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Long-term clinical outcomes and health-related quality of life in patients with isolated methylmalonic acidemia after liver transplantation:experience from the largest cohort study in China

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World Journal of Pediatrics 2024年第8期20卷 809-821页

作者： Yi-zhou Jiang guang-peng zhou Lin Wei Wei Qu Zhi-Gui Zeng Ying Liu Yu-Le Tan Jun Wang Zhi-Jun Zhu Li-Ying Sun Department of Critical Liver Diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityNo.101 Lu Yuan Dong RoadTong-Zhou DistrictBeijing110112China Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation No.101 Lu Yuan Dong RoadTong-Zhou DistrictCapital Medical UniversityBeijing110112China

Background Liver transplantation(LT)has been proposed as a viable treatment option for selected methylmalonic acidemia(MMA)***,there are still controversies regarding the therapeutic value of LT for *** systematic assessment of health-related quality of life(HRQoL)-targeted MMA children before and after LT is also *** study aimed to comprehensively assess the long-term impact of LT on MMA,including multiorgan sequelae and HRQoL in children and *** We retrospectively evaluated 15 isolated MMA patients undergoing LT at our institution between June 2013 and March ***-and post-transplant data were compared,including metabolic profiles,neurologic consequences,growth parameters,and *** further assess the characteristics of the HRQoL outcomes in MMA,we compared the results with those of children with biliary atresia(BA).Results All patients had early onset MMA,and underwent LT at a mean age of 4.3 *** 1.3–8.2 years of follow-up,the patient and graft survival rates were 100%.Metabolic stability was achieved in all patients with liberalized dietary protein *** was a significant overall improvement in height Z scores(P=0.0047),and some preexisting neurological complications remained stable or even improved after *** the Pediatric Quality of Life Inventory(PedsQL™)generic core scales,the mean total,physical health,and psychosocial health scores improved significantly posttransplant(P<0.05).In the family impact module,higher mean scores were noted for all subscales post-LT,especially family function and daily activities(P<0.01).However,the total scores on the generic core scales and transplant module were significantly lower(Cohen’s d=0.57–1.17)when compared with BA *** particular,social and school functioning(Cohen’s d=0.86–1.76),treatment anxiety,and communication(Cohen’s d=0.99–1.81)were far behind,with a large effect *** This large single-center study of the mainland of China sh

关键词： Health-related quality of life.Inborn error of metabolism Isolated methylmalonic acidemia Liver transplantation:Outcomes

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Mn(OAc)_2/Schiff base as a new efficient catalyst system for the Henry reaction of nitroalkanes with aldehydes

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Chinese Chemical Letters 2012年第6期23卷 690-694页

作者： guang peng zhou Yong Hai Hui Ning Ning Wan Qiu Ju Liu Zheng Feng Xie Ji De Wang Key Laboratory of Oil and Gas Fine Chemicals Ministry of Education and Xinjiang Uyghur Autonomous RegionCollege of Chemistry and Chemical Engineering Xinfiang University Urumqi 830046 China

A series of novel Schiff bases bearing triazole structure were synthesized and characterized by LR and NMR. Mn（OAc）2/Schiff base efficiently catalyzed Henry reaction of nitroalkanes with aldehydes to produce the corresponding products of ^-nitroalcohols, under mild conditions with high yields （up to 99%）. A reaction mechanism is proposed based on the experimental results.

关键词： Schiff base Henry reaction Aldehydes Nitroalkanes

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Multiple damage zones around hydraulic fractures generated by high-frequency pulsating hydraulic fracturing

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Petroleum Science 2024年第4期21卷 2688-2702页

作者： Yan peng Sheng-Jie Wei guang-Qing Zhang Da-Wei zhou Chuang-Chao Xu School of Petroleum Engineering China University of Petroleum PetroChina Changqing Oilfield Company

