sensors for multipoint partial discharge detection in power transformers. Commercial available fused-silica parts (a wafer, a fiber ferrule, and a mating sleeve) and a cleaved optical single mode fiber were bonded together to form an extrinsic Fabry-Perot acoustic sensor. Two lasers with center wavelengths separated by a quarter of the period of sensor interference fringes were used to probe acousticwave- induced diaphragm vibration. A coarse wavelength-division multiplexing (CWDM) add/drop multiplexer was used to separate the reflected two wavelengths before two photo detectors. Optical couplers were used to distribute mixed laser light to each sensor-detector module for multiplexing purpose. Sensor structure, detection system design and experiment results are presented.

Analysis of globotriaosylceramide (Gb3) isoforms/analogs in unfractionated leukocytes, B lymphocytes and monocytes from Fabry patients using ultra-high performance liquid chromatography/tandem mass spectrometry.

PubMed

Toupin, Amanda; Lavoie, Pamela; Arthus, Marie-Françoise; Abaoui, Mona; Boutin, Michel; Fortier, Carole; Ménard, Claudia; Bichet, Daniel G; Auray-Blais, Christiane

2018-07-26

Fabry disease is an X-linked lysosomal storage disorder with marked variability in the phenotype and genotype. Glycosphingolipids such as globotriaosylceramide (Gb 3 ) isoforms/analogs, globotriaosylsphingosine (lyso-Gb 3 ) and analogs, and galabiosylceramide (Ga 2 ) isoforms/analogs may accumulate in biological fluids and different organs. The aims of this study were to: 1) develop/validate a novel UHPLC-MS/MS method for relative quantitation of Gb 3 in leukocytes (unfractionated white blood cells), B lymphocytes and monocytes; 2) evaluate these biomarkers in a cohort of Fabry patients and healthy controls; and 3) assess correlations between these biomarkers, treatment and genotype. Whole blood, plasma and urine samples from 21 Fabry patients and 20 healthy controls were analyzed. Samples were purified by liquid-liquid extraction and analyzed by UHPLC-MS/MS in positive electrospray ionization. Methylated Gb 3 isoforms were detected, showing that a methylation process occurs at the cellular level. Our results show that there were no significant differences in the distribution of the different Gb 3 isoforms/analogs in blood cells between Fabry patients and healthy controls. In leukocyte, Gb 3 [(d18:1)(C14:0)], Gb 3 [(d18:1)(C16:0)], Gb 3 [(d18:1)(C16:0)]Me, Gb 3 [(d18:1)(C16:1)], Gb 3 [(d18:1)(C18:0)], Gb 3 [(d18:1)(C18:1)], Gb 3 [(d18:1)(C20:1)], Gb 3 [(d18:1)(C24:2)], Gb 3 [(d18:1)(C26:1)] and total Gb 3 allowed good discrimination between male Fabry patients and male controls, patients having higher biomarker levels than controls. Regarding B lymphocytes and monocytes, the same tendency was observed without reaching statistical significance. A positive concordance between mutation types and biomarker levels in white blood cells was established. Our results might provide a deeper mechanistic comprehension of the underlying biochemical processes of Gb 3 biomarkers in white blood cells of Fabry patients. Copyright © 2018 Elsevier B.V. All rights reserved.

Agalsidase-beta therapy for advanced Fabry disease: a randomized trial.

PubMed

Banikazemi, Maryam; Bultas, Jan; Waldek, Stephen; Wilcox, William R; Whitley, Chester B; McDonald, Marie; Finkel, Richard; Packman, Seymour; Bichet, Daniel G; Warnock, David G; Desnick, Robert J

2007-01-16

Fabry disease (alpha-galactosidase A deficiency) is a rare, X-linked lysosomal storage disorder that can cause early death from renal, cardiac, and cerebrovascular involvement. To see whether agalsidase beta delays the onset of a composite clinical outcome of renal, cardiovascular, and cerebrovascular events and death in patients with advanced Fabry disease. Randomized (2:1 treatment-to-placebo randomization), double-blind, placebo-controlled trial. 41 referral centers in 9 countries. 82 adults with mild to moderate kidney disease; 74 of whom were protocol-adherent. Intravenous infusion of agalsidase beta (1 mg per kg of body weight) or placebo every 2 weeks for up to 35 months (median, 18.5 months). The primary end point was the time to first clinical event (renal, cardiac, or cerebrovascular event or death). Six patients withdrew before reaching an end point: 3 to receive commercial therapy and 3 due to positive or inconclusive serum IgE or skin test results. Three patients assigned to agalsidase beta elected to transition to open-label treatment before reaching an end point. Thirteen (42%) of the 31 patients in the placebo group and 14 (27%) of the 51 patients in the agalsidase-beta group experienced clinical events. Primary intention-to-treat analysis that adjusted for an imbalance in baseline proteinuria showed that, compared with placebo, agalsidase beta delayed the time to first clinical event (hazard ratio, 0.47 [95% CI, 0.21 to 1.03]; P = 0.06). Secondary analyses of protocol-adherent patients showed similar results (hazard ratio, 0.39 [CI, 0.16 to 0.93]; P = 0.034). Ancillary subgroup analyses found larger treatment effects in patients with baseline estimated glomerular filtration rates greater than 55 mL/min per 1.73 m2 (hazard ratio, 0.19 [CI, 0.05 to 0.82]; P = 0.025) compared with 55 mL/min per 1.73 m2 or less (hazard ratio, 0.85 [CI, 0.32 to 2.3]; P = 0.75) (formal test for interaction, P = 0.09). Most treatment-related adverse events were mild or

Charles Darwin's mitochondria.

PubMed

Hayman, John

2013-05-01

Charles Darwin's long-term illness has been the subject of much speculation. His numerous symptoms have led to conclusions that his illness was essentially psychogenic in nature. These diagnoses have never been fully convincing, however, particularly in regard to the proposed underlying psychological background causes of the illness. Similarly, two proposed somatic causes of illness, Chagas disease and arsenic poisoning, lack credibility and appear inconsistent with the lifetime history of the illness. Other physical explanations are simply too incomplete to explain the range of symptoms. Here, a very different sort of explanation will be offered. We now know that mitochondrial mutations producing impaired mitochondrial function may result in a wide range of differing symptoms, including symptoms thought to be primarily psychological. Examination of Darwin's maternal family history supports the contention that his illness was mitochondrial in nature; his mother and one maternal uncle had strange illnesses and the youngest maternal sibling died of an infirmity with symptoms characteristic of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome), a condition rooted in mitochondrial dysfunction. Darwin's own symptoms are described here and are in accord with the hypothesis that he had the mtDNA mutation commonly associated with the MELAS syndrome.

Charles L. Brewer Award for Distinguished Teaching of Psychology: Neil Lutsky

ERIC Educational Resources Information Center

American Psychologist, 2011

2011-01-01

The American Psychological Foundation (APF) Charles L. Brewer Award for Distinguished Teaching of Psychology recognizes an outstanding career contribution to the teaching of psychology. The 2011 recipient of the Distinguished Teaching Award is Neil Lutsky. Dorothy W. Cantor, president of the APF, will present the APF Distinguished Teaching Award…

Charles W. Stuber: Maize geneticist and pioneer of marker-assisted selection

USDA-ARS?s Scientific Manuscript database

Charles W. Stuber is considered a pioneer of quantitative genetic mapping and marker-assisted selection in maize. The achievements of his four decade career in research include the development of genetic marker systems used in maize and adapted in many other crops, the first methods and studies to i...

33 CFR 80.505 - Cape Henlopen, DE to Cape Charles, VA.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 33 Navigation and Navigable Waters 1 2011-07-01 2011-07-01 false Cape Henlopen, DE to Cape Charles, VA. 80.505 Section 80.505 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND SECURITY INTERNATIONAL NAVIGATION RULES COLREGS DEMARCATION LINES Fifth District § 80.505 Cape Henlopen, DE...

Fiber Optic Fabry-Perot Current Sensor Integrated with Magnetic Fluid Using a Fiber Bragg Grating Demodulation

PubMed Central

Xia, Ji; Wang, Qi; Liu, Xu; Luo, Hong

2015-01-01

An optical fiber current sensor based on Fabry-Perot interferometer using a fiber Bragg grating demodulation is proposed. Magnetic fluid is used as a sensitive medium in fiber optical Fabry-Perot (F-P) cavity for the optical characteristic of magnetic-controlled refractive index. A Fiber Bragg grating (FBG) is connected after the F-P interferometer which is used to reflect the optical power at the Bragg wavelength of the interference transmission spectrum. The corresponding reflective power of the FBG will change with different external current intensity, due to the shift on the interference spectrum of the F-P interferometer. The sensing probe has the advantages of convenient measurement for its demodulation, low cost and high current measurement accuracy on account of its sensing structure. Experimental results show that an optimal sensitivity of 0.8522 nw/A and measurement resolution of 0.001 A is obtained with a FBG at 1550 nm with 99% reflectivity. PMID:26184201

Fiber Optic Fabry-Perot Current Sensor Integrated with Magnetic Fluid Using a Fiber Bragg Grating Demodulation.

PubMed

Xia, Ji; Wang, Qi; Liu, Xu; Luo, Hong

2015-07-09

An optical fiber current sensor based on Fabry-Perot interferometer using a fiber Bragg grating demodulation is proposed. Magnetic fluid is used as a sensitive medium in fiber optical Fabry-Perot (F-P) cavity for the optical characteristic of magnetic-controlled refractive index. A Fiber Bragg grating (FBG) is connected after the F-P interferometer which is used to reflect the optical power at the Bragg wavelength of the interference transmission spectrum. The corresponding reflective power of the FBG will change with different external current intensity, due to the shift on the interference spectrum of the F-P interferometer. The sensing probe has the advantages of convenient measurement for its demodulation, low cost and high current measurement accuracy on account of its sensing structure. Experimental results show that an optimal sensitivity of 0.8522 nw/A and measurement resolution of 0.001 A is obtained with a FBG at 1550 nm with 99% reflectivity.

