Gaucher disease in a patient with membranoproliferative glomerulonephritis: case report.

BACKGROUND: Gaucher disease (GD) is a rare autosomal recessive inherited, lysosomal storage disoder that involves liver, spleen, lung, bone, bone marrow even central nervous. However, GD associated membranoproliferative glomerulonephritis (MPGN) is seldom reported. CASE PRESENTATION: Here we described a case of 35-year-old man suffering from GD with hepatosplenomegaly, ascites, bone destruction, myelofibrosis and MPGN. Renal biopsy revealed MPGN and Gaucher cells presented in the glomeruli capillaries. ß-glucosidase activity was 1.95nmol/1 h/mg and gene detection demonstrated that one homozygous pathogenic variant Leu483Pro in GBA. He received the treatment of oral prednisone and mycophenolate mofetil and his ascites and renal outcomes had been significantly improved. CONCLUSIONS: Therapy of prednisone and mycophenolate mofetil may be an optional choice for patients with Gaucher disease who have no opportunity to use enzyme treatment.

A Wrinkled Ag/CNTs-PDMS Composite Film for a High-Performance Flexible Sensor and Its Applications in Human-Body Single Monitoring.

In this paper, a flexible Ag/CNTs-PDMS (polydimethylsi-loxane) composite film sensor based on the novel design philosophy was prepared. Its force-electric effect mechanism is based on the generation of micro-cracks in the Ag film during external forcing, leading to resistance variation. Experimental results find that Ag film thickness has a strong influence on the sensor's sensitivity, which exhibits a tendency of first increasing and then decreasing the Ag film thickness, and also has an optimal thickness of 4.9 µm for the maximum sensitivity around 30. The sensitive mechanism can be theoretically explained by using the quantum tunneling effect. Due to the use of the wrinkled carbon nanotubes (CNTs) film, this sensor has advantages, such as high sensitivity, large strain range, good stability and durability, cheap price, and suitability for large-scale production. Preliminary applications on human-body monitoring reveal that the sensor can detect weak tremors and breathe depth and rate, and the corresponding heartbeat response. It provides possibilities to diagnose early Parkinson's disease and exploit an early warning system for sudden infant death syndrome and sleep apnea in adults. In addition, as a force-electric effect sensor, it is expected to have broad application areas, such as a man-machine cooperation, and a robotic system.

Flexible photodetectors based on reticulated SWNT/perovskite quantum dot heterostructures with ultrahigh durability.

Recently, single-walled carbon nanotube (SWNT) films have been regarded as a promising channel material for flexible photodetectors due to their high intrinsic carrier mobility, conductivity, and mechanical flexibility. However, the application of SWNTs in photonic devices is limited due to their weak light absorption and the absence of a gain mechanism. Here, we demonstrate a high-performance flexible photodetector that consists of a reticulated SWNT film covered with a thin film of CsPbI3 perovskite colloidal quantum dots. The unique hierarchical reticulated structure of the SWNTs provides such films with extremely high tensile strength and great extensibility, which can ensure the appropriate toughness for achieving flexible photodetectors. Meanwhile, the perovskite quantum dots enhance light absorption, thereby sensitizing the creation of free electrical carriers within the SWNTs. This hybrid photodetector exhibits an extended photonic response and gain compared with the original pure SWNT devices. In addition, the device exhibits good robustness against repetitive bending and stretching, suggesting its applicability as a large-area wearable flexible photodetector.

Functionalized Graphene for Mechanical Property Enhancement of Polymer Composites.

Because of its high strength and high toughness, graphene has been widely used in mechanical reinforced composites. In general, the mechanical enhancement depends mainly on the properties of graphene itself and the number of surface chemical functional groups attached on it. In this paper, we report a method to improve the mechanical performance of polymer by using a kind of functionalized reduced graphene oxide (F-RGO), i.e., the F-RGO is prepared by chemical treatment, and then the F-RGO/polyvinylidene fluoride (PVDF) composite films are obtained by spin coating. Because the chemical treatment can increase the number of functional groups on the surface and edge of F-RGO, these functional groups make the F-RGO sheets strongly coupled with PVDF molecules, so as to achieve the purpose of mechanical enhancement. The experimental results reveal that the mechanical properties of the F-RGO/PVDF composite films are improved for 42% times, when comparing with regular RGO/PVDF composite films.

Synthesis and enhanced microwave absorption properties: a strongly hydrogenated TiO2 nanomaterial.

