Successful Pregnancy and Lactation Outcome in a Patient With Gaucher Disease Receiving Enzyme Replacement Therapy, and the Subsequent Distribution and Excretion of Imiglucerase in Human Breast Milk

Background: Enzyme replacement therapy (ERT) with imiglucerase is a well-established, effective treatment for Gaucher disease. However, there have been no published reports regarding the excretion of imiglucerase into human breast milk and its effects on the nursing infant. Objective: This letter reports on the successful pregnancy and lactation of a patient with Gaucher disease receiving treatment with imiglucerase, and the subsequent distribution and excretion of imiglucerase in human breast milk. Methods: A 39-year-old Japanese female (height, 164 cm; weight, 55 kg) with Gaucher disease had 2 successful pregnancies and continued ERT through both. The study was conducted 6 months after the first delivery. She was administered a 1-hour infusion of imiglucerase 60 U/kg that coincided with her regular every-2-week regimen. Serum and breast-milk samples were obtained before and up to 24 hours after administration. Breast-milk samples were also obtained from 10 nursing mothers with galactorrhea as controls. Results: The preinfusion level of breast-milk beta-glucocerebrosidase was 0.008 nmol/h/mL. The peak of serum beta-glucocerebrosidase activity (0.119 nmol/h/mL) was obtained at the end of the 1-hour infusion period. Slightly increased enzymatic activity (0.016 nmol/h/mL) was observed in the first breast milk sampled after imiglucerase infusion. Conclusions: We report a case of successful pregnancy and breastfeeding in a Japanese patient with Gaucher disease. A small amount of imiglucerase was found to be excreted into human breast milk, but only in the first milk produced after infusion. (Clin Ther. 2010;32:2048-2052)ArticleCLINICAL THERAPEUTICS. 32(12):2048-2052 (2010)journal articl

Development of the sensitizer for generating higher-energy photons under diluted condition via the triplet-triplet annihilation-supported upconversion

It was previously reported that photon upconversion can occur in the solution containing anthracene and the Pt complex of octaethylporphyrin (PtOEP) via the triplet-triplet annihilation process. In this study, by employing the modified Pt complex of the dual anthracene-tethered porphyrin, DA-PtP as a sensitizer, it is demonstrated that shorter-wavelength light can be generated under diluted condition. We synthesized DA-PtP and compared upconversion properties by changing the type of sensitizers. Accordingly, it was shown that the photon upconversion proceeded with the xenon lamp (540 nm) in the presence of DA-PtP. Furthermore, it was found that the emission band in the shorter wavelength light in the near UV region was observed from the solution containing DA-PtP even under diluted condition. From the mechanistic investigation, it was proposed that the anthracene moieties in DA-PtP might inhibit to form agglomeration with the free anthracene. As a result, reabsorption of the higher-energy light generated from upconversion could be suppressed

Towards high-resolution ptychographic x-ray diffraction microscopy

Ptychographic x-ray diffraction microscopy is a lensless imaging technique with a large field of view and high spatial resolution, which is also useful for characterizing the wavefront of an x-ray probe. The performance of this technique is degraded by positioning errors due to the drift between the sample and illumination optics. We propose an experimental approach for correcting the positioning errors and demonstrate success by two-dimensionally reconstructing both the wavefront of the focused x-ray beam and the complex transmissivity of the weakly scattering objects at the pixel resolution of better than 10 nm in the field of view larger than 5 μm. This method is applicable to not only the observation of organelles inside cells or nano-mesoscale structures buried within bulk materials but also the characterization of probe for single-shot imaging with x-ray free electron lasers. © 2011 American Physical Society.Yukio Takahashi, Akihiro Suzuki, Nobuyuki Zettsu, Yoshiki Kohmura, Yasunori Senba, Haruhiko Ohashi, Kazuto Yamauchi, and Tetsuya Ishikawa. Phys. Rev. B 83(21), 214109 (2011)

Fermi resonance interaction in hetero-dimer and trimer ions containing aniline+

Vibrational spectra of hetero-dimer and trimer ions containing aniline+ are measured by infrared photodissociation spectroscopy. For the dimer ions, the NH2-bending overtone band gains its intensity through Fermi resonance interaction with the hydrogen-bonded NH stretching fundamental. Unperturbed frequencies of the NH2-bending overtone are calculated to be in the range of 3255-3276 cm-1, suggesting that the frequency is almost intact upon cluster formation. For the trimer ions, Fermi resonance interaction occurs mainly between the NH2-bending overtone and the stretching fundamental of the NH oscillator involved in the stronger hydrogen bond

Infrared Photodissociation Spectroscopy of [Aniline-(Water)1-8]+ : Structural Change from Branched and Cyclic to Proton Transferred Forms

第19回化学反応討論会, 2003年6月11日-6月13日, 仙台市戦災復興記念館(仙台

Infrared Photodissociation Spectroscopy of [Aniline-(Water)1-8]+ : Structural Change from Branched and Cyclic to Proton Transferred Forms

