Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002986 (Fabry)
 5,646 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An erbium-doped fiber laser that emits a series of spectrally scanned pulses is used to monitor an array of fiber Bragg grating (FBG) sensors. The cavity for this Fabry-Perot laser is formed by two spectrally selective reflectors: a rotating mirror-grating combination for scanning the reflectance peak wavelength and a fiber Fabry-Perot interferometer (FFPI) with a periodic reflectance spectrum. During a scan of the rotating mirror, the laser produces a set of Q-switched pulses over the 1522-1568-nm spectral range at each of the FFPI reflectance peak wavelengths. This laser is used to simultaneously demonstrate wavelength-division multiplexing of FBGs with reflectance peaks in different spectral regimes and time-division multiplexing of FBGs with overlapping spectra. The spectral location of the FBG peaks was determined to an accuracy of 1.4 pm.
Opt Lett 2003 Sep 15
PMID:Spectrally scanned, repetitively pulsed erbium-doped fiber laser for spectral and temporal multiplexing of fiber Bragg grating sensors. 1367 24

I present a new derivation of the analytic form for the phase shift near resonance and the optical penetration length upon reflection from a distributed dielectric mirror consisting of a quarter-wave stack. The requirement of proper termination to achieve high reflectivity is suspended to investigate large optical penetration depths. Separate equations, derived for N and N + 1/2 layer pairs, are convenient for the design of tunable Fabry-Perot filters with a specified tuning range. The analysis is also applicable to distributed Bragg reflectors, vertical-cavity surface-emitting lasers, and resonant photodiodes. I show that the penetration length can sharply reduce the overly broad free spectral range of an ultrathin Fabry-Perot filter that might be useful in applications such as tunable wavelength filters for wavelength division multiplexing applications. The results also demonstrate regimes of zero dispersion and of superluminal reflection in the dielectric mirrors, which are of particular interest in photonic bandgap structures.
Appl Opt 2003 Sep 20
PMID:Theory of quarter-wave-stack dielectric mirrors used in a thin fabry-perot filter. 1452 31

A single-mode and highly side-mode-suppressed 1.55-microm Fabry-Perot laser diode (FPLD) is achieved by feedback injection with an erbium-doped fiber laser (EDFL). For selection of the strongest longitudinal mode from the gain spectrum of the FPLD for lasing in the EDFL, the FPLD is operated at just below the threshold condition and is feedback injected by 0.02% of the EDFL output power. The lasing mode and center wavelength of the proposed single-mode FPLD source are decided by cross-correlated gain profiles of the EDFL and the FPLD; however, the effect of FPLD injection modes is found to be more pronounced. The optimized lasing linewidth (system limitation) and side-mode suppression ratio of 0.01 nm and > 49 dB are obtained, which are far better than those of a FPLD at free-running condition. The worst linewidths at 3- and 10-dB decay are observed to be at approximately 0.016 and 0.05 nm, respectively. Linear wavelength tuning of as much as 4.5 nm (from 1558.7 to 1563.2 nm) by adjustment of the temperature of the FPLD from 10 degrees C to 40 degrees C at just below threshold is reported. The wavelength-tuning slope is approximately 0.14 nm/degrees C under temperature accuracy of 0.1 degrees C.
Appl Opt 2003 Sep 20
PMID:Linewidth reduction and wavelength tuning of an erbium-doped fiber laser by use of a single-mode-selected and side-mode-suppressed Fabry-Perot laser diode. 1452 34

We have observed the emission spectra of three-dimensional Rayleigh-Fabry-Perot cavity whispering-gallery modes from photonic quantum ring lasers that give rise to uniform angular mode spacing in space. The observed angular distribution (0 degrees approximately 30 degrees) of the emission modes is linear with respect to the mode number. For example, the average angular mode spacing is 0.95 degrees with a standard deviation of 0.098 degrees for a 20-microm-diameter laser, whose potential angle-resolving power is promising for angle-sensing applications in the future.
Appl Opt 2003 Sep 20
PMID:Extremely uniform angular distributions of the three-dimensional emission spectra of photonic quantum ring lasers. 1452 38

A simple and robust system is presented to generate wavelength-tunable optical short pulses by use of two gain-switched Fabry-Perot laser diodes in a mutual pulse injection-seeding scheme. The operating wavelength of the optical pulses is flexibly selected by adjustment of a tunable filter, and its intensity is enhanced with an erbium-doped fiber amplifier. The side-mode suppression ratio achieved by the system is larger than 26 dB over a wavelength region of 25 nm and higher than 31 dB within an 18-nm wavelength.
Appl Opt 2003 Sep 20
PMID:Wavelength-tunable optical short-pulse generation by mutual pulse injection seeding of two gain-switched Fabry-Perot laser diodes. 1452 41

