Child ; Congresses as Topic ; Coronary Disease ; diagnosis ; etiology ; therapy ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Incidence ; Injections, Intravenous ; International Cooperation ; Mucocutaneous Lymph Node Syndrome ; epidemiology ; etiology ; therapy

American Heart Association ; Anti-Bacterial Agents ; administration & dosage ; therapeutic use ; Cardiac Surgical Procedures ; Child ; Drug Resistance, Bacterial ; Drug Therapy, Combination ; Endocarditis ; drug therapy ; surgery ; Endocarditis, Bacterial ; drug therapy ; surgery ; Humans ; Practice Guidelines as Topic ; United States

Anthracyclines ; administration & dosage ; adverse effects ; Antineoplastic Agents ; administration & dosage ; adverse effects ; Biomarkers ; blood ; Cardiomyopathies ; chemically induced ; diagnosis ; prevention & control ; Cardiotonic Agents ; therapeutic use ; Child ; Child, Preschool ; Echocardiography ; Heart ; drug effects ; Heart Diseases ; chemically induced ; diagnosis ; prevention & control ; Humans ; Risk Factors ; Survival Analysis ; Troponin I ; analysis

Objective To evaluate the effect of staged repair for tetralogy of Fallot (TOF) associated with pulmonary artery hy- poplasty.Methods From June 1996 to June 2006,37 patients with TOF were operated on.There were 26 males and 11 females. Their age was 5 months to 17 years(mean 3.6 years) and weight was 4.6～38.0 kg.All patients were diagnosed as TOF with pulmo- nary artery hypoplasty by cardiac catheterization.The mean pre-operative arterial saturation of the patients was (68.2?6.5) %,Mc- Coon ratio was 0.95?0.26 (0.81～1.17) and Nakata index was 82.7?21.6(71.6～97.5) mm~3/m~2.At the time of the first surgi- cal procedure,17 patients underwent central aortopulmonary shunt,13 patients received modified Blalock-Taussig shunt in the left side and 7 patients had modified Blalock-Taussig shunt in the right side.Results There were no easly operative deaths and no late deaths after the first stage repair.Pleural effusion after shunt occurred in 5 patients.The mean arterial saturation was significantly increased to (91.3?10.4) %,P

Objective To explore the major work stresses and coping styles of psychiatric male nurses.Methods 95 psychiatric male nurses from a grade Ⅲ class A hospital in Beijing were surveyed by Nurse Job Stressors Questionnaires(NJSQ) and Simplified Coping Style Questionnaire (SCSQ).Results There was no significant difference in the total scores of JSQ among psychiatric male nurses with different position titles ( P＞0.05).However,statistical differences in the scores of work load and work time allocation between nurses-in-charge group,nurses group and nurse practitioners group[ (12.5±2.5) vs (8.7 ±3.7) vs (9.8 ±3.9) ;F=4.311,P＜0.05 ].The scores of married male nurses group in the source of work stress and work time allocation ( 10.6 ± 3.8) were higher than the score of single nurses group (7.8 ± 3.2 ) and showed significant difference (t =-3.198,P＜0.05 ).The scores of experienced male nurses group and unexperienced nurses group in the source of work stress were ( 14.9 ± 4.3 ) and ( 13.0 ± 4.3 ) respectively; the scores in work time allocation were ( 10.8 ± 3.6) and ( 8.5 ± 3.8 ) ; the scores in patients care were ( 19.3 ± 7.0) and ( 16.1± 6.8 ) ; the scores in management and relationships were ( 13.3 ± 5.5 ) and ( 10.7 ± 5.5 ).All the scores of experienced group were significantly higher than the unexperienced group ( t =- 2.230,- 3.021,- 2.229,- 2.268,respectively;P ＜ 0.05 ).There was no significant differences found between the score of coping mode and position title,years of work,marital status,education or age.Conclusions Psychiatric male nurses with different position title,work experience and marital status have more work stress.They should learn self-control,release pressure and cope with it actively.

Objective To study the effect of psychological intervention on patients with PanicDisorder. Methods 42 patients with Panic Disorder were randomly divided into study group (treated withabnormal nursing combined with psychological intervention)and control group(with abnormal nursing)for 4weeks, and curative effects assessed by the SCL-90, HAMA and HAMD. Results There were significantdifferences in scores of the HAMA(7.83±3.12) in study group than in control group(11.42±4.02) (P＜0.01), and that of in scores of the HAMD (19.45±3.92)in study group than in control group(18.67±4.33)at the end of 4th week(P＜0.01) ,and that of in scores of anxiety, depression, somatization, and personalrelationship of the SCL-90 in study group than in control group at the end of 4th week (P＜0.05).Conclusions Psychological intervention can improve the mental problems of patients with Panic Disorder,such as anxiety, depression, cognition and coping styles.

