ABSTRACT
Objective:To explore the pathogenic genes, clinical characteristics and treatment follow-up of children with congenital long QT syndrome (LQTS).Methods:Clinical data of 20 cases diagnosed with congenital LQTS and underwent gene testing from April 15, 2011 to April 15, 2021 in Department of Pediatric Cardiology, Shandong Provincial Hospital Affiliated to Shandong University were retrospectively collected and analyzed using independent sample t-test and Fisher′ s exact probability method. Results:LQTS-related gene mutations were detected in all the 20 cases, and pathogenic or suspected pathogenic mutations were identified in 18 cases (90.0%). Five LQTS mutation genes were discovered, including KCNQ1, KCNH2, SCN5A, CACNA1C and AKAP9.Eighteen cases (90.0%) had positive symptoms, and 13 cases (65.0%) had definite inducements.The inducement of symptoms in children with LQTS type 1(LQT1) was related to exercise, the causes of syncope in LQT1 and Jervell-Lange-Nielsen syndrome type 1 (JLNS1) with complex heterozygous mutations were exercise or emotional agitation; the causes of syncope in LQTS type 2 (LQT2) were unrelated to exercise; severe exercise in LQTS type 3 (LQT3) resulted in symptoms; and seizure in LQTS type 8 (LQT8) was non-induced.The corrected QT(QTc) interval of 20 cases was (553.1±66.6) ms, with a range of 460-707 ms, among which 17 cases showed QTc≥480 ms.The electrocardiogram(ECG) manifestations of children with various types of LQTS were different.There was no significant difference in QTc between different genders, or between children with syncope and those without syncope (all P>0.05). The follow-up time was (3.4±2.3) years, ranging from 0 to 8.3 years.Seventeen children received treatment[beta blockers and implantable cardiovertor-defibrillator(ICD)] and 3 cases did not.By the end of the follow-up, 1 child died, 19 cases survived, and 2 cases of the surviving children lost consciousness. Conclusions:There is a high consistency between genetic diagnosis and clinical diagnosis of congenital LQTS.The positive rate of gene detection is 90.0%.The clinical manifestations and ECG characteristics vary with genotypes.Beta blockers are protective.ICD therapy can prevent sudden cardiac death when oral medication does not respond.
ABSTRACT
Objective To evaluate the safety and efficacy of percutaneous balloon pulmonary valvuloplasty (PBPV)in the treatment of the children with pulmonary stenosis (PS),and to observe the long - term prognosis and analyze the influencing factors. Methods The total of 230 children were collected,who had been diagnosed with pul-monary valve stenosis and had undergone percutaneous balloon pulmonary valvuloplasty between November 1987 and November 2015 in Shandong Provincial Hospital Affiliated to Shandong University. Their ages ranged from 4 months to 17 years,and the follow - up duration lasted from 1 month to 29 years. The data included clinical data and long - term follow - up data of hospitalized children,and the echocardiography data from the healthy peers in the same period. Then the data were analyzed statistically. Results In this study,228 cases of children were successfully performed PBPV, and the success rate was 99％(228 / 230 cases). The pulmonary transvalvular gradient (△P)of preoperation,24 hours postoperatively,half a year postoperatively,2 years postoperatively,5 years postoperatively,and 10 years postope-ratively was (63. 5 ± 23. 8)mmHg (1 mmHg = 0. 133 kPa),(26. 2 ± 11. 1)mmHg,(24. 8 ± 9. 8)mmHg,(20. 9 ± 8. 9)mmHg,(18. 1 ± 8. 7)mmHg,(15. 3 ± 7. 3)mmHg and (15. 3 ± 7. 3)mmHg,respectively. The immediate post-operative △P was significantly lower than that of preoperation (P < 0. 01),and the △P of the most children decreased in the long - term follow - up. The results of Logistic regression analysis showed that valve dysplasia with right ventricu-lar outflow tract stenosis and the immediate postoperative residual transvalvular gradient degree were the risk factors for long term curative effect of PBPV in children who could not reach the best standard. The restenosis rate was 4. 6％(3 /65 cases)with children followed up for more than 10 years. The incidence of long - term follow - up pulmonary valve regurgitation (83％)was significantly higher than that before operation (58％)and short term (68％)after operation, and the degree of regurgitation also increased (P < 0. 05),while the degree of regurgitation of the tricuspid regurgitation decreased gradually during the follow - up (P < 0. 05);the right ventricular diastolic diameter of the patients at 10 years or more after the operation was measured as (19. 27 ± 3. 03)mm,which was significantly higher than that (15. 24 ± 2. 89)mm of the healthy children of at the same term healthy age (P < 0. 05). Conclusions The PBPV has a high success rate in the treatment of children with PS,and it has good medium - long - term curative effect,less com-plications and lower restenosis rate. Therefore,PBPV can be used as the first choice for PS. However,the incidence and degree of pulmonary regurgitation has an increasing trend after PBPV and the right ventricular diastolic diameter is still larger than that of the healthy children. Therefore,the long - term follow - up is necessary out of the hospital.
