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Pediatric Investigation 2024年第2期8卷 135-138页

作者： samantha Gilg sebastian Acosta Rohit s.Loomba Claire Rizk Gary E.stapleton David Faraoni Fabio savorgnan Department of Pediatrics Divisions of Critical Care Medicine and CardiologyTexas Children’s Hospital and Baylor College of MedicineHoustonTexasUSA Department of Pediatrics Division of CardiologyTexas Children’s Hospital and Baylor College of MedicineHoustonTexasUSA Department of Pediatrics The Heart InstituteAdvocate Children’s HospitalChicago Medical School and Rosalind Franklin University of Medicine and ScienceOak LawnIllinoisUSA Department of Anesthesiology Arthur S.Keats Division of Pediatric Cardiovascular AnesthesiaPerioperative and Pain MedicineTexas Children’s HospitalBaylor College of MedicineHoustonTexasUSA

The standard of care in the management of d-transposition of the great arteries(TGA)includes the arterial switch operation(AsO).1,2 Although some institutions have begun to publish the results of performing the AsO within the first few hours of life,most centers continue to"electively"delay AsO for several days to allow for a reduction in pulmonary vascular resistance and the stabilization of renal and liver function.1,2 The early initiation of prostaglandin E1(PGE)infusion to maintain ductal patency is a nearly universal component of therapy in order to maintain adequate arterial oxygen saturation and systemic oxygen delivery during the pre-operative period via intercirculatory mixing at the atrial septal and patent ductus arteriosus levels1,3.

关键词： balloon maintain stabilization

Cardiac Piezo1 Exacerbates Lethal Ventricular Arrhythmogenesis by Linking Mechanical stress with Ca^(2+) Handling After Myocardial Infarction

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Research 2024年第1期2023卷 583-594页

作者： sheng-an su Yuhao Zhang Wudi Li Yutao Xi Yunrui Lu Jian shen Yuankun Ma Yaping Wang Yimin shen Lan Xie Hong Ma Yao Xie Meixiang Xiang Department of Cardiology Cardiovascular Key Laboratory of Zhejiang ProvinceThe Second Affiliated HospitalZhejiang University School of MedicineHangzhou 310009China Department of Endocrinology The Second Affiliated HospitalZhejiang University School of MedicineHangzhou 310009China texas Heart Institute HoustonTX 77030USA

Ventricular arrhythmogenesis is a key cause of sudden cardiac death following myocardial infarction(MI).Accumulating data show that ischemia,sympathetic activation,and inflammation contribute to ***,the role and mechanisms of abnormal mechanical stress in ventricular arrhythmia following MI remain *** aimed to examine the impact of increased mechanical stress and identify the role of the key sensor Piezo1 in ventricular arrhythmogenesis in *** with increased ventricular pressure,Piezo1,as a newly recognized mechano-sensitive cation channel,was the most up-regulated mechanosensor in the myocardium of patients with advanced heart ***1 was mainly located at the intercalated discs and T-tubules of cardiomyocytes,which are responsible for intracellular calcium homeostasis and intercellular communication.

关键词： ventricular homeostasis Ventricular

Quantifying Kinematic Tremor in an NGLY1-Deficient Individual: A Case study

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Case Reports in Clinical Medicine 2024年第1期13卷 25-36页

作者： Brock Futrell Christopher Alexander Malaya Dacia Martinez Diaz Christian Alfaro Hannah Elizabeth Gustafson subhalakshmi Chandrasekaran Rhea Mohan Phatak Bernhard suter Charles shannon Layne Department of Health and Human Performance University of Houston Houston USA Center for Neuromotor and Biomechanics Research University of Houston Houston USA Department of Computer science and Engineering Texas A&M University College Station USA Blue Bird Circle Rett Center Texas Children’s Hospital Houston USA Baylor College of Medicine Houston USA Center for NeuroEngineering and Cognitive science University of Houston Houston USA

