We present an approach to safely expose the hepatic hilum for revision procedures after left lobe living donor liver transplantation. A 14-year-old adolescent girl who had undergone left lobe living donor liver transplantation experienced repeated episodes of cholangitis. Because treatment with interventional techniques failed, surgical revision was indicated. The right thoracoabdominal approach was selected to minimize dissection. Intraoperative findings showed adhesive kinking of the Roux-Y limb just distal to the bilioenteric anastomosis, and a side-to-side jejunojejunostomy was performed. The thoracoabdominal approach leads to easy and excellent reoperative exposure of the hilar site of a left lobe liver graft.

Bacterial infection associated with the use of medical or dental devices is a serious concern. Although devices made of ethylene vinyl acetate (EVA) are often used in the oral cavity, there are no established standards for their storage. We investigated bacterial survival on EVA sheets under various storage conditions to establish a standard for hygienic storage of such dental devices. Bacterial counts were evaluated, which showed a significant decrease after washing with sterilized water, mechanical brushing and rinsing, and using Mouthguard Cleaner as compared to untreated samples. In addition, no bacteria were detected on samples stored 2 days or longer in a ventilated environment, whereas they were detected for up to 14 days on samples without any cleaning stored in a closed environment. Bacterial counts for the untreated samples gradually declined, while surviving bacteria on samples treated with sterilized water and mechanical brushing showed a rapid decrease. Additionally, bacterial identification using polymerase chain reaction (PCR) revealed that Streptococcus oralis was dominantly detected on salivary samples after 14 days of storage among both two subjects. For effective hygienic storage of dental devices made of EVA, washing with sterilized water is important to remove absorbed salivary compounds along with storage in a ventilated environment. International Journal of Oral Science advance online publication, 16 March 2012; doi:10.1038/ijos.2012.13.

We report two patients with Leigh syndrome that showed a combination of facial dysmorphism and MRI imaging indicating an SURF1 deficiency, which was confirmed by sequence analysis. Case 1 is a 3-year-old girl with failure to thrive and developmental delay. She presented with tachypnea at rest and displayed facial dysmorphism including frontal bossing, lateral displacement of inner canthi, esotropia, maxillary hypoplasia, slightly upturned nostril, and hypertrichosis dominant on the forehead and extremities. Case 2 is an 8-year-old boy with respiratory failure. He had been diagnosed as selective complex IV deficiency. Case 2 displayed facial dysmorphism and hypertrichosis. Since both patients displayed characteristic facial dysmorphism and MRI findings, we sequenced the SURF1 gene and identified two heterozygous mutations; c.49+1 G>T and c.752_753del in Case 1, and homozygous c.743 C>A in Case 2. For patients with Leigh syndrome showing these facial dysmorphism and hypertrichosis, sequence analysis of the SURF1 gene may be useful.

Dynamic visual acuity (DVA) is defined as the ability to discriminate the fine parts of a moving object. DVA is generally better in athletes than in non-athletes, and the better DVA of athletes has been attributed to a better ability to track moving objects. In the present study, we hypothesized that the better DVA of athletes is partly derived from better perception of moving images on the retina through some kind of perceptual learning. To test this hypothesis, we quantitatively measured DVA in baseball players and non-athletes using moving Landolt rings in two conditions. In the first experiment, the participants were allowed to move their eyes (free-eye-movement conditions), whereas in the second they were required to fixate on a fixation target (fixation conditions). The athletes displayed significantly better DVA than the non-athletes in the free-eye-movement conditions. However, there was no significant difference between the groups in the fixation conditions. These results suggest that the better DVA of athletes is primarily due to an improved ability to track moving targets with their eyes, rather than to improved perception of moving images on the retina.

Hepatoportal sclerosis accompanied by dense elastic fibre deposition is generally regarded as the primary lesion in the development of idiopathic portal hypertension (IPH). This study was performed to clarify the mechanism of elastic fibre deposition in the peripheral portal tracts of IPH liver in relation to serum anti-endothelial cell antibodies (AECA). In-vitro experiments were performed using human dermal microvascular endothelial cells (HMVEC) and patients' sera. The presence of serum AECA was assayed by a cell-based enzyme-linked immunosorbent assay (ELISA) using HMVEC. Immunohistochemical analysis of elastin was performed using liver tissue sections of IPH patients. IPH sera contained one or more AECA that could bind to the vascular endothelial cells of the peripheral portal tracts of the liver. When the value of AECA greater than the mean ± 2 standard deviations of healthy controls was regarded as positive, the positive detection rate of either immunoglobulin (Ig)G, IgA or IgM AECA in IPH sera was 30%(10 of 33 cases). IPH sera induced the expression of elastin in HMVEC, which appeared to be associated with the presence of AECA. Apoptosis was also induced in HMVEC by the stimulation with IPH sera. In vivo, elastin expression was observed in the endothelial cells of the peripheral portal tracts of IPH livers in a proportion of cases. The disease pathogenesis of IPH seems to be heterogeneous, and this study elucidated a possible contribution of the induction of elastin expression in the portal vessels to hepatoportal sclerosis of IPH, which might be linked to serum AECA as a causative factor.

