Objective Mutations in polymerase gamma gene (POLG) are believed to be an important cause of early and juvenile onset of non-syndromic intractable epilepsy. The aim of this study is to investigate the incidence/prevalence of POLG pathogenic variants in epilespy patients of Han population through sequencing it.Methods Han Chinese patients with seizures prior valproic acid (VPA) exposure at Shanghai Children's Hospital were collected from 2015 to 2019. The clinical diagnosis was based on the 2014 Consensus Statement of Epilepsy by the International League against Epilepsy (ILAE). Blood sampling were performed before VPA treatment. The POLG gene DNA was sequenced by either the first or the next generation sequencing (NGS). The POLG variant burden was illustrated. Liver functions were tested to describe whether they experienced VPA toxicity. Results Totally 216 Han Chinese patients were included, aged from 1 month to 15 years old, 102 were male and 114 were female. The onset age was 1 month old to 13 years old, and the epilepsy course ranged from 2 weeks to about 3 years. VPA treatment was delivered for the generalized or intractable partial seizures at standard dosage. No patient experienced hepatic toxicity following VPA exposure. DNA sequencing data showed no patient had either a homozygous mutation or compound heterozygous mutation of POLG. Single heterozygous mutations of c.1150G>T and p.D384Y were found in 2 patients, and single heterozygous mutation of c.156_158dupGCA was found in 1 patient. None of these variants showed clinical significance. Conclusions Functional modifying POLG homozygous mutations and compound heterozygous mutations were not detected and VPA toxicity was not seen in the current study. POLG mutation frequency might be rare in Han Chinese, and standard VPA therapeutic dosage might be safe for Han Chinese patients.

Objective To study the clinical efficacy and safety of extracorporeal membrane oxygenation (ECMO) in critically ill neonates.Method From November 2016 to September 2018,the clinical data of 5 cases who received ECMO treatment in NICU of our hospital were retrospectively analyzed.The indication of ECMO was reversible respiratory failure irresponsive to conventional therapy.The treatment mode was V-A ECMO.Oxygenation index (OI),vasoactive-inotropic score,blood lactate before and 24 h after ECMO were recorded.Complications of ECMO were also studied.Paired t-test was used to compare the pre and post treatment parameters.Result Among the 5 cases,4 cases were male and 1 case was female.3 cases were diagnosed with meconium aspiration syndrome,2 cases pulmonary hypertension.OI[(9.5 ± 1.8) vs.(60.6 ± 19.4)],vasoactive-inotropic score[(19.5 ± 12.0) points vs.(204.0 ± 143.8) points]and blood lactate [(2.8 ± 1.5) mmol/L vs.(9.6 ± 3.6) mmol/L]) were all significantly decreased at 24 h after ECMO treatment (P ＜ 0.05).During follow-up,3 cases survived,2 cases died.All the 5 cases showed thrombocytopenia,3 cases developed renal failure and received continuous renal replacement therapy,1 case got intracranial hemorrhage.2 of the 3 survived cases developed neurological impairment and need long term follow-up and rehabilitation therapy.Conclusion ECMO treatment has remarkable effects on critically ill neonates and may actually save lives,but the risk of complications are quite high.

Objective To evaluate the value of nested polymerase chain reaction (PCR) for the rap-id detection of pathogens in children with severe pneumonia. Methods We prospectively enrolled the pa-tients with severe community-acquired pneumonia admitted to pediatric intensive care unit (PICU) in Shang-hai Children′s Hospital from January 2017 to June 2018. The sputum for PCR were collected within 24 h after PICU admission. Both nested PCR and routine microbiological methods were performed. Respiratory Panel (R-Panel) based on nested PCR could detect 17 kinds of respiratory pathogen at the same time. Results A total of 65 patients were enrolled in this study and the samples were detected using both R-Panel and routine microbiological method. (1) A total of 15 patients (23. 08 %) showed positive routine microbiological de-tection including 13 cases with virus-positive and 2 cases with mycoplasma-positive; (2) A total of 38 pa-tients (58. 46%) showed positive results using R-Panel within 2 h including 46 cases with virus-positive and 5 cases with mycoplasma-positive. The mainly primary infection was human rhinovirus and enterovirus in 15 cases (23. 08%),followed by adenovirus in 10 cases (15. 38%); and the positive rate of 2 or more patho-gens was 18. 46% (12/65);(3) The rate of adenovirus-positive using R-Panel was significantly higher than that using routine microbiological methods (15. 38 % vs. 4. 62%,χ2 ＝4. 188,P＝0. 041); the sensitivity of R-Panel for detection of adenovirus,respiratory syncytial virus,and parainfluenza virus was significantly high-er than those of routine microbiological methods (100% vs. 30%,χ2 ＝107. 692;50. 00% vs. 16. 67%,χ2 ＝ 24. 442;100% vs. 80%,χ2 ＝22. 222;100% vs. 40%,χ2 ＝85. 714; all P <0. 001). Conclusion R-Panel using nested PCR is a rapid,sensitive,and specific method for the detection of pathogens in children with severe community acquired pneumonia,which is valuable for targeted therapy in time.

