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Tissue-resident macrophages exacerbate lung injury after remote sterile damage

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Cellular & Molecular Immunology 2024年第4期21卷 332-348页

作者： Hanhui Zhong Jingjing Ji Jinling Zhuang Ziying Xiong Pengyun Xie Xiaolei Liu Jundi Zheng Wangli Tian Xiaoyang Hong Jing Tang The department of Anesthesiology Affiliated Hospital of Guangdong Medical UniversityZhanjiangGuangdongChina The department of Critical Care Medicine General Hospital of Southern Theater Command of PLAGuangzhouChina The department of Respiratory Medicine Guangdong Provincial Hospital of Integrated Chinese and Western MedicineFoshanChina Pediatric Intensive Care Unit Senior Department of Pediatricsthe Seventh Medical Center of PLA General HospitalBeijingChina

Remote organ injury,which is a common secondary complication of sterile tissue damage,is a major cause of poor prognosis and is difficult to ***,we report the critical role of tissue-resident macrophages in lung injury after trauma or stroke through the inflammatory *** found that depleting tissue-resident macrophages rather than disrupting the recruitment of monocyte-derived macrophages attenuated lung injury after trauma or *** findings revealed that the release of circulating alarmins from sites of distant sterile tissue damage triggered an inflammatory response in lung-resident macrophages by binding to receptor for advanced glycation end products(RAGE)on the membrane,which activated epidermal growth factor receptor(EGFR).Mechanistically,ligand-activated RAGE triggered EGFR activation through an interaction,leading to Rab5-mediated RAGE internalization and EGFR phosphorylation,which subsequently recruited and activated P38;this,in turn,promoted RAGE translation and trafficking to the plasma membrane to increase the cellular response to RAGE ligands,consequently exacerbating *** study also showed that the loss of RAGE or EGFR expression by adoptive transfer of macrophages,blocking the function of RAGE with a neutralizing antibody,or pharmacological inhibition of EGFR activation in macrophages could protect against trauma-or stroke-induced remote lung ***,our study revealed that targeting the RAGE-EGFR signaling pathway in tissue-resident macrophages is a potential therapeutic approach for treating secondary complications of sterile damage.

关键词： tissue-resident macrophage RAGE remote sterile damage

Height and body mass index trajectories from 1975 to 2015 and prevalence of stunting,underweight and obesity in 2016 among children in Chinese cities:findings from five rounds of a national survey

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World Journal of pediatrics 2024年第4期20卷 404-412页

作者： Xin‑Nan Zong Hui Li Ya‑Qin Zhang department of Growth and Development Capital Institute of PediatricsBeijing 100020China

Background A more comprehensive assessment of growth and nutrition in children is required in China due to rapid socioeconomic *** aimed to investigate height and body mass index(BMI)trajectories from 1975 to 2015 and the prevalence of stunting and obesity in 2016 among children in Chinese *** A total of 904,263 children from birth to 6.9 years were collected from a series of nationally representative surveys in *** and BMI trajectories and prevalence of stunting,underweight,overweight and obesity were *** The average height level of Chinese urban children under 7 years presented a positive secular trend from 1975 to 2015;however,a slowing tendency occurred in 2005‒*** apparent increase was observed at the 5th,50th and 95th percentiles of BMI in urban children aged 3 years and older,with a more prominent increase at the 95th *** total prevalence of stunting and underweight under 7 years was 1.4%and 2.0%,*** total prevalence of overweight and obesity under 7 years was 12.6%and 4.3%,respectively,with 12.7%and 4.9%for boys,12.6%and 3.6%for girls,12.1%and 4.0%in urban areas and 13.1%and 4.5%in suburban rural *** The average height level of Chinese urban children has reached World Health Organization child growth standards since 2005 and presented a slowing tendency in secular trend in 2005‒*** attention and efforts and public health interventions should be urgently made to combat overweight and obesity among preschool children.

关键词： Body mass index Children Height Obesity Stunting

Genetic variant reanalysis reveals a case of Sandhoff disease with onset of infantile epileptic spasm syndrome

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Acta Epileptologica 2024年第1期7卷 67-73页

作者： Qi Zhang Liping Zou Qian Lu Qiuhong Wang Shuo Dun Jing Wang Medical School of Chinese PLA Beijing100853China department of pediatrics the First Medical Center of PLA General HospitalBeijing100853China

Background Sandhoff disease(SD)i s an autosomal recessive lysosomal disease with clinical manifestations such as epilepsy,psychomotor retardation and developmental ***,infantile SD with onset of infantile epilepsy spasm syndrome(IESS)is extremely *** presentation The case presented here was a 22-month-old boy,who presented with IESS and psychomotor retardation/regression at 6 months of *** patient showed progressive aggravation of seizures and excessive startle *** whole exome sequencing data,which initially revealed negative results,were reanalyzed and indicated a homozygous mutation at the c.1613+4del splice site of the HEXB *** activities ofβ-hexosaminidase A and total hexosaminidase were significantly *** fundus examination showed cherry red spots at the *** IESS can be an epileptic phenotype of infantile *** phenotypes should be adequately collected in genetic *** the case of negative sequencing results,gene variant reanalysis can be performed when the patients show clinically suspicious indications.