ABSTRACT
Objective To investigate the feasibility and safety of transcatheter closure of coronary artery fistula (CAF)with Amplatzer vascular PlugⅡ(AVPⅡ)in pediatric patients.Methods Between June 2012 and October 2015,5 children aged 0.9 to 7.0 years old and weighted 10 to 21 kg with CAF were admitted to the Department of Pediatric Cardiology in Shandong Provincial Hospital Affiliated to Shandong University.Aortic root angiography was used first to confirm the origin,shape,branches,drainage and the diameter of the orifice of CAF by deploying the pigtail catheter.The AVPⅡwas retrogradely deployed into targeted artery through guiding catheter and aortic angiography was performed before releasing the plug.Results All the 5 children underwent transcatheter closure by AVPⅡsuccessful-ly.Two cases were involved with right coronary -right ventricular fistula,1 case of left anterior descending coronary -right ventricular fistula (residual fistula after surgical repair),and 1 case of left circumflex coronary -left atrial fistula. Four children had a single fistula,and 1 case had double fistulas.The diameter of the orifice ranged from 2.00 to 5.96 mm,and the selected occluders from 8 to 14 mm.The ratio of diameter of occluder to fistula orifice ranged from 2.3 to 3.4.All the patients were followed up for 4 to 44 months.Two patients developed instant minor and modera-te aortic re-gurgitation.No other complications such as thrombosis,embolization,residual shunt,arrhythmia,coronary dissection or perforation occurred.Conclusions Transcatheter closure of CAF by AVPⅡin pediatric patients is feasible and safe. Aortic regurgitation should be noted,especially during the procedure.
ABSTRACT
Objective To investigate the effect of CTLA4-Ig chimera protein on mice mortality, histopathological changes, viral fiters, expression of CTLA4 protein on infiltrated T lymphocyte and the balance of Thl/Th2 in mice myocarditis caused by coxsackie virus B3 (CVB3). Methods A total of 106 four to six week-old male BALB/c mice were used in the experiments, which were divided into CTLA4-Ig group (n = 16), CVB3 group (n=40), IgG group (n =40) and normal control group(n = 10) randomly. The mice in CVB3 group, IgG group and CTLA4-Ig group were inoculated intraperitoneally with 0. 15 ml CVB3 and the mice in norreal control group with 0. 15 ml Eagle. The mice in IgG group and CTLA4-1g group were inoculated with IgG (0. I mg/kg) and CTLA4-Ig(0. 1 mg/kg) at 6 h and 72 h post inoculation(p, i. ), respectively, The surplus mice in each group were sacrificed at day 7 p.i. Light microscope was used to quantify the inflammation. The expression of CVB3 mRNA in mycardium were semi-quantified by real-time quantitative polymerase chain reaction (RQ-PCR). The expression of CTLA4 protein were analyzed by immunohistochemistry. The levels of IL-2, IL-4 and 1FN-γ in serum were measured by ELISA. Results The mice mortality, histopathological score and CVB3 mRNA in CTLA4-Ig group were lower than that in CVB3 group ( P < 0.05, P < 0.01, P < 0. 05, respectively). The expression of CTLA4 was significantly increased in CTLA4-Ig therapy group (P < 0.05 ). The serum level of IFN-γ of mice in CVB3 group were significantly higher than that in normal control group( P < 0.01 ). The serum level of IL-4 of mice in CVB3 group were much lower than that in normal control group( P < 0.01 ). The serum level of IL-2 in CVB3 group had no statistical significance with that in normal control group ( P > 0.05 ). The serum level of IFN-γ in mice of CTLA4-Ig group were much lower than that in CVB3 group ( P <0.01 ) and lgG group (P < 0. 01 ). The serum level of IL-4 of mice in CTLA4-Ig group were significantly higher than that in CVB3 group (P<0.01) and IgG group (P<0.01). The serum level of IL-2 in CTLA4-Ig group had no statistical significance with that in CVB 3 control group and lgG group ( P > 0. 0 5 ) . Conclusion CTLA4-Ig may relieve inflammation and reduce mice mortality by blocking the costimulation signals for T lymphocyte activation and reinforcing Th2 response.
ABSTRACT
OBJECTIVE:To investigate the effects of Periplogenin, a compound extracted and purified from traditional Chinese medicine Cortex Periplocae, on mast cell degranulation and histamine release in mice and rats. METHODS: Sensitization was induced by injecting pertussis vaccine injected in rats' abdominal cavity and ovalbumin injected in mice's hind leg to detect mast cell degranulation reaction and prepare anti-serum; rats were injected intraperitoneally with the diluted serum sample taken form sensitized rats to detect mast cell degranulation reaction; and the concentration of histamine was determined by fluorescence spectrophotometry. RESULTS: Periplogenin showed significant inhibitory effect on histamine release of mast cell cultured in vitro, and at experimental dose it could decrease histamine release by(69.4?8.6)% in an obvious dose-depe-ndent manner. Periplogenin also showed obvious inhibitory effect on histamine release of mast cells in antigen-sensitized rats,and at a concentration of 20 ?g?mL-1,it could decrease histamine release by 73.55%, and at an oral dose of 50 mg?kg-1, it could dose-dependently reduce histamine release by above 80% in sensitized rats. CONCLUSION: Periplogenin showed significant inhibitory effects on histamine release of mast cells either cultured in vitro or in antigen-sensitized rats. Oral administration of Periplogenin can result in the significant reduction of histamine release of mast cells in rats. In view of the role that the mast cell histamine release and degranulation play in inflammation reaction, periplogenin can be regarded as one of the active anti-inflammation components of traditional Chinese medicine Cortex Periplocae.