Pulsating hydraulic fracturing(PHF) is a promising fracturing method and can generate a dynamic periodic pressure. The periodic pressure can induce fatigue failure of rocks and decrease initiation pressure of fracture. If the frequency of periodic pressure exceeds 10 Hz, the distribution of pressure along the main fracture will be heterogeneous, which is much different from the one induced by the common fracturing method. In this study, the impact of this special spatial feature of pressure on hydraulic fracture is mainly investigated. A coupled numerical simulation model is first proposed and verified through experimental and theoretical solutions. The mechanism of secondary fracture initiation around the main fracture is then discovered. In addition, sensitivity studies are conducted to find out the application potential of this new method. The results show that(1) this coupled numerical simulation model is accurate. Through comparison with experimental and theoretical data, the average error of this coupled model is less than 1.01%.(2) Even if a reservoir has no natural fracture, this heterogeneous distribution pressure can also cause many secondary fractures around the main fracture.(3) The mechanism of secondary fracture initiation is that this heterogeneous distribution pressure causes tensile stress at many locations along the main fracture.(4) Through adjusting the stimulation parameters, the stimulation efficiency can be improved. The average and amplitude of pressure can increase possibility of secondary fracture initiation. The frequency of this periodic pressure can increase number of secondary fractures. Even 6 secondary fractures along a 100 m-length main fracture can be generated.(5) The influence magnitudes of stimulation parameters are larger than ones of geomechanical properties, therefore, this new fracturing method has a wide application potential.

关键词： Tight reservoir Reservoir stimulation Numerical simulation Secondary fracture Fracture initiation

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Compromised therapeutic value of pediatric liver transplantation in ethylmalonic encephalopathy: A case report

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World Journal of Gastroenterology 2020年第40期26卷 6295-6303页

作者： guang-peng zhou Wei Qu Zhi-Jun Zhu Li-Ying Sun Lin Wei Zhi-Gui Zeng Ying Liu Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Liver Transplantation Center Intensive Care UnitBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation(LT) is emerging as a novel therapeutic option for EE;however, worldwide experience is still *** SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in AsianPacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4-and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic *** LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolismcorrecting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.

关键词： Ethylmalonic encephalopathy Liver transplantation Mitochondrial disorder ETHE1 Pediatric Case report

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Understand the Temperature Sensing Behavior of Solid-state Polymerized PEDOT Hybrid Based on X-ray Scattering Studies

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Chinese Journal of Polymer Science 2024年第1期42卷 105-112,I0010页

作者： Zhen-Hang He guang-Feng Liu Ze-Kun zhou Zhen Liu Yi-Shu Zeng peng Zhang School of Materials Science and Engineering Key Laboratory for Polymeric Composite and Functional Materials of Ministry of EducationSun Yat-sen UniversityGuangzhou 510275China Shanghai Advanced Research Institute(Zhangjiang Laboratory) Chinese Academy of SciencesShanghai 201204China

Poly(3,4-ethylenedioxythiophene)(PEDOT) is one of the most successful conductive polymers that recently has been used in wearable sensors for human health monitoring. In this work, we prepared a series of PEDOT hybrids consisting of PEDOT, sodium poly(styrene sulfonate)(PSSNa) and polyethylene oxide(PEO), and their preparation could be scaled-up via an adapted solid-state polymerization process. The resistance of the as-prepared PEDOT:PSS/PEO hybrid shows clear temperature response, i.e., it decreases almost linearly with the temperature increase. To understand this phenomenon, the in situ synchrotron radiation wide-and small-angle X-ray scattering(WAXS/SAXS) characterizations were undertaken to study the temperature-dependent microstructure change of the PEDOT:PSS/PEO hybrid. It demonstrated that PEDOT formed conductive paths in the hybrids, which were not destroyed by the PEO crystallization. As temperature increased, the PEO crystals' melting and the accompanying reorganization of PEDOT chains endowed the hybrid sample temperature responsiveness. Based on these fundamental knowledges, the hybrid materials were used to fabricate flexible wearable sensor that showing temperature sensing performance with an accuracy of 1 ℃. These findings shed lights on the scalable manufacturing of wearable sensors for body temperature monitoring.

关键词： Solid-state polymerization PEDOT hybrid Structure-property correlation X-ray scattering

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Research on the mechanism of the two-dimensional ultrasonic surface burnishing process to enhance the wear resistance for aluminum alloy

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Friction 2024年第3期12卷 490-509页

作者： Zhen-Yu zhou Qiu-Yang ZHENG Yu LI Cong DING guang-Jian peng Zhong-Yu PIAO College of Mechanical Engineering Zhejiang University of TechnologyHangzhou 310023China Key Laboratory of Special Purpose Equipment and Advanced Processing Technology Ministry of Education and Zhejiang ProvinceZhejiang University of TechnologyHangzhou 310014China

