Duchenne Muscular Dystrophy (DMD) is a rapidly progressive, lethal neuromuscular disorder, present from birth, which occurs almost exclusively in males. We have reviewed contemporary evidence of ...burden, epidemiology, illness costs and treatment patterns of DMD. This systematic review adhered to published methods with information also sought from the web and contacting registries. Searches were carried out from 2005 to June 2015. The population of interest was individuals with clearly defined DMD or their carers.
Nine thousand eight hundred fifty titles were retrieved from searches. Fifty-eight studies were reviewed with three assessed as high, 33 as medium and 22 as low quality. We found two studies reporting birth and four reporting point prevalence, three reporting mortality, 41 reporting severity and/or progression, 18 reporting treatment patterns, 12 reporting quality of life, two reporting utility measures, three reporting costs of illness and three treatment guidelines. Birth prevalence ranged from 15.9 to 19.5 per 100,000 live births. Point prevalence per 100,000 males was for France, USA, UK and Canada, 10.9, 1.9, 2.2 and 6.1 respectively. A study of adult DMD patients at a centre in France found median survival for those born between 1970 and 1994 was 40.95 years compared to 25.77 years for those born between 1955 and 1969. Loss of ambulation occurred at a median age of 12 and ventilation starts at about 20 years. There was international variation in use of corticosteroids, scoliosis surgery, ventilation and physiotherapy. The economic cost of DMD climbs dramatically with disease progression - rising as much as 5.7 fold from the early ambulatory phase to the non-ambulatory phase in Germany.
This is the first systematic review of treatment, progression, severity and quality of life in DMD. It also provides the most recent description of the burden, epidemiology, illness costs and treatment patterns in DMD. There are evidence gaps, particularly in prevalence and mortality. People with DMD seem to be living longer, possibly due to corticosteroid use, cardiac medical management and ventilation. Future research should incorporate registry data to improve comparability across time and between countries and to investigate the quality of life impact as the condition progresses.
Next-generation sequencing (NGS) provides a better approach to gene expression profiling with several advantages. The power of NGS along with novel molecular techniques and computational tools allow ...the researchers to perform the gene expression profiling to reveal transcriptional complexity of an organism and answering several biological questions. Although many studies for gene expression profiling related to various aspects have been performed in animal systems revealing unprecedented levels of complexity of transcriptomes, their use is still limited in plant biology. This review describes the use of NGS technologies with respect to gene expression profiling, bioinformatics challenges associated with data analysis and advances made so far in the plant biology research. We anticipate many more studies in recent future, which will surely advance our understanding of the complexity of plant genomes.
There has been considerable progress over the last decade in development of the perovskite solar cells (PSCs), with reported performances now surpassing 25.2% power conversion efficiency. Both ...long‐term stability and component costs of PSCs remain to be addressed by the research community, using hole transporting materials (HTMs) such as 2,2′,7,7′‐tetrakis(N,N′‐di‐pmethoxyphenylamino)‐9,9′‐spirbiuorene(Spiro‐OMeTAD) and polybis(4‐phenyl)(2,4,6‐trimethylphenyl)amine (PTAA). HTMs are essential for high‐performance PSC devices. Although effective, these materials require a relatively high degree of doping with additives to improve charge mobility and interlayer/substrate compatibility, introducing doping‐induced stability issues with these HTMs, and further, additional costs and experimental complexity associated with using these doped materials. This article reviews dopant‐free organic HTMs for PSCs, outlining reports of structures with promising properties toward achieving low‐cost, effective, and scalable materials for devices with long‐term stability. It summarizes recent literature reports on non‐doped, alternative, and more stable HTMs used in PSCs as essential components for high‐efficiency cells, categorizing HTMs as reported for different PSC architectures in addition to use of dopant‐free small molecular and polymeric HTMs. Finally, an outlook and critical assessment of dopant‐free organic HTMs toward commercial application and insight into the development of stable PSC devices is provided.
This review covers the latest development in dopant‐free organic hole transporting materials which have been employed in different perovskite solar cell structures. The criteria (appropriate highest occupied molecular orbital/lowest unoccupied molecular orbital, high thermal stability, good solubility, high purity, and amorphous nature) for the rational design of these novel pristine dopant‐free organic hole transporting materials as well as their deposition methods and commercialization aspects are discussed.