Room used at one point by Charles Edison, the inventor's ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

Room used at one point by Charles Edison, the inventor's son, as his office while president of the Edison companies in the 1930s. The large metal tanks to the right are part of a modern halon gas fire suppression system for the building. - Thomas A. Edison Laboratories, Building No. 5, Main Street & Lakeside Avenue, West Orange, Essex County, NJ

Hyperspectral Infrared Imaging of Flames Using a Spectrally Scanning Fabry-Perot Filter

NASA Technical Reports Server (NTRS)

Rawlins, W. T.; Lawrence, W. G.; Marinelli, W. J.; Allen, M. G.; Piltch, N. (Technical Monitor)

2001-01-01

The temperatures and compositions of gases in and around flames can be diagnosed using infrared emission spectroscopy to observe molecular band shapes and intensities. We have combined this approach with a low-order scanning Fabry-Perot filter and an infrared camera to obtain spectrally scanned infrared emission images of a laboratory flame and exhaust plume from 3.7 to 5.0 micrometers, at a spectral resolution of 0.043 micrometers, and a spatial resolution of 1 mm. The scanning filter or AIRIS (Adaptive Infrared Imaging Spectroradiometer) is a Fabry-Perot etalon operating in low order (mirror spacing = wavelength) such that the central spot, containing a monochromatic image of the scene, is viewed by the detector array. The detection system is a 128 x 128 liquid-nitrogen-cooled InSb focal plane array. The field of view is controlled by a 50 mm focal length multielement lens and an V4.8 aperture, resulting in an image 6.4 x 6.4 cm in extent at the flame and a depth of field of approximately 4 cm. Hyperspectral images above a laboratory CH4/air flame show primarily the strong emission from CO2 at 4.3 micrometers, and weaker emissions from CO and H2O. We discuss techniques to analyze the spectra, and plans to use this instrument in microgravity flame spread experiments.

High-Risk Screening for Fabry Disease: Analysis by Tandem Mass Spectrometry of Globotriaosylceramide (Gb3 ) in Urine Collected on Filter Paper.

PubMed

Auray-Blais, Christiane; Lavoie, Pamela; Boutin, Michel; Abaoui, Mona

2017-04-06

Fabry disease is a complex, panethnic lysosomal storage disorder. It is characterized by the accumulation of glycosphingolipids in tissues, organs, the vascular endothelium, and biological fluids. The reported incidence in different populations is quite variable, ranging from 1:1400 to 1:117,000. Its complexity lies in the marked genotypic and phenotypic heterogeneity. Despite the fact that it is an X-linked disease, more than 600 mutations affect both males and females. In fact, some females may be affected as severely as males. The purpose of this protocol is to focus on the high-risk screening of patients who might have Fabry disease using a simple, rapid, non-invasive high performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) method for urinary globotriaosylceramide (Gb 3 ) analysis. Urine filter paper samples are easily collected at home by patients and sent by regular mail. This method has been successfully used for high-risk screening of patients with ophthalmologic manifestations and in an on-going study for high-risk screening of Fabry disease in patients with chronic kidney diseases. © 2017 by John Wiley & Sons, Inc. Copyright © 2017 John Wiley & Sons, Inc.

Antiproteinuric therapy and Fabry nephropathy: factors associated with preserved kidney function during agalsidase-beta therapy

PubMed Central

Warnock, David G; Thomas, Christie P; Vujkovac, Bojan; Campbell, Ruth C; Charrow, Joel; Laney, Dawn A; Jackson, Leslie L; Wilcox, William R; Wanner, Christoph

2015-01-01

Background Nephropathy is an important feature of classical Fabry disease, which results in alpha-galactosidase A deficiency and cellular globotriaosylceramide accumulation. We report the safety and efficacy of antiproteinuric therapy with ACE inhibitors or angiotensin II receptor blockers (ARBs) in a study of classical Fabry patients receiving recombinant agalsidase-beta therapy. Methods and design The goal was maintenance of urine protein to creatinine ratio (UPCR) <0.5 g/g or a 50% reduction in baseline UPCR for 24 patients at eight study sites. The change in estimated glomerular filtration rate (eGFR) was assessed over 21 months of treatment. Results 18 out of 24 patients achieved the UPCR goal with eGFR slopes that were significantly better than six patients who did not achieve the UPCR goal (−3.6 (−4.8 to −1.1) versus −7.0 (−9.0 to −5.6) mL/min/1.73 m2/year, respectively, p=0.018). Despite achieving the UPCR goal, 67% (12/18 patients) still progressed with an eGFR slope <−2 mL/min/1.73 m2/year. Regression analysis showed that increased age at initiation of agalsidase-beta therapy was significantly associated with worsened kidney outcome. Hypotension and hyperkalaemia occurred in seven and eight patients, respectively, which required modification of antiproteinuric therapy but was not associated with serious adverse events. Conclusions This study documents the effectiveness of agalsidase-beta (1 mg/kg/2 weeks) and antiproteinuric therapy with ACE inhibitors and/or ARB in patients with severe Fabry nephropathy. Patients had preservation of kidney function if agalsidase-beta treatment was initiated at a younger age, and UPCR maintained at or below 0.5 g/g with antiproteinuric therapy. Trial registration number NCT00446862. PMID:26490103

Charles Darwin and the 1835 earthquake at Concepcion, Chile

USGS Publications Warehouse

Spall, H.

1981-01-01

On a stormy night in October 1836, H.M.S Beagle hove to and dropped anchor at Falmouth, a remote harbor in southwest England. Charles Darwin, the ship's naturalist, came ashore to take the mail coach to Shrewsbury. This was inauspicious end to an epic 5-year voyage around the coast of South America, the results of which were to have a tumultuous impact on scientific thought that has lasted to this day. 

The Voyage of the Beagle: Field Work Lessons from Charles Darwin.

ERIC Educational Resources Information Center

Smith, Louis M.

1987-01-01

Analyzes Charles Darwin's letters to his family during his voyage on H.M.S. Beagle. Relates the information to the development of Darwin's professional identity and the degree to which the concepts, field methods, and research methods revealed in Darwin's personal correspondence are useful to students of educational administration. (MD)

[Charles II: From spell to genitourinary pathology].

PubMed

García-Escudero López, Angel; Arruza Echevarría, A; Padilla Nieva, J; Puig Giró, R

2009-04-01

We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with. It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presented an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter's syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract. As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monastery.

Refractive index and absorption detector for liquid chromatography based on Fabry-Perot interferometry

DOEpatents

Yeung, E.S.; Woodruff, S.D.

1984-06-19

A refractive index and absorption detector are disclosed for liquid chromatography. It is based in part on a Fabry-Perot interferometer and is used for the improved detection of refractive index and absorption. It includes a Fabry-Perot interferometer having a normally fixed first partially reflecting mirror and a movable second partially reflecting mirror. A chromatographic flow-cell is positioned between the mirrors along the optical axis of a monochromatic laser beam passing through the interferometer. A means for deriving information about the interference fringes coming out of the interferometer is used with a mini-computer to compute the refractive index of the specimen injected into the flow cell. The minicomputer continuously scans the interferometer for continuous refractive index readings and outputs the continuous results of the scans on a chart recorder. The absorption of the specimen can concurrently be scanned by including a second optical path for an excitation laser which will not interfere with the first laser, but will affect the specimen so that absorption properties can be detected. By first scanning for the refractive index of the specimen, and then immediately adding the excitation laser and subsequently scanning for the refractive index again, the absorption of the specimen can be computed and recorded. 10 figs.

Refractive index and absorption detector for liquid chromatography based on Fabry-Perot interferometry

DOEpatents

Yeung, Edward S.; Woodruff, Steven D.

1984-06-19

A refractive index and absorption detector for liquid chromatography. It is based in part on a Fabry-Perot interferometer and is used for the improved detection of refractive index and absorption. It includes a Fabry-Perot interferometer having a normally fixed first partially reflecting mirror and a movable second partially reflecting mirror. A chromatographic flow-cell is positioned between the mirrors along the optical axis of a monochromatic laser beam passing through the interferometer. A means for deriving information about the interference fringes coming out of the interferometer is used with a mini-computer to compute the refractive index of the specimen injected into the flow cell. The minicomputer continuously scans the interferometer for continuous refractive index readings and outputs the continuous results of the scans on a chart recorder. The absorption of the specimen can concurrently be scanned by including a second optical path for an excitation laser which will not interfere with the first laser, but will affect the specimen so that absorption properties can be detected. By first scanning for the refractive index of the specimen, and then immediately adding the excitation laser and subsequently scanning for the refractive index again, the absorption of the specimen can be computed and recorded.

Modeling of low-finesse, extrinsic fiber optic Fabry-Perot white light interferometers

NASA Astrophysics Data System (ADS)

Ma, Cheng; Tian, Zhipeng; Wang, Anbo

2012-06-01

This article introduces an approach for modeling the fiber optic low-finesse extrinsic Fabry-Pérot Interferometers (EFPI), aiming to address signal processing problems in EFPI demodulation algorithms based on white light interferometry. The main goal is to seek physical interpretations to correlate the sensor spectrum with the interferometer geometry (most importantly, the optical path difference). Because the signal demodulation quality and reliability hinge heavily on the understanding of such relationships, the model sheds light on optimizing the sensor performance.

STS-84 Commander Charles Precourt suits up

NASA Technical Reports Server (NTRS)

1997-01-01

STS-84 Commander Charles J. Precourt adjusts the helmet of his launch and entry suit during final prelaunch preparations in the Operations and Checkout Building. This is Precourts third space flight, but his first as commander. Precourt and six other crew members will depart shortly for Launch Pad 39A, where the Space Shuttle Atlantis awaits liftoff during an approximate 7-minute launch window which opens at about 4:08 a.m. This will be the sixth docking of the Space Shuttle with the Russian Space Station Mir. The exact liftoff time will be determined about 90 minutes prior to launch, based on the most current location of Mir.

STS-91 Commander Charles Precourt participates in CEIT

NASA Technical Reports Server (NTRS)

1998-01-01

STS-91 Commander Charles Precourt inspects the windows of the cockpit from inside of the orbiter Discovery during the Crew Equipment Interface Test, or CEIT, in KSC's Orbiter Processing Facility Bay 2. During CEIT, the crew have an opportunity to get a hands-on look at the payloads with which they'll be working on- orbit. The STS-91 crew are scheduled to launch aboard the Shuttle Discovery for the ninth and final docking with the Russian Space Station Mir from KSC's Launch Pad 39A on May 28 at 8:05 EDT.

STS-71 Pilot Charles J. Precort arrival in T-38

NASA Technical Reports Server (NTRS)

1995-01-01

STS-71 Pilot Charles J. Precourt arrives at the KSC Shuttle Landing Facility in one of the T-38 aircraft traditionally flown by the astronaut corps. The seven STS-71 crew members flew into KSC from Johnson Space Center as final preparations are under way toward the scheduled liftoff on June 23 of the Space Shuttle Atlantis on the first mission to dock with the Russian Space Station Mir. KSC-95EC-870 - Mir 19 Flight Engineer Nikolai M. Budarin arrives at KSC Mir 19 Flight Engineer Nikolai M. Budarin hitches a ride with STS-71 Pilot Charles J. Precourt in a T-38. Budarin, Precourt and the rest of the STS-71 crew arrived at KSC's Shuttle Landing Facility the same day the countdown clock began ticking toward a scheduled liftoff on Friday, June 23. During the historic flight of the Space Shuttle Atlantis on STS- 71, the crew will perform the first U.S. docking with the Russian Space Station Mir. Budarin and Mir 19 Mission Commander Anatoly Solovyev will transfer to Mir during the flight, and the three crew members currently on Mir will return to Earth in the orbiter.

