Patients with erythrokeratodermia cardiomyopathy syndrome exhibit congenital, generalised erythrokeratoderma and dilated cardiomyopathy during early childhood. We report a case of erythrokeratodermia cardiomyopathy syndrome in a 15-year-old male patient and focus this report on cardiac features that were present.

The Japanese second deep ice coring project was carried out at Dome Fuji, Antarctica. Following the drilling of the pilot hole in 2001, deep ice core drilling led by the Japanese Antarctic Research Expedition (JARE) was conducted over four austral summer seasons, beginning with the 2003/04 season and reached a depth of 3035.22 m near the bedrock in January 2007. The new drill was designed and developed with the goals of (1) solving the problems encountered during the first JARE deep coring drill and (2) achieving more efficient drilling. In particular, the maximum core length that can be drilled at one time was increased from 2.30 m to 3.84 m and the chip storage efficiency was enhanced by a special pipe with many small holes. This paper gives an outline of the improved drilling system, the progress of drilling and various drilling data.

Brown rice (BR) and white rice (WR) produce different glycaemic responses and their consumption may affect the dietary management of obesity. In the present study, the effects of BR and WR on abdominal fat distribution, metabolic parameters and endothelial function were evaluated in subjects with the metabolic syndrome in a randomised cross-over fashion. In study 1, acute postprandial metabolic parameters and flow- and nitroglycerine-mediated dilation (FMD and NMD) of the brachial artery were determined in male volunteers with or without the metabolic syndrome after ingestion of either BR or WR. The increases in glucose and insulin AUC were lower after ingestion of BR than after ingestion of WR (P= 0·041 and P= 0·045, respectively). FMD values were decreased 60 min after ingestion of WR (P= 0·037 v. baseline), but the decrease was protected after ingestion of BR. In study 2, a separate cohort of male volunteers (n 27) with the metabolic syndrome was randomised into two groups with different BR and WR consumption patterns. The values of weight-based parameters were decreased after consumption of BR for 8 weeks, but returned to baseline values after a WR consumption period. Insulin resistance and total cholesterol and LDL-cholesterol levels were reduced after consumption of BR. In conclusion, consumption of BR may be beneficial, partly owing to the lowering of glycaemic response, and may protect postprandial endothelial function in subjects with the metabolic syndrome. Long-term beneficial effects of BR on metabolic parameters and endothelial function were also observed.

In order to achieve waste heat recovery using thermoelectric systems, thermoelectric materials showing high conversion efficiency over wide temperature range and high resistance against oxidation are indispensable. A silicide material with good n-type thermoelectric properties and oxidation resistance has been discovered. The composition and crystal structure of the silicide are found out Mn3Si4Al2 (abbreviated as 342 phase) and hexagonal CrSi2 structure, respectively. Element substitution of Mn with 3d transition metals is succeeded. Enhancement of Seebeck coefficient is observed in a Cr-substituted sample. The maximum dimensionless thermoelectric figure of merit ZT is 0.3 at 573 K in air for the Mn2.7Cr0.3Si4Al2 sample. Electrical resistivity of the Mn3Si4Al2 bulk sample holds constant value for 48 h at 873 K in air. This is due to formation of oxide passive layer on the surface of the bulk sample. The 342 phase is a promising n-type material with a good oxidation resistance in the middle temperature range of 500-800 K.

Compound formation properties of very finely layered Mg/Al laminate composite (“super laminate composite”) were investigated. Almost uniform Mg17Al12 compound was obtained by heat treatment of the super laminate at 673K in less than 0.6ks(10 minutes). The rate control process of the compound formation is thought to be the diffusion of Mg in Al. Hydrogenation properties of thus obtained Mg17Al12 compound were also studied and its absorption capacity and dissociation pressure were almost the same as those previously reported material, which was prepared by a longer time heat treatment.

We have prepared Mg/Pd laminate composites with (Mg/Pd)=6, 3 and 2.5 atom ratios, by a super lamination technique. The homogeneous Mg-Pd intermetallic compounds, Mg6Pd, Mg3Pd and Mg5Pd2, are formed during the initial activation process. We investigated the hydrogen storage properties of these materials. The compounds can reversibly absorb and desorb a large amount of hydrogen, up to 1.46˜0.9 H/M, at 573 K. Except for the Mg5Pd2-hydrogen system, the pressure composition-isotherms show two plateaux. The mechanism of the phase transition during hydrogenation/dehydrogenation was analyzed by in-situ XRD measurements. These intermetallic compounds absorb and desorb hydrogen through reversible multistage disproportionation and recombination processes.

