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Pediatric asthma severity score is associated with critical care interventions 被引量:2
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作者 Danielle K Maue Nadia Krupp Courtney M Rowan 《World Journal of Clinical Pediatrics》 2017年第1期34-39,共6页
AIM To determine if a standardized asthma severity scoring system(PASS) was associated with the time spent on continuous albuterol and length of stay in the pediatric intensive care unit(PICU).METHODS This is a single... AIM To determine if a standardized asthma severity scoring system(PASS) was associated with the time spent on continuous albuterol and length of stay in the pediatric intensive care unit(PICU).METHODS This is a single center,retrospective chart review study at a major children's hospital in an urban location.To qualify for this study,participants must have been admitted to the PICU with a diagnosis of status asthmaticus.There were a total of 188 participants between the ages of two and nineteen,excluding patients receiving antibiotics for pneumonia.PASS was calculated upon PICU admission.Subjects were put into one of three categories based on PASS: ≤ 7(mild),8-11(moderate),and ≥ 12(severe).The groups were compared based on different variables,including length of continuous albuterol and PICU stay.RESULTS The age distribution across all groups was similar.The median length of continuous albuterol was longest in the severe group with a duration of 21.5 h(11.5-27.5),compared to 15(7.75-23.75) and 10(5-15) in the moderate and mild groups,respectively(P = 0.001).Thelength of stay was longest in the severe group,with a stay of 35.6 h(22-49) compared to 26.5(17-30) and 17.6(12-29) in the moderate and mild groups,respectively(P = 0.001).CONCLUSION A higher PASS is associated with a longer time on continuous albuterol,an increased likelihood to require noninvasive ventilation,and a longer stay in the ICU.This may help safely distribute asthmatics to lower and higher levels of care in the future. 展开更多
关键词 ASTHMA NONINVASIVE ventilation CRITICAL CARE ALBUTEROL
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Interprofessional, multiple step simulation course improves pediatric resident and nursing staff management of pediatric patients with diabetic ketoacidosis 被引量:4
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作者 Linnea M Larson-Williams Amber Q Youngblood +6 位作者 Dawn Taylor Peterson J Lynn Zinkan Marjorie L White Hussein Abdul-Latif Leen Matalka Stephen N Epps Nancy M Tofil 《World Journal of Critical Care Medicine》 2016年第4期212-218,共7页
AIM To investigate the use of a multidisciplinary, longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary, multiple step simulati... AIM To investigate the use of a multidisciplinary, longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary, multiple step simulation course was developed by faculty and staff using a modified Delphi method from the Pediatric Simulation Center and pediatric endocrinology department. Effectiveness of the simulation for the residents was measured with a pre- and post-test and a reference group not exposed to simulation. A follow up post-test was completed 3-6 mo after the simulation. Nurses completed a survey regarding the education activity. RESULTS Pediatric and medicine-pediatric residents(n = 20) and pediatric nurses(n = 25) completed the simulation course. Graduating residents(n = 16) were used as reference group. Pretest results were similar in the control and intervention group(74% ± 10% vs 76% ± 15%, P = 0.658). After completing the intervention, participants improved in the immediate post-test in comparison to themselves and the control group(84% ± 12% post study; P < 0.05). The 3-6 mo follow up post-test results demonstrated knowledge decay when compared to their immediate post-test results(78% ± 14%, P = 0.761). Residents and nurses felt the interdisciplinary and longitudinal nature of the simulation helped with learning.CONCLUSION Results suggest a multidisciplinary, longitudinal simulation improves immediate post-intervention knowledge but important knowledge decay occurs, future studies are needed to determine ways to decrease this decay. 展开更多
关键词 INTERDISCIPLINARY Education SIMULATION Diabetic KETOACIDOSIS PEDIATRICS
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Confidence level of pediatric trainees in management of shock states
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作者 Kavita Morparia Julie Berg Sonali Basu 《World Journal of Critical Care Medicine》 2018年第2期31-38,共8页