Fiber Fabry-Perot Interferometric Sensor for the Measurement of Electric Current Flowing through a Fuse

NASA Astrophysics Data System (ADS)

Park, Jaehee

2007-06-01

A fiber Fabry-Perot inteferometric sensor bonded close to a fusing element has been studied for the measurement of electric current flowing through a fuse. The phase shift of the sensor output signal is proportional to the square of the electric current passing through the fuse and the sensitivity is 0.827°/mA2.

A sensitive and stable confocal Fabry-Pérot interferometer for surface ultrasonic vibration detection

NASA Astrophysics Data System (ADS)

Ding, Hong-sheng; Tong, Li-ge; Chen, Geng-hua

2001-08-01

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.

Enhanced Bulk-Edge Coulomb Coupling in Fractional Fabry-Perot Interferometers.

PubMed

von Keyserlingk, C W; Simon, S H; Rosenow, Bernd

2015-09-18

Recent experiments use Fabry-Perot (FP) interferometry to claim that the ν=5/2 quantum Hall state exhibits non-Abelian topological order. We note that the experiments appear inconsistent with a model neglecting bulk-edge Coulomb coupling and Majorana tunneling, so we reexamine the theory of FP devices. Even a moderate Coulomb coupling may strongly affect some fractional plateaus, but very weakly affect others, allowing us to model the data over a wide range of plateaus. While experiments are consistent with the ν=5/2 state harboring Moore-Read topological order, they may have measured Coulomb effects rather than an "even-odd effect" due to non-Abelian braiding.

Application of the CCD Fabry-Perot Annular Summing Technique to Thermospheric O(1)D.

NASA Astrophysics Data System (ADS)

Coakley, Monica Marie

1995-01-01

This work will detail the verification of the advantages of the Fabry-Perot charge coupled device (CCD) annular summing technique, the development of the technique for analysis of daysky spectra, and the implications of the resulting spectra for neutral temperature and wind measurements in the daysky thermosphere. The daysky spectral feature of interest is the bright (1 kilo-Rayleigh) thermospheric (OI) emission at 6300 A which had been observed in the nightsky in order to determine winds and temperatures in the vicinity of the altitude of 250 km. In the daysky, the emission line sits on top of a bright Rayleigh scattered continuum background which significantly complicates the observation. With a triple etalon Fabry-Perot spectrometer, the continuum background can be reduced while maintaining high throughput and high resolution. The inclusion of a CCD camera results in significant savings in integration time over the two more standard scanning photomultiplier systems that have made the same wind and temperature measurements in the past. A comparable CCD system can experience an order of magnitude savings in integration time over a PMT system. Laboratory and field tests which address the advantages and limitations of both the Fabry-Perot CCD annular summing technique and the daysky CCD imaging are included in Chap. 2 and Chap. 3. With a sufficiently large throughput associated with the spectrometer and a CCD detector, rapid observations (~4 minute integrations) can be made. Extraction of the line width and line center from the daysky near-continuum background is complicated compared to the nightsky case, but possible. Methods of fitting the line are included in Chap. 4. The daysky O ^1D temperatures are consistent with a lower average emission height than predicted by models. The data and models are discussed in Chap. 5. Although some discrepancies exist between resulting temperatures and models, the observations indicate the potential for other direct measurements

[The North African plague and Charles Nicolle's theory of infectious diseases].

PubMed

Ben, Néfissa Kmar; Moulin, Anne Marie

2010-01-01

Many infectious diseases were described in North Africa in 18th-19th centuries by European travellers. Most of them were allegedly imported by new migrant populations coming from sub-Saharan, European or Middle East countries. Plague outbreaks have been described since the Black Death as diseases of the Mediterranean harbours. Charles Nicolle and his collaborators at the Pasteur Institute were witnesses to the extinction of plague and typhus fever in Tunisia. Both could be considered as endemo-epidemic diseases propagated by ancient nomad communities for centuries. Typhus was exported to other countries; plague was imported by Mediterranean travellers but also hid in unknown wild-animal reservoirs. The role of the bite of a rat's flea was not confirmed and the pneumonic form might have prevailed in the medieval North African cities. Association between plague, typhus, flu and other causes of immune deficiencies could explain the high morbidity and mortality caused by plague in the past. The authors comment the local history of plague at the light of the evolutionary laws of infectious disease proposed by Charles Nicolle in 1930.

Faraday-Active Fabry-Perot Resonator: Transmission, Reflection, and Emissivity

NASA Technical Reports Server (NTRS)

Liptuga, Anatoliy; Morozhenko, Vasyl; Pipa, Viktor; Venger, Evgen; Kostiuk, Theodor

2011-01-01

The propagation of light within a semiconductor Faraday-active Fabry-Perot resonator (FAFR) is investigated theoretically and experimentally. It is shown that an external magnetic field radically changes the angular and spectral characteristics of transmission, reflection and emissivity of the resonator not only for polarized, but also for unpolarized light. Suppression of interference patterns and phase inversion of the interference extrema were observed in both monochromatic and polychromatic light. The investigations were carried out for the plane-parallel plates of n-InAs in the spectral range of free charge carrier absorption. The results can be used to create new controllable optical and spectroscopic devices for investigation of Faraday-active material properties and for control of parameters of plane-parallel layers and structures.

A Fabry-Perot interferometric imaging spectrometer in LWIR

NASA Astrophysics Data System (ADS)

Zhang, Fang; Gao, Jiaobo; Wang, Nan; Wu, Jianghui; Meng, Hemin; Zhang, Lei; Gao, Shan

2017-02-01

With applications ranging from the desktop to remote sensing, the long wave infrared (LWIR) interferometric spectral imaging system is always with huge volume and large weight. In order to miniaturize and light the instrument, a new method of LWIR spectral imaging system based on a variable gap Fabry-Perot (FP) interferometer is researched. With the system working principle analyzed, theoretically, it is researched that how to make certain the primary parameter, such as, wedge angle of interferometric cavity, f-number of the imaging lens and the relationship between the wedge angle and the modulation of the interferogram. A prototype is developed and a good experimental result of a uniform radiation source, a monochromatic source, is obtained. The research shows that besides high throughput and high spectral resolution, the advantage of miniaturization is also simultaneously achieved in this method.

HTS Fabry-Perot resonators for the far infrared

NASA Astrophysics Data System (ADS)

Keller, Philipp; Prenninger, Martin; Pechen, Evgeny V.; Renk, Karl F.

1996-06-01

We report on far infrared (FIR) Fabry-Perot resonators (FPR) with high temperature superconductor (HTS) thin films as mirrors. For the fabrication of FPR we use two parallel MgO plates covered with YBa2Cu3O7-delta thin films on adjacent sides. We have measured the far-infrared transmissivity at 10 K with a Fourier transform infrared spectrometer. Very sharp resonances can be observed for frequencies below 6 THz where the MgO is transparent. The finesse (width of the first order resonance) is comparable to the FPR with metallic meshes as reflectors that are applied in the FIR spectroscopy and astronomy. We have also shown that thin films of gold are not adequate substitute to HTS thin films and not suitable for the fabrication of high-quality FPR due to the ohmic losses.

Utilisation des fibres optiques dans un interférométre stellalre: demonstration en laboratoire des possibilités d'imagerie, étude et conception de lignes à retard métrique

NASA Astrophysics Data System (ADS)

Delage, L.; Simohamed, L. M.; Reynaud, F.

2018-04-01

This paper, "Utilisation des fibres optiques dans un interférométre stellalre: demonstration en laboratoire des possibilités d'imagerie, étude et conception de lignes à retard métrique," was presented as part of International Conference on Space Optics—ICSO 1997, held in Toulouse, France.

Astronaut Charles Duke examines surface of boulder at North Ray crater

NASA Technical Reports Server (NTRS)

1972-01-01

Astronaut Charles M. Duke Jr., lunar module pilot of the Apollo 16 lunar landing mission, examines the surface of a large boulder at North Ray crater during the third Apollo 16 extravehicular activity (EVA-3) at the Descartes landing site. This picture was taken by Astronaut John W. Young, commander. Note the chest-mounted 70mm Hasselblad camera.

33 CFR 80.505 - Cape Henlopen, DE to Cape Charles, VA.

Code of Federal Regulations, 2010 CFR

2010-07-01

... to Cape Charles, VA. (a) A line drawn from the seaward extremity of Indian River Inlet North Jetty to Indian River Inlet South Jetty Light. (b) A line drawn from Ocean City Inlet Light 6, 225° true across Ocean City Inlet to the submerged south breakwater. (c) A line drawn from Assateague Beach Tower Light...

A Fabry-Perot spectrometer for high-resolution observation of the Sun

NASA Astrophysics Data System (ADS)

Kneer, F.; Hirzberger, H.

2001-12-01

Fabry-Perot interferometers (FPIs) are powerful instruments for spectro-polarimetry of the Sun with high spatial resolution. They allow easy image reconstruction of two-dimensional fields of view. Some examples of high quality results obtained with the ``Göttingen'' FPI spectrometer, mounted in the Vacuum Tower Telescope at the Observatorio del Teide/Tenerife, are presented in a poster to this workshop. We thus concentrate on the design of a new instrument for the 1.5 m GREGOR solar telescope. We discuss the pros and cons of telecentric and collimated mounting and describe the expected performance, especially the spectral resolution, of our design.

High precision optical fiber Fabry-Perot sensor for gas pressure detection

NASA Astrophysics Data System (ADS)

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.

Downstream Fabry-Perot interferometer for acoustic wave monitoring in photoacoustic tomography.

PubMed

Nuster, Robert; Gruen, Hubert; Reitinger, Bernhard; Burgholzer, Peter; Gratt, Sibylle; Passler, Klaus; Paltauf, Guenther

2011-03-15

An optical detection setup consisting of a focused laser beam fed into a downstream Fabry-Perot interferometer (FPI) for demodulation of acoustically generated optical phase variations is investigated for its applicability in photoacoustic tomography. The device measures the time derivative of acoustic signals integrated along the beam. Compared to a setup where the detection beam is part of a Mach-Zehnder interferometer, the signal-to-noise ratio of the FPI is lower, but the image quality of the two devices is similar. Using the FPI in a photoacoustic tomograph allows scanning the probe beam around the imaging object without moving the latter.

Fiber Fabry-Perot interferometer sensor for measuring resonances of piezoelectric elements

NASA Astrophysics Data System (ADS)

da Silva, Ricardo E.; Oliveira, Roberson A.; Pohl, Alexandre A. P.

