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- 저자 : Steve Sims (검색결과 4 건)
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Musculoskeletal and neurocognitive clinical significance of adult hypophosphatasia
김세민,Funda Korkmaz,Steve Sims,Vitaly Ryu,Tony Yuen,Mone Zaidi 대한골다공증학회 2023 Osteoporosis and Sarcopenia Vol.9 No.4
Hypophosphatasia (HPP), also called Rathbun disease, is a rare genetic disorder that is caused by the loss-of-function mutation in the ALPL gene encoding tissue nonspecific alkaline phosphatase. Doctor Rathbun first described the case of a 3-week-old infant who presented with severe osteopenia, rickets, and multiple radiographic fractures, and died shortly after of epileptic seizure and respiratory distress. The term “hypophosphatasia” was coined as the patients’ alkaline phosphatase levels were significantly low. Since then, our understanding of HPP has evolved, and now we appreciate causative genetic mutation and the broad spectrum of clinical presentation depending on the age of onset, severity, and skeletal involvement: perinatal, infantile, childhood, adult and odontohypophosphatasia. The new development of enzyme replacement with asfostase alfa has saved the lives of severe form of hypophosphatasia. However, it is still unclear and remains challenging how to manage adult HPP that often presents with mild and non-specific symptoms such as muscle pain, joint stiffness, fatigue, anxiety, or low bone mass, which are common in the general population and not necessarily attributed to HPP. In this review, we will present 3 unique cases of adult HPP and discuss the pathophysiology, clinical presentation particularly neuromuscular and neurocognitive symptoms and management of adult HPP.
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Ryu, Jeongjae,Kim, Jaegyu,Oh, Jinwon,Lim, Seongjin,Sim, Joo Yong,Jeon, Jessie S.,No, Kwangsoo,Park, Steve,Hong, Seungbum Elsevier 2019 Nano energy Vol.55 No.-
<P><B>Abstract</B></P> <P>As future generations of wearable electronics are expected to be directly worn, fiber-based electronics are expected to become increasingly more important in the coming years, as they can be weaved into textiles to provide higher comfort, durability, and integrated multi-functionalities. Herein, we demonstrate an intrinsically stretchable multi-functional hollow fiber capable of harvesting mechanical energy and detecting strain. For energy harvesting, we have utilized a stretchable ferroelectric layer composed of P(VDF-TrFE) in a matrix of elastomer, sandwiched between stretchable electrodes composed of multi-walled carbon nanotubes and PEDOT:PSS. We have demonstrated voltage and current generation under stretching and normal pressure, with output voltage and current as high as 1.2 V and 10 nA, respectively. Furthermore, the hollow architecture enabled the harvesting of pressure coming from internal liquid flow, adding another dimension of harvesting mechanical energy. The stretchable electrodes were used as strain sensors, which exhibited high gauge factor of 80–177 in the 0–50% strain range, along with low hysteresis and durability. These features render our multi-functional fiber highly suitable for wearable electronic applications in the near future.</P> <P><B>Highlights</B></P> <P> <UL> <LI> We developed an intrinsically stretchable multi-functional hollow fiber. </LI> <LI> Strain can be detected with high sensitivity (gauge factor up to 177). </LI> <LI> The fiber can harvest energy coming from various external mechanical stimuli. </LI> <LI> Our fiber has potential in the field of wearable, and robotic applications. </LI> </UL> </P> <P><B>Graphical abstract</B></P> <P>[DISPLAY OMISSION]</P>
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Kim, Jong Soo,Wood, Sebastian,Shoaee, Safa,Spencer, Steve J.,Castro, Fernando A.,Tsoi, Wing Chung,Murphy, Craig E.,Sim, Myungsun,Cho, Kilwon,Durrant, James R.,Kim, Ji-Seon The Royal Society of Chemistry 2015 Journal of Materials Chemistry C Vol.3 No.35
<P>We report detailed analysis of the thin film morphology (molecular packing, molecular conformational order, and vertical phase separation) - performance (charge transport, photocurrent generation, and photovoltaic performance) relationships under nanowire formation and subsequent thermal annealing in polymer:fullerene blends. Nanowires of poly(3-hexylthiophene) (P3HT) are formed by controlled precipitation from solution and blended with [6,6]-phenyl-C61-butyric acid methyl ester (PCBM) to form bulk heterojunction thin films. The formation of nanowires and further thermal annealing result in increased molecular order of the P3HT, where the short-range conformational order is maximised by annealing at 100 °C and decreases when annealed at higher temperatures, but the quality of long-range molecular packing and lamellar packing distance increase with annealing temperature up to 150 °C. The long-range order correlates strongly with an increase in hole mobility, but the reduction in short-range conformational order indicates a slight reduction in planarity of the conjugated backbone in this aggregated polymer morphology. Photoconductive atomic force microscopy reveals enhanced connectivity of the hole transporting nanowire network as a result of thermal annealing. Additionally, we find that the nanowire morphology results in a favourable vertical phase separation, with PCBM enrichment at the electron-extracting surface in the conventional architecture, which is contrary to the non-nanowire case. This effect is further encouraged by thermal annealing, resulting in an enhancement of open-circuit voltage, and represents a morphological advantage over conventional P3HT:PCBM devices. Our study identifies an important interplay between long-range and short-range molecular order in charge generation, transport, extraction, and hence solar cell device performance.</P>
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Oh, Jinwon,Yang, Jun Chang,Kim, Jin-Oh,Park, Hyunkyu,Kwon, Se Young,Lee, Serin,Sim, Joo Yong,Oh, Hyun Woo,Kim, Jung,Park, Steve American Chemical Society 2018 ACS NANO Vol.12 No.8
<P>Tactile sensors that can mechanically decouple, and therefore differentiate, various tactile inputs are highly important to properly mimic the sensing capabilities of human skin. Herein, we present an all-solution processable pressure insensitive strain sensor that utilizes the difference in structural change upon the application of pressure and tensile strain. Under the application of strain, microcracks occur within the multiwalled carbon nanotube (MWCNT) network, inducing a large change in resistance with gauge factor of ∼56 at 70% strain. On the other hand, under the application of pressure to as high as 140 kPa, negligible change in resistance is observed, which can be attributed to the pressure working primarily to close the pores, and hence minimally changing the MWCNT network conformation. Our sensor can easily be coated onto irregularly shaped three-dimensional objects (e.g., robotic hand) via spray coating, or be attached to human joints, to detect bending motion. Furthermore, our sensor can differentiate between shear stress and normal pressure, and the local strain can be spatially mapped without the use of patterned electrode array using electrical impedance tomography. These demonstrations make our sensor highly useful and important for the future development of high performance tactile sensors.</P> [FIG OMISSION]</BR>
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