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Use of Patient-specific “4D” Tele-Education to Enhance Actual and Perceived Knowledge in Congenital Heart Disease (CHD) Patients

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Congenital Heart Disease 2024年第1期19卷 5-17页

作者： Molly Clarke Karin Hamann Nancy Klein Laura Olivieri Yue-Hin Loke Department of Inpatient Cardiology Boston Children’s HospitalBoston02115USA Department of Cardiology Children’s National HospitalWashington20001USA Department of Cardiology Noninvasive Cardiac ImagingUniversity of Pittsburgh Medical Center Children’s Hospital of PittsburghPittsburgh15224USA

Background:Patients with congenital heart disease(CHD)will transition to lifelong adult congenital cardiac ***,their structural heart disease is challenging to convey via two-dimensional *** study utilized a tele-educational environment,with personalized three-dimensional(3D)modeling and health Details(3D+Details=“4D”),to improve actual and perceived knowledge,both important components of transition readiness in CHD ***:Participants aged≥13 years with a history of CHD and cardiac magnetic resonance imaging(MRI)studies were *** MRI datasets were then used to segment and create 3D heart models(using Mimics,Materialize Inc.).Participantsﬁrst completed the MyHeart Questionnaire,a validated survey of actual knowledge.A tele-educational session was then scheduled,during which participants were shown a 3D model of a normal heart,followed by their personal 3D heart model and speciﬁc health *** then repeated the actual knowledge survey,in addition to questionnaires assessing perceived knowledge pre-and post-session,as well as a satisfaction ***:Twenty-two patients were *** knowledge increased from 75%±15%to 89%±20%(p=0.00043)and perceived knowledge increased inﬁve of seven *** knowledge correlated with perceived knowledge(r=0.608,ps ranked the 3D model as“very satisfactory”and ninety-ﬁve percent ranked the educational session as“very help-ful”or“extremely helpful.”Conclusions:The use of“4D”tele-education increased both actual and perceived knowledge and may help improve transition readiness in CHD patients.

关键词： Congenital heart disease tele-education transition

Inpatient management of iron deficiency anemia in pediatric patients with inflammatory bowel disease: A single center experience

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World Journal of Clinical Pediatrics 2024年第1期13卷 107-117页

作者： Krishanth Manokaran Jonathan spaan Giulio Cataldo Christopher Lyons Paul D Mitchell Tatyana sare LoriA Zimmerman Paul A Rufo Center for Inflammatory Bowel Disease Boston Children's HospitalBostonMA 02115United States Institutional Centers for Clinical and Translational Research Boston Children's HospitalBostonMA 02115United States

BACKGROUND screening for iron deficiency anemia(IDA)is important in managing pediatric patients with inflammatory bowel disease(IBD).Concerns related to adverse reactions may contribute to a reluctance to prescribe intravenous(IV)iron to treat IDA in this *** To track the efficacy and safety of IV iron therapy in treating IDA in pediatric IBD patients admitted to our *** A longitudinal observational cohort study was performed on 236 consecutive pediatric patients admitted to our tertiary IBD care center between september 2017 and December 2019.92 patients met study criteria for IDA,of which 57 received IV iron,17 received oral iron,and 18 were discharged prior to receiving iron *** Patients treated with IV iron during their hospitalization experienced a significant increase of 1.9(±0.2)g/dL in mean(±sE)hemoglobin(Hb)concentration by the first ambulatory follow-up,compared to patients who received oral iron 0.8(±0.3)g/dL or no iron 0.8(±0.3)g/dL(P=0.03).One out of 57(1.8%)patients that received IV iron therapy experienced an adverse *** Our findings demonstrate that treatment with IV iron therapy is safe and efficacious in improving Hb and iron levels in pediatric patients with IDA and active IBD.

关键词： Iron deficiency anemia Pediatric inflammatory bowel disease Intravenous iron therapy Inflammatory bowel disease

Interleukins in Epilepsy:Friend or Foe

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Neuroscience Bulletin 2024年第5期40卷 635-657页

作者： Yuan Dong Xia Zhang Ying Wang Neuropsychiatry Research Institute The Affiliated Hospital of Qingdao UniversityQingdao UniversityQingdao266000China Program in Cellular and Molecular Medicine Boston Children’s HospitalBostonMA02115USA

Epilepsy is a chronic neurological disorder with recurrent unprovoked seizures,affecting~65 million *** in patients with epilepsy and animal models suggests a contribution of neuroinflammation to epileptogenesis and the development of ***(ILs),as one of the major contributors to neuroinflammation,are intensively studied for their association and modulatory effects on ictogenesis and *** are commonly divided into pro-and anti-inflammatory cytokines and therefore are expected to be pathogenic or neuroprotective in ***,both protective and destructive effects have been reported for many *** may be due to the complex nature of ILs,and also possibly due to the different disease courses that those ILs are involved *** this review,we summarize the contributions of different ILs in those processes and provide a current overview of recent research advances,as well as preclinical and clinical studies targeting ILs in the treatment of epilepsy.