2011-05-01

The development of a fiber extrinsic Fabry-Perot interferometer for measuring vibration amplitude and resonances of piezoelectric elements is reported. The signal demodulation method based on the use of an optical spectrum analyzer allows the measurement of displacements and resonances with high resolution. The technique consists basically in monitoring changes in the intensity or the wavelength of a single interferometric fringe at a point of high sensitivity in the sensor response curve. For sensor calibration, three signal processing techniques were employed. Vibration amplitude measurement with 0.84 nm/V sensitivity and the characterization of the piezo resonance is demonstrated.

Photonic crystal fiber Fabry-Perot interferometers with high-reflectance internal mirrors

NASA Astrophysics Data System (ADS)

Fan, Rong; Hou, Yuanbin; Sun, Wei

2015-06-01

We demonstrated an in-line micro fiber-optic Fabry-Perot interferometer with an air cavity which was created by multi-step fusion splicing a muti-mode photonic crystal fiber (MPCF) to a standard single mode fiber (SMF). The fringe visibility of the interference pattern was up to 20 dB by reshaping the air cavity. Experimental results showed that such a device could be used as a highly sensitive strain sensor with the sensitivity of 4.5 pm/μɛ. Moreover, it offered some other outstanding advantages, such as the extremely compact structure, easy fabrication, low cost, and high accuracy.

Astronaut Joseph Kerwin takes blood sample from Astronaut Charles Conrad

NASA Technical Reports Server (NTRS)

1973-01-01

Scientist-Astronaut Joseph P. Kerwin (right), Skylab 2 science pilot and a doctor of medicine, takes a blood sample from Astronaut Charles Conrad Jr., Sylab 2 commander, as seen in this reproduction taken from a color television transmission made by a TV camera aboard the Skylab 1 and 2 space station cluster in Earth orbit. The blood sampling was part of the Skylab Hematology and Immunology Experiment M110 series.

Modified Fabry-Perot interferometer for displacement measurement in ultra large measuring range

NASA Astrophysics Data System (ADS)

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.

Popular Education in Nineteenth-Century Virginia: The Efforts of Charles Fenton Mercer.

ERIC Educational Resources Information Center

Hunt, Thomas C.

1981-01-01

Although Charles Fenton Mercer's attempts to move 19th-century Virginia to a collectively supported comprehensive system of public education failed, he authored one of the first definitive plans in this country for an organized system of education under control of the state. His contributions are discussed. (RM)

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

PubMed

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 P<0.001, respectively), and higher frequencies of gastrointestinal pain occurred during follow-up. In conclusion, after 2 years of observation, all groups showed a stable clinical disease course with respect to serious clinical events. However, patients under agalsidase-β dose-reduction and switch or a direct switch to agalsidase-α showed a decline of renal function independent of the eGFR formula used. Copyright © 2016 by the American Society of Nephrology.

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

PubMed Central

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

2016-01-01

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 P<0.001, respectively), and higher frequencies of gastrointestinal pain occurred during follow-up. In conclusion, after 2 years of observation, all groups showed a stable clinical disease course with respect to serious clinical events. However, patients under agalsidase-β dose-reduction and switch or a direct switch to agalsidase-α showed a decline of renal function independent of the eGFR formula used. PMID:26185201

Charles Theodore Dotter: The Father of Intervention

PubMed Central

Payne, Misty M.

2001-01-01

The 1st percutaneous transluminal angioplasty marked a new era in the treatment of peripheral atherosclerotic lesions. The early techniques used in peripheral percutaneous transluminal angioplasty form the basis for subsequent percutaneous intervention both in the peripheral and coronary arteries and are largely the contribution of Charles Dotter. Dotter was the 1st to describe flow-directed balloon catheterization, the double-lumen balloon catheter, the safety guidewire, percutaneous arterial stenting, and more. This practical genius dedicated his considerable energy to the belief that there is always a better way to treat disease. His personal contributions to clinical medicine, research, and teaching have saved millions of limbs and lives all over the world. PMID:11330737

STS-84 Commander Charles Precourt arriving for TCDT

NASA Technical Reports Server (NTRS)

1997-01-01

STS-84 Commander Charles J. Precourt arrives at KSCs Shuttle Landing Facility for the Terminal Countdown Demonstration Test (TCDT), a dress rehearsal for launch. Precourt will lead the six other crew members when they travel to Mir next month aboard the Space Shuttle Atlantis. STS-84 Mission Specialist C. Michael Foale will be dropped off on Mir to become a member of the Mir 23 crew, replacing U.S. astronaut Jerry M. Linenger, who will return to Earth on Atlantis after about four months on the orbiting station. STS-84 will be the sixth Shuttle-Mir docking. Liftoff is targeted for May 15.

VTT's Fabry-Perot interferometer technologies for hyperspectral imaging and mobile sensing applications

NASA Astrophysics Data System (ADS)

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.

Fiber Fabry-Perot sensors for detection of partial discharges in power transformers.

PubMed

Yu, Bing; Kim, Dae Woong; Deng, Jiangdong; Xiao, Hai; Wang, Anbo

2003-06-01

A diaphragm-based interferometric fiberoptic sensor that uses a low-coherence light source was designed and tested for on-line detection of the acoustic waves generated by partial discharges inside high-voltage power transformers. The sensor uses a fused-silica diaphragm and a single-mode optical fiber encapsulated in a fused-silica glass tube to form an extrinsic Fabry-Perot interferometer, which is interrogated by low-coherence light. Test results indicate that these fiber optic acoustic sensors are capable of faithfully detecting acoustic signals propagating inside transformer oil with high sensitivity and wide bandwidth.

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

NASA Astrophysics Data System (ADS)

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

2012-02-01

High efficiency reflecting volume Bragg gratings (VBGs) recorded in PTR glass plates have shown un-preceded performances that make them very good candidates for narrowband spectral filtering with sub-nanometer spectral widths. However, decreasing the bandwidth to value below 30-50 pm is very challenging as it requires increasing the thickness of the RBG to more than 15-20 mm. To overcome this limitation, we propose a new approach which is a monolithic Fabry-Perot cavity which consists from a reflecting VBG with a multilayer dielectric mirror (MDM) deposited on its surface. A VBG with a grating vector perpendicular to its surface and a MDM produce a Fabry-Perot resonator with a single transmission band inside of the reflection spectrum of the VBG. We present a theoretical description of this new class of filters that allow achieving a single ultra-narrowband resonance associated with several hundred nanometers rejection band. Then we show the methods for designing and fabricating such filter. Finally, we present the steps that we followed in order to fabricate a first prototype for 852 nm and 1062 nm region that demonstrates a 30 pm bandwidth, 90+% transmission at resonance and a good agreement with theoretical simulation.

Dublin and Irish politics in the age of Charles Lucas.

PubMed

Hill, J

2015-09-01

In addition to his contributions to medicine, Charles Lucas had a long career in politics, starting in the 1740s as a guild representative on the lower house of Dublin corporation, and culminating in his election to the Irish House of Commons in 1761. By examining the background in Dublin and Irish politics, this paper explores Lucas' impact on the electorate, and how it was that he was able to win a parliamentary seat in Dublin and retain it for a decade while he campaigned in support of a range of important Patriot issues. Lucas had none of the qualifications that would normally be required for a successful politician. His father held some land, but as a younger son who had to make a living, Charles was apprenticed to a Dublin apothecary. Nor did he have the political connections that might have compensated for a lack of land, wealth, or status. But Lucas possessed other advantages, notably an education that enabled him to read the city's medieval charters, identifying areas where the Dublin freemen had lost 'ancient rights', and some experience of publishing, so that he could appeal to the electorate. Lucas' remarkable political success stemmed from both local circumstances and his own personal qualities.

Advertising eugenics: Charles M. Goethe's campaign to improve the race.

PubMed

Schoenl, William; Peck, Danielle

2010-06-01

Over the last several decades historians have shown that the eugenics movement appealed to an extraordinarily wide constituency. Far from being the brainchild of the members of any one particular political ideology, eugenics made sense to a diverse range of Americans and was promoted by professionals ranging from geneticists and physicians to politicians and economists.(1) Seduced by promises of permanent fixes to national problems, and attracted to the idea of a scientifically legitimate form of social activism, eugenics quickly grew in popularity during the first decades of the twentieth century. Charles M. Goethe, the land developer, entrepreneur, conservationist and skilled advertiser who founded the Eugenics Society of Northern California, exemplifies the broad appeal of the eugenics movement. Goethe played an active role within the American eugenics movement at its peak in the 1920s. The last president of the Eugenics Research Association,(2) he also campaigned hard against Mexican immigration to the US and he continued open support for the Nazi regime's eugenic practices into the later 1930s.(3) This article examines Goethe's eugenic vision and, drawing on his correspondence with the leading geneticist Charles Davenport, explores the relationship between academic and non-academic advocates of eugenics in America. Published by Elsevier Ltd.

High quality factor surface Fabry-Perot cavity of acoustic waves

NASA Astrophysics Data System (ADS)

Xu, Yuntao; Fu, Wei; Zou, Chang-ling; Shen, Zhen; Tang, Hong X.

2018-02-01

Surface acoustic wave (SAW) resonators are critical components in wireless communications and many sensing applications. They have also recently emerged as a subject of study in quantum acoustics at the single phonon level. Acoustic loss reduction and mode confinement are key performance factors in SAW resonators. Here, we report the design and experimental realization of high quality factor Fabry-Perot SAW resonators formed in between the tapered phononic crystal mirrors patterned on a GaN-on-sapphire material platform. The fabricated SAW resonators are characterized by both an electrical network analyzer and an optical heterodyne vibrometer. We observed standing Rayleigh waves inside the cavity, with an intrinsic quality factor exceeding 1.3 × 104 at ambient conditions.

75 FR 41376 - Safety Zone; Fireworks Display, Potomac River, Charles County, MD

Federal Register 2010, 2011, 2012, 2013, 2014

2010-07-16

...-AA00 Safety Zone; Fireworks Display, Potomac River, Charles County, MD AGENCY: Coast Guard, DHS. ACTION... this temporary rule, call or e-mail Mr. Ronald L. Houck, Sector Baltimore Waterways Management Division, Coast Guard; telephone 410-576-2674, e- mail Ronald.L[email protected] . If you have questions on viewing...

School Renovation and the Importance of Maintenance. [Interview with Charles Boney, Jr.].

ERIC Educational Resources Information Center

Butterfield, Eric

Charles Boney, Jr., Director of Boney Architects, offers his views on school districts that build schools that have to be replaced too soon, general problems concerning renovation of older buildings, and keeping maintenance costs down. He discusses the types of flooring materials school districts should consider using to lower maintenance costs…

Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: assessing cardiac effects of long-term enzyme replacement therapy.