关键词： Infantile Sandhoff disease Gene variant reanalysis HEXB gene Infantile epilepsy spasm syndrome Cherry red spot Human phenotype ontology

Sirolimus can promote the disappearance of renal angiomyolipoma associated with tuberous sclerosis complex:a prospective cohort study

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World Journal of pediatrics 2024年第6期20卷 602-610页

作者： Shuo Dun Yang-Yang Wang Lin Wan Qiu-Hong Wang Qian Lu Xiao-Yan Yang Qi Zhang Hui-Min Chen Lu-Peng Qiu Li-Ping Zou Medical School of Chinese PLA Beijing 100853China department of pediatrics The First Medical CenterChinese PLA General HospitalBeijing 100853China Center of Epilepsy Beijing Institute for Brain DisordersBeijing 100069China

Background Renal angiomyolipoma(RAML)is the most common kidney lesion in patients with tuberous sclerosis complex(TSC),affecting about 80%of *** is a benign tumor that grows over time,usually bilaterally,and can easily lead to kidney complications such as acute ***,we investigated the efficacy and safety of sirolimus in children with TSC-associated RAML and explored the factors affecting tumor disappearance under sirolimus treatment through subgroup *** A prospective cohort study was *** was initiated at 1 mg/(m^(2)×day),and dose adjustments were made by a 2-week titration period to attain a trough blood concentration of 5-10 ng/*** disappearance of RAML in chil-dren after sirolimus treatment was observed,and Cox regression was used to screen the factors affecting tumor *** One hundred and twenty-six patients who met the criteria were *** 3 months,6 months,12 months,and 24 months of follow-up,tumors disappeared in 18(14.3%),30(23.8%),39(31.0%),and 42(33.3%)children,*** disappeared in 50(39.7%)children by the last visit of each individual,and 30(60%)of them occurred within 6 *** multivariate Cox regression analysis showed that patients with a smaller maximum tumor diameter at baseline had a higher tumor disappearance ***-six(29%)patients had stomatitis during the entire treatment period,and no serious *** Sirolimus could promote the disappearance of TSC-related *** disappearance rate was correlated with the maximum diameter at baseline,and the smaller the tumor was,the higher the disappearance *** is well tolerated in the treatment of RAML associated with TSC.

关键词： Angiomyolipoma mTOR inhibitor Sirolimus Tuberous sclerosis

Hematochezia due to rectal invasion by an internal iliac artery aneurysm: A case report

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World Journal of Clinical Cases 2024年第11期12卷 1980-1989页

作者： Fang Li Bin Zhao Yong-Qiang Liu Guo-Qing Chen Rong-Feng Qu Chao Xu Zhui Long Jin-Song Wu Mao Xiong Wei-Hang Liu Li Zhu Xiao-Ling Feng Lei Zhang department of general Surgery Chongqing General HospitalChongqing 401147China

BACKGROUND This case report presents the rare occurrence of hematochezia due to an internal iliac artery aneurysm leading to an arterioenteric fistula,expanding the differential diagnosis for gastrointestinal *** emphasizes the importance of considering vascular origins in cases of atypical hematochezia,particularly in the absence of common gastrointestinal causes,and highlights the role of imaging and multidisciplinary management in diagnosing and treating such unusual *** SUMMARY A 75-year-old man with a history of hypertension presented with 12 d of hematochezia,experiencing bloody stools 7-8 times per *** computed tomography(CT)scans revealed an aneurysmal rupture near the right internal iliac artery with suspected hematoma *** levels progressively decreased to 7 g/*** arterial angiography and iliac arterycovered stent placement were performed,followed by balloon *** initial stabilization,minor rectal bleeding and abdominal pain persisted,leading to further diagnostic *** identified a neoplasm and potential perforation at the proximal *** exploratory laparotomy confirmed the presence of a hematoma and an aneurysm invading the rectal wall,necessitating partial rectal resection,intestinal anastomosis,and *** recovery was successful,with no further bleeding incidents and normal follow-up CT and colonoscopy results after six *** In cases of unusual gastrointestinal bleeding,it is necessary to consider vascular causes for effective diagnosis and intervention.

