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Query: UMLS:C0002986 (Fabry)
 5,646 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A tunable single-mode laser is obtained by using a weakly coupled cavity structure involved in a coaxially packaged Fabry-Perot laser diode. The cleaved end facet of the coupling fiber becomes an optical reflector and forms an external cavity with a laser facet. The single-mode oscillation condition is controlled and stabilized by tuning the operating temperature. The tuning range is about 10 nm with the side-mode suppression ratio of more than 27 dB when the temperature changes from 11.5 degrees C to 25 degrees C. Direct modulation characteristics were investigated, and our results show that a shorter external cavity can bear deeper modulation depth.
Opt Lett 2006 Sep 01
PMID:Tunable single-mode Fabry-Perot laser diode using a built-in external cavity and its modulation characteristics. 1690 27

We present an overview of the properties and applications of optical bistability in vertical-cavity semiconductor optical amplifiers (VCSOAs). The basic physics and analytical models of this optical nonlinearity are discussed. Experimental results obtained from a VCSOA operated in the 850 nm wavelength region are presented. Counterclockwise hysteresis loops are obtained over a range of initial phase detuning and bias currents. One hysteresis loop is observed experimentally with an input power as low as 2 muW when the device is biased at 98% of its lasing threshold. Numerical simulations based on the Fabry-Perot resonator model and rate equations we developed show good agreement with our experimental observations. In addition, a low input intensity high contrast (10:1) optical and gate and 2R regeneration are demonstrated. We believe that bistable VCSOAs can significantly advance the prospect of a dense two-dimensional array of low-switching-intensity all-optical logic and memory elements.
Appl Opt 2006 Sep 01
PMID:Optical bistability in vertical-cavity semiconductor optical amplifiers. 1691 70

A method for measuring the length of an optical fiber by use of an optical fiber ring laser pulse source is proposed and demonstrated. The key element of the optical fiber ring laser is a gain-switched Fabry-Perot laser diode operated in a self-injection seeding scheme. This method is especially suitable for measuring a medium or long fiber, and a resolution of 0.1 m is experimentally achieved. The measurement is implemented by accurately determining the pulse frequency that can maximize the output power of the fiber ring laser. The measurement results depend only on the refractive index of the fiber corresponding to this single wavelength, instead of the group index of the fiber, which represents a great advantage over both optical time-domain reflectometry and optical low-coherence reflectometry methods.
Appl Opt 2006 Sep 01
PMID:Measuring optical fiber length by use of a short-pulse optical fiber ring laser in a self-injection seeding scheme. 1691 84

Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Questionnaires were completed by 566 Fabry patients, of whom 316 reported ear-related symptoms. Pure-tone audiograms from 86 patients, performed before starting enzyme replacement therapy, were analysed and compared with age- and sex-specific normal values (International Organization for Standardization, ISO 7029). When compared to an age-matched population (ISO 7029), 74% of patients had a threshold elevated above the 95th centile in at least one tested frequency. All frequencies were affected to a similar degree. However, only 14 patients (16%) were clinically affected by hearing impairment according to the age-independent World Health Organization (WHO) classification (mean threshold at 0.5, 1 and 2 kHz worse than 25 dB). Hearing loss was sensorineural in 63 patients (73%) of whom 7 patients (8%) had also a conductive component. One patient had a purely conductive hearing loss. Episodes of sudden hearing loss seemed to occur more frequently than in the general population. Men were affected earlier and more severely than women. Hearing in Fabry disease is significantly worse than in an age-matched general population but leads to clinically relevant hearing impairment in only 16% of cases. It resembles accelerated presbycusis with an additional Fabry-specific strial-type hearing loss.
Eur J Clin Invest 2006 Sep
PMID:Hearing loss in Fabry disease: data from the Fabry Outcome Survey. 1691 49

Fabry disease is an X-linked lysosomal storage disorder characterized by multi-organ dysfunction, including hearing loss - mainly sensorineural. The recent introduction of enzyme replacement therapy (ERT) has resulted in improvements in renal and cardiac function, pain and quality of life. One study has also suggested small improvements in high-frequency hearing. In this paper, we study the effect of ERT on hearing in patients in the Europe-wide database - the Fabry Outcome Survey (FOS). Twenty-six patients in FOS had pure-tone audiometry performed up to 6 months before starting ERT with agalsidase alpha and after a median of 12 months of treatment. We assessed changes in hearing thresholds, expressed as deviations from the 50th centile of the normal population (International Organization for Standardization ISO 7029) to correct for age-related non-specific hearing deterioration. Hearing did not change significantly in ears with normal hearing (less than 10 dB deviation from the 50th centile of ISO 7029) or those with severe hearing loss (more than 40 dB deviation from the 50th centile of ISO 7029) at baseline. In ears with a mild or moderate hearing loss at baseline, hearing thresholds, expressed as deviations from the normal 50th centile, improved significantly by 4-7 dB at most frequencies (P < 0.05). Agalsidase alpha stabilizes, and possibly improves, hearing in Fabry patients who have not already progressed to severe hearing loss. Further follow-up of these patients will determine the longer-term effects of ERT.
Eur J Clin Invest 2006 Sep
PMID:Agalsidase alpha and hearing in Fabry disease: data from the Fabry Outcome Survey. 1691 50

