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Chinese expert consensus statement on the diagnosis and treatment of Hirschsprung disease

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Chinese Medical Journal 2024年第5期137卷 505-507页

作者： Tianqi Zhu Weibing Tang Jiexiong Feng the Chinese Research Group of Hirschsprung Disease Department of Pediatric surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyHubei Clinical Center of Hirschsprung’s Disease and Allied DisordersWuhanHubei 430030China Department of Neonatal surgery Children’s Hospital of Nanjing Medical UniversityNanjingJiangsu 210008China 不详

Hirschsprung disease(HsCR)is a congenital anomaly of the intestine caused by the developmental absence of the enteric nervous system,which results in variable lengths of intestinal dysfunction and requires surgical intervention.^([1])Due to the complex etiology,multiple surgical procedures and postoperative complications,the diagnosis and treatment strategies of HsCR have always been the focus of pediatric surgeons around the *** pediatric surgical experts with rich clinical experience from almost all national and provincial children’s medical centers in China constituted the Chinese Research Group of Hirschsprung disease(CRGHsCR).

关键词： diagnosis intestine treatment

A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial switch Operation

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Congenital Heart Disease 2024年第1期19卷 65-68页

作者： Michael A.Rebolledo Jane s.Yao Jason N.Johnson Umar s.Boston Benjamin R.Waller III Division of Pediatric Cardiology Department of PediatricsThe University of Tennessee Health Science CenterThe Heart InstituteLe Bonheur Children’s HospitalMemphisUSA College of Medicine The University of Tennessee Health ScienceMemphisUSA Division of Pediatric Cardiothoracic surgery Department of SurgeryThe University of Tennessee Health Science CenterHeart InstituteLe Bonheur Children’s HospitalMemphisUSA

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the *** review the clinical and imaging data that support our *** had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and *** case may represent one example of the experimental surgical work done prior to *** Jatene’s description of theﬁrst successful arterial switch performed in 1975.

关键词： Adult congenital heart disease arterial switch operation great vessel anomaly imaging(all modalities)

Preoperative serum cortisone levels are associated with cognition in preschool‑aged children with tetralogy of Fallot after corrective surgery:new evidence from human populations and mice

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World Journal of Pediatrics 2024年第2期20卷 173-184页

作者： si-Yu Ma Yu-Ting Liu Yue-shuang Cun Qiang Wang Ming-Cui Fu Ke-De Wu Xin-Yu Cai shu-Ting Cheng Nishant Patel Min Da Liang Hu Zhuoga Deqin Xue-Jun Kang Ming Yang Xu-Ming Mo Department of Cardiothoracic surgery Children’s Hospital of Nanjing Medical University72 Guangzhou RoadNanjing 210008China Department of Radiology Children’s Hospital of Nanjing Medical University72 Guangzhou RoadNanjing 210008China Key Laboratory of Child Development and Learning science Research Center For Learning ScienceSchool of Biological Sciences&Medical EngineeringMinistry of EducationSoutheast UniversityNanjing 210096China Medical school of Nanjing University Nanjing 210093China

Background Tetralogy of Fallot(TOF)is the most common cyanotic congenital heart *** with TOF would be confronted with neurological impairment across their *** study aimed to identify the risk factors for cerebral morphology changes and cognition in postoperative preschool-aged children with *** We used mass spectrometry(Ms)technology to assess the levels of serum metabolites,Wechsler preschool and primary scale of intelligence-Fourth edition(WPPsI-Ⅳ)index scores to evaluate neurodevelopmental levels and multimodal magnetic resonance imaging(MRI)to detect cortical morphological *** Multiple linear regression showed that preoperative levels of serum cortisone were positively correlated with the gyrification index of the left inferior parietal gyrus in children with TOF and negatively related to their lower visual spaces index and nonverbal ***,preoperative spO_(2) was negatively correlated with levels of serum cortisone after adjusting for all ***,after intervening levels of cortisone in chronic hypoxic model mice,total brain volumes were reduced at both postnatal(P)11.5 and P30 *** Our results suggest that preoperative serum cortisone levels could be used as a biomarker of neurodevelopmental impairment in children with *** study findings emphasized that preoperative levels of cortisone could influence cerebral development and cognition abilities in children with TOF.

