Liver transplantation for type IV glycogen storage disease

TYPE IV glycogen storage disease is a rare autosomal recessive disorder (also called Andersen's disease1 or amylopectinosis) in which the activity of branching enzyme alpha-1, 4-glucan: alpha-1, 4-glucan 6-glucosyltransferase is deficient in the liver as well as in cultured skin fibroblasts and other tissues.2,3 This branching enzyme is responsible for creating branch points in the normal glycogen molecule. In the relative or absolute absence of this enzyme, an insoluble and irritating form of glycogen, an amylopectin-like polysaccharide that resembles plant starch, accumulates in the cells. The amylopectin-like form is less soluble than normal glycogen, with longer outer and inner chains. © 1991, Massachusetts Medical Society. All rights reserved

Measurements of Far-UV Emission from Elliptical Galaxies at z=0.375

The ``UV upturn'' is a sharp rise in spectra of elliptical galaxies shortward of rest-frame 2500 A. It is a ubiquitous phenomenon in nearby giant elliptical galaxies, and is thought to arise primarily from low-mass evolved stars on the extreme horizontal branch and beyond. Models suggest that the UV upturn is a very strong function of age for these old stellar populations, increasing as the galaxy gets older. In some models the change in UV/optical flux ratio is a factor of 25 over timescales of less than 3 Gyr. To test the predictions for rapid evolution of the UV upturn, we have observed a sample of normal elliptical galaxies in the z=0.375 cluster Abell 370 with the Faint Object Camera aboard the Hubble Space Telescope. A combination of two long-pass filters was used to isolate wavelengths shortward of rest-frame 2700 A, providing a measurement of the UV upturn at a lookback time of approximately 4 Gyr. Surprisingly, the four elliptical galaxies observed show a range of UV upturn strength that is similar to that seen in nearby elliptical galaxies, with an equivalent 1550-V color ranging from 2.9-3.4 mag. Our result is inconsistent with some models for the UV upturn; other models are consistent only for a high redshift of formation (z_f >= 4).Comment: 4 pages, Latex. 1 figure. To appear in ApJL. Uses emulateapj.sty and apjfonts.sty. Revision includes minor ApJ edits & fixes typo

The Short Rotation Period of Nereid

We determine the period, p = 11.52 \pm 0.14 h, and a light curve peak-to-peak amplitude, a = 0.029 \pm 0.003 magnitudes, of the Neptunian irregular satellite Nereid. If the light curve variation is due to albedo variations across the surface, rather than solely to the shape of Nereid variations, the rotation period would be a factor of two shorter. In either case, such a rotation period and light curve amplitude, together with Nereid's orbital period, p=360.14 days, imply that Nereid is almost certainly in a regular rotation state, rather than the chaotic rotation state suggested by Schaefer and Schaefer (1988,2000) and Dobrovolskis (1995). Assuming that Nereid is perfectly spherical, the albedo variation is 3% across the observed surface. Assuming a uniform geometric albedo, the observed cross sectional area varies by 3%. We caution that the lightcurve found in this paper only sets limits on the combination of albedo and physical irregularity and that we cannot determine the orientation of Nereid's spin axis from our data.Comment: Accepted by ApJ Letters, 11 pages (incl. 1 figure

Shwartzman reaction after human renal homotransplantation.

In three human recipients, five renal homografts were destroyed within a few minutes to hours after their revascularization in the new host. The kidneys, removed one to 54 days later, had cortical necrosis. The major vessels were patent, but the arterioles and glomeruli were the site of fibrin deposition. There was little or no fixation of host immunoglobulins in the homografts. The findings were characteristic of a generalized Shwartzman reaction. Although the cause (or causes) of the Shwartzman reaction in our patients is not known, they may have been conditioned by the bacterial contamination and hemolysis that often attend hemodialysis, by immunosuppression and by the transplantation itself. Some of the patients have preformed lymphocytotoxic antibodies. Thus, certain patients may be predisposed. High-risk patients should be recognized and treated prophylactically with anticoagulants

Quark condensates in the chiral bag with the NJL interaction

We discuss the quark condensate of the vacuum inside the baryon. We analyze the 1+1 dimensional chiral bag in analogy with the realistic 3+1 dimensional one. The Nambu--Jona-Lasinio (NJL) type interaction is used to investigate the quark condensate in the chiral bag. Considering the strong meson-quark coupling, we solve the mean field solution to the scalar and pseudoscalar channels by including the chiral Casimir effects. The self-consistent equation allows a finite value of the quark condensate and hence a finite dynamical quark mass inside the bag. It is shown that an approximate Cheshire Cat picture holds for massive quarks