PubMed

Messalli, G; Imbriaco, M; Avitabile, G; Russo, R; Iodice, D; Spinelli, L; Dellegrottaglie, S; Cademartiri, F; Salvatore, M; Pisani, A

2012-02-01

Anderson-Fabry disease is a multisystemic disorder of lipid metabolism secondary to X-chromosome alterations and is frequently associated with cardiac manifestations such as left ventricular (LV) hypertrophy, gradually leading to an alteration in cardiac performance. The purpose of this study was to monitor, using magnetic resonance imaging (MRI), any changes produced by enzyme replacement therapy with agalsidase beta at the cardiac level in patients with Anderson-Fabry disease. Sixteen (ten men, six women) patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI before starting enzyme replacement therapy (baseline study) and after 48 months of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). After 48 months of treatment, a significant reduction in LV mass and wall thickness was observed: 187±59 g vs. 149±44 g, and 16±3 mm vs. 13±3 mm, respectively. A significant reduction in T2 relaxation time was noted at the level of the interventricular septum (81±3 ms vs. 67±7 ms), at the apical level (80±8 ms vs. 63±6 ms) and at the level of the lateral wall (82±8 ms vs. 63±10 ms) (p<0.05). No significant variation was observed in ejection fraction between the two studies (65±3% vs. 64±2%; p>0.05) (mean bias 1.0); however, an improvement was noted in the New York Heart Association (NYHA) class of the majority of patients (12/16) (p<0.05). In patients with Anderson-Fabry disease undergoing enzyme replacement therapy with agalsidase beta, MRI documented a significant reduction in myocardial T2 relaxation time, a significant decrease in maximal myocardial thickness and in total LV mass. MRI did not reveal significant improvements in LV global systolic function; however, improvement in NYHA functional class was noted, consistent with improved diastolic function.

Charles C. Fries in Japan: A Case Study in Methodological Reform.

ERIC Educational Resources Information Center

Henrichsen, Lynn E.

The success of Charles C. Fries' Oral Approach to teaching English as a second language (ESL) as promoted in Japan by the English Language Exploratory Committee in the late 1950s and early 1960s is examined according to Jack Richards' four major factors that affect the course of a teaching method: appeals to facts; appeals to authority; form of…

A Lifetime of Language Testing: An Interview with J. Charles Alderson

ERIC Educational Resources Information Center

Brunfaut, Tineke

2014-01-01

Professor J. Charles Alderson grew up in the town of Burnley, in the North-West of England, and is still based in the North West but in the ancient city of Lancaster. From Burnley to Lancaster, however, lies a journey and a career that took him all around the world to share his knowledge, skills, and experience in language testing and to learn…

Enzyme replacement therapy in a patient with Fabry disease and the development of IgE antibodies against agalsidase beta but not agalsidase alpha.

PubMed

Tanaka, Akemi; Takeda, Taisuke; Hoshina, Takao; Fukai, Kazuyoshi; Yamano, Tsunekazu

2010-12-01

Fabry disease is an X-linked inherited lysosomal storage disorder caused by an inborn deficiency of the enzyme α-galactosidase A. Enzyme replacement therapy (ERT) with agalsidase alpha or beta isozymes is an effective treatment. Cross-reactivity of immunoglobulin G (IgG) antibodies with agalsidase alpha and beta has been reported, but no such reaction has been recorded for IgE antibodies. We present the case of a patient with Fabry disease who developed antiagalsidase beta IgE antibodies without cross-reactivity to agalsidase alpha. A 17-year-old boy with Fabry disease had suffered from severe atopic dermatitis since infancy, and he complained for several years of peripheral pain during the summer months and when exercising. Fabry disease was confirmed by family history and a positive enzyme test, and ERT was commenced. Following infusion of agalsidase beta (1.0 mg/kg), the patient complained of a high temperature in his hands and feet, and purulent eczema developed. The infusion dose was reduced to 0.2 mg/kg, but the hyperthermia did not change, although its duration decreased. After three infusions, eosinophilia developed (9.4%; 573 cells/μl blood) and remained unresolved after four infusions with agalsidase beta. Treatment with this enzyme was discontinued, and agalsidase alpha (0.2 mg/kg) started. This produced immediate resolution of the eosinophilia, which has been maintained during follow-up. In conclusion, this patient developed IgE antibodies against agalsidase beta, which demonstrated no cross-reactivity to agalsidase alpha. These findings emphasize the importance of analyzing IgE antibodies against both enzymes when patients exhibit severe infusion-related events.

Proton and gamma irradiation of Fabry-Perot quantum cascade lasers for space qualification

DOE PAGES

Myers, Tanya L.; Cannon, Bret D.; Brauer, Carolyn S.; ...

2015-01-20

Fabry-Perot quantum cascade lasers (QCLs) were characterized following irradiation by high energy (64 MeV) protons and Cobalt-60 gamma rays. Seven QCLs were exposed to radiation dosages that are typical for a space mission in which the total accumulated dosages from both radiation sources varied from 20 krad(Si) to 46.3 krad(Si). In conclusion, the QCLs did not show any measurable changes in threshold current or slope efficiency suggesting the suitability of QCLs for use in space-based missions.

Evaluation of a Magneto-optical Filter and a Fabry-perot Interferometer for the Measurement of Solar Velocity Fields from Space

NASA Technical Reports Server (NTRS)

Rhodes, E. J., Jr.; Cacciani, A.; Blamont, J.; Tomczyk, S.; Ulrich, R. K.; Howard, R. F.

1984-01-01

A program was developed to evaluate the performance of three different devices as possible space-borne solar velocity field imagers. Two of these three devices, a magneto-optical filter and a molecular adherence Fabry-Perot interferometer were installed in a newly-constructed observing system located at the 60-foot tower telescope at the Mt. Wilson Observatory. Time series of solar filtergrams and Dopplergrams lasting up to 10 hours per day were obtained with the filter while shorter runs were obtained with the Fabry-Perot. Two-dimensional k (sub h)-omega power spectra which show clearly the well-known p-mode ridges were computed from the time series obtained with the magneto-optical filter. These power spectra were compared with similar power spectra obtained recently with the 13.7-m McMath spectrograph at Kitt Peak.

Transversely coupled Fabry-Perot resonators with Bragg grating reflectors.

PubMed

Saber, Md Ghulam; Wang, Yun; El-Fiky, Eslam; Patel, David; Shahriar, Kh Arif; Alam, Md Samiul; Jacques, Maxime; Xing, Zhenping; Xu, Luhua; Abadía, Nicolás; Plant, David V

2018-01-01

We design and demonstrate Fabry-Perot resonators with transverse coupling using Bragg gratings as reflectors on the silicon-on-insulator (SOI) platform. The effects of tailoring the cavity length and the coupling coefficient of the directional coupler on the spectral characteristics of the device are studied. The fabricated resonators achieved an extinction ratio (ER) of 37.28 dB and a Q-factor of 3356 with an effective cavity length of 110 μm, and an ER of 8.69 dB and a Q-factor of 23642 with a 943 μm effective cavity length. The resonator structure presented here has the highest reported ER on SOI and provides additional degrees of freedom compared to an all-pass ring resonator to tune the spectral characteristics.

Mutant α-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin

PubMed Central

Ishii, Satoshi; Chang, Hui-Hwa; Kawasaki, Kunito; Yasuda, Kayo; Wu, Hui-Li; Garman, Scott C.; Fan, Jian-Qiang

2007-01-01

Fabry disease is a lysosomal storage disorder caused by the deficiency of α-Gal A (α-galactosidase A) activity. In order to understand the molecular mechanism underlying α-Gal A deficiency in Fabry disease patients with residual enzyme activity, enzymes with different missense mutations were purified from transfected COS-7 cells and the biochemical properties were characterized. The mutant enzymes detected in variant patients (A20P, E66Q, M72V, I91T, R112H, F113L, N215S, Q279E, M296I, M296V and R301Q), and those found mostly in mild classic patients (A97V, A156V, L166V and R356W) appeared to have normal Km and Vmax values. The degradation of all mutants (except E59K) was partially inhibited by treatment with kifunensine, a selective inhibitor of ER (endoplasmic reticulum) α-mannosidase I. Metabolic labelling and subcellular fractionation studies in COS-7 cells expressing the L166V and R301Q α-Gal A mutants indicated that the mutant protein was retained in the ER and degraded without processing. Addition of DGJ (1-deoxygalactonojirimycin) to the culture medium of COS-7 cells transfected with a large set of missense mutant α-Gal A cDNAs effectively increased both enzyme activity and protein yield. DGJ was capable of normalizing intracellular processing of mutant α-Gal A found in both classic (L166V) and variant (R301Q) Fabry disease patients. In addition, the residual enzyme activity in fibroblasts or lymphoblasts from both classic and variant hemizygous Fabry disease patients carrying a variety of missense mutations could be substantially increased by cultivation of the cells with DGJ. These results indicate that a large proportion of mutant enzymes in patients with residual enzyme activity are kinetically active. Excessive degradation in the ER could be responsible for the deficiency of enzyme activity in vivo, and the DGJ approach may be broadly applicable to Fabry disease patients with missense mutations. PMID:17555407

Antiproteinuric therapy and Fabry nephropathy: factors associated with preserved kidney function during agalsidase-beta therapy.

PubMed

Warnock, David G; Thomas, Christie P; Vujkovac, Bojan; Campbell, Ruth C; Charrow, Joel; Laney, Dawn A; Jackson, Leslie L; Wilcox, William R; Wanner, Christoph

2015-12-01

Nephropathy is an important feature of classical Fabry disease, which results in alpha-galactosidase A deficiency and cellular globotriaosylceramide accumulation. We report the safety and efficacy of antiproteinuric therapy with ACE inhibitors or angiotensin II receptor blockers (ARBs) in a study of classical Fabry patients receiving recombinant agalsidase-beta therapy. The goal was maintenance of urine protein to creatinine ratio (UPCR) <0.5 g/g or a 50% reduction in baseline UPCR for 24 patients at eight study sites. The change in estimated glomerular filtration rate (eGFR) was assessed over 21 months of treatment. 18 out of 24 patients achieved the UPCR goal with eGFR slopes that were significantly better than six patients who did not achieve the UPCR goal (-3.6 (-4.8 to -1.1) versus -7.0 (-9.0 to -5.6) mL/min/1.73 m(2)/year, respectively, p=0.018). Despite achieving the UPCR goal, 67% (12/18 patients) still progressed with an eGFR slope <-2 mL/min/1.73 m(2)/year. Regression analysis showed that increased age at initiation of agalsidase-beta therapy was significantly associated with worsened kidney outcome. Hypotension and hyperkalaemia occurred in seven and eight patients, respectively, which required modification of antiproteinuric therapy but was not associated with serious adverse events. This study documents the effectiveness of agalsidase-beta (1 mg/kg/2 weeks) and antiproteinuric therapy with ACE inhibitors and/or ARB in patients with severe Fabry nephropathy. Patients had preservation of kidney function if agalsidase-beta treatment was initiated at a younger age, and UPCR maintained at or below 0.5 g/g with antiproteinuric therapy. NCT00446862. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Point of View: Waving to Ray Charles--Missing the Meaning of Disabilities

ERIC Educational Resources Information Center

Kauffman, James M.