Due to its improved physical and chemical performances, a strongly hydrogenated TiO2 was designed and produced successfully by using a sealing-transfer reduction method at a relatively low temperature (425 °C). The microstructures, electromagnetic and microwave absorbing properties were investigated in detail. Experimental results revealed that: (1) the minimum reflection loss (RL) value of the hydrogenated TiO2 up to -53.8 dB (99.999 99% of EM wave attenuation) was reached at 11.2 GHz, and the RL values below -20 dB (99%) were obtained in a frequency range of 7.3-16.8 GHz. (2) Compared to pristine TiO2 and black TiO2 in other reports, the present hydrogenated TiO2 exhibited greatly improved microwave absorption performance. Moreover, the mechanism was also discussed. It was demonstrated that the excellent microwave absorption performance of the black TiO2 arose from the strong dielectric loss, excellent impedance matching and attention loss due to associated relaxation and interfacial polarization. It is expected that the hydrogenated TiO2 exhibits great potential applications in the area of high performance microwave absorbing materials. In addition, it is believed that the black TiO2 @ magnetic metals composites will display an excellent microwave absorbing property.

Present Perspectives of Advanced Characterization Techniques in TiO2-Based Photocatalysts.

TiO2 is the most investigated photocatalyst because of its nontoxicity, low cost, chemical stability, and strong photooxidative ability. Because of the morphology- and structure-dependent photocatalytic properties of TiO2, accurate characterization of the crystal and electronic structures of TiO2-based materials and their performance during the photocatalytic process is crucial not only for understanding the photocatalytic mechanism but also for providing experimental guidelines as well as a theoretical framework for the synthesis of high performance photocatalysts. In this review, we focused on the advanced characterization techniques that are utilized in the studies on the TiO2 structures and photocatalytic performance of TiO2 and TiO2-based materials. It is therefore anticipated that this review can provide a novel perspective to understand the fundamental aspects of photocatalysis and inspire the development of new photocatalysts with superior performances.

Highly Sensitive, Durable, and Multifunctional Sensor Inspired by a Spider.

Sensitivity, durability, and multifunction are the essential requirements for a high-performance wearable sensor. Here, we report a novel multifunctional sensor with high sensitivity and durability by using a buckled spider silk-like single-walled carbon nanotubes (SSL-SWNTs) film as the conducting network and a crack-shaped Au film as the sensitive transducer. Its high sensitivity is inspired by the crack-shaped structure of the spider's slit organs, while the high durability is inspired by the mechanical robustness of the spider silk. Similar to the spider's slit organs that can detect slight vibrations, our sensor also exhibits a high sensitivity especially to tiny strain. The proposed quantum tunneling model is consistent with experimental data. In addition, this sensor also responds sensitively to temperature with the sensitivity of 1.2%/°C. Because of the hierarchical structure like spider silk, this sensor possesses combined superiority of fast response (<60 ms) and high durability (>10 000 cycles). We also fabricate a wearable device for monitoring various human physiological signals. It is expect that this high-performance sensor will have wide potential applications in intelligent devices, fatigue detection, body monitoring, and human-machine interfacing.

Bioinspired Single-Walled Carbon Nanotubes as a Spider Silk Structure for Ultrahigh Mechanical Property.

Due to its unique hierarchical structure, natural spider silk features exceptional mechanical properties such as high tensile strength and great extensibility, making it one of the toughest materials. Herein, we design bioinspired spider silk single-walled carbon nanotubes (BISS-SWCNTs) that combine the hierarchical structure of spider silk and the high strength and conductivity of SWCNTs. To imitate the hierarchical structure, Fe nanoparticles are embedded on the surface of directly synthesized SWCNTs skeleton followed by coating an amorphous carbon layer. The carbon layer forms the spider silk-featured skin-core structure with SWCNTs, thus making the tube junction tougher. The embedded Fe nanoparticles act as glue spots for preventing interfacial slippages between the BISS-SWCNTs and the reinforced matrix. With only 2.1 wt % BISS-SWCNTs added, the tensile strength and Young's modulus of the BISS-SWCNTs/PMMA composites can be improved by 300%. More importantly, the BISS-SWCNTs also retain the high conductivity and transmittance of the pristine SWCNTs film. This unique bioinspired material will be of great importance in applications of multifunctional composite materials and has important implications for the future of biomimetic materials.

A rational design for the separation of metallic and semiconducting single-walled carbon nanotubes using a magnetic field.

