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pulmonary immune cells and inflammatory cytokine dysregulation are associated with mortality of IL-1R1^(-/-) mice infected with influenza virus(H1N1)

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Zoological Research 2017年第3期38卷 146-154页

作者： Lei Guo Yan-Cui Wang Jun-Jie Mei Ruo-Tong Ning Jing-Jing Wang Jia-Qi Li Xi Wang Hui-Wen Zheng Hai-Tao Fan Long-Ding Liu Department of Viral Immunology Institute of Medical Biology Chinese Academy of Medical Science and Peking Union Medical College Division of Neonatology Department of Pediatrics Children's Hospital of Philadelphia Perelman School of Medicine University of Pennsylvania

Respirovirus infection can cause viral pneumonia and acute lung injury （ALl）. The interleukin-1 （IL-1） family consists of proinflammatory cytokines that play essential roles in regulating immune and inflammatory responses in vivo. IL-1 signaling is associated with protection against respiratory influenza virus infection by mediation of the pulmonary anti-viral immune response and inflammation. We analyzed the infiltration lung immune leukocytes and cytokines that contribute to inflammatory lung pathology and mortality of fatal H1N1 virus-infected IL-1 receptor 1 （IL-1R1） deficient mice. Results showed that early innate immune cells and cytokine/chemokine dysregulation were observed with significantly decreased neutrophil infiltration and IL-6, TNF-α, G-CSF, KC, and MIP-2 cytokine levels in the bronchoalveolar lavage fluid of infected IL-1R1^-/- mice in comparison with that of wild type infected mice. The adaptive immune response against the H1N1 virus in IL-1 R1^-/- mice was impaired with downregulated anti-viral Thl cell, CD8＋ cell, and antibody functions, which contributes to attenuated viral clearance. Histological analysis revealed reduced lung inflammation during early infection but severe lung pathology in late infection in IL-1R1^-/- mice compared with that in WT infected mice. Moreover, the infected IL-1R1^--/ mice showed markedly reduced neutrophil generation in bone marrow and neutrophil recruitment to the inflamed lung. Together, these results suggest that IL-1 signaling is associated with pulmonary anti-influenza immune response and inflammatory lung injury, particularly via the influence on neutrophil mobilization and inflammatory cytokine/chemokine production.

关键词： Influenza Lung inflammation IL-1 receptor 1 Neutrophil

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ACTA2 mutation is responsible for multisystemic smooth muscle dysfunction syndrome with seizures:A case report and review of literature

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World Journal of Clinical Cases 2021年第29期9卷 8789-8796页

作者： Wen-Xian Yang Hang-Hu Zhang Jia-Ni Hu Li Zhao Yan-Yun Li Xiao-Li Shao Department of pediatrics Shaoxing University School of MedicineShaoxing 312000Zhejiang ProvinceChina Department of pediatrics Shaoxing Peoples’HospitalThe First Affiliated Hospital of Shaoxing UniversityShaoxing 312000Zhejiang ProvinceChina Department of Radiology Shaoxing Peoples’HospitalThe First Affiliated Hospital of Shaoxing UniversityShaoxing 312000Zhejiang ProvinceChina

BACKGROUND ACTA2 gene is a specific gene that encodes actinα*** smooth muscle dysfunction syndrome(MSMDS)is a multisystem disease characterized by aortic and cerebrovascular lesions caused by ACTA2 gene *** have been many reports of cardiac,pulmonary and cerebrovascular lesions caused by MSMDS;however,few studies have focused on seizures caused by *** SUMMARY Our patient was a girl aged 7 years and 8 mo with recurrent cough,asthma and seizures for 7 *** was diagnosed with severe pneumonia,congenital heart disease,cardiac insufficiency,and malnutrition in the local *** ultrasonography revealed congenital heart disease,patent ductus arteriosus(with a diameter of 0.68 cm),left coronary arteriectasis,patent oval foramen(0.12 cm),tricuspid and pulmonary regurgitation,and pulmonary *** magnetic resonance imaging and magnetic resonance angiography indicated stiffness in the brain vessels,together with multiple aberrant signaling shadows in bilateral paraventricular regions.A heterozygous mutation(c.536G>A)was identified in the ACTA2 gene,resulting in generation of ***,the girl was diagnosed with MSMDS combined with *** patient had 4 episodes of seizures before treatment,and no onset of seizure was reported after oral administration of sodium valproate for 1 *** MSMDS has a variety of clinical manifestations and unique cranial imaging *** injury and white matter injury may lead to *** detection can confirm the diagnosis and prevent missed diagnosis or misdiagnosis.