A novel tunable fiber ring laser configuration with a combination of bidirectional Raman amplification and dual erbium-doped fiber (EDF) amplification is proposed for realizing high optical signal-to-noise ratio (SNR), long-distance, quasi-distributed fiber Bragg grating (FBG) sensing systems with large capacities and low cost. The hybrid Raman-EDF amplification configuration arranged in the ring laser can enhance the optical SNR of FBG sensor signals significantly owing to the good combination of the high gain of the erbium-doped fiber amplifier (EDFA) and the low noise of the Raman amplification. Such a sensing system can support a large number of FBG sensors because of the use of a tunable fiber Fabry-Perot filter located within the ring laser and spatial division multiplexing for expansion of sensor channels. Experimental results show that an excellent optical SNR of approximately 60 dB has been achieved for a 50 km transmission distance with a low Raman pump power of approximately 170 mW at a wavelength of 1455 nm and a low EDFA pump power of approximately 40 mW at a wavelength of 980 nm, which is the highest optical SNR achieved so far for a 50 km long FBG sensor system, to our knowledge.
Opt Lett 2006 Sep 15
PMID:Long-distance fiber Bragg grating sensor system with a high optical signal-to-noise ratio based on a tunable fiber ring laser configuration. 1693 57

An optical binary switch for aircraft applications is demonstrated. A fiber Fabry-Perot interferometer (FFPI) bonded to a cantilever is used as the sensing element. A white-light interferometry system with two bulk Michelson interferometers sharing the same motor-driven translation stage is utilized to monitor the elongation of the FFPI. The system exhibits excellent linearity as a force sensor; the experimental results are in good agreement with theoretical calculated values. With a properly set threshold value, the system produces a binary output.
Opt Lett 2006 Sep 15
PMID:Fabry-Perot optical binary switch for aircraft applications. 1693 61

Hypohidrosis is a classic feature of Fabry disease; in contrast, hyperhidrosis has only been rarely described. The aim of the study is to characterise the baseline descriptive data on hyperhidrosis (frequency, age at onset, sex ratio and outcome with and without enzyme replacement therapy) in hemizygous male and heterozygous female patients with Fabry disease. We describe case histories of five patients with Fabry disease and hyperhidrosis seen at three different centres. We have also analysed a cohort of 21 paediatric patients in the UK and a large European cohort of patients enrolled in the Fabry Outcome Survey (FOS). Five patients (three female, two male) with hyperhidrosis were originally identified, although each had additional symptoms related to Fabry disease. The age at onset of hyperhidrosis was less than 18 years in four cases. In the cohort of 21 paediatric patients (12 female, nine male), one female had hyperhidrosis; the age at onset of this symptom was 11 years. In the FOS cohort, 66 of 714 patients with Fabry disease had hyperhidrosis (44 of 369 females, 11.9%; 22 of 345 males, 6.4%). The female predominance was observed in seven of nine countries from which data were analysed. Hyperhidrosis is an increasingly recognised feature of the Fabry disease phenotype. It is more prevalent in females than in males and often appears in childhood or adolescence. The efficacy of enzyme replacement therapy on this recently recognised symptom should be assessed.
Int J Clin Pract 2006 Sep
PMID:Hyperhidrosis: a new and often early symptom in Fabry disease. International experience and data from the Fabry Outcome Survey. 1693 46

Analytical techniques known in the literature are used to (i) identify all the planar waveguide modes in four top-emitting organic light-emitting diode (OLED) structures over the visible spectrum, and (ii) compute both TM and TE power spectra for classically radiating dipoles in the emissive layers of these OLED structures. Peaks in the computed power spectra are identified with the waveguide modes in the OLED devices, and areas associated with these peaks are used to estimate the excitation probability of the waveguide modes. In cases where ambiguities arise because of overlapping peaks, it is shown that computed power spectra can be approximated as sums of Lorentzian line shapes. It is found that for all four structures, the dipoles couple almost 80% of their radiant energy into TM modes with only about 20% going into TE modes. Furthermore, except for a narrow spectral band, the excited TM modes are primarily short-range surface plasmon polaritons. Excitations in the narrow spectral band correspond to TM and TE Fabry-Perot microcavity modes. Finally, the analysis shows that, in the absence of grating couplers, only light in the microcavity modes escapes into the air cover.
Appl Opt 2006 Sep 20
PMID:Excitation of waveguide modes in organic light-emitting diode structures by classical dipole oscillators. 1694 96

Fabry disease is an X-linked metabolic storage disorder due to the deficiency of lysosomal alpha-galactosidase A, and the subsequent accumulation of glycosphingolipids, primarily globotriaosylceramide, throughout the body. Males with classical Fabry disease develop early symptoms including pain and hypohidrosis by the second decade of life reflecting disease progression in the peripheral and autonomic nervous systems. An insidious cascade of disease processes ultimately results in severe renal, cardiac, and central nervous system complications in adulthood. The late complications are the main cause of late morbidity, as well as premature mortality. Disease presentation in female heterozygotes may be as severe as in males although women may also remain asymptomatic. The recent introduction of enzyme replacement therapy to address the underlying pathophysiology of Fabry disease has focused attention on the need for comprehensive, multidisciplinary evaluation and management of the multi-organ system involvement. In anticipation of evidence-based recommendations, an international panel of physicians with expertise in Fabry disease has proposed guidelines for the recognition, evaluation, and surveillance of disease-associated morbidities, as well as therapeutic strategies, including enzyme replacement and other adjunctive therapies, to optimize patient outcomes.
Genet Med 2006 Sep
PMID:Fabry disease: guidelines for the evaluation and management of multi-organ system involvement. 1698 Aug 9


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