NGLY1 Deficiency is an autosomal recessive congenital disorder that has been identified in less than 100 individuals. Most individuals with NGLY1 Deficiency display hyperkinetic movement disorders, including choreiform, athetoid, dystonic myoclonic, dyskinetic, and dysmetric movements. Developing a consistent and concise consensus on the classification and evaluation of tremors is essential to forward the research and treatment of tremors. It has also been reported that some individuals with NGLY1 Deficiency demonstrate tremor, but such tremor has never been formally investigated. The primary objective of this study is to determine if an individual with NGLY1 Deficiency demonstrates an identifiable tremor during a series of arm movements and, if so, describe the frequency and power characteristics of that tremor. Arm movement kinematics were obtained using a 16-camera Vicon system, and time series trajectory waveforms for three planes of a marker placed on the hand were developed. Custom MATLAB scripts were utilized to compute Fast Fourier Transformations of the data within the identified waveform segments. A mean frequency of 2.30 Hz (sD = 1.05) with a mean power of 5.02 |P1(f)| (sD = 4.63) suggests that our participant’s kinematic data did display a persistent tremor in both hands across all tasks and movement planes. Analyses of the reaching hand and the non-reaching hand suggest the participant displayed an action tremor in both postural and intention (kinetic) tremors. Future directions should include assessing additional individuals with NGLY1 Deficiency to determine if the tremor is a distinguishable disorder behavior. Additionally, evaluating other anatomical sites, such as the elbow, head, and lower limbs, would provide further insights into the characteristics of this tremor.

关键词： Tremor NGLY Fast Fourier Transformation Reaching Hyperkinetic Movement

Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant

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World Journal of Gastroenterology 2014年第32期20卷 11062-11068页

作者： Mary Elizabeth M Tessier sanjiv Harpavat Ross W shepherd Girish s Hiremath Mary L Brandt Amy Fisher John A Goss Department of Pediatrics Baylor College of Medicine and Texas Children’s Hospital Department of Pediatrics Baylor College of Medicine and Texas Children’s Hospital Houston Department of surgery Baylor College of Medicine and Texas Children’s HospitalHouston

Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.

关键词： Biliary atresia Liver transplantation Pediatric liver disease Pediatric end-stage liver disease Kasai operation Newborn screening surgical outcomes Living-related donor transplantation split liver transplantation ABO-incompatible liver transplantation

Diagnostic and therapeutic role of endoscopic retrograde cholangiopancreatography in biliary rhabdomyosarcoma

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World Journal of Gastroenterology 2008年第30期14卷 4823-4825页

作者： Ryan W Himes Isaac Raijman Milton J Finegold Heidi V Russell Douglas s Fishman section of Pediatric Gastroenterology Hepatology & Nutrition Baylor College of Medicine Texas Children's Hospital Digestive Disease Associates of Houston Department of Pathology Baylor College of Medicine Texas Children’s Hospital texas children’s Cancer Center Baylor College of Medicine section of Pediatric Gastroenterology Hepatology & Nutrition Baylor College of Medicine Texas Children’s Hospital

Biliary rhabdomyosarcoma (BRMs) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMs, in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used both diagnostically and therapeutically, thus obviating the need for surgery and its attendant risks of morbidity and mortality. We conclude that ERCP is an effective alternative to surgery for BRMs in some patients.

关键词： Pancreaticobiliary Malignancy Pediatric Endoscopic retrograde cholangiopancreatography Jaundice

3-Dimensional Kinematic Comparison of Arm Movements between an Individual with NGLY1 Deficiency and a Neurotypical Individual

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Case Reports in Clinical Medicine 2024年第4期13卷 122-146页

作者： Charles s. Layne Christopher A. Malaya Brock Futrell Dacia Martinez Diaz Christian Alfaro Hannah E. Gustafson subhalakshmi Chandrasekaran Rhea M. Phatak Bernhard suter Department of Health and Human Performance University of Houston Houston TX USA Center for Neuromotor and Biomechanics Research University of Houston Houston TX USA Center for Neuro Engineering and Cognitive science University of Houston Houston TX USA Department of Computer science and Engineering Texas A&M University College Station TX USA Blue Bird Circle Rett Center Texas Children&#8217s Hospital Houston TX USA Baylor College of Medicine Houston TX USA