AIM To assess overall confidence level of trainees in assessing and treating shock, we sought to improve awareness of recurrent biases in clinical decision-making to help address appropriate educational interventions.... AIM To assess overall confidence level of trainees in assessing and treating shock, we sought to improve awareness of recurrent biases in clinical decision-making to help address appropriate educational interventions.METHODS Pediatric trainees on a national listserv were offered the opportunity to complete an electronic survey anonymously. Four commonly occurring clinical scenarios were presented, and respondents were asked to choose whether or not they would give fluid, rank factors utilized in decision-making, and comment on confidence level in their decision. RESULTS Pediatric trainees have a very low confidence level for assessment and treatment of shock. Highest confidence level is for initial assessment and treatment of shock involving American College of Critical Care Medicine/Pediatric Advanced Life Support recommendations. Children with preexisting cardiac comorbidities are at high risk ofunder-resuscitation. CONCLUSION Pediatric trainees nationwide have low confidence in managing various shock states, and would benefit from guidance and teaching around certain common clinical situations. 展开更多
关键词 Fluid BOLUS Shock Medical education Central VENOUS pressure DECISION-MAKING PEDIATRIC advanced life support guidelines
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Protein-mediated interactions in the dynamic regulation of acute inflammation
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作者 RYAN STARK 《BIOCELL》 SCIE 2023年第6期1191-1198,共8页
Protein-mediated interactions are the fundamental mechanism through which cells regulate health and disease.These interactions require physical contact between proteins and their respective targets of interest.These t... Protein-mediated interactions are the fundamental mechanism through which cells regulate health and disease.These interactions require physical contact between proteins and their respective targets of interest.These targets include not only other proteins but also nucleic acids and other important molecules as well.These proteins are often involved in multibody complexes that work dynamically to regulate cellular health and function.Various techniques have been adapted to study these important interactions,such as affinity-based assays,mass spectrometry,and fluorescent detection.The application of these techniques has led to a greater understanding of how protein interactions are responsible for both the instigation and resolution of acute inflammatory diseases.These pursuits aim to provide opportunities to target specific protein interactions to alleviate acute inflammation. 展开更多
关键词 Protein interactions INFLAMMATION SEPSIS RNA DNA THERAPEUTICS
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A Case Report of Lemierre’s Syndrome with Multisystem Dysfunction in the Setting of COVID-19 Exposure in a Pediatric Patient
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作者 Kathleen McMahon Odiraa Nwankwor +2 位作者 Christina Palladino Laura Czulada Cameron Jacobs 《Case Reports in Clinical Medicine》 2021年第9期246-251,共6页
A 17-year-old male with no significant past medical history presented to the emergency department with severe sepsis of unknown etiology. The patient was found to have septic thrombophlebitis of the internal jugular v... A 17-year-old male with no significant past medical history presented to the emergency department with severe sepsis of unknown etiology. The patient was found to have septic thrombophlebitis of the internal jugular vein with multiorgan dysfunction and septic embolization to both lungs. The patient was also noted to have COVID-19 IgM antibodies and multiple close COVID-19 exposures prior to the patient’s emergency department presentation. Here, we present the prolonged and complicated hospitalization of this patient and a review of this rare but important disease. 展开更多
关键词 Lemierre’s Syndrome SEPSIS COVID-19 THROMBOPHLEBITIS Septic Embolism
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Establishment of a Rat Model of Capillary Leakage Syndrome Induced by Cardiopulmonary Resuscitation After Cardiac Arrest
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作者 Xiao-lei ZHANG Ye CHENG +4 位作者 Chun-lin XING Jia-yun YING Xue YANG Xiao-di CAI Guo-ping LU 《Current Medical Science》 SCIE CAS 2023年第4期708-715,共8页