关键词： Cognition Cortisone MRI Neurodevelopment Tetralogy of Fallot

soy lecithin in combination with soy isoflavones improves learning and memory impairments in rats by modulating cerebral vasodilation

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Food science and Human Wellness 2024年

作者： Dajun Li Xianyun Wang Chengyan Qi Huini Ding Jing shen Hongmei Huang Yuandi Xi school of Public Health， Beijing Key Laboratory of Environmental Toxicology， Capital Medical University Department of Clinical Nutrition， Beijing children's hospital， Capital Medical University， National Center for children's Health

Identifying a potential dietary non-pharmacological treatment to prevent cerebrovascular damage in Alzheimer's disease is crucial for alleviating cognitive decline in older adults and enhancing quality of life. This study featured the combined supplementation of soy lecithin (sL) and soy isoflavones (sIF)， using in vivo animal models， in vitro vascular ring preparation， and cell studies to investigate the potential advantages and mechanisms of sL combined with sIF on cognitive function and cerebrovascular health from multiple perspectives. The results show that Aβ can significantly induce learning and memory impairment in rats， as well as pathological changes in brain blood vessels， exacerbating damage to cerebral vasodilation function and subsequently reducing cerebral blood flow (CBF) in the brain. The above-mentioned phenomena induced by Aβ can be significantly improved by the combined intervention of sL and sIF. Further research has revealed that the combined intervention of sL and sIF can reverse the downregulation of the PI3K/PIP3/PDK-1/Akt/eNOs signaling pathway and phosphorylated protein expression induced by Aβ in rat brain vascular tissues and bEND.3 cells. silencing PDK-1 expression in bEND.3 cells showed that the upregulation effect of sL and sIF on Akt and eNOs disappeared. Here we find that prophylactically supplementation with sL in conjunction with sIF appears to effectively activate the PI3K/PIP3/PDK-1/Akt/eNOs pathway within cerebral vascular. This activation improves cerebrovascular vasodilation， offering potential protective effects for both cerebral vascular health and cognitive function.

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BMP9 induces osteogenic differentiation through up-regulating LGR4 via the mTORC1/ stat3 pathway in mesenchymal stem cells

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Genes & Diseases 2024年第3期11卷 468-483页

作者： Jie Zhang Jinhai Jiang Hang Liu shiyu Wang Kaixin Ke siyuan Liu Yue Jiang Lu Liu Xiang Gao Baicheng He Yuxi su Department of Pharmacology School of PharmacyChongqing Medical UniversityChongqing 400016China Key Laboratory of Biochemistry and Molecular Pharmacology of Chongqing Chongqing Medical UniversityChongqing 400016China Department of Orthopedics Second Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China orthopedics Department Children's Hospital of Chongqing Medical UniversityChongqing 400014China China International science and Technology Cooperation Base of Child Development and Critical Disorders Jiangxi Hospital Affiliated Children's Hospital of Chongqing Medical UniversityJiangxi 330000China National Clinical Research Center for Child Health and Disorders China

Bone defects and non-union are prevalent in clinical orthopedy,and the outcomes of current treatments are often *** tissue engineering offers a promising approach to treating these conditions *** morphogenetic protein 9(BMP9)can commit mesenchymal stem cells to osteogenic lineage,and a knowledge of the underlying mechanisms may help advance the field of bone tissue ***-rich repeats con-taining G protein-coupled receptor 4(LGR4),a member of G protein-coupled receptors,is essential for modulating bone *** study is aimed at investigating the impact of LGR4 on BMP9-induced osteogenesis in mesenchymal stem cells as well as the underlying *** marrow stromal cells from BMp9-knockout mice exhibited diminished LGR4 expression,and exogenous LGR4 clearly restored the impaired osteogenic potency of the bone marrow stromal ***,LGR4 expression was increased by BMP9 in C3H10T1/2 ***4 augmented the benefits of BMP9-induced osteogenic markers and bone formation,whereas LGR4 inhibition restricted these ***,the BMP9-induced li-pogenic markers were increased by LGR4 *** protein levels of Raptor and p-stat3 were elevated by *** knockdown or p-stat3 suppression attenuated the osteoblastic markers and LGR4 expression brought on by ***4 significantly reversed the blocking ef-fect of Raptor knockdown or p-stat3 suppression on the BMP9-induced osteoblastic *** interacts with p-stat3,and p-stat3 activates the LGR4 promoter *** conclusion,LGR4 boosts BMP9 osteoblastic potency in mesenchymal stem cells,and BMP9 may up-regulate LGR4 via the mTORC1/stat3 signal activation.