Feldspars are an attractive alternative to quartz for extending the dose range, and for dating volcanic terrains such as on Mars and Iceland. Unfortunately, charge stored in the feldspar lattice ...undergoes anomalous fading leading to an underestimation in the dose estimates. In this paper we use the time-resolved optically stimulated luminescence (TR-OSL) technique to investigate the processes that give rise to the signal following infrared (IR), green and blue stimulation, with an objective to understand tunnelling and charge transport during thermo-optical excitations. We show that the TR-OSL shape is governed by the energy of excitation and the subsequent charge recombination route through the excited state of the trap, the band tail states or the conduction band. The role of band tail states in charge recombination is specifically examined using the signal shown to decay over several ms; we identify two dominant recombination routes, viz., phonon (0.05–0.06 eV) assisted diffusion, and quantum mechanical tunnelling, depending on the energy state of the detrapped electron. As would be expected, diffusion in the band tails is identical for both resonant and non-resonant excitations, where in the latter case the band tail state occupancy likely arises from thermalisation of conduction band electrons. The important outcome of this study is a comprehensive physical model based on a single dosimetric trap that successfully explains wide-ranging luminescence phenomena in feldspars, in particular, the luminescence efficiency and thermal partitioning of charge in different energy states and the subsequent recombination routes. The model predicts three different systematic approaches to preferentially sampling the most stable signal. We finally present evidence for a non-fading signal using one of these methods based on pulsed IR stimulation.
The effect of martensite morphology and distribution in a ferrite matrix on the mechanical properties and the damage accumulation in uniaxial tension was investigated in two different ...automotive-grade dual phase DP600 steels. The two sheet steels had roughly 20% volume fraction of martensite but dissimilar chemical composition. A detailed analysis of microstructure and damage accumulation has been conducted as a function of strain. SEM analysis revealed that voids nucleation occurs by martensite cracking, separation of adjacent martensite regions, or by decohesion at the ferrite/martensite interface. Martensite morphology and distribution had a significant influence in the accumulation of damage. The steel with a more uniform distribution of martensite showed a slower rate of damage growth and a continuous void nucleation during the deformation process, which resulted in a higher void density before fracture. On the other hand, the steel with a centre-line of martensite through the sheet thickness exhibited accelerated void growth and catastrophic coalescence in the transverse orientation to the applied load.
In this report, highly efficient and humidity‐resistant perovskite solar cells (PSCs) using two new small molecule hole transporting materials (HTM) made from a cost‐effective precursor anthanthrone ...(ANT) dye, namely, 4,10‐bis(1,2‐dihydroacenaphthylen‐5‐yl)‐6,12‐bis(octyloxy)‐6,12‐dihydronaphtho7,8,1,2,3‐nopqrtetraphene (ACE‐ANT‐ACE) and 4,4′‐(6,12‐bis(octyloxy)‐6,12‐dihydronaphtho7,8,1,2,3‐nopqrtetraphene‐4,10‐diyl)bis(N,N‐bis(4‐methoxyphenyl)aniline) (TPA‐ANT‐TPA) are presented. The newly developed HTMs are systematically compared with the conventional 2,2′,7,7′‐tetrakis(N,N′‐di‐p‐methoxyphenylamino)‐9,9′‐spirbiuorene (Spiro‐OMeTAD). ACE‐ANT‐ACE and TPA‐ANT‐TPA are used as a dopant‐free HTM in mesoscopic TiO2/CH3NH3PbI3/HTM solid‐state PSCs, and the performance as well as stability are compared with Spiro‐OMeTAD‐based PSCs. After extensive optimization of the metal oxide scaffold and device processing conditions, dopant‐free novel TPA‐ANT‐TPA HTM‐based PSC devices achieve a maximum power conversion efficiency (PCE) of 17.5% with negligible hysteresis. An impressive current of 21 mA cm−2 is also confirmed from photocurrent density with a higher fill factor of 0.79. The obtained PCE of 17.5% utilizing TPA‐ANT‐TPA is higher performance than the devices prepared using doped Spiro‐OMeTAD (16.8%) as hole transport layer at 1 sun condition. It is found that doping of LiTFSI salt increases hygroscopic characteristics in Spiro‐OMeTAD; this leads to the fast degradation of solar cells. While, solar cells prepared using undoped TPA‐ANT‐TPA show dewetting and improved stability. Additionally, the new HTMs form a fully homogeneous and completely covering thin film on the surface of the active light absorbing perovskite layers that acts as a protective coating for underlying perovskite films. This breakthrough paves the way for development of new inexpensive, more stable, and highly efficient ANT core based lower cost HTMs for cost‐effective, conventional, and printable PSCs.
First time low‐cost anthanthrone dye based hole transporting materials (HTMs) 4,10‐bis(1,2‐dihydroacenaphthylen‐5‐yl)‐6,12‐bis(octyloxy)‐6,12‐dihydronaphtho7,8,1,2,3‐nopqrtetraphene (ACE‐ANT‐ACE) and 4,4′‐(6,12‐bis(octyloxy)‐6,12‐dihydronaphtho7,8,1,2,3‐nopqrtetraphene‐4,10‐diyl)bis(N,N‐bis(4‐methoxyphenyl)aniline) (TPA‐ANT‐TPA) end capped with dihydroacenaphthylene and triphenyleamine groups are designed and synthesized, respectively. Among both, dopant‐free TPA‐ANT‐TPA cut‐rate HTM ($67 g−1) exhibits higher performance with 17.5% efficiency and retains respectable performance after 50 h in 58% relative humidity than conventional expensive 2,2′,7,7′‐tetrakis(N,N′‐di‐p‐methoxyphenylamino)‐9,9′‐spirbiuorene.
PDF