Objective:Cardiopulmonary resuscitation(CPR)after cardiac arrest(CA)is one of the main causes of capillary leakage syndrome(CLS).This study aimed to establish a stable CLS model following the CA and cardiopulmonary re... Objective:Cardiopulmonary resuscitation(CPR)after cardiac arrest(CA)is one of the main causes of capillary leakage syndrome(CLS).This study aimed to establish a stable CLS model following the CA and cardiopulmonary resuscitation(CA-CPR)model in Sprague-Dawley(SD)rats.Methods:We conducted a prospective,randomized,animal model study.All adult male SD rats were randomly divided into a normal group(group N),a sham operation group(group S),and a cardiopulmonary resuscitation group(group T).The SD rats of the three groups were all inserted with 24-G needles through their left femoral arteries and right femoral veins.In group S and group T,the endotracheal tube was intubated.In group T,CA induced by asphyxia(AACA)was caused by vecuronium bromide with the endotracheal tube obstructed for 8 min,and the rats were resuscitated with manual chest compression and mechanical ventilation.Preresuscitation and postresuscitation measurements,including basic vital signs(BVS),blood gas analysis(BG),routine complete blood count(CBC),wet-to-dry ratio of tissues(W/D),and the HE staining results after 6 h were evaluated.Results:In group T,the success rate of the CA-CPR model was 60%(18/30),and CLS occurred in 26.6%(8/30)of the rats.There were no significant differences in the baseline characteristics,including BVS,BG,and CBC,among the three groups(P>0.05).Compared with pre-asphyxia,there were significant differences in BVS,CBC,and BG,including temperature,oxygen saturation(SpO_(2)),mean arterial pressure(MAP),central venous pressure(CVP),white blood cell count(WBC),hemoglobin,hematocrit,pH,pCO_(2),pO_(2),SO_(2),lactate(Lac),base excess(BE),and Na+(P<0.05)after the return of spontaneous circulation(ROSC)in group T.At 6 h after ROSC in group T and at 6 h after surgery in groups N and S,there were significant differences in temperature,heart rate(HR),respiratory rate(RR),SpO_(2),MAP,CVP,WBC,pH,pCO_(2),Na+,and K+among the three groups(P<0.05).Compared with the other two groups,the rats in group T showed a significantly increased W/D weight ratio(P<0.05).The HE-stained sections showed consistent severe lesions in the lung,small intestine,and brain tissues of the rats at 6 h after ROSC following AACA.Conclusion:The CA-CPR model in SD rats induced by asphyxia could reproduce CLS with good stability and reproducibility. 展开更多
关键词 cardiac arrest cardiopulmonary resuscitation capillary leakage model
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Characteristics of In-Hospital Patients with Congenital Heart Disease Requiring Rapid Response System Activations: A Japanese Database Study
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作者 Taiki Haga Tomoyuki Masuyama +3 位作者 Yoshiro Hayashi Takahiro Atsumi Kenzo Ishii Shinsuke Fujiwara 《Congenital Heart Disease》 SCIE 2022年第1期31-43,共13页
Objectives:This study aimed to study the characteristics of in-hospital deterioration in patients with congenital heart disease who required rapid response system activation and identify risk factors associated with 1... Objectives:This study aimed to study the characteristics of in-hospital deterioration in patients with congenital heart disease who required rapid response system activation and identify risk factors associated with 1-month mortality.Methods:We retrospectively analysed data from a Japanese rapid response system registry with 35 participating hospitals.We included consecutive patients with congenital heart disease who required rapid response system activation between January 2014 and March 2018.Logistic regression analyses were performed to examine the associations between 1-month mortality and other patient-specific variables.Results:Among 9,607 patients for whom the rapid response system was activated,only 82(0.9%)had congenital heart disease.Only few patients with congenital heart disease were being treated at the cardiology and cardiovascular surgery departments(12.3%and 9.9%,respectively).Moreover,the incidences of rapid-response events after intensive care unit discharge or surgery were low(6.8% and 12.2%,respectively).The most common reason for rapid response system activation was respiratory dysfunction(desaturation:35.4%,tachypnoea:25.6%,and new dyspnoea:19.5%).Rapid