OBJECTIVEThe value of plasma brain natriuretic peptide (BNP) and N-terminal proBNP (NT-proBNP) can reflect cardiac function and therefore can be used for diagnosing congestive heart failure (CHF) and evaluating cardiac function. There are few reports, however, on the value of BNP and NT-proBNP in pediatric cases of congenital heart defect. The aim of this study was to assess the value of plasma NT-proBNP in the diagnosis of CHF and evaluation of cardiac function in pediatric patients with ventricular septal defect (VSD).

METHODSFifty-one patients with VSD aged from 2 months to 2 years old (mean 7.9 months) were enrolled. According to the modified Ross Score, the patients were divided into three groups, no CHF group (20 patients), mild CHF group (18 patients) and moderate to severe CHF group (13 patients). Fifteen age-matched normal children were used as controls. Plasma NT-proBNP was measured using enzyme immunoassay. All patients had complete echocardiographic study, including measurement of left ventricular end diastolic volume index (LVEDVI), left ventricular end systolic wall stress (LVSEWS), heart rate corrected mean velocity of circumferential fiber shortening (mVcFc), left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), and contractility index (Con). The correlation between plasma NT-proBNP level and modified Ross Score and echocardiographic cardiac functional indexes was determined. The sensitivity, specificity and ROC curve of plasma NT-proBNP for diagnosing CHF was studied.

RESULTSPlasma NT-proBNP was positively correlated with modified Ross Score (r = 0.75, P < 0.01). Plasma NT-proBNP concentration in moderate to severe CHF group (2061 +/- 908) fmol/ml was significantly higher than that of mild CHF group (810 +/- 335) fmol/ml, and Plasma NT-proBNP concentration in mild CHF group was higher than that in no CHF group (309 +/- 68) fmol/ml. 97.14% of normal controls and subjects in no CHF group had their plasma NT-proBNP below 400 fmol/ml. 83.3% of children in mild CHF group had their plasma NT-proBNP between (400-1400) fmol/ml while in moderate and severe CHF group 84.6% of children had their plasma NT-proBNP beyond 1400 fmol/ml. Plasma NT-proBNP was also positively correlated with LVEDVI and LVSEWS. There was no correlation among mVcFc, LVEF, LVFS, Con and plasma NT-proBNP concentration. Using plasma NT-proBNP concentration > or = 400 fmol/ml as cut-point for diagnosing CHF, the sensitivity was 89.3%, the specificity was 91.2%, and the area under the ROC curve was 0.944.

CONCLUSIONSPlasma NT-proBNP level could be used to assess cardiac function and diagnose CHF in pediatric patients with VSD.

Echocardiography ; Female ; Heart Failure ; blood ; Heart Septal Defects, Ventricular ; blood ; diagnosis ; Humans ; Infant ; Male ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood ; Ventricular Function, Left

OBJECTIVETo study the influence of anastomoses sequence on the hemodynamics in off-pump coronary artery bypass grafting (CABG), and to investigate the strategy to stabilize the hemodynamics.

METHODSFrom March 2005 to March 2007, 67 patients with serious triple-vessel coronary artery lesions (male 45, female 22) with a age range from 44 to 81-years-old were enrolled for off-pump CABG. All the patients underwent left internal mammary artery-left anterior descending branch (LIMA-LAD) anastomose firstly, followed by the foreword anastomose of the other two vessels. According to the anastomose sequence of posterior descending branch (PDA) and obtuse marginal branch (OM), the patients were divided into three groups. Group I (n = 22) did the sequence of PDA-OM-aortic root (Ao). Group II (n = 14) did the sequence of Ao-PDA-OM. Group III (n = 31) did the sequence of Ao-OM-PDA. The hemodynamics markers, including heart rate (HR), central venous pressure (CVP), mean artery pressure (MAP), cardiac index (CI), left ventricular-stroke work index (LVSWI) and right ventricular stroke work index (RVSWI), were analyzed before LIMA-LAD anastomose to serve as baseline. And these markers were re-measured at LIMA-LAD anastomose, immediately after LIMA-LAD anastomose, at PDA anastomose and at OM anastomose.

RESULTSAll the patients survived after off-pump CABG. Compared with the baseline, there were no significant hemodynamic changes when doing LIMA-LAD anastomose. When the anastomose finished, the hemodynamic indices improved significantly. When performing OM anastomose, HR and CVP increased significantly, and MAP, CI, LVSWI and RVSWI decreased remarkably in group I and group III, while there were no significant changes of MAP, CI and RVSWI in group II . When performing PDA anastomose, HR and CVP increased significantly, and MAP, CI, LVSWI and RVSWI decreased remarkably in group I and group II, while there were no significant hemodynamic changes in group III except HR.

CONCLUSIONProximal anastomose first when performing off-pump CABG, followed by distal anastomoses of target vessels is beneficial to those who have hard exposure and difficult anastomose of OM and PDA.

