[Autosomal recessive polycystic kidney disease in a girl]. / å¸¸æŸ“è‰²ä½“éšæ€§é—ä¼ æ€§å¤šå›Šè‚¾1例.

A 5-year-old girl was admitted due to one episode of melena and one episode of hematemesis. Upon admission, gastroscopy revealed esophageal and gastric varices. Abdominal CT scan, MRI, and color Doppler ultrasound suggested cirrhosis, intrahepatic bile duct dilation, and bilateral kidney enlargement. Genetic testing identified compound heterozygous mutations in the PKHD1 gene: c.2264C>T (p.Pro755Leu) and c.1886T>C (p.Val629Ala). The c.2264C>T (p.Pro755Leu) mutation is a known pathogenic variant with previous reports, while c.1886T>C (p.Val629Ala) is a novel mutation predicted to have pathogenic potential according to Mutation Taster and PolyPhen2. The child was diagnosed with autosomal recessive polycystic kidney disease. In children presenting with gastrointestinal bleeding without obvious causes, particularly those with liver or kidney disease, consideration should be given to the possibility of autosomal recessive polycystic kidney disease, and genetic testing should be conducted for definitive diagnosis when necessary.

Distribution patterns of the contents of five active components in taproot and stolon of Glycyrrhiza uralensis.

Wild or cultivated Glycyrrhiza uralensis FISCHER (G. uralensis) are the main source of licorice, and they contain the similar compounds, such as the triterpenoid saponins and flavonoids, but above two kinds of the components contents are low level in the cultivated licorice. To produce the high quality cultivated licorices, researchers studied the affecting factors about the compounds producing in the plant of licorice, and then found that the growth years, genetic differences and water deficit are all the important factors. In this paper, we found that there were different distribution patterns of the main five active components (FAC) including glycyrrhizin, liquiritin, isoliquiritin, liquiritigenin and isoliquiritigenin in the taproot and stolon of G. uralensis and maybe they are also important influence factors to the FAC contents of the licorices. In wild G. uralensis, the contents of FAC tended to be lower in the younger parts of the stolon, and in the cultivated G. uralensis taproot, the contents of glycyrrhizin, liquiritin and isoliquiritin tended to increase from top to end, contrary to the contents of liquiritigenin and isoliquiritigenin, which increased first and then decreased. Our results will contribute to the analyses of factors which influence the quality of licorice, and provide some reference for cultivating high quality licorices for herbal medicine.

[Acquisition and storage methods for image data collected in Chinese medicine resources survey].

The acquisition and storage of the image data are important in the Chinese medicine resources survey, and it is important data and evidence for the process and the results. The image data of the Chinese medicinal materials' habitat, original plant or animal, processing in habitat, commodity form, the relative contents and workshop scenarios in the investigation are important for the compiling of the Color Atlas of National Chinese Medicine Resources, mapping the digital scattergram of the Chinese medicine resources, establishing the digital Chinese medicine plant herbarium and acquiring the documentary of the Chinese medicine resource survey. The content, procedures and methods of the video data collecting have been related and analyzed in this article to provide reference for the Chinese medicine resources survey.

[Wild-tending techniques study on Glycyrrhiza uralensis--effect of irrigation and rhizome length on survival ratio, yield and quality].

This research aimed at studying the effects of irrigation and rhizome length on the survival of ratio, yield and quality of Glycyrrhiza uralensis in wild tending condition. Employed the split-block design to carry out the field experiment, sampled with the quadrat method to measured the relative growth indexes and to estimate the yield, used the HPLC (high performance liquid chromatog- raphy ) method to measure the glycyrrhizin in the rhizome and adventitious root of the G. uralensis in this study. The quantity of the adventitious roots and the survival ratio were increased significantly as the length of the rhizome increased (P < 0.01), but the length of the rhizome had no remarkable effect on the content of glycyrrhizin. The average content of the glycyrrhizin in the adventitious root and rhizome could reach 3.03% and 2.12% after 3-year wild tending, respectively, and this results indicated that the quality of the glycyrrhiza using this method was much better than that from cultured glycyrrhiza with the reproducing method of seeding. so using the rhizome as reproductive material to produce the glycyrrhiza under the wild tending condition could get the high quality glycyrrhiza quick- ly and steadily, this phenomenon could be explained by the Hypothesis of synthetic inertia of the medicinal components from the wild material of G. uralensis. But the maximum yield with this method was just more than 945 kg x hm(-2) in this study. So the further work of how to increase the yield in the practical application with the method found in this study need to be done in the next research.