The World Congress of Nephrology was held in Berlin, Germany, June 8-12, 2003. The meeting offered the newest advances in basic and clinical nephrology science and was attended by about 9,000 scientists and clinicians from around the world. During the congress, results of the treatment of Fabry's disease with enzyme replacement therapy, the results of the treatment of anemia in patients with chronic kidney disease with new erythropoietic agents (darbepoetin alfa, continuous erythropoiesis receptor activator), and the management of secondary hyperparathyroidism and calcium-phosphorus disorders in uremia with calcimimetic agents and new phosphate binders, such as lanthanum carbonate, were discussed. Furthermore, recent studies evaluating the efficacy and safety of new immunosuppressive agents and their combination for the treatment of renal transplant recipients were also presented.
Drug News Perspect 2003 Sep
PMID:Recent advances in nephrology. 1466 43

During the last 5 years 2057 children under the age of 5 with various neurologic symptoms with the suspected diagnosis of lysosomal storage diseases were referred to our hospital from different universities and state hospitals. We were able to separate sphingolipidoses by lysosomal enzyme screening. A total of 300 patients (15%) with sphingolipidoses were diagnosed; there were deficiencies of arylsulfatase A [metachromatic leukodystrophy (MLD)] in 93 (31%), hexosaminidase [Sandhoff disease (SHD)] in 62 (20.7%), hexosaminidase A [Tay-Sachs disease (TSD)] in 15 (5%), beta-galactosidase (GM1 gangliosidosis) in 35 (11.7%), alpha-galactosidase (Fabry disease) in one (0.3%) cerebroside beta-galactosidase (Krabbe disease) in 65 (21.7%) and glucosylceramidase (Gaucher disease) in 29 (9.6%). SHD (20.7%), MLD (31%) and Krabbe disease (21.7%) were common. Prenatal enzymatic diagnosis was made in 70 at risk pregnancies, 64 for TSD and SHD, three for MLD and three for GM1 gangliosidosis by using chorionic villus biopsy in 54, cord blood samples in 12 and cultured amniotic fluid cells in four. Seventeen fetuses were found to be affected. We have calculated the relative frequency and minimum incidence of sphingolipidoses in Turkey. The combined incidence of sphingolipidoses is 4.615 per 100,000 live births. The calculated incidences are 1.43, 0.95, 1, 0.23, 0.54, 0.45, 0.015 per 100,000 live births for MLD, SHD, Krabbe, Gaucher, TSD, GM1 gangliosidosis and Fabry diseases, respectively. The real incidence, which covers all subtypes of this group of diseases, should be greater than this number. The results suggested that, as a group, sphingolipidoses are relatively common and represent an important health problem in Turkey and some rare autosomal recessive diseases of Turkey are due to 'founder effect' created by consanguineous marriages.
Brain Dev 2004 Sep
PMID:Sphingolipidoses in Turkey. 1527 96

Therapeutic enzymes are often recognized as foreign by the immune system of patients undergoing enzyme replacement therapy. The antibodies that develop may alter pharmacokinetics and biodistribution of the therapeutic protein, may be able to neutralize the activity of the enzyme, or may cause immune reactions in certain patients. We have explored treatment regimens to reduce the antibody response to human alpha-galactosidase A (r-halphaGAL) in Fabry (alphaGAL knock-out) and normal BALB/c mice. A wide variety of treatment modalities were tested, including high dose tolerance induction, increased frequency of therapeutic doses and immunosuppressive drugs in combination with administration of enzyme. The most substantial effects were observed in mice injected intravenously with r-halphaGAL in combination with methotrexate (MTX), which significantly lowered r-halphaGAL-specific serum antibody levels. A short course of treatment with MTX was able to reduce antibody and spleen cell proliferative responses to long-term r-halphaGAL treatment. MTX was able to suppress the development of r-halphaGAL-specific IgG in antigen-primed mice. However, MTX was not effective in dampening robust ongoing antibody responses. These experiments provide a framework for the design of clinical protocols to prevent the drug-specific antibody responses of patients undergoing enzyme replacement therapy.
Clin Exp Immunol 2004 Sep
PMID:Methotrexate reduces antibody responses to recombinant human alpha-galactosidase A therapy in a mouse model of Fabry disease. 1532 Aug 98

A unique case of a man with Fabry's disease and associated small vessel vasculopathy manifesting as recurrent episodes of intermittent claudication is described. The case highlights the concept that microvascular involvement due to local accumulation of glycosphingolipid in the smooth muscle fibres of vessel walls may be responsible for claudicant symptoms in such patients rather than the more classical macrovascular insufficiency.
J Clin Neurosci 2004 Sep
PMID:Intermittent claudication -- atypical presentation, diagnosis and treatment. 1533 51

A 1500-nm-band laser signal is upconverted to the mid-visible part of the spectrum by quasi-phase matched, sum-frequency generation with an 800-nm-band laser pump in a periodically poled KTiOPO4. For an appropriate combination of the two fundamental wavelengths, an acceptance bandwidth of 40-60 nm cm for the pump wavelength is attainable simultaneously with a temperature acceptance bandwidth of 60-70 degrees C cm in an angularly noncritical condition. Using a distributed feedback laser at 1590 nm and a Fabry-Perot laser at 807 nm, we demonstrate a temperature tolerance as large as 60 degrees C with a 10-mm-long crystal.
Appl Opt 2004 Sep 01
PMID:Type II quasi-phase-matched sum-frequency mixing of diode lasers in KTiOPO4 with broad spectral and temperature acceptance bandwidths. 1544 79


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