NGLY1 Deficiency is an ultra-rare autosomal recessively inherited disorder. Characteristic symptoms include among others, developmental delays, movement disorders, liver function abnormalities, seizures, and problems with tear formation. Movements are hyperkinetic and may include dysmetric, choreo-athetoid, myoclonic and dystonic movement elements. To date, there have been no quantitative reports describing arm movements of individuals with NGLY1 Deficiency. This report provides quantitative information about a series of arm movements performed by an individual with NGLY1 Deficiency and an aged-matched neurotypical participant. Three categories of arm movements were tested: 1) open ended reaches without specific end point targets;2) goal-directed reaches that included grasping an object;3) picking up small objects from a table placed in front of the participants. Arm movement kinematics were obtained with a camera-based motion analysis system and “initiation” and “maintenance” phases were identified for each movement. The combination of the two phases was labeled as a “complete” movement. Three-dimensional analysis techniques were used to quantify the movements and included hand trajectory pathlength, joint motion area, as well as hand trajectory and joint jerk cost. These techniques were required to fully characterize the movements because the NGLY1 individual was unable to perform movements only in the primary plane of progression instead producing motion across all three planes of movement. The individual with NGLY1 Deficiency was unable to pick up objects from a table or effectively complete movements requiring crossing the midline. The successfully completed movements were analyzed using the above techniques and the results of the two participants were compared statistically. Almost all comparisons revealed significant differences between the two participants, with a notable exception of the 3D initiation area as a percentage of the complete movement. The stati

关键词： NGLY1 Deficiency Developmental Disorders Kinematics 3 Dimensional Analyses

Mitochondria-targeted atovaquone promotes anti-lung cancer immunity by reshaping tumor microenvironment and enhancing energy metabolism of anti-tumor immune cells

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Cancer Communications 2024年第3期44卷 448-452页

作者： Donghai Xiong Zheng Yin Mofei Huang Yian Wang Micael Hardy Balaraman Kalyanaraman stephen T Wong Ming You Center for Cancer Prevention Houston Methodist Neal Cancer CenterHouston Methodist HospitalWeill Cornell MedicineHoustonTexasUSA Department of systems Medicine and Bioengineering Houston Methodist Cancer CenterHouston Methodist HospitalWeill Cornell MedicineHoustonTexasUSA Aix Marseille Univ Centre Nationaldela Recherche Scientifique(CNRS)Institut de Chimie Radicalaire(ICR)UnitéMixte de Recherche(UMR)7273MarseilleFrance Department of Biophysics Medical College of WisconsinMilwaukeeWisconsinUSA

Dear Editor,Atovaquone(ATO),a mitochondrial inhibitor,has anti-cancer effects[1].Based on ATO,we developed mitochondria-targeted atovaquone(Mito-ATO)that had even stronger anti-tumor efficacy than ATO[2].We syn-thesized Mito-ATO by attaching the bulky triphenylphos-phonium(TPP)group to ATO via a ten-carbon alkyl chain(supplementary file of methods;supplementary Figure s1).

关键词： metabolism immunity targeted

Disparate outcomes in Hispanic patients with metabolic dysfunctionassociated steatotic liver disease/steatohepatitis and type 2 diabetes: Large cohort study

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World Journal of Diabetes 2024年第5期15卷 886-897页

作者： Joseph Matthew Gosnell George Golovko Esteban Arroyave Akshata Moghe Michael L Kueht Omar Abdulsaldarriaga Kevin H McKinney Heather L stevenson Monique R Ferguson Department of Pathology University of Texas Medical BranchGalvestonTX 77555United States Department of Pharmacology&Toxicology University of Texas Medical BranchGalvestonTX 77555United States Department of Medicine University of Texas Health Science Center at HoustonHoustonTX 77030United States Department of surgery University of Texas Medical BranchGalvestonTX 77555United States Center for Tropical Diseases Department of Internal MedicineUniversity of Texas Medical BranchGalvestonTX 77555United States Department of Internal Medicine University of Texas Medical BranchGalvestonTX 77555United States

BACKGROUND Metabolic dysfunction-associated steatotic liver disease(MAsLD)and metabolic dysfunction-associated steatohepatitis(MAsH)are a growing health burden across a significant portion of the global patient ***,these conditions seem to have disparate rates and outcomes between different ethnic *** combination of MAsLD/MAsH and type 2 diabetes increases the risk of hepatocellular carcinoma(HCC),and Hispanic patients experience the greatest burden,particularly those in south *** To compare outcomes between Hispanic and non-Hispanic patients in the United states,while further focusing on the Hispanic population within southeast texas to determine whether the documented disparity in outcomes is a function of geographical circumstance or if there is a more widespread reason that all clinicians must account for in prognostic *** This cohort analysis was conducted with data obtained from TriNetX,LLC(“TriNetX”),a global federated health research network that provides access to deidentified medical records from healthcare organizations *** cohort networks were used:University of texas Medical Branch(UTMB)hospital and the United states national database collective to determine whether disparities were related to geographic regions,like southeast *** This study findings revealed Hispanics/Latinos have a statistically significant higher occurrence of HCC,type 2 diabetes mellitus,and liver fibrosis/cirrhosis in both the United states and the UTMB Hispanic/Latino *** mortality in Hispanics/Latinos was lower within the United states group and not statistically elevated in the UTMB *** This would appear to support that Hispanic patients in southeast texas are not uniquely affected compared to the national Hispanic population.

