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Carnitine palmitoyltransferase 2 (CPT2) deficiency, the most common inherited disease of the mitochondrial long-chain fatty acid (LCFA) oxidation, may result in distinct clinical phenotypes, namely a mild adult muscular form and a severe hepatocardiomuscular disease with an onset in the neonatal period or in infancy. In order to understand the mechanisms underlying the difference in severity between these phenotypes, we analyzed a cohort of 20 CPT2-deficient patients being affected either with the infantile (seven patients) or the adult onset form of the disease (13 patients). Using a combination of direct sequencing and denaturing gradient gel electrophoresis, 13 CPT2 mutations were identified, including five novel ones, namely: 371G>A (R124Q), 437A>C (N146T), 481C>T (R161W), 983A>G (D328G), and 1823G>C (D608H). After updating the spectrum of CPT2 mutations (n=39) and genotypes (n=38) as well as their consequences on CPT2 activity and LCFA oxidation, it appears that both the type and location of CPT2 mutations and one or several additional genetic factors to be identified would modulate the LCFA flux and therefore the severity of the disease.  相似文献   
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We report a case of noma having occurred in an adult female patient with HIV. The strain was characterized as HIV-1 group M subtype G. In order to explore the interactions between HIV/AIDS and this disease, we purpose systematic HIV screening for any case of noma, especially for adult patients.  相似文献   
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Tandem configuration-containing perovskite and silicon solar cells are promising candidates for realizing a high power conversion efficiency of 30% at reasonable costs. Silicon solar cells with planar front surfaces used in tandem devices cause high optical losses, which significantly affects their efficiency. Moreover, some studies have explored the fabrication of perovskites on textured silicon cells. However, due to improper texturing, light trapping is not ideal in these devices, which reduces the efficiency. In this work, we optimized the pyramid height of textured silicon cells and efficiently characterized them to achieve enhanced light trapping. Two different kinds of perovskites, namely, Cs0.17FA0.6Pb(Br0.17I0.7)3 and Cs0.25FA0.6Pb(Br0.20I0.7)3 with wide bandgaps were conformally deposited on textured silicon cells, and the performance of these flat and fully textured tandem devices was numerically analyzed. The thickness of each layer in the tandem cell was optimized in a way to ensure a perfect current match between the top perovskite and bottom silicon subcells. The results indicated that the textured tandem configuration enhances light absorption over a broad spectral range due to the optimized pyramid height compared to flat surfaces. Eventually, the photovoltaic parameters of the proposed tandem cell were compared with the already existing structures, and our design supports large values of open circuit voltage (Voc) = 1.78 V, short circuit current density (Jsc) = 20.09 mA cm−2, fill factor (FF) = 79.01%, and efficiency (η) = 28.20% compared to other kinds of tandem solar cells.

Tandem configuration-containing perovskite and silicon solar cells are promising candidates for realizing a high power conversion efficiency of 30% at reasonable costs.  相似文献   
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Improving the photon absorption in thin-film solar cells with plasmonic nanoparticles is essential for the realization of extremely efficient cells with substantial cost reduction. Here, a comprehensive study of solar energy enhancement in a cadmium telluride (CdTe) thin-film solar cell based on the simple design of a square array of plasmonic titanium nanoparticles, has been reported. The excitation of localized plasmons in the metallic nanostructures together with the antireflection coating (ARC) significantly enhances the absorption of photons in the active CdTe layer. The proposed structure attained super absorption with a mean absorbance of more than 97.27% covering a wide range from visible to near-infrared (i.e., from 300 nm to 1200 nm), presenting a 90% absorption bandwidth over 900 nm, and the peak absorption is up to 99.9%. For qualitative analysis, the photocurrent density is also estimated for AM 1.5 solar illumination (global tilt), whose value reaches 40.36 mA cm−2, indicating the highest value reported to date. The impact of nanoparticle dimensions, various metal materials, shapes, and random arrangement of nanoparticles on optical absorption are discussed in detail. Moreover, the angle insensitivity is essentially validated by examining the absorption performance with oblique incidences and it is found that the solar cell keeps high absorption efficiency even when the incidence angle is greater than 0°. Therefore, these findings suggest that the proposed broadband structure has good prospect in attaining high power conversion efficiency while reducing the device cost.

Improving the photon absorption in thin-film solar cells with plasmonic nanoparticles is essential for the realization of extremely efficient cells with substantial cost reduction.  相似文献   
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We describe a comprehensive surveillance system involving infection control practitioners, surgeons, administrative staff, and patients aimed at improving the postdischarge surveillance of surgical site infections. The system was able to detect 22 infections out of 538 procedures, 95% of which were detected during the postdischarge period.  相似文献   
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Objectives

Rank Preserving Structural Failure Time models are one of the most commonly used statistical methods to adjust for treatment switching in oncology clinical trials. The method is often applied in a decision analytic model without appropriately accounting for additional uncertainty when determining the allocation of health care resources. The aim of the study is to describe novel approaches to adequately account for uncertainty when using a Rank Preserving Structural Failure Time model in a decision analytic model.

Methods

Using two examples, we tested and compared the performance of the novel Test-based method with the resampling bootstrap method and with the conventional approach of no adjustment. In the first example, we simulated life expectancy using a simple decision analytic model based on a hypothetical oncology trial with treatment switching. In the second example, we applied the adjustment method on published data when no individual patient data were available.

Results

Mean estimates of overall and incremental life expectancy were similar across methods. However, the bootstrapped and test-based estimates consistently produced greater estimates of uncertainty compared with the estimate without any adjustment applied. Similar results were observed when using the test based approach on a published data showing that failing to adjust for uncertainty led to smaller confidence intervals.

Conclusions

Both the bootstrapping and test-based approaches provide a solution to appropriately incorporate uncertainty, with the benefit that the latter can implemented by researchers in the absence of individual patient data.  相似文献   
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