Microstructures and hydrogen storage properties of Mg/Cu super-laminates were compared to clarify the effect of initial activation. The initial activation change micro/nano-structures of Mg/Cu super-laminates into Mg2Cu with layered structure in fine grain size of about 1μm and pores highly dispersed between layers in sub-micrometer size. Large surface area, dense defects and short diffusion distance for the reaction enable Mg/Cu super-laminates to absorb hydrogen very quickly.

A small p-n diode array was fabricated on a polycrystalline Si substrate and the electrical characteristics were measured for each small diode to evaluate the distribution of energy conversion efficiency in the substrate. The crystal qualities in conjunction with the electrical characteristics were also evaluated. We found large variations in measuring the current-voltage (I-V) characteristics of the p-n diode. We also observed variations in quality even in diodes without any grain boundaries at the p-n junction. Therefore, we evaluated crystalline quality using various techniques to compare the diode characteristics. We found clear evidence in photo-luminescence (PL) mapping, where grains, including degraded diodes, were darker in the mapping, implying lower PL intensities than the others. The PL spectra obtained from the “dark grains” included D-lines indicating the existence of dislocations. We could conclude that the electrical characteristics of p-n diodes were not only affected by grain boundaries but also by crystalline defects evaluation such as dislocations. We observed a Secco-etched surface for crystalline defects evaluation using an optical microscope. The origins of etch pits were also determined by transmission electron microscope (TEM) and three different types of defects were confirmed.

Super-laminates have been attracting attention since co-authors Ueda et al. reported that Mg/Cu super-laminates showed reversible hydrogenation and dehydrogenation at 473K. The Mg/Cu super-laminates were prepared by a repetitive fold and roll method. Initial activation at 573 K led the super-laminates to absorb hydrogen at 473K. TEM observations of micro/nano-structures in the super-laminates were performed in order to clarify the process of hydrogenation and dehydrogenation at 473K, The as-rolled Mg/Cu super-laminates have laminated structures in size of sub-micrometer thickness composed of Mg and Cu layers with dense lattice defects. The super-laminates after initial activation keep laminated structure and have uniformly distributed pores with a sub-micrometer diameter. It is considered that these micro/nano-structures of Mg/Cu super-laminates lead to lower dehydrogenation temperature and better kinetics, which would contribute to achieve high performance hydrogen storage materials.

Assessing the total energy expenditure (TEE) and the levels of physical activity in free-living conditions with non-invasive techniques remains a challenge. The purpose of the present study was to investigate the accuracy of a new uniaxial accelerometer for assessing TEE and physical-activity-related energy expenditure (PAEE) over a 24 h period in a respiratory chamber, and to establish activity levels based on the accelerometry ranges corresponding to the operationally defined metabolic equivalent (MET) categories. In study 1, measurement of the 24 h energy expenditure of seventy-nine Japanese subjects (40 (SD 12) years old) was performed in a large respiratory chamber. During the measurements, the subjects wore a uniaxial accelerometer (Lifecorder; Suzuken Co. Ltd, Nagoya, Japan) on their belt. Two moderate walking exercises of 30 min each were performed on a horizontal treadmill. In study 2, ten male subjects walked at six different speeds and ran at three different speeds on a treadmill for 4 min, with the same accelerometer. O2 consumption was measured during the last minute of each stage and was expressed in MET. The measured TEE was 8447 (SD 1337) kJ/d. The accelerometer significantly underestimated TEE and PAEE (91·9 (SD 5·4) and 92·7 (SD 17·8) % chamber value respectively); however, there was a significant correlation between the two values (r 0·928 and 0·564 respectively; P<0·001). There was a strong correlation between the activity levels and the measured MET while walking (r2 0·93; P<0·001). Although TEE and PAEE were systematically underestimated during the 24 h period, the accelerometer assessed energy expenditure well during both the exercise period and the non-structured activities. Individual calibration factors may help to improve the accuracy of TEE estimation, but the average calibration factor for the group is probably sufficient for epidemiological research. This method is also important for assessing the diurnal profile of physical activity.

The Orientation control of (Ca,Sr)CuO2 film has been investigated under various sputtering conditions from the view point of controlling the “self-texture” and epitaxy. The films with (001), (110) and (101) orientations were able to be obtained by changing partial gas pressure during sputtering. These orientations were observed on glass and MgO(100) single crystal substrates. The cohesive energy between the film and the MgO single crystal substrate was calculated to evaluate the change in orientation on glass and MgO substrates.