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Thalidomide induces γ-globin expression in erythroid progenitor cells, but its efficacy on patients with transfusion-dependent β-thalassemia (TDT) remains unclear. In this phase 2, multi-center, randomized, double-blind clinical trial, we aimed to determine the safety and efficacy of thalidomide in TDT patients. A hundred patients of 14 years or older were randomly assigned to receive placebo or thalidomide for 12 weeks, followed by an extension phase of at least 36 weeks. The primary endpoint was the change of hemoglobin (Hb) level in the patients. The secondary endpoints included the red blood cell (RBC) units transfused and adverse effects. In the placebo-controlled period, Hb concentrations in patients treated with thalidomide achieved a median elevation of 14.0 (range, 2.5 to 37.5) g/L, whereas Hb in patients treated with placebo did not significantly change. Within the 12 weeks, the mean RBC transfusion volume for patients treated with thalidomide and placebo was 5.4 ± 5.0 U and 10.3 ± 6.4 U, respectively ( P  < 0.001). Adverse events of drowsiness, dizziness, fatigue, pyrexia, sore throat, and rash were more common with thalidomide than placebo. In the extension phase, treatment with thalidomide for 24 weeks resulted in a sustainable increase in Hb concentrations which reached 104.9 ± 19.0 g/L, without blood transfusion. Significant increase in Hb concentration and reduction in RBC transfusions were associated with non β0 / β0 and HBS1L - MYB (rs9399137 C/T, C/C; rs4895441 A/G, G/G) genotypes. These results demonstrated that thalidomide is effective in patients with TDT.

Inorganic superionic conductors possess high ionic conductivity and excellent thermal stability but their poor interfacial compatibility with lithium metal electrodes precludes application in all-solid-state lithium metal batteries 1 , 2 . Here we report a LaCl 3 -based lithium superionic conductor possessing excellent interfacial compatibility with lithium metal electrodes. In contrast to a Li 3 MCl 6 (M = Y, In, Sc and Ho) electrolyte lattice 3 – 6 , the UCl 3 -type LaCl 3 lattice has large, one-dimensional channels for rapid Li + conduction, interconnected by La vacancies via Ta doping and resulting in a three-dimensional Li + migration network. The optimized Li 0.388 Ta 0.238 La 0.475 Cl 3 electrolyte exhibits Li + conductivity of 3.02 mS cm −1 at 30 °C and a low activation energy of 0.197 eV. It also generates a gradient interfacial passivation layer to stabilize the Li metal electrode for long-term cycling of a Li–Li symmetric cell (1 mAh cm −2 ) for more than 5,000 h. When directly coupled with an uncoated LiNi 0.5 Co 0.2 Mn 0.3 O 2 cathode and bare Li metal anode, the Li 0.388 Ta 0.238 La 0.475 Cl 3 electrolyte enables a solid battery to run for more than 100 cycles with a cutoff voltage of 4.35 V and areal capacity of more than 1 mAh cm −2 . We also demonstrate rapid Li + conduction in lanthanide metal chlorides (LnCl 3 ; Ln = La, Ce, Nd, Sm and Gd), suggesting that the LnCl 3 solid electrolyte system could provide further developments in conductivity and utility. A LaCl 3 -based lithium superionic conductor is developed that has excellent interfacial compatibility with lithium metal electrodes, with its optimized Li 0.388 Ta 0.238 La 0.475 Cl 3 electrolyte exhibiting good Li + conductivity and low activation energy.

Fabricating a robust interfacial layer on the lithium metal anode to isolate it from liquid electrolyte is vital to restrain the rapid degradation of a lithium metal battery. Here, we report that the solution-processed metal chloride perovskite thin film can be coated onto the lithium metal surface as a robust interfacial layer to shield the lithium metal from liquid electrolyte. Via phase analysis and density functional theory calculations, we demonstrate that the perovskite layer can allow fast lithium ion shuttle under a low energy barrier of 0.45 eV without the collapse of framework. Such perovskite modification can realize stable cycling of LiCoO 2 |Li cells with an areal capacity of 2.8 mAh cm −2 using thin lithium metal foil (50 μm) and limited electrolyte (20 μl mAh −1 ) for over 100 cycles at 0.5 C. The metal chloride perovskite protection strategy could open a promising avenue for advanced lithium metal batteries. Metal halide perovskite is well-known for the high absorption coefficient; however, its Li-ion transport property remains poorly explored. Here the authors coat an ion conducting metal chloride perovskite interfacial layer on the Li metal anode, enabling good battery performance.

