Objective To study the antagonism to chemokine MCP-1 of MC148 gene which homogued human?chemokines mediated by adenoviral vector.Methods MC148 was amplified from DNA of molluscum contagiosum virus (MCV) isolates and sequenced. The product was co-tranfected into adenovirus vector to construct recombinant Ad-MC148. MC148P protein was obtained from the supernatant of 293 cells transfected with recombinant Ad-MC148 and served as antagonist against the recruitment of monocyte to MCP-1 in chemotaxis inhibition assay.Results MC148 gene was cloned, the sequence of which was identical to that reported in GenBank. Recombinant Ad-MC148 was constructed successfully. MC148P could antagonize the chemotaxis of monocyte to MCP-1. Conclusions MC148 could antagonize chemokine MCP-1 and inhibit chemotaxis of monocyte. MC148 is an antagonist against MCP-1. This study might provide a novel way to control immune response.

Objective To understand the patients of congenital microtia malformation families knowledge of skin expander and influencing factors. Methods Self-made questionnaire to sample survey of 500 cases of our department (Plastic Surgery Hospital, Chinese Academy of Medical Sciences, the second microtia concer) patients′ families. Results 47.8％(239/500) of 500 patients of expander knowledge level is high, 41.2％(206/500) pass the exam, 11.0％(55/500) fall the exam, only 13.4％(67/500) really have a comprehensive understanding on expander achieve excellent. Scores of male and female were (16.06 ± 1.99) points and (16.39 ± 2.16) points, t = 1.752, P > 0.05, there was no statistically significant difference comparing the 2 group. Patients′ families score of different cultural levels, respectively (14.06 ± 2.36), (14.98 ± 2.02), (16.54 ± 2.00), (16.73 ± 1.88) points, F = 21.736, P < 0.01, difference of four groups was statistically significant. Different age patients′families score ( 16.21 ± 1.96), (16.62 ± 2.14), (14.86 ± 2.11), (13.98 ± 2.02), (13.73 ± 1.88) points, F = 15.685, P > 0.05, there was no statistically significant difference comparing the 5 groups. Patients with different professional families score (13.25 ± 2.19), (13.79±2.27), (16.08±1.89), (14.10±2.08), (14.13±2.35), (14.45±2.09), (14.56±1.75), (16.84± 1.81) points, F = 2.737, P < 0.01, difference of eight groups was statistically significant. Conclusions Congenital microtia patients′families skin expander knowledge needs to be improved, it is necessary to take various forms, conduct for families of expander knowledge through propaganda and education.

Objective:To investigate the levels of periphreal blood free carnitine and amino acids in healthy pregnant women in the third trimester and their association with maternal, fetal, and neonatal cardiac function and structure.Methods:This prospective descriptive study included healthy singleton pregnancies who underwent routine obstetric examination and delivered in two district maternal and child health hospitals (one in the urban and one in the suburb an area) in Beijing from June 2017 to February 2018. All recruiters had serology Down's syndrome screening test at (18±1) gestational weeks. Besides measurement of amino acids and free carnitine levels in whole blood and urine samples by liquid chromatography-tandem mass spectrometry, all cases underwent maternal and fetal echocardiography at (35±1) weeks of gestation. And neonatal echocardiography was performed after delivery to assess the heart function and structure. Antenatal factors were also collected, including maternal education background, age at first marriage and conception, gravidity, and folic acid supplement in early pregnancy. Statistical analysis was performed using t-test, ANOVA, Chi-square test, Pearson correlation coefficient, and Kappa test. Results:A total of 493 mother-neonate dyads were enrolled in this study. Blood free carnitine levels in the healthy pregnant women in the third trimester ranged from 5.09 to 59.17 μmol/L (reference value: 10.00-50.00 μmol/L) with an average value of (13.03±3.87) μmol/L. None was found with structural abnormalities by cardiac ultrasound, showing an average left ventricular end diastolic diameter (LVEDD) and end systolic diameter (LVESD) of (45.70±3.08) mm and (29.17±3.12) mm, respectively, and left ventricular ejection fraction (LVEF) of all cases were over 55%. No cardiac malformation was detected by the third-trimester fetal echocardiography. The average birth weight of the 493 newborns was (3 340±313) g. Those whose birth weight <2 500 g and >4 000 g were accounted for 1.0% (5 cases) and 3.0% (15 cases) with the average maternal blood free carnitine level of (13.25±2.17) μmol/L (10.46-19.21 μmol/L) and (12.64±2.50) μmol/L (8.78-17.73 μmol/L) ( t=0.42, P>0.05). The average LVEDD and LVESD of the 493 newborns were (17.21±1.27) mm and (11.03±1.30) mm, respectively. For the 64 newborns (13.0%) whose LVEF<60%, the maternal blood free carnitine level was (12.93±2.78) μmol/L (7.34-22.13 μmol/L), showing no statistical difference ( t=-0.29, P>0.05) with those 59 neonates (12.0%) whose LVEF over 75% and maternal carnitine level of (13.09±3.24) μmol/L (8.66-27.49 μmol/L). All cases were divided into four groups based on the quartiles of maternal blood free carnitine level and no significant difference in maternal or neonatal LVEDD or LVEF was observed among these groups (all P>0.05). Conclusions:Blood free carnitine concentration in healthy pregnant women in the third trimester is at the lower limit of normal range, and no significant effect on maternal cardiac function and fetal cardiac structure is seen. However, the effect of low maternal carnitine level in the third trimester on children's myocardial function and whether carnitine should be supplemented in the third trimester are worthy of further investigation with larger sample size.