The gradient nanostructure is machined on the aluminum(Al)alloy by the two-dimensional ultrasonic surface burnishing process(2D-USBP).The mechanism of why the gradient nanostructure enhances wear resistance is *** mechanical properties and microstructure characterization for the gradient nanostructure are performed by operating a nanoindenter,transmission electron microscopy(TEM),and electron backscattered diffraction(EBSD).Dry wear tests are performed on the samples before and after machining to evaluate the wear resistance and *** effect of the gradient nanostructure on the wear resistance is explored by developing the crystal plasticity(CP)finite element and molecular dynamics(MD)*** characterization results show that the 2D-USBP sample prepared a gradient structure of~600μm thick on the aluminum surface,increasing the surface hardness from 1.13 to 1.71 GPa and reducing the elastic modulus from 78.84 to 70.14 *** optimization of the surface microstructure and the increase of the mechanical properties effectively enhance the wear resistance of the sample,with 41.20%,39.07%,and 54.58% of the wear scar areas for the 2D-USBP treated samples to the original samples under 5,10,and 15 N loads,*** gradient nanostructure hinders the slip of dislocations inside the sample during the wear process and reduces the size and scope of plastic deformation;meanwhile,the resistance to deformation,adhesion,and crack initiation and propagation of the sample surface is improved,resulting in enhanced wear resistance.

关键词： structural superlubricity lattice registry elastic deformation strained solitons crystal plasticity molecular dynamics simulations

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Prognostic Factors Related to In-hospital Death in Children with Biliary Atresia:Analysis of a Nationwide Inpatient Database

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Journal of Clinical and Translational Hepatology 2023年第2期11卷 416-424页

作者： Yu Shi Yi-zhou Jiang guang-peng zhou Ying Shi Lan-Xia Gan Yuan-Yuan Kong Hai-Bo Wang Zhi-Jun Zhu Li-Ying Sun Liver Transplantation Center Beijing Friendship HospitalCapital Medical UniversityBeijingChina National Clinical Research Center for Digestive Diseases Beijing Friendship HospitalCapital Medical UniversityBeijingChina Department of Critical Liver Diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityBeijingChina Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijingChina National Clinical Research Center for Cardiovascular Diseases Fuwai Hospital Chinese Academy of Medical SciencesShenzhenGuangdong ProvinceChina China Standard Medical Information Research Center ShenzhenGuangdongChina Clinical Trial Unit Precision Medicine InstituteFirst Affiliated Hospital of Sun Yat-Sen UniversityGuangzhouChina

Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to *** study aimed to identify the possible risk factors affecting in-hospital death in BA patients in ***:We collected data from the Hospital Quality Monitoring System,a national inpatient *** patients aged up to 2 years old with a diagnosis of BA were *** subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no *** regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital ***:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of *** proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years *** signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital *** no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe ***,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.

关键词： Biliary atresia In-hospital death Liver transplantation Kasai portoenterostomy

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Metasurface-assisted low-frequency performance enhancement of ultra-broadband honeycomb absorber based on carbon nanotubes

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Nano Research 2024年第9期17卷 8542-8551页

作者： Cai-Liang Wang Shuang Bai Pei-Yan Zhao Tao zhou Hui-Ya Wang Jun-peng Wang Luo-Xin Wang guang-Sheng Wang School of Chemistry Beihang UniversityBeijing 100191China Aerospace Science and Industry Corporation of Wuhan Magnetoelectricity Co. Ltd.Wuhan 430070China College of Materials Science and Engineering Key Laboratory for New Textile Materials and Applications of Hubei ProvinceWuhan Textile UniversityWuhan 430200China School of Chemistry and Chemical Engineering Lingnan Normal UniversityZhanjiang 524048China School of Materials Science and Engineering Zhejiang Sci-Tech UniversityHangzhou 310018China

Here,we present a unique method to enhance the low-frequency absorption performance of a honeycomb absorber by integrating a *** geometrical dimensions of the proposed metasurface have been numerically *** introduction of the metasurface allows exploitation of its robust resonance and superior impedance matching in low-frequency bands,thereby improving microwave absorption *** incorporation of the metasurface does not impact the wave transmission performance of the honeycomb core absorber at high-frequency band,thus preserving its high-frequency *** broadens the absorption range,leading to an expanded *** results reveal that the composite absorber(CA)exhibits strong absorption performance with an incident angle stability up to 45°for both transverse electric(TE)and transverse magnetic(TM)*** absorption mechanism of the CA has been investigated by using an equivalent circuit model and electromagnetic field analysis.A prototype was designed,fabricated,and tested to validate the proposed *** simulation and measurement results demonstrate that the prototype can achieve an average absorption rate exceeding 90%across a 1.0-18.0 GHz *** study introduces an innovative technique for creating microwave absorbers for low-frequency wideband applications.

