Hypertrophic cardiomyopathy associated with malformation syndrome in children / 中国实用儿科杂志

Hypertrophic cardiomyopathy(HCM) is defined by the presence of increased left ventricular(LV)wall thickness that is not solely explained by abnormal loading conditions. It includes familial HCM caused by mutations in the genes encoding the cardiac muscle branch contraction-associated protein,as well as hereditary HCM caused by mutations in genes encoding non-muscle-contracting system-associated proteins. Malformation syndromes are symptom complex characterized by multi-system and multi-organ abnormality. HCM can be the only or major manifestation of its cardiovascular system lesions. The article will briefly introduce HCM associated with malformation syndrome in children.

Precision diagnosis and treatment for hypertrophic cardiomyopathy in children: Current opportunities and challenges / 中国实用儿科杂志

Hypertrophic cardiomyopathy(HCM)is the second commonest cardiac muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. HCM is highly heterogeneous in pediatric population,and currently the precision diagnosis of the etiology of the disease faces enormous challenges. In addition,prospective and randomized controlled clinical studies in the diagnosis,treatment and prognosis of pediatric HCM are extremely rare. Evidence-based consensus or guidelines needs to be developed urgently for personalized risk assessment and treatment,as well as standardized family management and genetic counseling.

Clinical features of children with Epstein-Barr virus-related acute liver failure: an analysis of four cases / 中国当代儿科杂志

A retrospective analysis was performed for the clinical data of four children with Epstein-Barr virus (EBV)-related acute liver failure. There were two boys and two girls with a median age of 10 months (range 8.5-44 months). Of the four children, three were diagnosed with infectious mononucleosis (IM), among whom two met the diagnostic criteria of hemophagocytic lymphohistiocytosis (HLH), and one was diagnosed with past EBV infection. All the children had positive EBV DNA in blood and all had pyrexia, hepatomegaly, and jaundice on admission. Three children had the symptom of splenomegaly, ascites, or vomiting. Two children had enlargement of cervical lymph nodes, skin rash, or pleural effusion. One child had gastrointestinal bleeding or stage 2 hepatic encephalopathy. All the children had an abnormal lymphocyte count of <10%, and only one child had leukocytosis and thrombocytopenia. Among the four children, alanine aminotransferase level increased by 10-100 times; total bilirubin level increased by 3-5 times; lactate dehydrogenase level increased by many 10 times; prothrombin time prolonged significantly. All the children were given antiviral therapy with intravenously injected acyclovir or ganciclovir, as well as hepatocyte growth factor to promote hepatocyte growth and hormone to alleviate inflammatory response. Two children were given plasma exchange in addition, among whom one was given the combination of continuous venovenous hemodiafiltration. Two children with HLH were given chemotherapy according to the HLH-2004 regimen. Three children survived, and one child with HLH died of multiple organ failure. It is concluded that EBV infection can cause acute liver failure and that early use of multimodality therapy including blood purification may be beneficial for prognosis in these children.

Association between S100B gene polymorphisms and hand, foot and mouth disease caused by enterovirus 71 infection / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the association between rs9722 polymorphisms in the S100B gene and hand, foot and mouth disease (HFMD) caused by enterovirus 71.</p><p><b>METHODS</b>A total of 124 HFMD children with enterovirus 71 infection were enrolled as subjects, and 56 healthy children were enrolled as control group. The rs9722 polymorphisms in the S100B gene were detected for both groups, and the serum level of S100B protein was measured for 74 HFMD children.</p><p><b>RESULTS</b>The rs9722 locus of the S100B gene had three genotypes, CC, CT, and TT, and the genotype frequencies were in accordance with Hardy-Weinberg equilibrium. Compared with the control group, the HFMD group had significant increases in the frequencies of TT genotype and T allele (P<0.01). Children with severe HFMD caused by enterovirus 71 infection had significantly higher frequencies of TT genotype and T allele than those with moderate or mild HFMD (P<0.05). Compared with the cured patients, the patients with poor prognosis had significant increases in the frequencies of TT genotype and T allele in the rs9722 locus of the S100B gene (P<0.05). Among the 74 children with HFMD, the children with TT genotype had the highest serum level of S100B protein, and those with CC genotype had the lowest level (P<0.01).</p><p><b>CONCLUSIONS</b>T allele in the rs9722 locus of the S100B gene might be a risk factor for severe HFMD caused by enterovirus 71 infection.</p>

Comparing of the values of four criteria for the diagnosis of infective endocarditis in children / 中华实用儿科临床杂志