关键词： Metabolic dysfunction-associated steatotic liver disease Metabolic dysfunction-associated steatohepatitis Hispanic Diabetes mellitus TriNetX Non-alcoholic fatty liver disease Non-alcoholic steatohepatitis steatotic liver disease Hepatocellular carcinoma

Oomycete Nudix effectors display WY-Nudix conformation and mRNA decapping activity

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Journal of Integrative Plant Biology 2024年第8期66卷 1548-1552页

作者： Baodian Guo Qinli Hu Bangwei Wang Deqiang Yao Haonan Wang Guanghui Kong Chenyang Han suomeng Dong Fengquan Liu Weiman Xing Yuanchao Wang Department of Plant Pathology Nanjing Agricultural UniversityNanjing 210095China Institute of Plant Protection Jiangsu Academy of Agricultural SciencesNanjing 210014China Department of Molecular Genetics University of Texas Southwestern Medical CenterDallasTexas 75390USA Institute of Aging&Tissue Regeneration Renji HospitalShanghai Jiao Tong University School of MedicineShanghai 200127China College of Horticulture Nanjing Agricultural UniversityNanjing 210095China College of Agriculture Guizhou UniversityGuiyang 550025China College of Life sciences Shanghai Normal UniversityShanghai 200234China

Phytopathogens secrete effectors to promote colonization of their host *** effectors modulate host immune responses by interfering with various pathways,but little is known about their biochemical *** recent reports indicate that some phytopathogen effectors have Nudix hydrolase activity,including Avr3b from Phytophthora sojae(Dong et al.,2011),AvrM14 from Melampsora lini (McCombe et al.,2023),and RipN from Ralstonia solanacearum (sun et al.,2019).

关键词： plants Phytophthora biochemical

A sTUDY ON CHINEsE PHENYLALANINE HYDROXYLAsE GENE REsTRICTION sITE POLYMORPHIsM

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science China Chemistry 1988年第12期31卷 1447-1453页

作者：曾溢滔,黄淑帧,陈美珏,张美兰,任兆瑞,徐永华,彭素芬,sAVIO L. C. WOO,胡流清 Laboratory of Medical Genetics shanghai children's hospital Laboratory of Medical Genetics shanghai children's hospital Laboratory of Medical Genetics shanghai children's hospital Laboratory of Medical Genetics shanghai children's hospital Laboratory of Medical Genetics shanghai children's hospital shanghai Institute of Cell Biology Academia sinica shanghai Institute of Cell Biology Academia sinica Department of Cell Biology Baylor College of Medicine Houston texas 77030 U.s.A. Department of Cell Biology Baylor College of Medicine Houston texas 77030 U.s.A.

Human phenylalanine hydroxylase (PAH) cDNA was applied as a hybridization probe to analyzing the following 8 restriction fragment length polymorphisms (RFLP) in the PAH genes of 80 normal and 28 phenylketonuric Chinese patients: BgⅢ, 3.6kb/1.7kb; EcoRI, 17kb/11kb; EcoRV, 30kb/25kb; HindⅢ, 4.2kb/4.0kb; MspIa, 23kb/19kb; MspIb, 4.0kb/2.2kb; PvuIIa, 19kb/6.0kb and PvuIIb, 11.5kb/9.1kb. The frequencies of the above RFLP in normal Chinese and PKU patients are: 0.13, 0.83, 0.77, 0.81, 0.12, 0.04, 0.70, 0.10 and 0.12, 0.93, 0.89, 0.81, 0.04, 0, 0.69, 0.04, respectively. This study reveals significant differences between the frequencies of individual RFLPs when these values are compared with those of the Caucasians. Finally, the detection of RFLPs in the PAH gene in the Chinese population will permit prenatal diagnosis of PKU in China.

关键词： phenylketonuria Chinese PAH gene polymorphism.