关键词： low frequency metasurface radar absorption impedance matching

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Review of Fiber-Optic Localized Surface Plasmon Resonance Sensors:Geometries,Fabrication Technologies,and Bio-Applications

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Photonic Sensors 2024年第2期14卷 31-56页

作者： Mengdi LU Chen WANG Ruizhi FAN Ming LIN Jianye guang Wei peng Affiliated Cancer Hospital Dalian University of TechnologyShenyang 110042China School of Physics Dalian University of TechnologyDalian 116024China

Localized surface plasmon resonance(LSPR)biosensors,which enable nanoscale confinement and manipulation of light,offer the enhanced sensitivity and electromagnetic energy *** integration of LSPR with the fiber-optic technology has led to the development of compact and versatile sensors for miniaturization and remote *** comprehensive review explores various sensor configurations,fiber types,and geometric shapes,highlighting their benefits in terms of sensitivity,integration,and performance *** techniques such as focused non-chemical bonding strategies and self-assembly of nanoparticles are discussed,providing control over nanostructure morphology and enhancing sensor ***-applications of fiber-optic LSPR(FOLSPR)sensors are detailed,specifically in biomolecular interactions and analysis of proteins,pathogens and cells,nucleic acids(DNA and RNA),and other small molecules(organic compounds and heavy metal ions).Surface modification and detection schemes are emphasized for their potential for label-free and real-time *** challenges and prospects of FOLSPR sensors are addressed,including the developments in sensitivity,fabrication techniques,and measurement *** with emerging technologies such as nanomaterials is highlighted as a promising direction for future ***,this review provides insights into the advancements and potential applications of FOLSPR sensors,paving the way for sensitive and versatile optical biosensing platforms in various fields.

关键词： Fiber-optic localized surface plasmon resonance biosensor nanoparticles geometric configuration fabrication technology

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Comparision between portosystemic shunts and endoscopic therapy for prevention of variceal re-bleeding:a systematic review and meta-analysis

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Chinese Medical Journal 2019年第9期132卷 1087-1099页

作者： guang-peng zhou Li-Ying Sun Lin Wei Wei Qu Zhi-Gui Zeng Ying Liu Yi-zhou Jiang Zhi-Jun Zhu Liver Transplantation Center Beijing Friendship HospitalCapital Medical UniversityBeijing 100005China Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijing 100005China National Clinical Research Center for Digestive Diseases Beijing 100005China Intensive Care Unit Beijing Friendship HospitalCapital Medical UniversityBeijing 100005China

Background:Portosystemic shunts,including surgical portosystemic shunts and transjugular intra-hepatic portosystemic shunt (TIPS),may have benefit over endoscopic therapy (ET) for treatment of variceal bleeding in patients with cirrhotic portal hypertension;however,whether there being a survival benefit among them remains *** study was to compare the effect of three above-mentioned therapies on the short-term and long-term survival in patient with ***:Using the terms "variceal hemorrhage or variceal bleeding or variceal re-bleeding" OR "esophageal and gastric varices" OR "portal hypertension" and "liver cirrhosis," the Cochrane Central Register of Controlled Trials,PubMed,Embase,and the references of identified trials were searched for human randomized controlled trials (RCTs) published in any language with full texts or abstracts (last search June 2017).Risk ratio (RR) estimates with 95% confidence interval (CI) were calculated using random effects model by Review *** quality of the included studies was evaluated using the Cochrane Collaboration’s tool for the assessment of the risk of ***:Twenty-six publications comprising 28 RCTs were included in this *** studies included a total of 2845 patients:496 (4 RCTs) underwent either surgical portosystemic shunts or TIPS,1244 (9 RCTs) underwent either surgical portosystemic shunts or ET,and 1105 (15 RCTs) underwent either TIPS or *** was no significant difference in overall mortality and 30-day or 6-week survival among three *** with TIPS and ET,separately,surgical portosystemic shunts were both associated with a lower bleeding-related mortality (RR = 0.07,95% CI = 0.01–0.32;P<0.001;RR = 0.17,95% CI = 0.06–0.51,P<0.005) and rate of variceal re-bleeding (RR = 0.23,95% CI = 0.10–0.51,P<0.001;RR = 0.10,95% CI = 0.04–0.24,P<0.001),without a significant difference in the rate of postoperative hepatic encephalopathy (RR = 0.52,95% CI = 0.25–1.00,P = 0.1

关键词： Portosystemic shunts Endoscopic therapy Variceal rebleeding Cirrhosis Meta-analysis

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