Objective Discussing the role of Yunnan baiyao in the newborn umbilical cord.Methods 120 cases newborns were selected and randomly divided into the control group of 60cases and the observation group of 60 cases according to birth order.The two kinds of newborns were both used single valve care,and were disinfected with 75%alcohol twice a day,Newborns of the observation group were dipped in Yunnan baiyao cream which was taken 75%alcohol to apply the root of umbilical and around after each nursing time.Observed the umbilical cord healing of two groups of newborns and take notes.Results In the observation group newborns,umbilical cord on the drying time [(3.1 ±1.1)d]and off time[(6.6 ±1.8)d]were better than the control group[(3.1 ±1.1)d,(8.0 ±3.2)d](t =7.8,2.95,all P <0.05);6 cases occurred redness and swelling around the belly button,2 cases occurred hemor-rhage,17 cases occurred secretions.which were better than that in control group(18 cases,6 cases,35 cases).The statistical analysis showed significant difference in the data of the two groups.Conclusion Yunnan baiyao can promote healing of umbilical cord,it should be popularize in clinical use.

Background The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients (13 males and 10 females,aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months,16 cases) and adult type (age of onset older than 12 months,7 cases).Four patients were diagnosed with CLMCA-A (three males and one female,aged ranging from 3 months to 2 years).The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination:Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography:pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in adult type.In echocardiography,the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement,left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA,and moderate to large mitral valve.It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery (RCA) from the right sinus of the aortic root and absence of LCA origin in angiography.After opacification of RCA,reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation.Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end,with diameter of only 1.1-2.0 mm.Treatment and prognosis:21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively.Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months (median 17 months).As for treatment of CLMCA-A,four patients took digoxin and diuretics without undergoing cardiac surgery.Their clinical symptoms improved during the close follow-ups.Conclusions ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children.In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.

Objective To investigate the prevalence of tuberculosis among silicosis patients and silica exposure patients,and to analysis the risk factors of tuberculosis among these population.Methods A total of 1 227 silica exposure patients from Wenling,Zhejiang were enrolled in this field study.Basic demographic information was collected and chest X-ray was taken for each patient.Sputum was collected for Mycobacterium tuberculosis culture and strain identification. In univariate analysis,t test was performed for continuous variables andχ2 test for categorical variables.In multivariate analysis,the odds ratio (OR )was calculated along with a 95 % confidence interval (CI )by binary Logistic regression. Results A total of 1 204 silica exposure patients had full basic information and 99.8% were male patients with mean age of (59.4 ± 6.8 )years.The patients in phase 0 + to phase Ⅲ were 172 (14.3%),255 (21 .2%),160 (13.3%)and 617 (51 .2%),respectively.The tuberculosis prevalence rate was about 7.3% among these population.The risk factors for tuberculosis including phase Ⅱ silicosis (OR =2.96, 95 %CI :1 .05 -8.32,P =0.04)and phase Ⅲ silicosis (OR=3.88,95 %CI :1 .58-9.56,P <0.01),and contacting with tuberculosis patients (OR=4.14,95 %CI :1 .91 -8.98,P <0.01).Patients complicated with tuberculosis lacked specific symptoms,but fever and weight loss were more frequent.Conclusion Tuberculosis is highly prevalent in silicotic patients,especially in patients with phase Ⅱ/Ⅲ silicosis and in patients with tuberculosis contact history.