2005-01-01

The author believes that some statements about education are as silly as waving to Ray Charles or another person who is blind. In particular, some of the statements about closing the achievement gap between students with disabilities and those without disabilities are extremely so. Educators need to demand that statements about education make…

Fabry-Perot Interferometer for Column CO2

NASA Technical Reports Server (NTRS)

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.

A Fabry-Perot Spectrometer for High-Resolution Observation of the Sun

NASA Astrophysics Data System (ADS)

Kneer, F.; Hirzberger, J.

Fabry-Perot interferometers (FPIs) are powerful instruments for spectro-polarimetry of the Sun with high spatial resolution. They allow easy image reconstruction of two-dimensional narrow-band fields of view. Some examples of high quality results obtained with the ``Göttingen'' FPI spectrometer, mounted in the Vacuum Tower Telescope at the Observatorio del Teide/Tenerife, are presented in a poster to this workshop. We thus concentrate on the design of a new instrument for the 1.5 m GREGOR solar telescope. We discuss the pros and cons of telecentric and collimated mounting and describe the expected performance, especially the spectral resolution, of our design.

Multimode fiber tip Fabry-Perot cavity for highly sensitive pressure measurement.

PubMed

Chen, W P; Wang, D N; Xu, Ben; Zhao, C L; Chen, H F

2017-03-23

We demonstrate an optical Fabry-Perot interferometer fiber tip sensor based on an etched end of multimode fiber filled with ultraviolet adhesive. The fiber device is miniature (with diameter of less than 60 μm), robust and low cost, in a convenient reflection mode of operation, and has a very high gas pressure sensitivity of -40.94 nm/MPa, a large temperature sensitivity of 213 pm/°C within the range from 55 to 85 °C, and a relatively low temperature cross-sensitivity of 5.2 kPa/°C. This device has a high potential in monitoring environment of high pressure.

Dryden's David Bushman explains the capabilities of the Altus UAV to NASA Langley's Charles Hudgins

NASA Image and Video Library

2003-05-27

David Bushman, unmanned aerial vehicle (UAV) mission manager in NASA Dryden's Airborne Science Program, explains the capabilities of the Altus UAV to Charles Hudgins of NASA Langley's Chemistry and Dynamics Branch.

Charles Bachman Moore (1920-2010)

NASA Astrophysics Data System (ADS)

Winn, William; Krehbiel, Paul

2011-02-01

Charles B. Moore passed away 2 March 2010 at the age of 89, following a long and varied scientific career in meteorology and the atmospheric sciences. He will be remembered best for his substantial contributions in the field of atmospheric electricity and for the students and faculty he guided as chairman of Langmuir Laboratory for Atmospheric Research and professor of physics at the New Mexico Institute of Mining and Technology. He possessed a unique sense of humor and an excellent memory that served as a reservoir of scientific and historical knowledge. Like many of his generation, Charlie's career was profoundly influenced by the Second World War. Following Pearl Harbor, he interrupted his undergraduate studies in chemical engineering at Georgia Institute of Technology to enlist in the Army Air Corps, where he became the chief weather equipment officer in the 10th Weather Squadron, setting up and operating remote meteorological stations behind enemy lines in the China-Burma-India theater. He served with distinction alongside Athelstan Spilhaus Sr., who had been one of Charlie's instructors in the Army meteorology program.

Astronaut Charles Duke photographed collecting lunar samples at Station 1

NASA Technical Reports Server (NTRS)

1972-01-01

Astronaut Charles M. Duke Jr., lunar module pilot of the Apollo 16 lunar landing mission, is photographed collecting lunar samples at Station no. 1 during the first Apollo 16 extravehicular activity at the Descartes landing site. This picture, looking eastward, was taken by Astronaut John W. Young, commander. Duke is standing at the rim of Plum crater, which is 40 meters in diameter and 10 meters deep. The parked Lunar Roving Vehicle can be seen in the left background.

Manfred Girbardt and Charles Bracker: outstanding pioneers in fungal microscopy.

PubMed

Bartnicki-Garcia, Salomon

2015-01-01

Midway through the twentieth century, the availability of new and improved optical and electronic microscopes facilitated rapid advances in the elucidation of the fine structure of fungal cells. In this Essay, I pay tribute to Manfred Girbardt (1919-1991) and Charles Bracker (1938-2012)—two individuals who, despite being separated by geography and the restrictions of the Cold War, both made equally fundamental discoveries in fungal cell ultrastructure and set high standards for specimen manipulation and image processing.

Astronaut Charles Conrad poses in shower facility in crew quarters

NASA Technical Reports Server (NTRS)

1973-01-01

Astronaut Charles Conrad Jr., Skylab 2 commander, smiles for the camera after a hot bath in the shower facility in the crew quarters of the Orbital Workshop of the Skylab 2 space station cluster in Earth orbit. In deploying the shower facility the shower curtain is pulled up from the floor and attached to the ceiling. The water comes through a push-button shower head attached to a flexible hose. Water is drawn off by a vacuum system.

Fabry disease: multidisciplinary evaluation after 10 years of treatment with agalsidase Beta.

PubMed

Juan, Politei; Hernan, Amartino; Beatriz, Schenone Andrea; Gustavo, Cabrera; Antonio, Michref; Eduardo, Tanus; Raul, Dominguez; Margarita, Larralde; Mariana, Blanco; Daniela, Gaggioli; Marina, Szlago

2014-01-01

Fabry disease is an X linked disorder of metabolism due to deficient α-galactosidase A activity. Enzyme replacement therapy (ERT) with agalsidase Beta was approved by EMA in 2001 and FDA in 2003. Six patients were enrolled. Baseline data was measured for renal, cardiac, and cerebrovascular functioning. We compared baseline quality of life scales with the current results. These parameters were assessed during the 10 years of follow-up period. Before ERT four patients showed normal eGFR, one stage 2 of CKD, and one hyperfiltration stage. All presented microalbuminuria and just two cases showed proteinuria. After 10 years of ERT, no patient showed decrease in renal functioning. One patient decreased from proteinuria to microalbuminuria range. Before treatment one case showed left ventricular (LV) hypertrophy and LV Mass Index was abnormal in two female patients. After 10 years echocardiographic values did not present progression to LVH and one female showed regression to normal values of LV posterior wall and interventricular septum. Brain MRI showed ischemic lesions in one female and vertebrobasilar dolichoectasia in one male. From baseline and during the follow-up period MRI did not progress to new ischemic lesions and there were no clinical signs of cerebrovascular damage. After 10 years quality of life showed improvement in all domains measured. Early treatment of agalsidase Beta is related to a better outcome regarding stability and regression of signs and symptoms in Fabry disease. Our results in patients with mild organ involvement showed good outcomes and support an early and continuous ERT.

A polymer-based Fabry-Perot filter integrated with 3-D MEMS structures

NASA Astrophysics Data System (ADS)

Zhang, Ping (Cerina); Le, Kevin; Malalur-Nagaraja-Rao, Smitha; Hsu, Lun-Chen; Chiao, J.-C.

2006-01-01

Polymers have been considered as one of the most versatile materials in making optical devices for communication and sensor applications. They provide good optical transparency to form filters, lenses and many optical components with ease of fabrication. They are scalable and compatible in dimensions with requirements in optics and can be fabricated on inorganic substrates, such as silicon and quartz. Recent polymer synthesis also made great progresses on conductive and nonlinear polymers, opening opportunities for new applications. In this paper, we discussed hybrid-material integration of polymers on silicon-based microelectromechanical system (MEMS) devices. The motivation is to combine the advantages of demonstrated silicon-based MEMS actuators and excellent optical performance of polymers. We demonstrated the idea with a polymer-based out-of-plane Fabry-Perot filter that can be self-assembled by scratch drive actuators. We utilized a fabrication foundry service, MUMPS (Multi-User MEMS Process), to demonstrate the feasibility and flexibility of integration. The polysilicon, used as the structural material for construction of 3-D framework and actuators, has high absorption in the visible and near infrared ranges. Therefore, previous efforts using a polysilicon layer as optical interfaces suffer from high losses. We applied the organic compound materials on the silicon-based framework within the optical signal propagation path to form the optical interfaces. In this paper, we have shown low losses in the optical signal processing and feasibility of building a thin-film Fabry-Perot filter. We discussed the optical filter designs, mechanical design, actuation mechanism, fabrication issues, optical measurements, and results.

Renal outcomes of agalsidase beta treatment for Fabry disease: role of proteinuria and timing of treatment initiation

PubMed Central

Ortiz, Alberto; Mauer, Michael; Linthorst, Gabor E.; Oliveira, João P.; Serra, Andreas L.; Maródi, László; Mignani, Renzo; Vujkovac, Bojan; Beitner-Johnson, Dana; Lemay, Roberta; Cole, J.Alexander; Svarstad, Einar; Waldek, Stephen; Germain, Dominique P.; Wanner, Christoph

2012-01-01

Background. The purpose of this study was to identify determinants of renal disease progression in adults with Fabry disease during treatment with agalsidase beta. Methods. Renal function was evaluated in 151 men and 62 women from the Fabry Registry who received agalsidase beta at an average dose of 1 mg/kg/2 weeks for at least 2 years. Patients were categorized into quartiles based on slopes of estimated glomerular filtration rate (eGFR) during treatment. Multivariate logistic regression analyses were used to identify factors associated with renal disease progression. Results. Men within the first quartile had a mean eGFR slope of –0.1 mL/min/1.73m2/year, whereas men with the most rapid renal disease progression (Quartile 4) had a mean eGFR slope of –6.7 mL/min/1.73m2/year. The risk factor most strongly associated with renal disease progression was averaged urinary protein:creatinine ratio (UP/Cr) ≥1 g/g (odds ratio 112, 95% confidence interval (95% CI) 4–3109, P = 0.0054). Longer time from symptom onset to treatment was also associated with renal disease progression (odds ratio 19, 95% CI 2–184, P = 0.0098). Women in Quartile 4 had the highest averaged UP/Cr (mean 1.8 g/g) and the most rapid renal disease progression: (mean slope –4.4 mL/min/1.73m2/year). Conclusions. Adults with Fabry disease are at risk for progressive loss of eGFR despite enzyme replacement therapy, particularly if proteinuria is ≥1 g/g. Men with little urinary protein excretion and those who began receiving agalsidase beta sooner after the onset of symptoms had stable renal function. These findings suggest that early intervention may lead to optimal renal outcomes. PMID:21804088

Renal outcomes of agalsidase beta treatment for Fabry disease: role of proteinuria and timing of treatment initiation.