关键词： Multi-systemic smooth muscle dysfunction syndrome ACTA2 gene Seizures Gene detection Case report

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Effects of General Anesthesia on the Results of Cardiac Catheterization in Pediatric Patients with Ventricular Septal Defect

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Congenital Heart Disease 2023年第2期18卷 235-243页

作者： Kiyotaka Go Taichi Kato Machiko Kito Yoshihito Morimoto Satoru Kawai Hidenori Yamamoto Yoshie Fukasawa Kazushi Yasuda Department of Pediatric Cardiology Aichi Children’s Health and Medical CenterObu474-8710Japan Department of pediatrics Nagoya University Graduate School of MedicineNagoya466-8560Japan

Background:There is no gold standard sedation method for pediatric cardiac *** congenital heart diseases with intracardiac shunts,hemodynamic parameters are prone to change depending on the ventilation conditions and anesthetics,although few studies have examined these *** purpose of this study was to investigate the effects of two different sedation methods on the hemodynamic ***:This study retrospectively evaluated consecutive patients with ventricular septal defect(VSD)below 1 year of age who underwent cardiac catheterization at Aichi Children’s Health and Medical Center,who were divided into age-and VSD diameter-matched general anesthesia(GA)and monitored anesthesia care(MAC)under the natural airway groups(n=40 each),for comparison of hemodynamic ***:In the GA group,arterial blood pH and arterial partial pressure of oxygen were significantly higher(ppulmonary artery pressure(ppulmonary blood flow index,systemic blood flow index,and pulmonary/systemic blood flow ratio between the two ***:Cardiac catheterization under GA in VSD patients results in different hemodynamic parameters compared to that under *** particular,when using pulmonary artery pressure and pulmonary vascular resistance measured under GA for judgment regarding the surgical indications or perioperative management,consideration should be given to the fact that these parameters might be lower compared to those measured under MAC.

关键词： Ventricular septal defect cardiac catheterization general anesthesia monitored anesthesia care

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Prenatal glucocorticoid contributed to rat lung dysplasia is related to asymmetric dimethylarginine/nitric oxide pathway

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Science Bulletin 2015年第16期60卷 1416-1425页

作者： Yu-Chieh Chen Li-Tung Huang You-Lin Tain Chih-Cheng Chen Jiunn-Ming Sheen Mao-Meng Tiao Chih-Min Tsai Ho-Chang Kuo Chao-Cheng Huang Kow-Aung ChangDepartment of Anesthesiology Chang Gung Memorial Hospital-Kaohsiung Medical Center Graduate Institute of Clinical Medical Science Chang Gung University College of medicine Hong-Ren Yu Department of pediatrics Chang Gung Memorial Hospital-Kaohsiung Medical Center Graduate Institute of Clinical Medical Science Chang Gung University College of Medicine Department of Pathology Chang Gung Memorial Hospital-Kaohsiung Medical Center Graduate Institute of Clinical Medical Science Chang Gung University College of Medicine