Objective: To analyze the gene mutations and clinical features of patients with Noonan syndrome and hypertrophic cardiomyopathy. Method: Determined the mutation domain in five cases diagnosed with Noonan syndrome and hypertrophic cardiomyopathy and identified the relationship between the mutant domain and hypertrophic cardiomyopathy by searching relevant articles in pubmed database. Result: Three mutant genes (PTPN11 gene in chromosome 12, RIT1 gene in chromosome 1 and RAF1 gene in chromosome 3) in five cases all had been reported to be related to hypertrophic cardiomyopathy. The reported hypertrophic cardiomyopathy relevant genes MYPN, MYH6 and MYBP3 had also been found in case 1 and 2. Patients with same gene mutation had different clinical manifestations. Both case 4 and 5 had RAF1 mutation (c.770C>T). However, case 4 had special face, low IQ, mild pulmonary artery stenosis, and only mild ventricular hypertrophy. Conclusion Noonan syndrome is a genetic heterogeneity disease. Our study identified specific gene mutations that could result in Noonan syndrome with hypertrophic cardiomyopathy through molecular biology methods. The results emphasize the importance of gene detection in the management of Noonan syndrome.

Objective To explore the effects of automatic and intelligent micro pump in changing vasoactive agents. Methods This research selected 60 patients with a great number of vasoactive agents (dopamine or adrenalin hydrochloride) after cardiac surgery of Cardiovascular Surgery in Chinese PLA General Hospital from April to November 2016. Those patients were divided into experimental group (n=30, changing agents with the automatic and intelligent micro pump) and control group (n=30, changing agents with the traditional method of dual pump). The fluctuation of blood pressure (BP) of patients between two groups was compared before and after changing agents. Results There was no significant difference in BP of both group patients before changing vasoactive agents (P> 0.05). The mean BP of patients in the control group was significantly higher than that in the experimental group 30 seconds after changing vasoactive agents (P=0.011). The degree of fluctuation of BP of patients in the control group was greater than that in the experimental group 30 seconds and one minute after changing agents with a significant difference (P<0.05). Conclusions The automatic and intelligent micro pump has a big advantage in maintaining a stable BP during changing agents by pump. It is worth to be used widely in clinical application.

Objective:To investigate the short-term and medium-term changes of the left ventricular ejection fraction (LVEF) and the predictive value of relevant electrocardiogram (ECG) indexes in children with dilated cardiomyopathy (DCM) complicated with complete left bundle branch block (CLBBB).Methods:Children clinically diagnosed with DCM in the Department of Heart Center, Women and Children′s Hospital, Qingdao University and Beijing Anzhen Hospital, Capital Medical University between November 2011 and August 2020 were retrospectively recruited.According to the combination of CLBBB, they were divided into CLBBB group and non-CLBBB group.Echocardiogram and ECG were regularly performed.Short-term and medium-term changes of LVEF based on the 1-5-year follow-up data were compared between groups.COX proportional hazards model and Kaplan-Meier multiplicative limit method were used to analyze the predictive value of ECG indexes of LVEF changes in children with DCM combined with CLBBB.Results:Ninety-four children with DCM were enrolled, including 35 cases in CLBBB group and 59 cases in non-CLBBB group.There was no difference in baseline LVEF between groups.However, significant differences were found in QRS duration, corre-cted QT interval(QTc), R peak time in lead V 5 (T V5R) and QRS notching or slurring between groups ( P<0.05). LVEF of all children showed an upward trend within one year after onset, while the Z value of eft ventricular end diastolic diameter(LVEDd) showed a downward trend, and the two indexes tended to be stable within 1 - 5 years.The Z value of LVEDd in CLBBB group was significantly higher than that of non-CLBBB group, while LVEF was significantly lower (all P<0.05). The mean LVEF of CLBBB group slightly fluctuated around 50%, that of LVEF in non-CLBBB group was 60%.The multivariate COX regression analysis showed that QRS duration ( HR=0.979; 95% CI: 0.960-0.999, P<0.05) and QTc ( HR=0.988; 95% CI: 0.979-0.998, P<0.05) were independent predictors of LVEF recovery in children with DCM.Kaplan-Meier method showed a significant difference of LVEF normalization between DCM children with different QRS durations ( P<0.05), which was also detected in those with QTc interval ( P<0.05). Conclusions:LVEF of children with DCM combined with CLBBB increases in the short term after standard treatment, and then being stable.CLBBB can affect the recovery of left ventricular systolic function in children with DCM.Moreover, QRS duration and QTc interval are independent predictors of LVEF recovery in DCM children.