PubMed

Warnock, David G; Ortiz, Alberto; Mauer, Michael; Linthorst, Gabor E; Oliveira, João P; Serra, Andreas L; Maródi, László; Mignani, Renzo; Vujkovac, Bojan; Beitner-Johnson, Dana; Lemay, Roberta; Cole, J Alexander; Svarstad, Einar; Waldek, Stephen; Germain, Dominique P; Wanner, Christoph

2012-03-01

The purpose of this study was to identify determinants of renal disease progression in adults with Fabry disease during treatment with agalsidase beta. Renal function was evaluated in 151 men and 62 women from the Fabry Registry who received agalsidase beta at an average dose of 1 mg/kg/2 weeks for at least 2 years. Patients were categorized into quartiles based on slopes of estimated glomerular filtration rate (eGFR) during treatment. Multivariate logistic regression analyses were used to identify factors associated with renal disease progression. Men within the first quartile had a mean eGFR slope of -0.1 mL/min/1.73m(2)/year, whereas men with the most rapid renal disease progression (Quartile 4) had a mean eGFR slope of -6.7 mL/min/1.73m(2)/year. The risk factor most strongly associated with renal disease progression was averaged urinary protein:creatinine ratio (UP/Cr) ≥1 g/g (odds ratio 112, 95% confidence interval (95% CI) 4-3109, P = 0.0054). Longer time from symptom onset to treatment was also associated with renal disease progression (odds ratio 19, 95% CI 2-184, P = 0.0098). Women in Quartile 4 had the highest averaged UP/Cr (mean 1.8 g/g) and the most rapid renal disease progression: (mean slope -4.4 mL/min/1.73m(2)/year). Adults with Fabry disease are at risk for progressive loss of eGFR despite enzyme replacement therapy, particularly if proteinuria is ≥1 g/g. Men with little urinary protein excretion and those who began receiving agalsidase beta sooner after the onset of symptoms had stable renal function. These findings suggest that early intervention may lead to optimal renal outcomes.

Gold medal award for life achievement in the practice of psychology: Charles Silverstein.

PubMed

2011-01-01

The American Psychological Foundation (APF) Gold Medal Awards recognize distinguished and enduring records of accomplishment in four areas of psychology: the application of psychology, the practice of psychology, psychology in the public interest, and the science of psychology. The 2011 recipient of the Gold Medal Award for Life Achievement in the Practice of Psychology is Charles Silverstein. Dorothy W. Cantor, president of the APF, will present the APF Gold Medal Awards at the 119th Annual Convention of the American Psychological Association on August 5, 2011, at 4:00 p.m. Members of the 2011 APF Board of Trustees are Dorothy W. Cantor, president; William Howell, vice president/secretary; Gerald Koocher, treasurer; Elisabeth R. Straus, executive vice president/executive director; Norman Anderson; David H. Barlow, Camilla Benbow; Sharon Stephens Brehm; Charles L. Brewer; Connie Chan; Anthony Jackson; Ronald F. Levant; Sandra Shullman; Archie L. Turner; and Kurt Geisinger, APA Board of Directors liaison.

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

PubMed

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

2017-10-01

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.

Enzyme therapy in Fabry disease: severe adverse events associated with anti-agalsidase cross-reactive IgG antibodies.

PubMed

Tesmoingt, Chloe; Lidove, Olivier; Reberga, Axele; Thetis, Marguerite; Ackaert, Chloe; Nicaise, Pascale; Arnaud, Philippe; Papo, Thomas

2009-11-01

To report a severe adverse event related to enzyme replacement therapy with agalsidase in an hemizygous male patient treated for Fabry disease. Retrospective analysis of clinical, radiological and biochemical data in a patient who suffered adverse events related to both agalsidase alfa and agalsidase beta treatments. A hemizygous male patient was first treated for Fabry disease with agalsidase alfa. After more than 1 year of therapy, infusion-related symptoms necessitated systemic steroids and antihistaminic therapy. Decline in kidney function prompted a switch for agalsidase beta. Anaphylactoid shock occurred after the second infusion. No serum IgE antibodies were disclosed. Skin-test reactivity to agalsidase beta was negative. Following a published rechallenge infusion protocol, agalsidase beta was reintroduced, leading to a second anaphylactoid shock episode. Enzyme replacement therapy was stopped and the patient was treated with symptomatic therapy only. This case was referred to the pharmacovigilance department. The negativity of immunological tests (specific anti-agalsidase IgE antibodies and skin tests) does not rule out the risk of repeated anaphylactoid shock following agalsidase infusion.

Enzyme therapy in Fabry disease: severe adverse events associated with anti-agalsidase cross-reactive IgG antibodies

PubMed Central

Tesmoingt, Chloe; Lidove, Olivier; Reberga, Axele; Thetis, Marguerite; Ackaert, Chloe; Nicaise, Pascale; Arnaud, Philippe; Papo, Thomas

2009-01-01

AIMS To report a severe adverse event related to enzyme replacement therapy with agalsidase in an hemizygous male patient treated for Fabry disease. METHODS Retrospective analysis of clinical, radiological and biochemical data in a patient who suffered adverse events related to both agalsidase alfa and agalsidase beta treatments. RESULTS A hemizygous male patient was first treated for Fabry disease with agalsidase alfa. After more than 1 year of therapy, infusion-related symptoms necessitated systemic steroids and antihistaminic therapy. Decline in kidney function prompted a switch for agalsidase beta. Anaphylactoid shock occurred after the second infusion. No serum IgE antibodies were disclosed. Skin-test reactivity to agalsidase beta was negative. Following a published rechallenge infusion protocol, agalsidase beta was reintroduced, leading to a second anaphylactoid shock episode. Enzyme replacement therapy was stopped and the patient was treated with symptomatic therapy only. This case was referred to the pharmacovigilance department. CONCLUSION The negativity of immunological tests (specific anti-agalsidase IgE antibodies and skin tests) does not rule out the risk of repeated anaphylactoid shock following agalsidase infusion. PMID:19917001

Enzyme replacement therapy in patients with Fabry disease: state of the art and review of the literature.

PubMed

Pisani, Antonio; Visciano, Bianca; Roux, Graciana Diez; Sabbatini, Massimo; Porto, Caterina; Parenti, Giancarlo; Imbriaco, Massimo

2012-11-01

Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency of the hydrolytic enzyme alpha galactosidase A, with consequent accumulation of globotrioasoyl ceramide in cells and tissues of the body, resulting in a multi-system pathology including end organ failure. In the classical phenotype, cardiac failure, renal failure and stroke result in a reduced median life expectancy. The current causal treatment for Fabry disease is the enzyme replacement therapy (ERT): two different products, Replagal (agalsidase alfa) and Fabrazyme (agalsidase beta), have been commercially available in Europe for almost 10 years and they are both indicated for long-term treatment. In fact, clinical trials, observational studies and registry data have provided many evidences for safety and efficacy of ERT in improving symptoms of pain, gastrointestinal disturbances, hypohidrosis, left ventricular mass index, glomerular filtration rate and quality of life. Few data are available on comparison of the two treatments and on the clinical course of the disease. This article reviews the published evidence for clinical efficacy of the two available enzyme preparations. Copyright © 2012 Elsevier Inc. All rights reserved.

Displacement and Strain Measurement up to 1000 °C Using a Hollow Coaxial Cable Fabry-Perot Resonator.

PubMed

Zhu, Chen; Chen, Yizheng; Zhuang, Yiyang; Huang, Jie

2018-04-24

We present a hollow coaxial cable Fabry-Perot resonator for displacement and strain measurement up to 1000 °C. By employing a novel homemade hollow coaxial cable made of stainless steel as a sensing platform, the high-temperature tolerance of the sensor is dramatically improved. A Fabry-Perot resonator is implemented on this hollow coaxial cable by introducing two highly-reflective reflectors along the cable. Based on a nested structure design, the external displacement and strain can be directly correlated to the cavity length of the resonator. By tracking the shift of the amplitude reflection spectrum of the microwave resonator, the applied displacement and strain can be determined. The displacement measurement experiment showed that the sensor could function properly up to 1000 °C. The sensor was also employed to measure the thermal strain of a steel plate during the heating process. The stability of the novel sensor was also investigated. The developed sensing platform and sensing configurations are robust, cost-effective, easy to manufacture, and can be flexibly designed for many other measurement applications in harsh high-temperature environments.

Control of resonant wavelength from organic light-emitting materials by use of a Fabry-Perot microcavity structure.

PubMed

Jung, Boo Young; Kim, Nam Young; Lee, Changhee; Hwangbo, Chang Kwon; Seoul, Chang

2002-06-01

We report the fabrication of Fabry-Perot microcavity structures with the organic light-emitting material tris-(8-hydroxyquinoline) aluminum (Alq3) and derive their optical properties by measuring their photoluminescence (PL) and absorption. Silver and a TiO2-SiO2 multilayer were used as metal and dielectric reflectors, respectively, in a Fabry-Perot microcavity structure. Three types of microcavity were prepared: type A consisted of [air[Ag[Alq3]Ag]glass]; type B, of [air[dielectric[Alq3]dielectric]glass]; and type C, of [air[Ag[Alq2]dielectric]glass]. A bare Alq3 film of [air[Alq3]glass] had its PL peak near 514 nm, and its full width at half-maximum (FWHM) was 80 nm. The broad FWHM of a bare Alq3 film was reduced to 15-27.5, 7-10.5, and 16-16.6 nm for microcavity types A, B, and C, respectively. Also, we could control the PL peak of the microcavity structure by changing the spacer thickness, the amount of phase change on reflection, and the angle of incidence.

Displacement and Strain Measurement up to 1000 °C Using a Hollow Coaxial Cable Fabry-Perot Resonator

PubMed Central

Chen, Yizheng; Zhuang, Yiyang

2018-01-01

We present a hollow coaxial cable Fabry-Perot resonator for displacement and strain measurement up to 1000 °C. By employing a novel homemade hollow coaxial cable made of stainless steel as a sensing platform, the high-temperature tolerance of the sensor is dramatically improved. A Fabry-Perot resonator is implemented on this hollow coaxial cable by introducing two highly-reflective reflectors along the cable. Based on a nested structure design, the external displacement and strain can be directly correlated to the cavity length of the resonator. By tracking the shift of the amplitude reflection spectrum of the microwave resonator, the applied displacement and strain can be determined. The displacement measurement experiment showed that the sensor could function properly up to 1000 °C. The sensor was also employed to measure the thermal strain of a steel plate during the heating process. The stability of the novel sensor was also investigated. The developed sensing platform and sensing configurations are robust, cost-effective, easy to manufacture, and can be flexibly designed for many other measurement applications in harsh high-temperature environments. PMID:29695063

APOLLO 16 ASTRONAUTS JOHN YOUNG AND CHARLES DUKE EXAMINE FAR ULTRAVIOLET CAMERA

NASA Technical Reports Server (NTRS)

1971-01-01

Apollo 16 Lunar Module Pilot Charles M. Duke, Jr., left and Mission Commander John W. Young examine Far Ultraviolet Camera they will take to the Moon in March. They will measure the universe's ultraviolet spectrum. They will be launched to the Moon no earlier than March 17, 1972, with Command Module Pilot Thomas K. Mattingly, II.