Prenatal glucocorticoids （GCs） have been used to induce maturation of preterm fetal lungs and prevent the development of respiratory distress syndrome of the premature. pulmonary surfactant induction has been regarded as the most important effect of prenatal GCs. However, report about the prolonged effects of prenatal GCs on the development of rat lung is of limited. In this study, we tried to investigate the acute and chronic modulation effects of prenatal dexamethasone （DEX） to asymmetric dimethylarginine （ADMA）/nitric oxide （NO） signal pathway of lung tissue. Pregnant Sprague Dawley rats at gestational day 14-20 were administered i.p. DEX （0.1 mg· kg-1 ·d-1）. Acute programming effects of prenatal DEX were assessed at postnatal day 7, and long-term programming effects of offspring were assessed at day 120. We found that repetitive prenatal DEX exposure contributes to DNA oxidative damage and alveolar tissue dysplasia. Prenatal DEX treatment decreased ADMA and increased iNOS expres- sion. Prenatal DEX treatment also increased TNF-α transcript expression and decreased HDAC2 protein expression at acute stage. In conclusion, repetitive prenatal DEX has prolonged stress damage effects on lung tissue.

关键词： Prenatal glucocorticoids ADMA DNA oxidative damage TNF-α HDAC2 Lung dysplasia

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Prognostic markers for idiopathic pulmonary arterial hypertension

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Chinese Medical Journal 2014年第21期127卷 3798-3802页

作者： Guo Xiaomin Jin Hongfang Du Junbao Department of pediatrics Peking University First Hospital Beijing 100034 China

Objective The objective of this study is to review the research on the prognostic markers of idiopathic pulmonary arterial hypertension （IPAH）.Date sources We searched literature from PubMed and CNKI databases both in English and Chinese up to *** selection Data about mortality and cut-off value are from clinical trials and identified by *** IPAH is an unexplained,progressive,and rare disease characterized by increased pulmonary artery pressure and pulmonary vascular *** diagnosis is difficult,mortality of IPAH is high,and the survival periods are only 2-3 years after *** in recent years have identified a range of prognostic markers for IPAH,including the 6-minute walking test,red blood cell distribution width,and platelet levels,as well as imaging *** in these markers are important sources of information to predict the prognosis of patients with IPAH,which carries significant benefits for treatment *** Even though the prognosis of IPAH has been investigated,the mortality is also *** accurate and meaningful assessment for the prognosis of IPAH is required.

关键词： pulmonary arterial hypertension prognosis prognostic markers

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Hydrogen sulfide and vascular relaxation

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Chinese Medical Journal 2011年第22期124卷 3816-3819页

作者： SUN Yan TANG Chao-shu DU Jun-bao JIN Hong-fang Department of pediatrics Peking University First Hospital Beiiing 100034 China

Objective To review the vasorelaxant effects of hydrogen sulfide （H2S） in arterial rings in the cardiovascular system under both physiological and pathophysiological conditions and the possible mechanisms involved. Data sources The data in this review were obtained from Medline and Pubmed sources from 1997 to 2011 using the search terms ＂hydrogen sulfide＂ and ＂vascular relaxation＂. Study selection Articles describing the role of hydrogen sulfide in the regulation of vascular activity and its vasorelaxant effects were selected. Results H2S plays an important role in the regulation of cardiovascular tone. The vasomodulatory effects of H2S depend on factors including concentration, species and tissue type. The H2S donor, sodium hydrosulfide （NariS）, causes vasorelaxation of rat isolated aortic rings in a dose-dependent manner. This effect was more pronounced than that observed in pulmonary arterial rings. The expression of KATp channel proteins and mRNA in the aortic rings was increased compared with pulmonary artery rings. H2S is involved in the pathogenesis of a variety of cardiovascular diseases. Downregulation of the endogenous H2S pathway is an important factor in the pathogenesis of cardiovascular diseases. The vasorelaxant effects of H2S have been shown to be mediated by activation of KATP channels in vascular smooth muscle cells and via the induction of acidification due to activation of the Cr/HCO3 exchanger. It is speculated that the mechanisms underlying the vasoconstrictive function of H2S in the aortic rings involves decreased NO production and inhibition of cAMP accumulation. Conclusion H2S is an important endogenous gasotransmitter in the cardiovascular system and acts as a modulator of vascular tone in the homeostatic regulation of blood pressure.

