Objective To explore the causes,clinical characteristics and prognosis of children′s completely at-rioventricular block(CAVB).Methods The clinical data of 73 patients with CAVB were analyzed retrospectively from January 2004 to December 201 3 at the Cardiology Department,Nanjing Children′s Hospital Affiliated to Nanjing Medi-cal University.Within those 73 patients,34 patients were male and the others were female,from 3 months old to 1 2.5 years old,the mean age of 6 years.Results There were 21 congenital CAVB patients and 52 acquired CAVB patients with myocarditis undergoing ventricular septal defect (VSD)closure operation.All congenital CAVB patients were re-fractory to drug therapy.Electrocardiogram and echocardiogram were performed in 1 9 cases without clinical symptoms during follow -up,but 2 cases had permanent pacemaker implanted.Among 27 fulminant myocarditis,Adams -Stokes attacks were found in 1 5 cases,3 cases had Adams -Stokes attack in 1 5 cases with sequelae of myocarditis,and 2 out of 6 cases undergoing VSD closure operation had Adams -Stokes attack,and other 4 cases without clinical symptoms were followed up periodically.The acquired CAVB patients were given energy composition and intravenous megavitamin C. The cases with fulminant myocarditis were given adrenal cortical hormone and intravenous gamma globulins simulta-neously.A total of 27 acquired CAVB patients were implanted temporary pacemaker and 5 with permanent pacemaker. Among 52 acquired CAVB patients,31 cases were cured,9 cases were improved,1 1 cases were ineffective,and 1 case died.Conclusions Most congenital CAVB children without clinical symptoms need clinical follow -ups.Myocarditis is a major cause of acquired CAVB.The CAVB prognosis caused by fulminant myocarditis may be related to antimely im-planting the temporary pacemaker timely.Permanent pacemaker should be implanted in patients who have no response to drug therapy with frequent Adams -Stokes or heart failure.

Mutations in the CACNA1C gene which encodes the α1C subunit of voltage dependent l-type Ca²⁺ channel can cause mental and cardiovascular diseases.It is the pathogenic gene of Timothy syndrome.Its cardiovascular-system phenotype mainly includes long QT syndrome, Brugada syndrome, short QT syndrome, etc.In recent years, it has been found that CACNA1C gene mutations can also lead to non-syndromic phenotypes, including congenital heart disease, cardiomyopathy, etc, further enriching the clinical phenotype of CACNA1C gene mutation.Now, the recent advances in heart disease phenotypes and mechanisms involved in CACNA1C gene mutations are reviewed.

Recent studies have found that children with congenital heart disease (CHD) are at increased risk of neurodevelopmental disorders (NDDs), including cognitive, adaptive, motor, speech and autism spectrum disorders.Structural and functional neuroimaging has indicated that brain abnormalities in children with CHD might be caused by an in utero developmental insult.Specific genetic abnormalities, particularly copy number variants(CNVs), have been increasingly implicated in both CHD and NDDs.Variations in genes involved in apolipoprotein E production, the Wnt signaling pathway, and histone modification, as well as in the 1q21.1, 16p13.1-11 and 8p23.1 genetic loci, are associated with CHD and NDDs.Understanding these associations is important for risk stratification, disease classification, improving screening and pharmacologic management of individuals with CHD.

Objective:To explore the risk factors of thrombocytopenia in children with patent ductus arteriosus (PDA) after transcatheter closure, and to establish a prediction model of thrombocytopenia after transcatheter closure of PDA.Methods:A total of 39 PDA children with thrombocytopenia after transcatheter closure treated in Children′s Hospital of Nanjing Medical University from November 2016 to January 2020 were selected.During the same period, 138 PDA children without thrombocytopenia after transcatheter closure were included in the control group. Logistic regression model was used to explore the possible risk factors of thrombocytopenia after transcatheter closure in PDA children, and a random forest model was established to predict the occurrence of thrombocytopenia after transcatheter closure of PDA. Results:After transcatheter closure of PDA, children developed thrombocytopenia within 1 to 7 days, and the platelet count recovered within 2 to 22 days. Logistic regression model suggested that the diameter of pulmonary artery end of arterial catheter ( OR=9.54, 95% CI: 2.08-48.84, P=0.004)and preoperative platelet count( OR=0.99, 95% CI: 0.98-0.99, P=0.001)were correlated with the occurrence of thrombocytopenia after transcatheter closure of PDA.The random forest model indicated that PDA inner diameter was the most important factor for predicting the occurrence of thrombocytopenia after transcatheter closure of PDA. Conclusions:A large diameter of arterial duct is an important risk factor and increased preoperative platelet count is a protective factor for thrombocytopenia after transcatheter closure of PDA.Diameter is of the greatest significance in predicting the occurrence of thrombocytopenia after transcatheter closure of PDA.