Photoelectrochemical catalysis is an attractive way to provide direct hydrogen production from solar energy. However, solar conversion efficiencies are hindered by the fact that light harvesting has so far been of limited efficiency in the near-infrared region as compared to that in the visible and ultraviolet regions. Here we introduce near-infrared-active photoanodes that feature lattice-matched morphological hetero-nanostructures, a strategy that improves energy conversion efficiency by increasing light-harvesting spectral range and charge separation efficiency simultaneously. Specifically, we demonstrate a near-infrared-active morphological heterojunction comprised of BiSeTe ternary alloy nanotubes and ultrathin nanosheets. The heterojunction’s hierarchical nanostructure separates charges at the lattice-matched interface of the two morphological components, preventing further carrier recombination. As a result, the photoanodes achieve an incident photon-to-current conversion efficiency of 36% at 800 nm in an electrolyte solution containing hole scavengers without a co-catalyst. The solar conversion efficiencies of photoelectrochemical catalysis are hindered by the light harvesting range. Here, the authors use near-infrared-active photoanodes that feature lattice-matched morphological hetero-nanostructures to realize efficient photoelectrochemical hydrogen production.

Objectives To investigate the value of colour Doppler echocardiography (CDE) in occlusion of elderly with atrial septal defect (ASD). Methods In total of 64 cases of elderly ASD, observe the location, size and residual edge using two-dimensional echocardiography (2DE), observe the atrial septal colourful shunt bunch flow signal and the heart valve colourful regurgitation bunch flow signal using colour Doppler flow imaging (CDFI) and measure the pulmonary artery pressure using continuous Doppler (CW) before the occlusion. If the patient had atrial fibrillation or multiple ASDs, transesophageal echocardiography (TEE) examination should be used to screen the patients according to the indication of ASD occlusion. During the procedure of occlusion of ASD, 2DE was used to monitor the position of occluder, CDFI was used to monitor residual shunt through the atrial septum and CDE was used to determine the efficacy of occlusion. Results According to the results of CDE, we selected 64 cases of elderly ASD, the implementation of all ASD occlusion were successful and the efficacy was satisfactory. Conclusions CDE plays a important role in ASD occlusion. Before ASD occlusion, 2DE is recommedated to observe the location, size and residual edge, CW is recommedated to measure the pulmonary artery pressure. TEE is the key to screen according to the indications of ASD occlusion for the patients with atrial fibrillation or multiple ASDs. All elderly patients with ASD should undergo coronary angiography before the occlusion to exclude the patients with coronary artery disease who can’t treat by stenting.

Background： Hypoparathyroidism-deafness-renal dysplasia （HDR） syndrome is an autosomal dominant disorder primarily caused by haploinsufficiency of GATA binding protein 3 （GATA3） gene mutations, and hearing loss is the most frequent phenotypic feature. This study aimed at identifying the causative gene mutation for a three-generation Chinese t;amily with HDR syndrome and analyzing auditory phenotypes in all familial HDR syndrome cases. Methods： Three affected family members underwent otologic examinations, biochemistry tests, and other clinical evaluations. Targeted genes capture combining next-generation sequencing was pertbrmed within the family. Sanger sequencing was used to confirm the causative mutation. The auditory phenotypes of all reported familial H DR syndrome cases analyzed were provided. Results： In Chinese family 712 l, a heterozygous nonsense mutation c.826C〉T （p.R276＊） was identified in GA TA3. All the three affected members suffered from sensorineural deafness and hypocalcemia; however, renal dysplasia only appeared in the youngest patient. Furthermore, an overview of thirty HDR syndrome families with corresponding GATA3 mutations revealed that hearing impairment occurred earlier in the younger generation in at least nine familial cases （30%） and two thirds of them were found to carry premature stop mutations. Conclusions： This study highlights the phenotypic heterogeneity of HDR and points to a possible genetic anticipation in patients with HDR, which needs to be further investigated.