关键词： hydrogen sulfide, vasorelaxing effects cardiovascular system

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ECIEN-2020 study:the effect of COVID-19 on admissions for non-COVID-19 diseases

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World Journal of pediatrics 2021年第1期17卷 85-91页

作者： Beatriz Ramos-Lacuey Mercedes Herranz Aguirre Clara Calderón Gallego Andrea Ilundain López de Munain Eva Gembero Esarte Laura Moreno-Galarraga Department of pediatrics CHNComplejo Hospitalario de NavarraServicio Navarro de SaludC/Irunlarrea 331080 PamplonaSpain Pediatric Infectious Diseases Department CHNComplejo Hospitalario de NavarraServicio Navarro de SaludPamplonaSpain Pediatric Hospitalization Department of PediatricsCHNComplejo Hospitalario de NavarraServicio Navarro de SaludPamplonaSpain IdiSNA Navarra Institute for Health ResearchPamplonaSpain Department of Preventive medicine and Public Health School of MedicineUniversity of NavarraPamplonaSpain Pediatric Pulmonology Department of PediatricsCHNComplejo Hospitalario de NavarraServicio Navarro de SaludPamplonaSpain

Background The pandemic caused by severe acute respiratory coronavirus 2(SARS-CoV-2)has had great effects on health systems worldwide,not only in relation to coronavirus disease 2019(COVID-19)cases but also affecting patients with other *** ECIEN-2020 is an observational study conducted in a tertiary referral hospital in Navarra,*** describes the effects of COVID-19 pandemic and the preventive measures adopted,in pediatric admissions for non-COVID-19 *** during March-June 2020(first wave of the COVID-19 pandemic in Spain)are described and compared with the same quarter in 2019.A sub-analysis was performed delving into *** characteristics(age,sex,past medical history),disease characteristics(symptoms,duration of symptoms,previous consultation in Primary Care Health Center),and admission characteristics(place and average stay)were *** A 33% reduction in the number of pediatric hospital admissions was observed,decreasing from 529 hospitaliza-tions in 2019 to 353 in 2020(Ppulmonary *** were no significant changes in average hospital-stay,percentage of intensive care unit admissions,or in admissions for other *** of patients admitted among those seen in the emergency department rose from 5.1%in 2019 to 10.9% in 2020,whereas the total number of consultations in the emergency department decreased by 68%.Conclusion The pandemic and the measures adopted due to SARS-CoV-2 have significantly decreased pediatric admissions for non-COVID-19 diseases,especially due to a reduction in the hospitalization for respiratory diseases.

关键词： Admission COVID-19 Hospitalization SARS-CoV-2

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Bedside cardiopulmonary ultrasonography evaluates lung water content in very low-weight preterm neonates with patent ductus arteriosus

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World Journal of Clinical Cases 2021年第8期9卷 1827-1834页

作者： Li-Fang Yu Chen-Ke Xu Min Zhao Lin Niu Xian-Mei Huang Zhi-Qun Zhang Department of Ultrasound Affiliated Hangzhou First People’s HospitalZhejiang University School of MedicineHangzhou 310006Zhejiang ProvinceChina Department of pediatrics Affiliated Hangzhou First People’s HospitalZhejiang University School of MedicineHangzhou 310006Zhejiang ProvinceChina

BACKGROUND Patent ductus arteriosus(PDA)is a common congenital heart abnormality in preterm neonates with a high incidence in neonates with very low birth *** PDA persists,interstitial lung water content increases,which could lead to abnormal circulation hemodynamics and pulmonary *** is important to perform early and reliable assessment of lung water content in very low-weight preterm neonates with persistent *** To evaluate the role of bedside cardiopulmonary ultrasonography in the lung water content assessment in very low-weight preterm neonates with persistent *** From January 2018 to March 2020,69 very low-weight preterm neonates with echocardiography-confirmed PDA were selected as the PDA *** the same time,89 very low-weight preterm neonates without PDA were randomly selected as the control *** neonates underwent echocardiography and 6-segment lung ultrasonography on the fourth day after *** clinical characteristics and main ultrasonography results were compared between the two ***’s analysis was used to analyze the correlation between lung ultrasonography score(LUS)and other related clinical and ultrasonography results in all *** the PDA group,PDA diameters were recorded,and the correlation with LUS and left atrium to aortic(LA/AO)dimension ratio were also ***/AO ratio is one of the ultrasonic diagnostic criteria for hemodynamically significant *** the ratio is≥1.5,it suggests the possibility of hemodynamic changes in persistent PDA.A receiver operating characteristic curve was established using the sensitivity of LUS to predict the hemodynamic changes in neonates with PDA as the ordinate and 1-specificity as the *** A total of 158 neonates were enrolled in this study,including 69 in the PDA group and 89 in the control *** were no statistical differences in sex,gestational age,birth weight,ventilator dependence,hospitalization length and left ventricular ejection f