response system interventions and intensive care unit transfers were required for 65.9% and 20.7%of patients,respectively.The mortality rate was 1.2% at the end of the rapid response system intervention and 11.0% after 1 month.Moreover,decreased respiratory rate and decreased heart rate at rapid response system activation were associated with increased 1-month mortality.The adjusted odds ratio was 1.10(95% confidence interval 1.02–1.19)and 1.02(95% confidence interval,1.00–1.04) for respiratory rate and heart rate,respectively.Conclusions:Rapid response systems were rarely activated after cardiac surgery and intensive care unit discharge,which were situations with a high risk of sudden deterioration in patients with congenital heart disease.Therefore,encouraging the use of the rapid response system in these departments will enable intervention by a third,specialised team for in-hospital emergencies and help provide comprehensive medical care to patients.Furthermore,1-month mortality was associated with vital signs at rapid response system activation.These findings may guide treatment selection for patients with congenital heart disease showing deterioration. 展开更多
关键词 Heart defects CONGENITAL EMERGENCIES clinical deterioration hospital rapid response team critical care This
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Promoting Medication Adherence to Asthma 被引量:1
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作者 Weerapong Lilitwat Yuttiwat Vorakunthada 《Health》 2018年第1期13-19,共7页
Promoting adherence to asthma treatment is an essential aspect of clinical practice. Approximately 60% of asthmatic patients are non-adherent to asthma regimen, resulting in adverse outcomes and higher costs of care. ... Promoting adherence to asthma treatment is an essential aspect of clinical practice. Approximately 60% of asthmatic patients are non-adherent to asthma regimen, resulting in adverse outcomes and higher costs of care. Non-adherence could be intentional (perceptions of asthma severity, self-manage therapy, fear of side-effects) or non-intentional (forgetful, cost, and misunderstandings). Adherence can be evaluated by patient’s reporting, dose counter, electronic metered dose inhaler but using pharmacy records is a more cost-effective method. The most successful strategies to improve patients’ adherence is to utilize the principle of patient-centered collaborative care and effective communication. Keys of communication skills consist of establishing a relationship, listening, collaborating on the treatment plan, time management, and implementing effective follow-up interventions. Interventions to improve adherence include providing reinforcement for patients’ efforts to change, providing feedback on progress, tailoring education to patients’ needs and circumstances and follow-up. Evaluation of health literacy is mandatory for prioritizing information from most to least critical, speaking slowly, avoiding medical jargon, and spending minimal extra time during each visit. Communication technology including texting or interactive voice response is another new strategy that can increase adherence. 展开更多
关键词 ADHERENCE ASTHMA EDUCATION COMMUNICATION HEALTH LITERACY
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Mortality and morbidity of acute hypoxemic respiratory failure and acute respiratory distress syndrome in infants and young children 被引量:11
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作者 ZHU Yan-feng XU Feng +11 位作者 LU Xiu-lan WANG Ying CHEN Jian-li CHAO Jian-xin ZHOU Xiao-wen ZHANG Jian-hui HUANG Yan-zhi YU Wen-liang XIE Min-hui YAN Chao-ying LU Zhu-jin SUN Bo 《Chinese Medical Journal》 SCIE CAS CSCD 2012年第13期2265-2271,共7页
Background Acute hypoxemic respiratory failure (AHRF) often develops acute respiratory distress syndrome (ARDS), and its incidence and mortalities in critically ill pediatric patients in China were 2% and 40% resp... Background Acute hypoxemic respiratory failure (AHRF) often develops acute respiratory distress syndrome (ARDS), and its incidence and mortalities in critically ill pediatric patients in China were 2% and 40% respectively. This study aimed at prospectively investigating incidence, causes, mortality and its risk factors, and any relationship to initial tidal volume (VT) levels of mechanical ventilation, in children 〈5 years of age with AHRF and ARDS. Methods In 12 consecutive months in 23 pediatric intensive care units (PICU), AHRF and ARDS were identified in those