Quantitative comparison of 2D and 3D late gadolinium enhancement MR imaging in patients with Fabry disease and hypertrophic cardiomyopathy.

PubMed

Morsbach, F; Gordic, S; Gruner, C; Niemann, M; Goetti, R; Gotschy, A; Kozerke, S; Alkadhi, H; Manka, R

2016-08-15

This study aims to determine whether the quantification of myocardial fibrosis in patients with Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) using a late gadolinium enhancement (LGE) singlebreath-hold three-dimensional (3D) inversion recovery magnetic resonance (MR) imaging sequence is comparable with a clinically established two-dimensional (2D) multi-breath-hold sequence. In this retrospective, IRB-approved study, 40 consecutive patients (18 male; mean age 50±17years) with Fabry disease (n=18) and HCM (n=22) underwent MR imaging at 1.5T. Spatial resolution was the same for 3D and 2D images (field-of-view, 350×350mm(2); in-plane-resolution, 1.2×1.2mm(2); section-thickness, 8mm). Datasets were analyzed for subjective image quality; myocardial and fibrotic mass, and total fibrotic tissue percentage were quantified. There was no significant difference in subjective image quality between 3D and 2D acquisitions (P=0.1 and P=0.3) for either disease. In patients with Fabry disease there were no significant differences between 3D and 2D acquisitions for myocardial mass (P=0.55), fibrous tissue mass (P=0.89), and total fibrous percentage (P=0.67), with good agreement between acquisitions according to Bland-Altman analyses. In patients with HCM there were also no significant differences between acquisitions for myocardial mass (P=0.48), fibrous tissue mass (P=0.56), and total fibrous percentage (P=0.67), with good agreement according to Bland-Altman analyses. Acquisition time was significantly shorter for 3D (25±5s) as compared to the 2D sequence (349±62s, P<0.001). In patients with Fabry disease and HCM, 3D LGE imaging provides equivalent diagnostic information in regard to quantification of myocardial fibrosis as compared with a standard 2D sequence, but at superior acquisition speed. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

FAST: A near-infrared imaging Fabry-Perot spectrometer

NASA Astrophysics Data System (ADS)

Krabbe, A.; Rotaciuc, V.; Storey, J. W. V.; Cameron, M.; Blietz, M.; Drapatz, S.; Hofmann, R.; Saemann, G.; Genzel, R.

1993-12-01

The near-infrared imaging spectrometer Fabry-Perot Array Spectrometer (FAST) provides a spectral resolution of 1000-2700 over a field of view of approximately 30 x 30 sq arcsec with a plate scale of 0.5-1.0 arcsec/pixel. FAST is based on a SBRC InSb 58 x 62 FPA working at 6 K, yielding a sensitivity 5(sigma), tint = 100 s, K band) of 1.1 x 10-4 erg/s (sq cm) (sr) at R = 1000. As a portable, self-contained instrument FAST has already been successfully deployed at several 2- and 4-m class telescopes. The optical and mechanical design, computer hardware and software control, as well as spectral calibration of the FPI, special treatment of the detectors, and the main data reduction steps are described. The necessity for such a dedicated system in the investigation of line-emission mechanisms in various galactic and extragalactic environments is illustrated by recently obtained results.

Fabry-Perot microcavity sensor for H2-breath-test analysis

NASA Astrophysics Data System (ADS)

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.

Linear FBG Temperature Sensor Interrogation with Fabry-Perot ITU Multi-wavelength Reference.

PubMed

Park, Hyoung-Jun; Song, Minho

2008-10-29

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.

Linear FBG Temperature Sensor Interrogation with Fabry-Perot ITU Multi-wavelength Reference

PubMed Central

Park, Hyoung-Jun; Song, Minho

2008-01-01

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. PMID:27873898

PCF-based Fabry-Perot interferometric sensor for strain measurement under high-temperature

NASA Astrophysics Data System (ADS)

Deng, Ming; Tang, Chang-Ping; Zhu, Tao; Rao, Yun-Jiang

2011-05-01

We report a simple and robust all-fiber in-line Fabry-Perot interferometer (FPI) with bubble cavity, which is fabricated by directly splicing a mutimode photonic crystal fiber to a conventional single mode fiber by using a commercial splicer. The fabrication process only involves fusion splicing and cleaving. The high-temperature strain characteristic of such a device is evaluated and experimental results shows that this FPI can be used as an ideal sensor for precise strain measurement under high temperatures of up to 750°C. Therefore, such a FPI sensor may find important applications in aeronautics or metallurgy areas.

77 FR 30028 - Manufacturer of Controlled Substances; Notice of Registration; Cambrex Charles City, Inc.

Federal Register 2010, 2011, 2012, 2013, 2014

2012-05-21

... DEPARTMENT OF JUSTICE Drug Enforcement Administration Manufacturer of Controlled Substances..., Charles City, Iowa 50616, made application by letter to the Drug Enforcement Administration (DEA) to be registered as a bulk manufacturer of the following basic classes of controlled substances: Drug Schedule...

Charles Valentine Riley, A Biography: ambition, genius, and the emergence of applied entomology

USDA-ARS?s Scientific Manuscript database

Charles Valentine Riley, 1843-1895, was a renowned entomologist and founder of the field of applied or economic entomology. This biography, supported by the scientific collaboration of Dr. Weber, is the first story of his fascinating life at the center of many of the foundational events of American...

Adaptive pathway development for Fabry disease: a clinical approach.

PubMed

Schuller, Yvonne; Arends, Maarten; Körver, Simon; Langeveld, Mirjam; Hollak, Carla E M

2018-06-01

Fabry disease (FD) is a rare X-chromosome-linked lysosomal storage disorder. Although initial expectations of enzyme replacement therapy (ERT) were high, it is now clear that real-world effectiveness is disappointing and evidence gathering has been inadequate. In retrospect, development of ERT for FD had several shortcomings. Little convincing evidence on the effectiveness existed at time of authorization. Also, post-marketing evaluation failed to generate sufficient and relevant data for adequate evaluation on effectiveness. Adaptive pathways might have benefitted ERT development by: (i) involving healthcare professionals, patients, health technology assessment bodies and payers in the development process; (ii) iterative development, starting with initial authorization in classical males; (iii) a clear real-world data collection plan; (iv) an independent disease registry; and (v) prescription control. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

Fabry disease

PubMed Central

2010-01-01

Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal α-galactosidase A activity. FD is pan-ethnic and the reported annual incidence of 1 in 100,000 may underestimate the true prevalence of the disease. Classically affected hemizygous males, with no residual α-galactosidase A activity may display all the characteristic neurological (pain), cutaneous (angiokeratoma), renal (proteinuria, kidney failure), cardiovascular (cardiomyopathy, arrhythmia), cochleo-vestibular and cerebrovascular (transient ischemic attacks, strokes) signs of the disease while heterozygous females have symptoms ranging from very mild to severe. Deficient activity of lysosomal α-galactosidase A results in progressive accumulation of globotriaosylceramide within lysosomes, believed to trigger a cascade of cellular events. Demonstration of marked α-galactosidase A deficiency is the definitive method for the diagnosis of hemizygous males. Enzyme analysis may occasionnally help to detect heterozygotes but is often inconclusive due to random X-chromosomal inactivation so that molecular testing (genotyping) of females is mandatory. In childhood, other possible causes of pain such as rheumatoid arthritis and 'growing pains' must be ruled out. In adulthood, multiple sclerosis is sometimes considered. Prenatal diagnosis, available by determination of enzyme activity or DNA testing in chorionic villi or cultured amniotic cells is, for ethical reasons, only considered in male fetuses. Pre-implantation diagnosis is possible. The existence of atypical variants and the availability of a specific therapy singularly complicate genetic counseling. A disease-specific therapeutic option - enzyme replacement therapy using recombinant human α-galactosidase A - has been recently introduced and its long term outcome is currently still being investigated. Conventional management consists of pain relief with analgesic drugs

Fiber-optic extrinsic Fabry-Perot interferometer sensors with three-wavelength digital phase demodulation.

PubMed

Schmidt, M; Fürstenau, N

1999-05-01

A three-wavelength-based passive quadrature digital phase-demodulation scheme has been developed for readout of fiber-optic extrinsic Fabry-Perot interferometer vibration, acoustic, and strain sensors. This scheme uses a superluminescent diode light source with interference filters in front of the photodiodes and real-time arctan calculation. Quasi-static strain and dynamic vibration sensing with up to an 80-kHz sampling rate is demonstrated. Periodic nonlinearities owing to dephasing with increasing fringe number are corrected for with a suitable algorithm, resulting in significant improvement of the linearity of the sensor characteristics.

Development of an embedded Fabry Perot Fiber Optic Strain Rosette Sensor (FP-FOSRS)

NASA Technical Reports Server (NTRS)

Carman, Gregory P.; Lesko, John J.; Case, Scott W.; Fogg, Brian; Claus, Richard O.

1992-01-01

We investigate the feasibility of utilizing a Fabry-Perot Fiber Optic Strain Rosette Sensor (FP-FOSRS) for the evaluation of the internal strain state of a material system. We briefly describe the manufacturing process for this sensor and point out some potential problem areas. Results of an embedded FP-FOSRS in an epoxy matrix with external resistance strain gauges applied for comparative purposes are presented. We show that the internal and external strain measurements are in close agreement. This work lays the foundation for embedding this sensor in actual composite laminas.

Infrared imaging microscopy of bone: illustrations from a mouse model of Fabry disease.

PubMed

Boskey, Adele L; Goldberg, Michel; Kulkarni, Ashok; Gomez, Santiago

2006-07-01

Bone is a complex tissue whose composition and properties vary with age, sex, diet, tissue type, health and disease. In this review, we demonstrate how infrared spectroscopy and infrared spectroscopic imaging can be applied to the study of these variations. A specific example of mice with Fabry disease (a lipid storage disease) is presented in which it is demonstrated that the bones of these young animals, while showing typical spatial variation in mineral content, mineral crystal size, and collagen maturity, do not differ from the bones of age- and sex-matched wild type animals.