Objective To evaluate the diagnostic value of A-F mnemonic performed by sonographers with limited experience in patients with acute chest pain. Methods This was a prospective observational study. Bedside cardiac ultrasound for patients with chest pain was performed by six sonographers with limited experience using A-F mnemonic, evaluating indexes including aortic dissection(A), both ventricles (B), regional wall motion abnormality (RWMA), left ventricular ejection fraction (LVEF) ≤ 50％ (C ,contractility), dimensions (D), pleural and pericardial effusion (E) and further abnormalities (F). Afterwards, experienced cardiac sonographers performed the same examinations, and the difference in the time of ultrasound examination was calculated. The diagnosis of experienced sonographers were referred as the control group, and kappa test was applied to analyze the sensitivity, specificity, positive predictive value and negative predictive value. Results There were 245 cases eligible for study, and 20 cases were excluded. Finally 225 cases of acute chest pain were included in the analysis, containing 158 fatal chest pain and 67 low-risk chest pain. The experienced sonographers diagnosed 20 cases of ascending aortic dissection, 5 cases of right ventricular dilatation, 72 cases of RWMA, 12 cases of LVEF ≤ 50％, 45 cases of left ventricular dilatation, 6 cases of hydropericardium, and 6 cases of other abnormalities. The consistency between beginners and experienced sonographers were as follows:completely same (hydropericardium, Kappa=1.000), highly consistent (ascending aortic dissection, Kappa=0.853, right ventricular dilatation, Kappa=0.931, and other abnormalities, Kappa=0.829), moderately consistent (RWMA, Kappa=0.768, LVEF ≤ 50％, Kappa=0.713 and left ventricular dilatation, Kappa=0.766). The sensitivity and negative predictive value of RWMA and LVEF ≤ 50％ and the positive predictive value of left ventricular dilatation in the beginner sonographers were lower than those in the experienced sonographers. Conclusions A-F mnemonic was a simple and practical way for the beginner sonographers to perform bedside cardiac ultrasound. It was of significant value in making correct diagnosis of most acute chest pain patients and providing quick and reliable information for clinicians.

Objective: To survey the conduction and evaluate the effectiveness of extracorporeal membrane oxygenation (ECMO) therapy in pediatric intensive care unit (PICU) in China mainland. Methods: In a questionnaire-based survey, we retrospectively reviewed the application of ECMO in children's hospital and general hospital in China mainland to summarize and analyze the categories of diseases and prognosis of children treated with ECMO therapy. Results: By December 31, 2017, a total of 23 hospitals using ECMO, including 22 tertiary referral hospitals and 1 secondary hospital, among which 16 were children′s hospitals and 7 were general hospitals. Thirty-seven ECMO equipment was available. A total of 518 patients treated with ECMO, within whom 323 (62.4%) successfully weaned from ECMO and 262 (50.6%) survived to discharge. Among 375 pediatric patients, 233 (62.1%) were successfully weaned from ECMO and 186 (49.6%) survived to discharge. Among 143 newborn patients, 90 (62.9%) successfully weaned from ECMO, 76 (53.1%) survived to discharge. ECMO was applied in veno-arterial (VA) mode to 501 (96.7%) patients, veno-venous (VV) mode to 14 (2.7%) patients, and VV-VA conversion mode to 3 (0.6%) patients. Sixty-nine patients required extracorporeal cardiopulmonary resuscitation (ECPR), including 20 newborn patients (29.0%) and 38 pediatric patients (71.0%), who were all with cardiovascular disease. Neonatal respiratory distress syndrome (26/61), persistent pulmonary hypertension of the newborn (PPHN) (12/61), and meconium aspiration syndrome (MAS) (11/61) are the most common pulmonary diseases in newborn patients; among whom, infants with PPHN had highest survival rate (10/12), followed by MAS (9/11). Among newborn patients with cardiovascular diseases, those who admitted were after surgery for congenital cardiac disease were the most common (54/82), while those with septic shock had the highest survival rate (2/3). In pediatric pulmonary diseases, acute respiratory distress syndrome was the most common (42/93), while plastic bronchitis was with the highest survival rate (4/4), followed by viral pneumonia (13/16). Among pediatric cardiovascular diseases, congenital cardiac defect was the most common (124/282), while fulminant myocarditis had the highest survival rate (54/77). Conclusion The application of ECMO as a rescue therapy for children with severe cardiopulmonary failure has dramatically developed in China mainland.