requiring 〉12 hour intratracheal mechanical ventilation and followed up for 90 days or until death or discharge. ARDS was diagnosed according to the American-European Consensus definitions. The mortality and ventilation free days (VFD) were measured as the primary outcome, and major complications, initial disease severity, and burden were measured as the secondary outcome. Results In 13 491 PICU admissions, there were 439 AHRF, of which 345 (78.6%) developed ARDS, resulting in incidences of 3.3% and 2.6%, and corresponding mortalities of 30.3% and 32.8% respectively along with 8.2 and 6.7 times of relative risk of death in those with pneumonia (62.9%) and sepsis (33.7%) as major underlying diseases respectively. No association was found in VT levels during the first 7 days with mortality, nor for V-r at levels 〈6, 6-8, 8-10, and 〉10 ml/kg in the first 3 days with mortality or length of VFD. By binary Logistic regression analyses, higher pediatric risk of mortality score III, higher initial oxygenation index, and age 〈1 year were associated with higher mortality or shorter VFD in AHRF. Conclusions The incidence and mortalities of AHRF and ARDS in children 〈5 years were similar to or lower than the previously reported rates (in age up to 15 years), associated with initial disease severity and other confounders, but causal relationship for the initial VT levels as the independent factor to the major outcome was not found. 展开更多
关键词 acute respiratory distress syndrome HYPOXEMIA respiratory failure MORBIDITY MORTALITY respiratory therapy
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Are incidence and severity of clubfoot related to the season of birth? 被引量:1
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作者 Da-Hang Zhao Wei-Wei Rao +5 位作者 Li Zhao Xuan Yang Jian-Lin Liu Zhen-Kai Wu Qing Du Xiao-Yan Yang 《World Journal of Pediatrics》 SCIE CSCD 2016年第3期360-363,共4页
Background:This study was designed to determine whether the occurrence of clubfoot follows a seasonal pattern in neonates from eastern and south-eastern China and to speculate the potential etiology of clubfoot.Method... Background:This study was designed to determine whether the occurrence of clubfoot follows a seasonal pattern in neonates from eastern and south-eastern China and to speculate the potential etiology of clubfoot.Methods:We reviewed 239 neonates with clubfeet during a period of 4 years as well as the monthly neonatal population of the Sixth National Population Census.Seasonal variations in terms of month of birth and severity were analyzed.Results:The incidence of clubfoot in neonates from eastern and south-eastern China showed seasonal variations,and the incidence was higher in autumn with a reference to the average birth rate in this corresponding area.No signifi cant difference was found in severity of clubfoot.Conclusion:This seasonal pattern is of signifi cant value to further understanding the etiology and pathogenesis of clubfoot in the corresponding area of China. 展开更多
关键词 CLUBFOOT ETIOLOGY SEASONALITY
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Performance of the PRISM I,PIM2,PELOD-2 and PRISM IV scoring systems in western China:a multicenter prospective study 被引量:1
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作者 Xue-Peng Zhang Yun-Xia Feng +14 位作者 Yang Li Guo-Yan Lu Xin-Yue Zhou Can-Zheng Wei Xi-Ying Gui Kai-Ying Yang Tong Qiu Jiang-Yuan Zhou Hua Yao Geng Zhang Wen-Qi Zhang Yu-Hang Hu Hong Wu Si-Yuan Chen Yi Ji 《World Journal of Pediatrics》 SCIE CAS CSCD 2022年第12期818-824,共7页
Background The aim of this study was to evaluate the performance of the four scoring tools in predicting mortality in pediatric intensive care units(PICUs)in western China.Methods This was a multicenter,prospective,co... Background The aim of this study was to evaluate the performance of the four scoring tools in predicting mortality in pediatric intensive care units(PICUs)in western China.Methods This was a multicenter,prospective,cohort study conducted in six PICUs in western China.The performances of the scoring systems were evaluated based on both discrimination and calibration.Discrimination was assessed by calculating the area under the receiver operating characteristic curve(AUC)for each model.Calibration was measured across defined groups based on mortality risk using the Hosmer-Lemeshow goodness-of-fit test.Results A total of 2034 patients were included in this study,of whom 127(6.2%)died.For the entire cohort,AUCs for Pediatric Risk