关键词： Epilepsy Interleukins Neuroinflammation

Toward Artificial Peptide Nanocapsules

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Nano-Micro Letters 2024年第10期16卷 240-243页

作者： Yuan Wang Bing Chen Laboratory for Biomaterials and Drug Delivery Department of AnesthesiologyDivision of Critical Care MedicineBoston Children's HospitalHarvard Medical SchoolBostonMA 02115USA College of Electronic and Optical Engineering and College of Flexible Electronics(Future Technology) Nanjing University of Posts and TelecommunicationsNanjing 210023People's Republic of China

HIGHLIGHTs The formation of peptide nanocapsules is facilitated by a gradient interface,where the differential solvent concentration drives the peptides to preferentially localize and *** peptide nanocapsules,characterized by their hollow structures,demonstrated potential as carriers for targeted drug delivery.1 Introduction Peptide nanocapsules are a type of nanoscale delivery system that encapsulates active substances within a shell composed of peptides,leveraging the unique properties of peptides such as biocompatibility and biodegradability[1].Historically,the development of peptide nanocapsules was inspired primordially by the natural biological processes.

关键词： Peptide nanocapsules self-assembly Drug delivery Nanomedicine

Therapeutic nucleic acids in regenerative medicine and tissue repair

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Nano Research 2024年第10期17卷 8942-8976页

作者： Qimanguli saiding Duotian Qin soohwan An Dylan Neal Patel Muhammad Muzamil Khan Na Kong Wei Tao Center for Nanomedicine and Department of Anesthesiology Brigham and Women’s HospitalHarvard Medical SchoolBostonMassachusetts 02115USA

In the vanguard of biomedical innovation,regenerative medicine emerges as a transformative paradigm,concentrating its efforts on rectifying tissue deficits and functional aberrations in patients through the strategic augmentation of endogenous cellular *** to this approach is nucleic acid therapy,which offers a novel pathway for tissue regeneration by modulating key signaling *** one of the most effective ways to regulate cell function,nucleic acids are crucial for various tissue regeneration;however,their in vivo therapeutic applications face substantial challenges,including nuclease degradation,cell membrane impermeability,and targeted intracellular ***-based gene delivery systems offer a solution for stable and localized drug delivery by enabling the controlled overexpression of therapeutic nucleic acids,producing functional regulatory *** review presents examples of nucleic acid applications in regenerative medicine,highlighting the synergy between nucleic acids and biomaterial *** underlines the importance of nucleic acid delivery techniques,the choice of therapeutic nucleic acids,and biomaterials for advancing tissue *** latest developments in designing nucleic acid biomaterial-based delivery vehicles are explored,the mechanism of nucleic acids in tissue regeneration is elucidated,and their limitations are discussed while considering future directions for the clinical translation of nucleic acid-based therapeutics.

关键词： tissue repair nucleic acids biomaterials delivery systems regenerative medicine

Expanding the toolbox of metal-free organic halide perovskite for X-ray detection

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Chinese Chemical Letters 2024年第9期35卷 102-110页

作者： Xiuwen Xu Quan Zhou Yacong Wang Yunjie He Qiang Wang Yuan Wang Bing Chen College of Electronic and Optical Engineering and College of Flexible Electronics(Future Technology) Nanjing University of Posts and TelecommunicationsNanjing 210023China Laboratory for Biomaterials and Drug Delivery Department of AnesthesiologyDivision of Critical Care MedicineBoston Children’s HospitalHarvard Medical SchoolBostonMA 02115United States

X-ray detection plays a crucial role across various aspects of our daily lives,encompassing medical diagnoses,security screenings,and non-destructive examinations in industrial *** the wide array of application contexts,a wealth of opportunities is entailed with the practical utilization of both organic and inorganic X-ray detection materials.A novel and promising contender in this realm is the emergence of metal-free organic halide perovskites(O-PVsKs),offering great opportunities and tremendous potential in X-ray *** potential can be attributed to the distinct crystalline configuration of O-PVsKs,where organic constituents are structured into an ABX3perovskite ***,O-PVsKs exhibit captivating characteristics reminiscent of organic materials,such as lightweight nature and modifiability,all while retaining the distinctive traits associated with halide perovskites ranging from diverse structures to tunable optoelectronic *** review article delves into the intrinsic attributes of O-PVsKs and critically examines the viability of O-PVsKs in X-ray detection,through which key features that distinguish O-PVsKs from traditional organic semiconductors and perovskites are *** is followed by a perspective given on their future avenues for exploration.

关键词： Metal-free perovskites X-ray detection Electronic band structure Carrier transport Ion migration

Evaluation of large language models for the classification of medical device software

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Bio-Design and Manufacturing 2024年第5期7卷 819-822页

作者： Yu Han Aaron Ceross Florence Bourgeois Paulo savaget Jeroen H.M.Bergmann Department of Engineering science University of OxfordOld Road CampusOxford OX37QDUK Harvard-MIT Center for Regulatory science Harvard Medical SchoolBostonMAUSA Computational Health Informatics Program(CHIP) Boston Children’s HospitalBostonMAUSA Department of Technology and Innovation The University of Southern DenmarkOdenseDenmark

Amidst the rapidly expanding integration of large language models(LLMs)across various sectors(ranging from everyday applications to specialized fields demanding stringent regulatory adherence),our investigation seeks to determine how well these models can support medical device software *** device classification functions to systematically categorize devices according to their designated use,associated risk levels,and requisite regulatory oversight,thereby providing a structured framework for ensuring safety and efficacy as mandated by regulatory authorities.