The Equal Rights Amendment: A Constitutional Basis for Equal Rights for Women

In the present legal structure, some laws exclude women from legalrights, opportunities, or responsibilities. Some are framed as legislationconferring special benefits, or protection, on women. Others create orperpetuate a separate legal status without indicating on their facewhether the position of women ranks below, or above, the positionof men. Many of the efforts to create a separate legal status for womenstem from a good faith attempt to advance the interests of women.Nevertheless, the preponderant effect has been to buttress the socialand economic subordination of women

First observation of 54Zn and its decay by two-proton emission

The nucleus 54Zn has been observed for the first time in an experiment at the SISSI/LISE3 facility of GANIL in the quasi-fragmentation of a 58Ni beam at 74.5 MeV/nucleon in a natNi target. The fragments were analysed by means of the ALPHA-LISE3 separator and implanted in a silicon-strip detector where correlations in space and time between implantation and subsequent decay events allowed us to generate almost background free decay spectra for about 25 different nuclei at the same time. Eight 54Zn implantation events were observed. From the correlated decay events, the half-life of 54Zn is determined to be 3.2 +1.8/-0.8 ms. Seven of the eight implantations are followed by two-proton emission with a decay energy of 1.48(2) MeV. The decay energy and the partial half-life are compared to model predictions and allow for a test of these two-proton decay models.Comment: 4 pages, 4 figures, accepted for publication in PR

Alternative job search strategies in remote rural and peri-urban labour markets: the role of social networks

This paper examines the importance of informal methods (especially social networking) to the job search strategies used by unemployed people. It compares three areas: a small rural town; a larger, more sparsely populated, remote rural area; and a centrally-located, peri-urban labour market. The analysis is based first on survey research undertaken with 490 job seekers across the study areas. Emerging issues were then followed up during a series of twelve focus groups. The survey research showed that job seekers in the rural study areas were significantly more likely to use social networks to look for work. However, those who had experienced repeated or long-term periods out of work, the unskilled and young people were significantly less likely to use such networks. Focus groups confirmed the perceived importance of social networking to the job search process in rural areas, in contrast to the more marginal role such methods appear to play in peri-urban settings. For many rural job seekers, formal job search activities conducted through Jobcentres were seen as largely symbolic, lacking the practical value of social networking. These results suggest that service providers seeking to assist unemployed people in rural areas need to address the problems faced by many disadvantaged job seekers who are currently caught between their lack of social network relations and the absence of local public employment service facilities in more remote communities

Local structures of polar wurtzites Zn_{1-x}Mg_{x}O studied by Raman and {67}Zn/{25}Mg NMR spectroscopies and by total neutron scattering

Local compositions and structures of Zn_{1-x}Mg_{x}O alloys have been investigated by Raman and solid-state {67}Zn/{25}Mg nuclear magnetic resonance (NMR) spectroscopies, and by neutron pair-distribution-function (PDF) analyses. The E2(low) and E2(high) Raman modes of Zn_{1-x}Mg_{x}O display Gaussian- and Lorentzian-type profiles, respectively. At higher Mg substitutions, both modes become broader, while their peak positions shift in opposite directions. The evolution of Raman spectra from Zn_{1-x}Mg_{x}O solid solutions are discussed in terms of lattice deformation associated with the distinct coordination preferences of Zn and Mg. Solid-state magic-angle-spinning (MAS) NMR studies suggest that the local electronic environments of {67}Zn in ZnO are only weakly modified by the 15% substitution of Mg for Zn. {25}Mg MAS spectra of Zn_{0.85}Mg_{0.15}O show an unusual upfield shift, demonstrating the prominent shielding ability of Zn in the nearby oxidic coordination sphere. Neutron PDF analyses of Zn_{0.875}Mg_{0.125}O using a 2x2x1 supercell corresponding to Zn_{7}MgO_{8} suggest that the mean local geometry of MgO_{4} fragments concurs with previous density functional theory (DFT)-based structural relaxations of hexagonal wurtzite MgO. MgO_{4} tetrahedra are markedly compressed along their c-axes and are smaller in volume than ZnO_{4} units by ~6%. Mg atoms in Zn_{1-x}Mg_{x}O have a shorter bond to the $c$-axial oxygen atom than to the three lateral oxygen atoms, which is distinct from the coordination of Zn. The precise structure, both local and average, of Zn_{0.875}Mg_{0.125}O obtained from time-of-flight total neutron scattering supports the view that Mg-substitution in ZnO results in increased total spontaneous polarization.Comment: 12 pages, 14 figures, 2 table