of Mortality Score(PRISM)I,Pediatric Index of Mortality 2(PIM2),Pediatric Logistic Organ Dysfunction Score-2(PELOD-2)and PRISM IV were 0.88[95%confidence interval(CI)0.85–0.92],0.84(95%CI 0.80–0.88),0.80(95%CI 0.75–0.85),and 0.91(95%CI 0.88–0.94),respectively.The Hosmer-Lemeshow goodness-of-fit Chi-square value was 12.71(P=0.12)for PRISM I,4.70(P=0.79)for PIM2,205.98(P<0.001)for PELOD-2,and 7.50(P=0.48)for PRISM IV[degree of freedom(df)=8].The standardized mortality ratios obtained with the PRISM I,PIM2,PELOD-2,and PRISM IV models were 0.87(95%CI,0.75–1.01),0.97(95%CI,0.85–1.12),1.74(95%CI,1.58–1.92),and 1.05(95%CI,0.92–1.21),respectively.Conclusions PRISM IV performed best and can be used as a prediction tool in PICUs in Western China.However,PRISM IV needs to be further validated in NICUs. 展开更多
关键词 Illness severity score Intensive care Mortality prediction PEDIATRIC
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Hospital-based emergency department visits in children with motor vehicle traffic accidents: estimates from the nationwide emergency department sample
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作者 Veerajalandhar Allareddy Ingrid M Anderson +3 位作者 Min Kyeong Lee Veerasathpurush Allareddy Sankeerth Rampa Romesh P Nalliah 《World Journal of Pediatrics》 SCIE CSCD 2015年第3期261-266,共6页
Background:The purpose of this study is to provide nationally representative estimates of children visiting hospital-based emergency departments(ED)for motor vechicle traffic accidents(MVTA)in the United States during... Background:The purpose of this study is to provide nationally representative estimates of children visiting hospital-based emergency departments(ED)for motor vechicle traffic accidents(MVTA)in the United States during the year of 2008.Methods:Nationwide Emergency Department Sample for 2008 was used.All pediatric(age<18 years)ED visits with external cause for injury ICD-9-diagnostic codes for MVTA were selected.Outcomes examined included discharge status following ED visit and presence of concomitant injuries.Descriptive statistics was used to summarize the estimates.Results:Totally 604027 hospital-based ED visits occurred in the United States among children(age≤18 years)due to MVTA.Following an ED visit,91%were discharged routinely,while 6%were admitted as inpatients into the same hospital.A total of 928 children died in the ED.A total of 34004 ED visits required inpatient admission into the same hospital and 768 patients died during hospitalization.Mean charge per ED visit was$1887 and total ED charges across the United States were close to$970 million.Among those admitted into the same hospital following ED visit(n=34004),the mean hospitalization charge was$53726 and total hospitalization charge across the entire United States were$1.8 billion.Conclusions:Study findings illustrate the burden associated with pediatric ED visits due to MVTA.Close to$970 million of hospital charges were incurred by children who made an ED visit due to a MVTA during 2008 and about$1.8 billion was incurred among those hospitalized following an ED visit. 展开更多
关键词 EMERGENCY HOSPITAL traffic accidents
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Outcomes of complete surgical repair versus palliative intervention in neonates with Tetralogy of Fallot
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作者 Mohammed Hamzah Hasan FOthman +3 位作者 Krystel Chedid Mohammed Alsabri Ibrahim Qattea Hany Aly 《Pediatric Investigation》 CAS CSCD 2022年第4期260-263,共4页
The ideal surgical approach when managing neonates with Tetralogy of Fallot(TOF)is still controversial.1 In 1945,subclavian artery to pulmonary artery anastomosis was created as palliation for patients with TOF.2 A de... The ideal surgical approach when managing neonates with Tetralogy of Fallot(TOF)is still controversial.1 In 1945,subclavian artery to pulmonary artery anastomosis was created as palliation for patients with TOF.2 A decade later,Lillehei et al.3 performed the first intracardiac repair for TOF with ventricular septal defect closure and right ventricular outflow obstruction relief.The surgical and perfusion techniques,as well as the postoperative care,have advanced and evolved since that time.Despite that evolution,the two concepts of surgical interventions had remained the same.There is still much debate on what is the ideal surgical approach to infants with TOF,an early primary surgical repair(EPSR)versus a two-staged surgical approach with either an initial temporary shunt for pulmonary blood flow or transcatheter cardiac palliation.The aim of this study was to determine if one strategy had lower mortality than the other and to assess the hospital length of stay and cost of charge in the study groups. 展开更多