Objective:To explore the clinical characteristics and mutation spectrum of ALPK3-related pediatric cardiomyopathy and craniofacial-skeletal abnormalities in children.Methods:The clinical data during a follow-up of 11 years including clinical features, echocardiogram, electrocardiogram, cardiac magnetic resonance, genetic testing, and other data of a child firstly diagnosed with ALPK3 gene-related cardiomyopathy and craniofacial-skeletal abnormalities in China were collected retrospectively. The literatures containing the keyword of "ALPK3 gene" published in the China National Knowledge Infrastructure, Wanfang database and PubMed were collected up to November 2020. Then, the clinical features and gene mutations of ALPK3 gene-related pediatric cardiomyopathy with craniofacial-skeletal features were summarized.Results:A female patient aged 10 months who presented with an enlarged heart for 2 months, was admitted to the hospital and initially diagnosed with endocardial elastic fibrosis. The echocardiography showed features of dilated left ventricle (LV) and LV systolic dysfunction. Low-set ears, webbed neck, a grade 2/6 systolic murmur at lower left sternal area and bilateral absent flexion creases of dig were observed. After treatment, the size and function of the heart recovered to normal at age 13 months. However, the ventricular septum and LV wall were thicker than normal values. Then, the diagnosis was revised to hypertrophic cardiomyopathy(HCM) and suspected congenital malformation syndrome. LV hypertrophy (LVH) progressed slowly before the age of 8 years and then progressed rapidly. At age 9 years, compound heterozygous ALPK3 mutations (c.721dup, p.Y241Lfs*42(exon 1) and c.4840C>T, p.R1614*(exon 10)) were detected in the proband and the mutations had not been reported previously. Then, the final diagnosis of ALPK3 gene-related pediatric cardiomyopathy with craniofacial-skeletal features was made. During the follow up of 11 years, regular follow-up echocardiographic images showed progressive LVH. At age 11 years, electrocardiogram showed LVH, ST-T changes in multiple-lead, T wave inversion, and prolonged QT intervals. Cardiac magnetic resonance showed biventricular hypertrophy and late gadolinium enhancement showed non-uniform enhancement of left and right ventricular myocardium. A total of 7 articles published in English were retrieved, and no Chinese literature was found. Twenty-eight cases were reported in the articles plus the patient in this study. Twenty-four mutations were reported worldwide, 18 patients carried homozygous mutations and 10 patients compound heterozygous mutations. Eleven patients showed dilated cardiomyopathy (DCM) at early stage of disease, and 10 of them transitioned to HCM at the disease progression stage. Eight patients presented with HCM at early stage of disease. Nine patients initially exhibited a mixed phenotype of DCM and HCM, and 6 of them eventually progressed to HCM. Electrocardiogram showed prolonged QT interval. Extracardiac features included short stature, special face, cleft palate, webbed neck, joint contracture, and scoliosis, etc.Conclusions:Progressive myocardial hypertrophy is a major feature of ALPK3 gene-related cardiomyopathy with craniofacial-skeletal malformations. Precise diagnosis depends on molecular genetic techniques. More cases should be accumulated for further analysis on the genotype-phenotype correlation and prognosis assessment.