Objective To develop a machine simultaneously integrating mechanized environmental cleaning and automatic mollusciciding and to evaluate its effectiveness of field application,so as to provide a novel Oncomelania hupensis snail control technique in the large?scale marshlands. Methods The machine simultaneously integrating mechanized environmental clean?ing and automatic mollusciciding,which was suitable for use in complex marshland areas,was developed according to the mech?anization and automation principles,and was used for O. hupensis snail control in the marshland. The effect of the machine on environmental cleaning and plough was evaluated,and the distribution of living snails was observed at various soil layers follow? ing plough. The snail control effects of plough alone and plough followed by mollusciciding were compared. Results The ma?chine could simultaneously complete the procedures of getting vegetation down and cut vegetation into pieces,plough and snail control by spraying niclosamide. After plough,the constituent ratios of living snails were 36.31% ,25.60% ,22.62% and 15.48% in the soil layers at depths of 0-5,6-10,11-15 cm and 16-20 cm respectively,and 61.91% living snails were found in the 0-10 cm soil layers. Seven and fifteen days after the experiment,the mortality rates of snails were 9.38% and 8.29% in the plough alone group,and 63.04% and 80.70% in the plough + mollusciciding group respectively(c27 d = 42.74,c215 d =155.56,both P values < 0.01). Thirty days after the experiment,the densities of snails were 3.02 snails/0.1 m2 and 0.53 snails/0.1 m2 in the soil surface of the plough alone group and the plough + mollusciciding group,which decreased by 64.92% and 93.60% ,respectively,and the decrease rate of snail density was approximately 30% higher in the plough + mollusciciding group than that in the plough alone group. Conclusions The machine simultaneously integrating mechanized environmental cleaning and automatic mollusciciding achieves the integration of mechanical environmental cleaning and automatic niclosamide spraying in the complex marshland areas,which provides a novel technique of field snail control in the large?scale setting in Chi?na.

Objective To clinically observe and verificate the advantages of treating infantile anorexia of spleen-dysfunction in transportation with infantile massage and Chinese medicine based on the thought of “invigo-rating spleen and nourishing qi”. Methods Multi-central large-sample randomized controlled trial was carried out in Guangzhou, Shenzhen, and Foshan. Five hundreds cases of infantile anorexia of spleen-dysfunction in transportation were randomized into infantile massage group, Chinese medicine group, infantile massage combined with Chinese medicine group and Western medicine group and two sessions of treatment were required in each group. Separately, during the enrollment, after 2 sessions of treatment, infantile anorexia signs and symptoms scale , the related therapeutic effect criterion from clinical guideline of new drugs for traditional Chinese medicine, the expression level of hemoglobin and blood zinc were adopted for efficacy assessment. Results A mong 4 groups , the recovery rate and the total effective rate of infantile massage combined with Chinese medicine group were better than those of other groups (P < 0.05). The expression level of hemoglobin and blood zinc of 4 groups both increased obviously (all P < 0.01), but more improvement was found in infantile massage combined with Chinese medicine group (both P < 0.05). Conclusion The treatment of infantile anorexia with infantile massage and Chinese medicine based on the thought of invigorating spleen and nourishing qi has a significant effect.