Inorganic superionic conductors possess high ionic conductivity and excellent thermal stability but their poor interfacial compatibility with lithium metal electrodes precludes application in all-solid-state lithium metal batteries . Here we report a LaCl -based lithium superionic conductor possessing excellent interfacial compatibility with lithium metal electrodes. In contrast to a Li MCl (M = Y, In, Sc and Ho) electrolyte lattice , the UCl -type LaCl lattice has large, one-dimensional channels for rapid Li conduction, interconnected by La vacancies via Ta doping and resulting in a three-dimensional Li migration network. The optimized Li Ta La Cl electrolyte exhibits Li conductivity of 3.02 mS cm at 30 °C and a low activation energy of 0.197 eV. It also generates a gradient interfacial passivation layer to stabilize the Li metal electrode for long-term cycling of a Li-Li symmetric cell (1 mAh cm ) for more than 5,000 h. When directly coupled with an uncoated LiNi Co Mn O cathode and bare Li metal anode, the Li Ta La Cl electrolyte enables a solid battery to run for more than 100 cycles with a cutoff voltage of 4.35 V and areal capacity of more than 1 mAh cm . We also demonstrate rapid Li conduction in lanthanide metal chlorides (LnCl ; Ln = La, Ce, Nd, Sm and Gd), suggesting that the LnCl solid electrolyte system could provide further developments in conductivity and utility.

Background To investigate the value of colour Doppler echocardiography (CDE) in the closure of patent ductus arteriosus (PDA) in the elderly. Methods Before the occlusion, screening the indicantions for twenty-five elderly patients with PDA by CDE. Using the shunt flow signal of colour Doppler flow imaging (CDFI) to estimate the pulmonary side diameter of PDA, compare with the angiocardiography and determine its accuracy through statistics. Using the continuous wave Doppler (CW) to measure peak velocity of tricuspid regurgitation and estimate cross-tricuspid pressure gradient according to the simplified Bernoulli equation ΔP = 4V2, together with the inherent right atrial pressure to estimate pulmonary artery pressure. Then compare cross-tricuspid pressure gradient and pulmonary artery pressure with cardiac catheterization. Determine its accuracy through statistics. All patients underwent interventional therapy and determine the efficacy of occlusion by CDE. Results all the occlusion of the elderly patients with PDA was successful through the examination by CDE. The diameter of the PDA pulmonary side estimated by the shunt flow signal had a positive correlation with angiocardiography (r = 0.71, P < 0.001). Pulmonary artery pressure estimated by the CW had a positive correlation with cardiac catheterization contrast (r = 0.63, P < 0.001). Conclusions CDE has an important value in the elderly during the procedure of patent ductus arteriosus occlusion. Using CDFI to estimate PDA pulmonary side diameter and CW to estimate pulmonary artery pressure before the occlusion is the key to screen the indication of occlusion. And it is also important for using CDFI to observe whether there was a residual PDA shunt after transcatheter closure.

Volatile aroma compounds make significant contributions to human perception of flowers. Osmanthus fragrans is a famous aroma plant and linalool along with its oxides are proved to be the dominant aroma active compounds. Although some terpene synthases (TPSs) have been characterized, a comprehensive study of the hub metabolic gene and its transcriptional regulation remain to be revealed. Here, we selected a specific cultivar Boyeyingui (BBYG) with the highest content of linalool among 20 wide-cultivated cultivars for genome and transcriptome sequencings. Among the 25 new putative OfTPSs, only OfTPS6, OfTPS7 could exclusively produce linalool in planta. Biochemical analysis demonstrated that OfTPS6, OfTPS7 were able to catalyze geranyl diphosphate (GPP) into linalool and a small proportion of other monoterpenes in vitro. Spatial and temporal correlation analysis further confirmed the transcript level of OfTPS7 was closely associated with linalool content in diverse cultivars and different tissues, suggesting OfTPS7 was the essential linalool synthase gene. Combined with yeast one-hybrid screen and weighted correlation network analysis (WGCNA), a nucleus-localized transcriptional factor OfWRKY33 was considered as a potential modulator. Y1H, dual-luciferase assay and electrophoretic mobility shift assay (EMSA) demonstrated that OfWRKY33 directly bound to the W-box of OfTPS7 promoter to stimulate its transcription. OfWRKY33 could coordinately induce the expressions of OfTPS7, 1-deoxy-d-xylulose 1 (OfDXS1), thereby promoting the linalool formation. The results first revealed the hub linalool synthase gene OfTPS7 and a novel TF participating in the complex transcriptional regulation of linalool biosynthesis in O. fragrans flowers.