关键词： everyday demanding thereby

Exome sequencing identifies a likely causative variant in 53%of families with ciliopathy-related features on renal ultrasound after excluding NPHP1 deletions

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Genes & Diseases 2024年第5期11卷 40-43页

作者： Konstantin Deutsch Verena Klambt Thomas M.Kitzler Tilman Jobst-schwan Ronen schneider Florian Buerger steve seltzsam sherif El Desoky Jameela A.Kari Farkhanda Hafeez Maria szczepanska Loai A.Eid Hazem s.Awad Muna Al-saffar Neveen A.soliman Velibor Tasic Camille Nicolas-Frank Kirollos Yousef Luca M.schierbaum sophia schneider Abdul Halawi Izzeldin Elmubarak Katharina Lemberg shirlee shril shrikant M.Mane Nancy Rodig Friedhelm Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsmedizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany Department of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany Department of Pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia Department of Pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan Department of Pediatrics Faculty of Medical Sciences in ZabrzeMedical University of Silesia in KatowiceKatowice 40-752Poland Dubai hospital and Al-Jalila children’s specialty hospital Kidney Center of ExcellenceDubai 4545United Arab Emirates Department of Genetics and Genomics UAE UniversityAbu Dhabi 15551United Arab Emirates Department of Pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative Medicine(ECRRM) Cairo 11511Egypt Medical Faculty skopje University Children’s HospitalSkopje 1000North Macedonia Department of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NPHP-RC)represent one of the most common causes of chronic kidney disease in the first three decades of life and are characterized by a broad genetic and clinical heterogeneity.1 To date,more than 90 genes have been identified that cause autosomalrecessive NPHP-RC if mutated,accounting for up to 60%of cases.1 Among these,homozygous deletions of NPHP1 are the most common *** genes localize to primary cilia,basal bodies,or the centrosome and lead to a primary ciliary disruption if mutated,thereby causing a broad phenotypical spectrum.1 Patients that suffer from NPHP-RC can either have an isolated renal phenotype,such as polyuria,polydipsia,decreased urinary concentration ability,and secondary enuresis due to loss of tubular function,or present with extrarenal symptoms including retinal degeneration,cerebellar vermis hypoplasia,or hepatic *** are often diagnosed in early adolescence,reaching end-stage renal disease before 25 years of age,but early onset and rapidly progressive forms of NPHP-RC also exist.1 Renal ultrasound indicates kidneys of normal or reduced renal length with increased echogenicity and corticomedullary cysts.

关键词： degeneration urinary likely

Dual‑Atom Nanozyme Eye Drops Attenuate Inflammation and Break the Vicious Cycle in Dry Eye Disease

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Nano-Micro Letters 2024年第6期16卷 439-457页

作者： Dandan Chu Mengyang Zhao shisong Rong Wonho Jhe Xiaolu Cai Yi Xiao Wei Zhang Xingchen Geng Zhanrong Li Xingcai Zhang Jingguo Li Henan Eye hospital Henan Provincial People’s HospitalPeople’s Hospital of Zhengzhou UniversityZhengzhou 450003People’s Republic of China Department of Ophthalmology Mass Eye and EarMass General BrighamHarvard Medical SchoolBostonMA 02114USA school of Engineering and Applied sciences Harvard UniversityCambridgeMA 02138USA College of Chemistry Zhengzhou UniversityZhengzhou 450001People’s Republic of China

Dry eye disease(DED)is a major ocular pathology worldwide,causing serious ocular discomfort and even visual *** incidence of DED is gradually increasing with the highfrequency use of electronic *** inflammation is core cause of the DED vicious cycle,reactive oxygen species(ROs)play a pivotal role in the vicious cycle by regulating inflammation from ***,current therapies merely targeting inflammation show the failure of DED ***,a novel dual-atom nanozymes(DAN)-based eye drops are *** antioxidative DAN is successfully prepared by embedding Fe and Mn bimetallic single-atoms in N-doped carbon material and modifying it with a hydrophilic *** in vitro and in vivo results demonstrate the DAN is endowed with superior biological activity in scavenging excessive ROs,inhibiting NLRP3 inflammasome activation,decreasing proinflammatory cytokines expression,and suppressing cell ***,the DAN effectively alleviate ocular inflammation,promote corneal epithelial repair,recover goblet cell density and tear secretion,thus breaking the DED vicious *** findings open an avenue to make the DAN as an intervention form to DED and ROsmediated inflammatory diseases.

关键词： Dry eye disease DAN Dual-atom nanozyme Vicious cycle NLRP3 inflammasome Nanomedicine