关键词： Patent ductus arteriosus Cardiopulmonary Ultrasonography Lung ultrasound score Very low-weight neonates Preterm

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An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

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Congenital Heart Disease 2021年第4期16卷 393-396页

作者： Sarah Pradhan Eileen Broomall Russel Hirsch The Heart Institute Cincinnati Children’s Hospital Medical CenterUniversity of Cincinnati College of MedicineCincinnatiOhioUSA Division of Neurology Cincinnati Children’s Hospital Medical CenterDepartment of PediatricsUniversity of Cincinnati College of MedicineCincinnatiOhioUSA

The differential diagnosis for irritability in children is *** patients with congenital heart disease,one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure(CVP).In patients with single-ventricle physiology,the second stage of palliation includes bidirectional Glenn,which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ***,early in the post-operative period,patients may experience a headache due to the acute increase in CVP,and symptoms improve over *** intracranial hypertension(IIH),also known as pseudotumor cerebri,is a rare neurologic disorder in children,characterized by raised intracranial pressure(ICP)in the absence of brain parenchymal lesions or cerebrospinal fluid(CSF)*** the pathogenesis of IIH is unknown,early recognition and treatment of IIH are important to prevent permanent vision *** are only rare reports of IIH in patients with Fontan *** our knowledge,we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

关键词： Single ventricle bidirectional Glenn shunt idiopathic intracranial hypertension pseudotumor cerebri

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Significance of platelet count in children admitted with bronchiolitis

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World Journal of Clinical pediatrics 2017年第2期6卷 118-123页

作者： Amar Al Shibli Najla Alkuwaiti May Hamie Dima Abukhater Muhammad B Noureddin Abdulla Amri Salwa Al Kaabi Aysha Al Kaabi Mariam Harbi Hassib Narchi Department of pediatrics Tawam Hospital Department of pediatrics College of Medicine and Health SciencesUnited Arab Emirates University

AIM To determine the true prevalence of thrombocytosis in children less than 2 years of age with bronchiolitis,its association with risk factors,disease severity and thromboembolic *** A retrospective observational medical chart review of 305 infants aged two years or less hospitalized for *** outcomes included disease severity,duration of hospital stay,admission to pediatric intensive care unit,or *** also included complications of thrombocytosis,including thromboembolic complications such as cerebrovascular accident,acute coronary syndrome,deep venous thrombosis,pulmonary embolus,mesenteric thrombosis and arterial thrombosis and also hemorrhagic complications such as bleeding(spontaneous hemorrhage in the skin,mucous membranes,gastrointestinal,respiratory,or genitourinary tracts).RESULTS The median age was 4.7 mo and 179 were males(59%).Respiratory syncytial virus was isolated in 268(84%),adenovirus in 23(7%) and influenza virus A or B in 13(4%).Thrombocytosis(platelet count > 500 × 109/L) occurred in 88(29%;95%CI:24%-34%),more commonly in younger infants with the platelet count declining with *** was no significant association with the duration of illness,temperature on admission,white blood cell count,serum C-reactive protein concentration,length of hospital stay or admission to the intensive care *** death,thrombotic or hemorrhagic events *** Thrombocytosis is common in children under two years of age admitted with *** is not associated with disease severity or thromboembolic complications.

关键词： Hospitalization Bronchiolitis Platelet count Thrombocytosis Infant Virus diseases

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