Adult ; Aged ; Aged, 80 and over ; Anastomosis, Surgical ; methods ; Coronary Artery Bypass, Off-Pump ; methods ; Coronary Artery Disease ; physiopathology ; surgery ; Coronary Vessels ; surgery ; Female ; Hemodynamics ; Humans ; Male ; Middle Aged ; Monitoring, Intraoperative ; Retrospective Studies

OBJECTIVERett syndrome (RTT) is a neurodevelopmental disorder occurring almost exclusively in females as sporadic cases due to de novo mutations in the methyl-CpG-binding protein 2 gene (MECP2). Familial cases of RTT are rare and are due to X-chromosomal inheritance from a carrier mother. Recently, DNA mutations in the MECP2 have been detected in approximately 84.7% of patients with RTT in China. To explain the sex-limited expression of RTT, it has been suggested that de novo X-linked mutations occur exclusively in male germ cells resulting therefore only in affected daughters. To test this hypothesis, we have analyzed the parental origin of mutations and the XCI status in 15 sporadic cases with RTT due to MECP2 molecular defects.

METHODSAllele-specific PCR was performed to amplify a fragment including the position of the mutation. The allele-specific PCR products were sequenced to determine which haplotype contained the mutation. It was then possible to determine the parent of origin by genotyping the single nucleotide polymorphism (SNP) in the parents. The degree of XCI and its direction relative to the X chromosome parent of origin were measured in DNA prepared from peripheral blood leucocytes by analyzing CAG repeat polymorphism in the androgen receptor gene (AR).

RESULTSExcept for 2 cases who had a frameshift mutation; all the remaining 13 cases had a C-->T transition mutation. Paternal origin has been determined in all cases with the C-->T transition mutation. For the two frameshift mutations, paternal origin has been determined in one case and maternal origin in the other. The frequency of male germ-line transmission in mutations is 93.3%. Except for 2 cases who were homozygotic at the AR locus, of the remaining 13 cases, 8 cases had a random XCI pattern; the other five cases had a skewed XCI pattern and they favor expression of the maternal origin allele.

CONCLUSIONDe novo mutations in sporadic RTT occur almost exclusively on the paternally derived X chromosome and that this is most probably the cause for the high female: male ratio observed in sporadic cases with RTT. Random XCI was the main XCI pattern in sporadic RTT patients. The priority inactive X chromosome was mainly of paternal origin.

Chromosome Aberrations ; Chromosomes, Human, X ; Female ; Humans ; Male ; Methyl-CpG-Binding Protein 2 ; genetics ; Mutation ; Polymorphism, Single Nucleotide ; Rett Syndrome ; genetics ; X Chromosome Inactivation

OBJECTIVERett syndrome (RTT) is an X-linked progressive neurodeveopmental disorder that almost exclusively affects girls, and is one of the most common causes of mental retardation in females, with an estimated prevalence of approximately 1 in 10,000 - 15,000 female individuals. Mutations in X-linked methyl-CpG-binding protein 2 (MECP2) gene, located on chromosome Xq28, have been found to be a cause of RS. A lot of mutations have been reported to be related to RS recently. Mutations are found in 70% - 85% of patients with classical RTT and in less than 50% of patients with atypical RS. Up to now, RTT is diagnosed based on a consistent counseling for clinical features and the established diagnostic criteria. The present study aimed to investigate frequency and type of mutation of MECP2 gene and if hot spot of mutation exits in patients with atypical RTT and find out the relationship between genotype and phenotype.

METHODSA systematic analysis of the entire coding region of MECP2 in 26 unrelated patients with atypical RTT was performed by polymerase chain reaction (PCR) and direct sequencing. Genomic DNA was extracted using standard procedures from the peripheral blood leukocytes of each patient. PCR amplification products were checked by 2% agarose gel electrophoresis and were subsequently sequenced with ABI 3730 Automated DNA Sequencer with both the forward and reverse primers. Mutational analyses were performed using normal human genomic MECP2 sequence as a reference (GenBank accession NO.AF030876).

RESULTSSeven mutations were identified in 12 of 26 patients. Most of the mutations were missense mutation; c.397C > T (R133C) was found in 3 of 26 patients; c.473C > T (T158M) and c.916C > T (R306C) were found in 2 of 26 patients, respectively; c.397A > G (R133H) and c.1005G > A (R335C) were found in 1 of 26 patients, respectively. One base pair deletion mutation (806delG) resulting in frameshift was found in 2 of 26 patients, and 1 base pair transversion at splice accept-site (IVS3-2A > T).

CONCLUSIONThe results of this study indicated that c.397C > T (R133C), c.473C > T (T158M) and c.916C > T (R306C) were hot spot mutations in MECP2 gene of patients with atypical RTT. There was some relationship between genotype and phenotype.

Base Sequence ; Child ; Female ; Gene Frequency ; Genetic Predisposition to Disease ; Humans ; Methyl-CpG-Binding Protein 2 ; genetics ; Molecular Sequence Data ; Mutation ; Mutation, Missense ; Phenotype ; Polymerase Chain Reaction ; Rett Syndrome ; genetics ; Sequence Analysis, DNA