OBJECTIVE: To assess the quality of life of patients with narcolepsy with cataplexy (NA-CA), narcolepsy without cataplexy (NA w/o CA), and idiopathic hypersomnia without long sleep time (IHS w/o LST) who were taking psychostimulant medication, and to ascertain which factors (including psychosocial and environmental variables) influence quality of life in this population. METHODS: In total, 185 patients who had received regular treatment were enrolled in the study (NA-CA, n=83; NA w/o CA, n=48; IHS w/o LST, n=54). Patients were asked to complete questionnaires including the Short Form-36 Health Survey (SF-36), the Epworth Sleepiness Scale (ESS), and items concerning psychosocial and environmental variables. RESULTS: All three diagnostic groups had significantly lower scores for most SF-36 domains compared with the Japanese normative data, and the ESS score was significantly reduced with treatment. Multiple logistic regression analyses revealed that several SF-36 domains were associated with the ESS score; autonomy in controlling own job schedule, experience of divorce or break up with a partner due to symptoms, experience of being forced to relocate or being dismissed due to symptoms, and perception of support from others. CONCLUSIONS: The severity of subjective sleepiness and psychological and environmental variables influenced quality of life in patients with these hypersomnias of central origin.

Previous simulation and experimental studies have demonstrated that the application of Variational Bayesian Multimodal EncephaloGraphy (VBMEG) to magnetoencephalography (MEG) data can be used to estimate cortical currents with high spatio-temporal resolution, by incorporating functional magnetic resonance imaging (fMRI) activity as a hierarchical prior. However, the use of combined MEG and fMRI is restricted by the high costs involved, a lack of portability and high sensitivity to body-motion artifacts. One possible solution for overcoming these limitations is to use a combination of electroencephalography (EEG) and near-infrared spectroscopy (NIRS). This study therefore aimed to extend the possible applications of VBMEG to include EEG data with NIRS activity as a hierarchical prior. Using computer simulations and real experimental data, we evaluated the performance of VBMEG applied to EEG data under different conditions, including different numbers of EEG sensors and different prior information. The results suggest that VBMEG with NIRS prior performs well, even with as few as 19 EEG sensors. These findings indicate the potential value of clinically applying VBMEG using a combination of EEG and NIRS.

Cardiomyocytes derived from human induced pluripotent stem cells (hiPS-CMs) hold great promise for development of in vitro research tools to assess cardiotoxicity, including QT prolongation. In the present study, we aimed to clarify the electrophysiological/pharmacological characteristics of hiPS-CMs using the patch-clamp technique. The hiPS cells were differentiated into beating cardiomyocytes by the embryoid body method. The expression of genes related to cardiac ion channels and differentiation markers in cardiomyocytes were detected by RT-PCR. Whole-cell patch-clamp recordings were performed using single hiPS-CMs dispersed from beating colonies. We confirmed voltage-dependence of major cardiac ion currents (I(Na), I(Ca), I(Kr), and I(Ks)) and pharmacological responses to ion-channel blockers. Action potential duration (APD) was prolonged by both I(Kr)/hERG and I(Ks) blockers, whereas it was shortened by an I(Ca) blocker, indicating that these ion current components contribute to action potential generation in hiPS-CMs. As for multiple ion channel blockers, terfenadine prolonged APD, but verapamil did not, results which were identical to clinically relevant pharmacological responses. These data suggest that patch-clamp assay using hiPS-CMs could be an accurate method of predicting the human cardiac responses to drug candidates. This study would be helpful in establishing an electrophysiological assay to assess the risk of drug-induced arrhythmia using hiPS-CMs.

This study examined the neurobehavioral changes and alteration in gene expression in the brains of metallothionein (MT)-I/II null mice exposed to low-levels of mercury vapor (Hg(0)) during postnatal development. MT-I/II null and wild-type mice were repeatedly exposed to Hg(0) at 0.030 mg/m(3)(range: 0.023-0.043 mg/m(3)), which was similar to the current threshold value (TLV), for 6 hr per day until the 20th day postpartum. The behavioral effects were evaluated with locomotor activity in the open field (OPF), learning ability in the passive avoidance response (PA) and spatial learning ability in the Morris water maze (MM) at 12 weeks of age. Hg(0)-exposed MT-I/II null mice showed a significant decrease in total locomotor activity in females, though learning ability and spatial learning ability were not affected. Immediately after Hg(0) exposure, mercury concentrations in the brain did not exceed 0.5 µg/g in any animals. Hg(0) exposure resulted in significant alterations in gene expression in the brains of both strains using DNA microarray analysis. The number of altered genes in MT-I/II null mice was higher than that in wild-type mice and calcium-calmodulin kinase II (Camk2a) involved in learning and memory in down-regulated genes was detected. These results provide useful information to elucidate the development of behavioral toxicity following low-level exposure to Hg(0).