Objective To compare the diagnostic results and values among Duke criteria,St Thomas criteria,the modified Duke criteria and domestic criteria for the diagnosis of infective endocarditis (IE) in children.Methods Forty-five children were clinically diagnosed IE,the surgical or the pathologic or autopsy findings were served as the golden criteria for the diagnosis of IE.The children were classified by Duke criteria,St Thomas criteria,the modified Duke criteria and domestic criteria respectively,so as to compare the sensitivity and specificity and accuracy of all criteria for the diagnosis of IE.Results During the forty-five children were clinically diagnosed IE,of whom 32 cases with IE were confirmed by the surgical or the pathologic or autopsy findings; 13 cases were ruled out from the diagnosis of IE.The specificity of Duke criteria,the modified Duke criteria and domestic criteria were all 100％,but the specificity were 92.3％ in St Thomas criteria for the 45 cases with clinically diagnosed IE and 26 cases with negative blood culture.For the children with clinically diagnosed IE,the sensitivity and the accuracy were 90.6％ and 93.3％ in domestic criteria,62.5％ and 73.3％ in Duke criteria and the modified Duke criteria,87.5％ and 88.9％ in St Thomas criteria.For the children with negative blood culture,the sensitivity and the accuracy were 76.9％ and 88.5％ in domestic criteria,7.7％ and 53.8％ in Duke criteria and the modified Duke criteria,69.2％ and 80.8％ in St Thomas criteria.The Youden index had no significant difference among domestic standard and Duke standard,Duke modified standard,St Thomas standard for children clinically diagnosed IE (P ＞ 0.05) ; however,the significant difference of the Youden index among domestic standard and Duke standard,Duke modified standard for children with negative blood culture were found(P ＜ 0.01),no significant difference of the Youden index between domestic standard and St Thomas standard was found (P ＞ 0.05).Conclusions With the addition of echocardiographic evidence of endocardial involvement,2 minor criteria as definite diagnostic criteria and some clinical minor criteria,the sensitivity and accuracy of the domestic criteria is superior to those of the Duke criteria,St Thomas criteria and the modified Duke criteria,and the domestic criteria is more suitable for the diagnosis of IE in Chinese children.

Effects of Astragaloside Ⅳ on Collagen of Myocardial Fibroblasts in Rats / 实用儿科临床杂志

Objective To investigate the dose- and time-effects of astragaloside Ⅳ(XGA) on collagen of myocardial fibroblasts in rats.Methods The myocardial fibroblasts of rats were separated by collagenase and trypsinase digestive method,and the cell culture system was established. After XGA in different concentrations and at different time points was administered in fibroblast culture systems,the mRNA expression levels of collagen,matrix metalloproteinases(MMP)-1,-2,-9,tissue inhibitor of metalloproteinase(TIMP)-1 and -2 were measured with reverse transcription-polymerase chain reaction(RT-PCR) test.Results After XGA administration with different doses and at different time points was adminstered,the gel electrophoresis product of RT-PCR in fibroblast culture system expressed the mRNA of type Ⅰ,Ⅲ and Ⅳ collagens,MMP-1,-2,-9,TIMP-1 and -2;but the mRNA expression levels of type Ⅰ,Ⅲ and Ⅳ collagens,TIMP-1 and -2 decreased and the mRNA expression levels of MMP-1,-2,and -9 increased compared to those before XGA administration;the mRNA expression of type Ⅰ,Ⅲ and Ⅳ collagens,MMP-1,-2,-9,TIMP-1 and -2 decreased or increased gradually with the increase of doses and the prolonged time of XGA use.The mRNA expression levels of type Ⅰ,Ⅲ and Ⅳ collagens,TIMP-1 and -2 were negatively related to the doses and action times of XGA(r=-0.927 to -0.637 P= 0 to 0.024);and the mRNA expression levels of MMP-1,-2 and -9 were positively related to the doses and action times of XGA(r=0.672 to 0.962 P=0 to 0.034).Conclusion XGA can markedly reduce the collagen formation of myocardial fibroblasts in rats,and its mechanisms are related to the inhibiting of collagen synthesis and the increase of collagen degradation.

Effect of milkvetch injection on immune function of children with tetralogy of Fallot after radical operation / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To evaluate the effect of milkvetch injection (MI) on immune function of children with tetralogy of Fallot (TOF) after radical operation.</p><p><b>METHODS</b>Forty-children with TOF were divided into two groups, the 20 in the control group treated with conventional treatment alone and the 20 in the treated group treated with conventional treatment plus 15 ml of MI every 12 hrs for 14 days. Changes of immunoglobulin, complements, lymphocyte phenotypes and cytokines were observed.</p><p><b>RESULTS</b>In the treated group, the abnormally increased levels of IgG, IgM, C3, C4, CD8+ and CD19+ began to lower at lst-2nd week after treatment, and basically restored to the levels of normal at 3rd-4th week; while the decreased levels of IgA, CD3+, CD4+, CD4+/CD8+ ratio, CD3+/HLA-DR+ and CD3+/CD16+ -CD56+ raised gradually from the 1st week and restored to normal range at 2nd-3rd week. The IL-6 and tumor necrosis factor-alpha (TNF-alpha) levels in the plasma and supernatant, produced in vitro by peripheral blood mononuclear cells (PBMC) decreased gradually at 1st week and restored to the normal level at 3rd-4th weeks. The different value before and after treatment of the above-mentioned indexes in the treated group were superior to those in the control group (P<0.05 or P<0.01).</p><p><b>CONCLUSION</b>MI could significantly improve the immune function of children with TOF after radical operation.</p>