The separation of metallic (m-) and semiconducting (s-) single-walled carbon nanotubes (SWNTs) without causing contamination and damage is a major challenge for SWNT-based devices. As a facile and nondestructive tool, the use of a magnetic field could be an ideal strategy to separate m-/s-SWNTs, based on the difference of magnetic susceptibilities. Here, we designed a novel magnetic field-assisted floating catalyst chemical vapor deposition system to separate m-/s-SWNTs. Briefly, m-SWNTs are attracted toward the magnetic pole, leaving s-SWNTs on the substrate. By using this strategy, s-SWNTs with a purity of 99% could be obtained, which is enough to construct high-performance transistors with a mobility of 230 cm(2) V(-1) s(-1) and an on/off ratio of 10(6). We also established a model to quantitatively calculate the percentage of m-SWNTs on the substrate and this model shows a good match with the experimental data. Furthermore, our rational design also provides a new avenue for the growth of SWNTs with specific chirality and manipulated arrangement due to the difference of magnetic susceptibilities between different diameters, chiralities, and types.

Diamond synthesis from carbon nanofibers at low temperature and low pressure.

In this article, we report a new route to synthesize diamond by converting "solid" carbon nanofibers with a Spark Plasma Sintering system under low temperature and pressure (even at atmospheric pressure). Well-crystallized diamond crystals are obtained at the tips of the carbon nanofibers after sintering at 1500 °C and atmospheric pressure. Combining with scanning electron microscopy, transmission electron microscopy, electron-energy loss spectroscopy and Raman spectroscopy observations, we propose the conversion mechanism as follows: the disorder "solid" carbon nanofibers→well crystallined carbon nanofibers→bent graphitic sheets→onion-liked rings→diamond single crystal→the bigger congregated diamond crystal. It is believed that the plasma generated by low-voltage, vacuum spark, via a pulsed DC in Spark Plasma Sintering process, plays a critical role in the low temperature and low pressure diamond formation. This Spark Plasma Sintering process may provide a new route for diamond synthesis in an economical way to a large scale.

Facile Synthesis of Carbon Nanosphere/NiCo2O4 Core-shell Sub-microspheres for High Performance Supercapacitor.

This paper introduced a process to prepare the carbon nanosphere (CNS)/NiCo2O4 core-shell sub-microspheres. That is: 1) CNSs were firstly prepared via a simple hydrothermal method; 2) a layer of NiCo2O4 precursor was coated on the CNS surface; 3) finally the composite was annealed at 350 °C for 2 hours in the air, and the CNS/NiCo2O4 core-shell sub-microspheres were obtained. This core-shell sub-microsphere was prepared with a simple, economical and environmental-friendly hydrothermal method, and was suitable for large-scale production, which expects a promising electrode candidate for high performance energy storage applications. Electrochemical experiments revealed that the composite exhibited remarkable electrochemical performances with high capacitance and desirable cycle life at high rates, such as: 1) the maximum specific capacitance was up to 1420 F/g at 1 A/g; 2) about 98.5% of the capacitance retained after 3000 charge-discharge cycles; 3) the capacitance retention was about 72% as the current density increase from 1 A/g to 10 A/g.

Strong magnetic field-assisted growth of carbon nanofibers and its microstructural transformation mechanism.

It is well-known that electric and magnetic fields can control the growth direction, morphology and microstructure of one-dimensional carbon nanomaterials (1-DCNMs), which plays a key role for its potential applications in micro-nano-electrics and devices. In this paper, we introduce a novel process for controlling growth of carbon nanofibers (CNFs) with assistance of a strong magnetic field (up to 0.5 T in the center) in a chemical vapor deposition (CVD) system. The results reveal that: 1) The CNFs get bundled when grown in the presence of a strong magnetic field and slightly get aligned parallel to the direction of the magnetic field; 2) The CNFs diameter become narrowed and homogenized with increase of the magnetic field; 3) With the increase of the magnetic field, the microstructure of CNFs is gradually changed, i.e., the strong magnetic field makes the disordered "solid-cored" CNFs transform into a kind of bamboo-liked carbon nanotubes; 4) We propose a mechanism that the reason for these variations and transformation is due to diamagnetic property of carbon atoms, so that it has direction selectivity in the precipitation process.

Strain induced chemical potential difference between monolayer graphene sheets.

Monolayer graphene sheets were deposited on a transparent and flexible polydimethylsiloxane (PDMS) substrate, and a tensile strain was loaded by stretching the substrate in one direction. It was found that an electric potential difference between stretched and static monolayer graphene sheets reached 8 mV when the strain was 5%. Theoretical calculations for the band structure and total energy revealed an alternative way to experimentally tune the band gap of monolayer graphene, and induce the generation of electricity.