Objective To explore the application value of dipstick dye immuno-assay (DDIA) for screening the schistosomiasis chemotherapy targets in the low endemic areas of Xiaogan City.Methods The residents aged 6-65 years in a village in the low endemic areas of schistosomiasis of Xiaogan City were selected and tested by the methods of fecal examination,DDIA,indirect hemagghitination (IHA),enzyme linked immunosorbent assay (ELISA) and inquiry,and the results of fecal examination were determined as the gold standard.Results The Youden' s indices of IHA,DDIA,ELISA and inquiry were 0.74,0.72,0.62 and 0.30,respectively,and the consistency rates of them were 93.38%,91.99%,81.53% and 70.03%,respectively.It took 16.70,4.95,4.12,5.63 and 2.44 Yuan screening one patient with the fecal examination,IHA,DDIA,ELISA and inquiry,respectively.Conclusion The validity of DDIA with simple operation and low cost for screening the schistosomiasis chemotherapy targets is satisfying,and the method is suitable for large scale screening in low endemic areas.

Clinical data and follow-up of a case of congenital disorder of glycosylation type Ia (CDG-Ia) combined with dilated cardiomyopathy admitted to the Department of Cardiology, Children′s Hospital of Nanjing Medical University were analyzed retrospectively.The 5-year-old female patient was admitted in December 2016 due to recu-rrent shortness of breath for 2 months.Clinical symptoms and signs included repeated attacks of shortness of breath, physical retardation, malnutrition, binocular esotropia, multiple episodes of hypoglycemia, hepatosplenomegaly, hypotonia and other multi-system damages.Cardiac echocardiography suggested the feature of dilated cardiomyopathy, including the significant enlargement of the left ventricle, and decreased systolic function.Genetic testing revealed a compound heterozygous mutation in the PMM2 gene, and as a result, the patient was diagnosed as CDG-Ia.The patient′s condition improved after symptomatic treatments such as Cedilanid, Dopamine, Dobutamine, Furosemide, as well as support treatments like myocardium nutrition, blood sugar maintenance, liver protection, etc.After discharge, the patient was given oral Digoxin, Betaloc, Captopril and diuretics, and hypoglycemia-controlling agents.The patient was followed up every 3-6 months.After more than 2 years of follow-up, the heart function and heart enlargement gradually returned to normal.During the Corona Virus Disease 2019 outbreak, self-withdrawal continued for 2 months.Re-examinations showed decreased cardiac function and enlarged left ventricle again.Medications were resumed again, and the patient was followed up closely.This case report suggested that CDG-Ia may be associated with dilated cardiomyopathy, and the cardiac phenotype may be improved by symptomatic supportive treatment.

Adhesive intestinal obstruction is the most common type of ileus, and conserva-tive treatment serves as its preferred treatment option. In the course of conservative treatment, gastrointestinal decompression will relieve symptoms, prevent ileus progression and promote gas-trointestinal function recovery, which has significant clinical effects. Currently, decompression effects of nasointestinal tubes and nasogastric tubes are controversial. There is a previous Meta-analysis evaluating decompression effects of these two methods, but this analysis includes non-randomized controlled trial and lacks research about Chinese patients. Therefore, the authors con-duct a Meta-analysis to evaluate decompression effects of nasointestinal tubes versus nasogastric tubes for adhesive intestinal obstruction.

OBJECTIVE To study the pharmacokinetic characters of Baihe -zhimu decoction （BZD） and its single herbs . METHODS A liquid chromatography -tandem mass spectrometry method was developed to simultaneously determine the blood concentrations of 7 effective components （neomangiferin，mangiferin，regaloside A ，regaloside Ⅰ，timosaponin B Ⅱ，timosaponin E and timosaponin A Ⅲ）in rats after oral administration of BZD extract ，single herb extract of Lilium brownii and Anemarrhena asphodeloides. The pharmacokinetic parameters were calculated . RESULTS The linear range of 7 effective components as neomangiferin were 1-1 000，1-1 000，0.1-100，0.4-400，1-1 000，0.1-100 and 0.5-500 ng/mL（all r＞0.998），respectively. The accuracy of the method ranged from 87.50% to 115.00%，and the RSDs of intra -day and inter -day precision were 0.62%-14.35%. RSDs of matrix factor were 2.68%-14.03%，and deviation of stability were within ±15%. Compared with L. brownii and A. asphodeloides，AUC0－24h，AUC0－∞ and cmax of 7 effective components in BZD were increased significantly （P＜0.05），while CL z/Fof 6 effective components （except timosaponin B Ⅱ）decreased significantly （P＜0.05）. CONCLUSIONS BZD can increase the absorption of effective ingredients in rats ，slow down their elimination and prolong their retention time ，indicating pharmacokinetic behaviors of effective components in BZD possess more advantages over those of single herbs .