关键词： Iliac artery aneurysm Hematoma Rectum Hematochezia Case report

Magnetic resonance-guided focused ultrasound for essential tremor:a prospective,single center,single-arm study

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Neural Regeneration Research 2024年第9期19卷 2075-2080页

作者： Rui Zong Xuemei Li Chunyu Yin Jianfeng He Dekang Zhang Xiangbing Bian Lichao Huang Jiayou Zhou Zhipei Ling Lin Ma Xin Lou Longsheng Pan Xinguang Yu Seniro department of Neurosurgery The First Medical Center of the PLA General HospitalBeijingChina Clinics of Cadre Department of OutpatientThe First Medical Center of the PLA General HospitalBeijingChina department of Radiology The First Medical Center of the PLA General HospitalBeijingChina

The safety and effectiveness of magnetic resonance-guided focused ultrasound thalamotomy has been broadly established and validated for the treatment of essential *** 2018,the first magnetic resonance-guided focused ultrasound system in Chinese mainland was installed at the First Medical Center of the PLA general *** prospective,single center,open-label,single-arm study was part of a worldwide prospective multicenter clinical trial(*** Identifier:NCT03253991)conducted to confirm the safety and efficacy of magnetic resonance-guided focused ultrasound for treating essential tremor in the local *** 2019 to 2020,10 patients with medication refractory essential tremor were recruited into this open-label,single arm *** treatment efficacy was determined using the Clinical Rating Scale for *** was evaluated according to the incidence and severity of adverse *** of the subjects underwent a unilateral thalamotomy targeting the ventral intermediate *** the baseline assessment,the estimated marginal mean of the Clinical Rating Scale for Tremor total score was 58.3±3.6,and this improved after treatment to 23.1±6.4 at a 12-month follow-up assessment.A total of 50 adverse events were recorded,and 2 were defined as *** most common intraoperative adverse events were nausea and *** most frequent postoperative adverse events were paresthesia and equilibrium *** of the adverse events were mild and usually disappeared within a few *** findings suggest that magnetic resonance-guided focused ultrasound for the treatment of essential tremor is effective,with a good safety profile,for patients in Chinese mainland.

关键词： efficacy essential tremor magnetic resonance-guided focused ultrasound(MRgFUS) movement disorder safety stereotactic therapy thalamotomy ventral intermediate nucleus

Advances in the molecular genetics of parathyroid tumors

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Chinese Medical Journal 2024年第3期137卷 376-378页

作者： Liwei Zhao Xianghui He department of general Surgery Tianjin Medical University General HospitalTianjin 300052China

To the Editor:Parathyroid tumors are endocrine tumors that can lead to disorders of calcium and phosphorus metabolism and involve multiple organ *** the past,the genetics of parathyroid tumors have been well established,and the significant roles of genes such as MEN1,CDC73,and CCND1 in the process of parathyroid tumorigenesis have been identified and confirmed[Table 1].However,with further research and the widespread use of sequencing technology,“new”roles for“old”genes and new pathogenic mechanisms have been discovered.

关键词： metabolism tumors parathyroid

Growth and development of children in China:achievements,problems and prospects

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World Journal of pediatrics 2024年第2期20卷 97-104页

作者： Xin-Nan Zong Hui Li department of Growth and Development Capital Institute of PediatricsBeijing 100020China

Growth reflects the general condition of society,and is a measure of the nutritional and hygienic status of a population[1,2].Nutrition is one of the most important components of children’s well-being[3].The Chinese government attaches great importance to children’s health and development,with a clear statement and consistent practice for“Children are the future and hope of the motherland”.

关键词： mother statement children

Stubborn Hypoxia in Neonates with D-Transposition of the Great Arteries after Arterial Switch Operation: Central Sleep Apnea as the Cause and Potential Indicator of Brain Immaturity

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Congenital Heart Disease 2024年第2期19卷 185-195页

作者： Camden L.Hebson Kyle Bliton Amr Y.Hammouda Kaitlyn Barr W.Hampton Gray Mohini Gunnett Waldemar F.Carlo department of pediatrics Division of Pediatric CardiologyUniversity of Alabama BirminghamBirmingham35233USA department of pediatrics Division of Pediatric PulmonologyUniversity of Alabama BirminghamBirmingham35233USA department of pediatrics University of Alabama BirminghamBirmingham35233USA department of Surgery Division of Cardiothoracic SurgeryUniversity of Alabama BirminghamBirmingham35233USA

D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive *** significant central sleep apnea is uncommon in non-premature infants,but when present indicates immature autonomic control of respiration likely due to a neurologic *** report the unanticipatedfinding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO,with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal *** this report,we will review each patient’s clinical course and then examine the literature on pediatric central sleep apnea,neurodevelopmental outcomes after ASO,and the important overlap of these entities in the care of patients going forward.

关键词： Transposition congenital heart disease sleep apnea polysomnography