Temporary Cardiac Pacemaker Therapy on 9 Children with Adam-Stoke Syndrome / 实用儿科临床杂志

Objective To explore the value of temporary cardiac pacemaker therapy on children with Adam-Stoke syndrome(ASS).Methods Nine children with ASS was implanted the emporary cardiac pacemaker under X-ray or blinding inserted beside bed.Results Seven children was successfully implanted the temporary cardiac pacemaker within 1-2 d of ASS attack,and two children was successfully implanted at 10 d and 20 d after ASS attack, respectively. Five patients was cured, two children was died, and two children was implanted the permanent cardiac pacemaker. During pacing, two patients had the electrode shifted,and one patient had the cardiac murmur,and one patient had the local skin infected.Conclusion Temporary cardiac pacemaker can successfully treat children with ASS attack;the earlier the implantation,the better the prognosis.

Changes of insulin like growth factor - 1 in blood and cerebrospinal fluid in children with viral encephalitis / 实用儿科临床杂志

Objective To study the changes of insulin- like growth factor- 1(IGF-1)in blood and cerebrospinal fluid (CSF) in children with viral encephalitis (VE).Methods The IGF-1 levels in blood and CSF were determined before treatment by ELISA in 25 children who admitted with VE, including 15 cases with severe VE and another 10 cases with mild VE, 10 children served as con-trols. Results Before treatment, the blood IGF-1 levels in VE group were significantly lower than those of controls, but the CSF IGF-1 levels were significantly higher than those of controls(P0.05), but the blood IGF-1 levels in serve VE group were significanfly lower than those of mild VE group and controls(P

Effect of Milkvetch Injection on cardiac function and hemodynamics in children with tetralogy of Fallot after radical operation / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To evaluate the effect of Milkvetch Injection (MI) on cardiac function and hemodynamics in children with tetralogy of Fallot after radical operation.</p><p><b>METHODS</b>Thirty-two children with tetralogy of Fallot were divided into two groups, the 20 patients in the control group treated with conventional treatment and the 12 patients in the treated group treated with 15 ml of MI every 12 hrs for 7 days. The cardiac function and hemodynamic parameters were determined by echocardiography and thermodilution catheter inserted in pulmonary artery.</p><p><b>RESULTS</b>Patients' cardiac function and hemodynamic parameters were improved significantly 1 hr after administering 15 ml of MI Injection, but restored to the level before medication in 2-4 hrs. Administering MI injection for successive 7 days could gradually improve patients' cardiac function and hemodynamic parameters after operation, and the improvement became more evident with the prolonging time of medication. The intubation time, intensive care unit duration and the time of intravenously administration of inotropic drugs in the treated group were significantly shorter than those in the control group (P < 0.01).</p><p><b>CONCLUSION</b>MI could significantly improve the hemodynamics and cardiac function, and shorten the recovery time in children with tetralogy of Fallot after radical operation.</p>

Prognosis and Etiological Treatment of Cardiogenic Syncope in Children / 实用儿科临床杂志

Objective To explore the prognosis and etiological treatment of cardiogenic syncope in children.Methods The cause,clinical manifestation,treatment and prognosis of 45 children with syncope were made in affiliated hospital of Qingdao medical university were su mmarized and analyzed.They were divided into arhythmia group and other groups,the former included myocarditis,dilated cardiomyopathy,and post-operation of congenital heart disease and the latter included tetralogy of fallot,pulmonary stenosis,hypertrophic cardiomyopathy,pericarclial effusion,and right atrial myxoma.Results There were 29 cases(64.44%) with arrhythmia in this group,of which there were 18 cases with complete atrioventricular block(CAVB),2 cases with second degree type Ⅱ atrioventricular block,3 cases with ventricular tachycardia,2 cases with bradycardia,2 cases with atrial flutter,1 case with supraventricular tachycardia,and 1 case with nonconducting atrial premature.There were 16 cases(35.56%) with other etiologies,of which there 8 cases with tetralogy of Fallot,2 cases with pulmonary stenosis,hypertrop-hic cardiomyopathy,pericardial effusion,and right atrial myxoma,respectively.Children with CAVB,bradycardia,or ventricular tachycardia resulted from viral myocarditis were given anti-virus medicine,protecting myocardium,neoepinephrine,and intravenous vitamin C 100-200 mg/(kg?d).Dexamethasone 1-2 mg/(kg?d) was given in earlier period for short course,and some children were given human ?-globulin.Eight cases were placed temporary pacemaker because of unsatisfactory therapeutic efficacy,and 1 case was placed permanent pacemaker.In all,38 cases(84.44%)were cured,1 case(2.22%)was improved,and 4 cases(8.88%) died.Conclusions Cardiogenic syncope is pediatric emergency and shall be treated immediately.Temporary pacemaker shall be placed at the right moment.