EGFR inhibitors sensitize non-small cell lung cancer cells to TRAIL-induced apoptosis.

Apoptosis induced by tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) can be regulated by the epidermal growth factor (EGF) signaling pathway. In this study, recombinant adenoviral vectors that encode TRAIL gene from the hTERT/RGD promoter (AdTRAIL) was combined with drugs including gefitinib, elotinib, and cetuximab that inhibit EGFR and the EGF signaling pathway in non-small cell lung cancer (NSCLC) cell lines to investigate their antitumor activity. In vitro, compared to single reagent, AdTRAIL combined with EGFR inhibitors reduced proliferation and enhanced apoptosis in H460, A549, and SW1573 cell lines. Western blot results suggested that these effects were relative to up-regulation of pro-apoptosis protein BAX and down-regulation of p-AKT. In vivo, AdTRAIL combined with cetuximab resulted in a significant growth reduction in H460 xenografts without damage to the main organs of nude mice. Histological examination and TUNEL analyses of xenografts showed that cetuximab enhanced cell apoptosis induced by AdTRAIL. These results indicate that EGFR inhibitors enhanced AdTRAIL anti-tumor activity in NSCLC cell lines and that inhibiting the AKT pathway played an important role in this enhancement.

Highly Bendable and Durable Waterproof Paper for Ultra-High Electromagnetic Interference Shielding.

An efficient electromagnetic interference (EMI) shielding paper with excellent water repellency and mechanical flexibility has been developed, by assembling silver nanowires (AgNWs) and hydrophobic inorganic ceramic on the cellulose paper, via a facile dip-coating preparation. Scanning electron microscope (SEM) observations confirmed that AgNWs were interconnected and densely coated on both sides of the cellulose fiber, which endows the as-prepared paper with high conductivity (33.69 S/cm in-plane direction) at a low AgNW area density of 0.13 mg/cm2. Owing to multiple reflections and scattering between the two outer highly conductive surfaces, the obtained composite presented a high EMI shielding effectiveness (EMI SE) of up to 46 dB against the X band, and ultrahigh specific EMI SE of 271.2 dB mm-1. Moreover, the prepared hydrophobic AgNW/cellulose (H-AgNW/cellulose) composite paper could also maintain high EMI SE and extraordinary waterproofness (water contact angle > 140°) by suffering dozens of bending tests or one thousand peeling tests. Overall, such a multifunctional paper might have practical applications in packaging conductive components and can be used as EMI shielding elements in advanced application areas, even under harsh conditions.

Layer-Structured Design and Fabrication of Cyanate Ester Nanocomposites for Excellent Electromagnetic Shielding with Absorption-Dominated Characteristic.

In this work, we propose novel layer-structured polymer composites (PCs) for manipulating the electromagnetic (EM) wave transport, which holds unique electromagnetic interference (EMI) shielding features. The as-prepared PCs with a multilayered structure exhibits significant improvement in overall EMI shielding effectiveness (EMI SE) by adjusting the contents and distribution of electrical and magnetic loss fillers. The layer-structured PCs with low nanofiller content (5 wt % graphene nanosheets (GNSs) and 15 wt % Fe3O4) and a thickness of only 2 mm exhibited ultrahigh electrical conductivity and excellent EMI SE, reaching up to 2000 S/m and 45.7 dB in the X-band, respectively. The increased EMI SE of the layer-structured PCs was mainly based on the improved absorption rather than the reflection of electromagnetic waves, which was attributed to the "absorb-reflect-reabsorb" process for the incident electromagnetic waves. This work may provide a simple and effective approach to achieve new EMI shielding materials, especially for absorption-dominated EMI shielding.