关键词 SURGICAL nates repair
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Status epilepticus due to fructose-1,6-bisphosphatase deficiency caused by FBP1 gene mutation 被引量:1
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作者 Shiyue Mei Chao Ma +2 位作者 Yibing Cheng Suyun Qian Zhipeng Jin 《Pediatric Investigation》 CSCD 2019年第2期122-126,共5页
Introduction:Fructose-1,6-bisphosphatase (FBPase) deficiency is a rare inherited disorder in gluconeogenesis,characterized by hypoglycemia,ketonuria,metabolic acidosis and convulsions.Case presentation:We describe two... Introduction:Fructose-1,6-bisphosphatase (FBPase) deficiency is a rare inherited disorder in gluconeogenesis,characterized by hypoglycemia,ketonuria,metabolic acidosis and convulsions.Case presentation:We describe two brothers with FBPase deficiency.The proband developed severe hypoglycemia and progressed to status epilepticus,and the brother showed slightly hypoglycemia with a good prognosis.Whole exome sequencing (WES) identified compound heterozygous variants [c.333+1333+2delinsTC and c.490G>A (p.Gly164Ser)] in fructose-1,6-bisphosphatase 1 gene in the two brothers,which were inherited from the father and the mother,respectively.Conclusion:Genetic analysis provided a solid basis for a definite diagnosis and the determination of precision therapies for the patient. 展开更多
关键词 Fructose-1 6-bisphosphatase DEFICIENCY HYPOGLYCEMIA Status epilepticus FBP1 MUTATION
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Noninvasive ventilation in a young infant with congenital central hypoventilation and 7-year follow-up 被引量:1
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作者 Zhifei Xu Yunxiao Wu +3 位作者 Bei Li Li Zheng Jingyuan Liu Kunling Shen 《Pediatric Investigation》 CSCD 2019年第4期261-264,共4页
Introduction:Congenital central hypoventilation syndrome(CCHS)is a rare disorder characterized by alveolar hypoventilation and autonomic system dysregulation secondary to mutations of the PHOX2B gene.Treatment consist... Introduction:Congenital central hypoventilation syndrome(CCHS)is a rare disorder characterized by alveolar hypoventilation and autonomic system dysregulation secondary to mutations of the PHOX2B gene.Treatment consists of assisted ventilation using positive-pressure ventilators via tracheostomy,bi-level positive airway pressure(BPAP)via a noninvasive interface,negative-pressure ventilators,or diaphragm pacing.The long-term use of BPAP in younger children at home has been less frequently reported.Case presentation:We present a case of a 2-month-old infant with CCHS who was successfully managed by BPAP without the need for tracheostomy and followed up for 7 years.Conclusion:CCHS is a rare disease that manifests as nocturnal desaturation and carbon dioxide retention in early life.Noninvasive ventilation can be successfully used in young infants via an appropriate mask. 展开更多
关键词 CONGENITAL CENTRAL HYPOVENTILATION syndrome PHOX2B gene NONINVASIVE ventilation
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Fatal encephalitis accompanied by ARDS in a child with HSV infection
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作者 Lili Xu Jun Liu +4 位作者 Yilin Pang Xiaolei Guan Suyun Qian Zhengde Xie Hengmiao Gao 《Pediatric Investigation》 2018年第3期198-202,共5页
INTRODUCTION Herpes simplex virus (HSV) is a ubiquitous human pathogen that causes a range of diseases from mild uncomplicated mucocutaneous infection to life-threatening conditions.1 HSV-1 is normally associated with... INTRODUCTION Herpes simplex virus (HSV) is a ubiquitous human pathogen that causes a range of diseases from mild uncomplicated mucocutaneous infection to life-threatening conditions.1 HSV-1 is normally associated with orofacial infections,whereas HSV-2 usually causes genital infections and can be transmitted from infected mothers to neonates.2 Less common manifestations of HSV infection,such as meningitis,encephalitis,3,4 hepatitis,5 and pneumonitis,6,7 can occur in both children and adults.After primary infection by HSV,the virus establishes a lifelong latent infection in the neuronal district,resulting in reactivation due to various triggering factors. 展开更多
关键词 ARDS CHILD HSV INFECTION
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