第19回化学反応討論会, 2003年6月11日-6月13日, 仙台市戦災復興記念館(仙台

Anomalous elastic softening of SmRu_{4}P_{12} under high pressure

The filled skutterudite compound SmRu_4P_{12} undergoes a complex evolution from a paramagnetic metal (phase I) to a probable multipolar ordering insulator (phase II) at T_{MI} = 16.5 K, then to a magnetically ordered phase (phase III) at T_{N} = 14 K. Elastic properties under hydrostatic pressures were investigated to study the nature of the ordering phases. We found that distinct elastic softening above T_{MI} is induced by pressure, giving evidence of quadrupole degeneracy of the ground state in the crystalline electric field. It also suggests that quadrupole moment may be one of the order parameters below T_{MI} under pressure. Strangely, the largest degree of softening is found in the transverse elastic constant C_{T} at around 0.5-0.6 GPa, presumably having relevancy to the competing and very different Gruneisen parameters \Omega of T_{MI} and T_{N}. Interplay between the two phase transitions is also verified by the rapid increase of T_{MI} under pressure with a considerably large \Omega of 9. Our results can be understood on the basis of the proposed octupole scenario for SmRu_4P_{12}.Comment: 7 pages, 7 figure

Efficacy and safety of monthly oral minodronate in patients with involutional osteoporosis

Summary Monthly minodronate at 30 or 50 mg had similar efficacy as 1 mg daily in terms of change in bone mineral density (BMD) and bone turnover markers with similar safety profiles. This new regimen provides patients with a new option for taking minodronate. Introduction Minodronate at a daily oral dose of 1 mg has been proven to have antivertebral fracture efficacy. In the present study, the efficacy and safety of oral minodronate at monthly doses of either 30 mg or 50 mg were compared with a daily dose of 1 mg. Methods A total of 692 patients with involutional osteoporosis were randomized to receive minodronate at either 30 or 50 mg monthly or a daily dose of 1 mg. The primary endpoint was the percent change from baseline in lumbar spine (LS) BMD at 12 months. Total hip BMD, bone turnover markers, serum calcium (Ca), and parathyroid hormone (PTH) levels were also evaluated. Results Minodronate at monthly doses of 30 or 50 mg were noninferior to the 1 mg daily dose in terms of change in LS-BMD. Changes in total hip BMD were also comparable. Although a transient decrease in serum Ca and increase in PTH levels were observed in all three groups at slightly different magnitudes and time courses, changes in bone turnover markers were comparable among the differentdosage groups with a similar time course. Safety profiles were also comparable. Conclusion Minodronate at monthly doses of 30 or 50 mg has similar efficacy to the daily 1 mg dose in terms of BMD and bone turnover markers with similar tolerability

The Nature of Ultra-Luminous Compact X-Ray Sources in Nearby Spiral Galaxies

Studies were made of ASCA spectra of seven ultra-luminous compact X-ray sources (ULXs) in nearby spiral galaxies; M33 X-8 (Takano et al. 1994), M81 X-6 (Fabbiano 1988b; Kohmura et al. 1994; Uno 1997), IC 342 Source 1 (Okada et al. 1998), Dwingeloo 1 X-1 (Reynolds et al. 1997), NGC 1313 Source B (Fabbiano & Trinchieri 1987; Petre et al. 1994), and two sources in NGC 4565 (Mizuno et al. 1999). With the 0.5--10 keV luminosities in the range 10^{39-40} ergs/s, they are thought to represent a class of enigmatic X-ray sources often found in spiral galaxies. For some of them, the ASCA data are newly processed, or the published spectra are reanalyzed. For others, the published results are quoted. The ASCA spectra of all these seven sources have been described successfully with so called multi-color disk blackbody (MCD) emission arising from optically-thick standard accretion disks around black holes. Except the case of M33 X-8, the spectra do not exhibit hard tails. For the source luminosities not to exceed the Eddington limits, the black holes are inferred to have rather high masses, up to ~100 solar masses. However, the observed innermost disk temperatures of these objects, Tin = 1.1--1.8 keV, are too high to be compatible with the required high black-hole masses, as long as the standard accretion disks around Schwarzschild black holes are assumed. Similarly high disk temperatures are also observed from two Galactic transients with superluminal motions, GRO 1655-40 and GRS 1915+105. The issue of unusually high disk temperature may be explained by the black hole rotation, which makes the disk get closer to the black hole, and hence hotter.Comment: submitted to ApJ, December 199

Stability of a metallic state in the two-orbital Hubbard model

Electron correlations in the two-orbital Hubbard model at half-filling are investigated by combining dynamical mean field theory with the exact diagonalization method. We systematically study how the interplay of the intra- and inter-band Coulomb interactions, together with the Hund coupling, affects the metal-insulator transition. It is found that if the intra- and inter-band Coulomb interactions are nearly equal, the Fermi-liquid state is stabilized due to orbital fluctuations up to fairly large interactions, while the system is immediately driven to the Mott insulating phase away from this condition. The effects of the isotropic and anisotropic Hund coupling are also addressed.Comment: 7 pages, 9 figure