Objective To investigate the clinicalmanifestation,monitoring and therapeutic measure of severe enterovirus 71 ( EV71 ) infection in children.MethodsForty-five cases of severe EV71 infectionwere admitted in our PICU from May 2010 to Sep 2011.The vital sign and arterial blood pressure,central venous pressure,mixed venous oxygen saturation,dynamic non-invasive heart function and urine volume were monitored.Forty-five cases were divided into 3 stages according to clinical manifestation:( 1 ) nervous system involvement stage; (2) respiratory system involvement stage; ( 3 ) circulatory system involvement stage ( compensation and decompensation).We adopted individualized remedy measure according to different stages.ResultsIn 45 cases,38 cases discharged from hospital,the cure rate was 84.4％.Among all the 38 cases,nervous system involvement was found in 19 cases,respiratory system involvement was found in 12 cases,circulatory system involvement was found in 7 cases.Seven cases died,who had circulation failure.ConclusionWe should identify severe EV71 infection early.Positive control of high fever,appropriate liquid treatment,control of high blood pressure,early respiratory support,preventment of circulation failure are the key measures for treatment.Individualized monitoring and treatment are effective in children with severe EV71 infection.

Objective To explore the clinical characteristics of critically ill children infected with pseudomonas aeruginosa(PA) and PA antibiotics resistance in pediatric intensive care unit (PICU).Methods Case records of children with PA infection admitted to PICU in children′s hospital affiliated to Shanghai Jiaotong University from Jan 2007 to Dec 2009 were reviewed for clinical characteristics,case fatality rate,prognosis and drug resistance.Results (1) Clinical features:12 cases were community-acquired infection and 46 cases were hospital-acquired infections in 58 cases.On the same period,hospital-wide surveillance obtained PA 232 strains,PICU obtained PA 112,the ratio was 48.3%.Twelve cases died and total mortality was 20.7%.The mortality was significantly difference between community-acquired infections (5 cases,41.6%)and hospital-acquired infections (7 cases,15.2%)(P<0.05).The main symptom of children with community-acquired infections were intestinal infection (5 cases) and sepsis (5 cases).The children had acute onset and developed to shock and multiple organ dysfunction syndrome rapidly.Laboratory examination revealed the white blood cell normal (7/12) and decreased in 5 cases (5/12).The value of C-reactive protein was increased significantly,and the concentration of blood endotoxin were also increased.In the hospital-acquired PA infection cases,the main symptom was respiratory abnormal (38 cases),worsen primary disease,extended staying days in PICU.(2)Drug resistance analysis:112 PA,69.8% of ceftazidime-resistant,72.8% of the imipenem-resistant.Conclusion There is significant difference of the clinical features between PA community-acquired infection and hospital-acquired infection.The former is mostly primary infections with high fatality rate.PA hospital-acquired infection has become an important pathogen of nosocomial infection in PICU.And it is important to prevent PA infection caused by a long term broad-spectrum antibiotics application and invasive medical procedures.

Objective To develop a new method to measure pleural effusion volume by ultrasound in critically ill patients. Methods Forty-six critically ill patients admitted to emergency ICU were involved.The height of effusion (H),area of effusion at the middle section (S), thickness of effusion at middle-back line (T1) and posterior axillary line (T2) were measured by ultrasound in supine position at the end of expiration. The measured volume of pleural effusion (Vc) was calculated by H×S,and the actual volume of drainage (V) within 2 hours was also recorded. The correlation of actual volume of pleural effusion (V)with effusion height (H) ,thickness (T1, T2), area (S) and the calculated volume (Vc) were analyzed to decide the most accurate index and method. Results There was much better correlation between actual volume of effusion and S, (H & S), Vc, than these between V and T1 ,T2, H in all patients and subgroup, Vc had good correlation with V and very close to V(the average difference was 56 ml) when the actual volume was less than 500 ml,there was no difference[(417 ± 94)ml vs (402±95)ml, t = 1.095, P = 0. 285]. Both Logistic regression analysis and receiver operating characteristic (ROC) curve showed S was the most reliable index to predict the actual volume to exceed 500 ml,400 ml,and 300 ml when compared with H,S,T1 and T2. The corresponding threshold was 30.3 cm2 , 28.3 cm2 and 23. 1 cm2 , with the sensitivity and specificity of 0. 77 and 0. 88,0.72 and 1.0,0.95 and 1.0, respectively. Conclusions This new method based on measuring the area of effusion by ultrasound is more efficient and reliable than those traditional ones to measure the volume of pleural effusion. It's clinically valuable and easy to perform, and deserves broad application.