关键词： BMP9 LGR4 mTORC1 Osteogenic differentiation stat3

Infant anogenital digitate keratoses:A common but often neglected disease

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Pediatric Investigation 2024年第2期8卷 157-158页

作者： Jiayue Wang Bin Zhang Department of Dermatology West China Second University HospitalKey Laboratory of Birth Defects and Related Diseases of Women and ChildrenMinistry of EducationSichuan UniversitySichuanChina Department of Dermatology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina

To the editor:Infant anogenital digitate keratoses(IADK)is a relatively common but often neglected disorder during *** is a recently described disease,and improved awareness of it is essential for precise diagnosis an... 详细信息

关键词： diagnosis neglected digit

Active Micro-Nano-Collaborative Bioelectronic Device for Advanced Electrophysiological Recording

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Nano-Micro Letters 2024年第7期16卷 244-264页

作者： Yuting Xiang Keda shi Ying Li Jiajin Xue Zhicheng Tong Huiming Li Zhongjun Li Chong Teng Jiaru Fang Ning Hu Department of Chemistry Zhejiang-Israel Joint Laboratory of Self-Assembling Functional MaterialsZJU-Hangzhou Global Scientific and Technological Innovation CenterZhejiang UniversityHangzhou 310058People’s Republic of China school of Basic Medical sciences Zhejiang Chinese Medical UniversityHangzhou 310053People’s Republic of China General surgery Department Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Children’s HealthHangzhou 310052People’s Republic of China Department of Lung Transplantation and General Thoracic surgery The First Affiliated HospitalZhejiang University School of MedicineHangzhou 310003People’s Republic of China Department of Orthopedics The Fourth Affiliated HospitalZhejiang University School of MedicineYiwu 322005People’s Republic of China school of Electronics and Information Technology Sun Yat-Sen UniversityGuangzhou 510006People’s Republic of China Department of Obstetrics and Gynecology The Tenth Affiliated HospitalSouthern Medical UniversityDongguan 523059People’s Republic of China Dongguan Key Laboratory of Major Diseases in Obstetrics and Gynecology Dongguan 523059People’s Republic of China

The development of precise and sensitive electrophysiological recording platforms holds the utmost importance for research in the fields of cardiology and *** recent years,active micro/nano-bioelectronic devices have undergone significant advancements,thereby facilitating the study of *** distinctive configuration and exceptional functionality of these active micro-nano-collaborative bioelectronic devices offer the potential for the recording of high-fidelity action potential signals on a large *** this paper,we review three-dimensional active nano-transistors and planar active micro-transistors in terms of their applications in electroexcitable cells,focusing on the evaluation of the effects of active micro/nano-bioelectronic devices on electrophysiological *** forward to the possibilities,challenges,and wide prospects of active micro-nano-devices,we expect to advance their progress to satisfy the demands of theoretical investigations and medical implementations within the domains of cardiology and neuroscience research.

关键词： Active micro/nano collaborative bioelectronic device Three-dimensional active nano-transistor Planar active microtransistor Electrophysiology

Epitranscriptomic Regulation of NMDA Receptors Rears its Ugly Head in Chemotherapy-Induced Neuropathic Pain

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Neuroscience Bulletin 2024年第8期40卷 1209-1211页

作者： Wing Lam Yu Gerald W.Zamponi Department of Clinical Neurosciences Hotchkiss Brain InstituteAlberta Children's Hospital Research InstituteUniversity of CalgaryCalgaryAB T2N 4N1Canada

number of different cancer chemotherapy agents such as cisplatin,oxaliplatin,and paclitaxel can lead to nerve damage,thereby giving rise to neuropathic pain states that present with mechanical and cold allodynia[1].Although these pain conditions often resolve after completion of the chemotherapy,a significant portion of patients exhibit longlasting pain that can persist longer than 12 months with a negative impact on the quality of life of affected individuals[1].

关键词： chemotherapy pain damage

Interleukin-10 suppresses lipid metabolism-mediated intestinal inflammation

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Life Metabolism 2024年第3期3卷 3-5页

作者： Tristram A.J.Ryan Ivan Zanoni Division of Immunology and Division of Gastroenterology Boston Children’s Hospital and Harvard Medical SchoolBostonMA 02115United States

In a recent paper published in Nature,York et *** that the anti-inflammatory cytokine interleukin(IL)-10 regulates sphingolipid metabolism to limit NF-κB-mediated *** of Il10 in mice,or genetic mutation of IL10 in humans,predisposes to inflammatory bowel disease,which may be overcome by restoring homeostatic sphingolipid metabolism.

关键词： metabolism inflammation al.