Pulmonary arterial hypertension(PAH)is caused by pulmonary vascular remodeling, pulmonary arteriolar spasm, proliferation of vascular endothelial cells and smooth muscle cells, which gradually increase the pulmonary arterial pressure(PAP), eventually leads to right heart failure and even death.In recent years, due to the research of pathogenesis and the development of new drugs, the prognosis of patients with PAH has been significantly improved.The targeted drugs of PAH mainly act on different targets in three classic pathways of nitric oxide(NO), prostacyclin I 2(PGI 2)and endothelin-1(ET-1). Its application, combined application, surgical treatment and other methods have significantly improved the survival rate and life quality of patients with PAH.Compared with adults, children are more likely to have PAH associated with congenital heart disease(PAH-CHD)and idiopathic PAH(IPAH). As the onset age is young and pulmonary vascular lesions have not yet emerged, children can have a better prognosis.This paper reviews new treatment in children with PAH.

There is no consensus regarding pathogenesis of viral myocarditis in pediatric patients.It makes a big challenge in clinical diagnosis and treatment.The myocyte injury is wildly recognized to be initiated by viral proliferation and secondary host immune response.It may persist and lead to dilated cardiomyopathy.In serious cases, it could even end up with heart failure, cardiogenic shock and sudden death.A growing number of scholars consider that host immune response is the main cause of severe cardiomyopathy.This review outlines the current progress of immune response in viral myocarditis, aiming to assist the clinical diagnosis and treatment.

Hemoptysis in childhood is a common clinical manifestation whose severity depends on the rate at which hemoptysis occurs and the degree of bleeding.Hemoptysis occurs for a variety of reasons,among which hemopty-sis correlated with vessels is one of the factors.Although the incidence rate is not common,it is mostly the first cause of hemoptysis.Hemoptysis correlated with vessels exit a relatively backward diagnosis in clinical which due to the gradual process of the diagnosis,and its treatment and prognosis of the disease depends on the type,number,morphology of ab-normal vessels and degree of bleeding.It is important to earlier recognize hemoptysis correlated with vessels,develop a standardized diagnostic procedure,setup appropriate treatment plans and timing for medical staff so as to improve the diagnosis and treatment of the disease and reduce the incidence of complications and hemoptysis recurrence rate.

Objective To investigate pediatric hemoptysis recurrence related to vascular malforma-tions after transcatheter occlusion and offer suitable preventive actions for reducing the rate of hemoptysis recurrence.Methods The clinical data of 27 children,collected form Department of Cardiology,Children's Hospital of Chongqing Medical University between June 2012 and April 2017,with hemoptysis related to vascular malformations were retrospectively analyzed.The clinical manifestation,image feature and occlusion program of children with hemoptysis recurrence were re-analyzed and evaluated. Results All 27 children with hemoptysis received transcatheter occlusion, whose vascular malformations included bronchial-pulmonary artery fistula (24 cases,88.9%) and pulmonary arteriovenous fistulas (3 cases,11.1%) by angiography. Six cases,approximately 26.1%,suffered from recurrent hemoptysis after therapy,and the interval time of hemoptysis recurrence was roughly (5.6 ± 2.3)months.It indicated mycoplasma pneumonia infection in all children with hemoptysis recurrence, and re-angiography showed that more abnormal vessels were found, other minor vessels except for vessels occluded grew thick and large,vessels except for vessels occluded were recanalization in children with recurrent hemoptysis.Conclusion Recurrence is the common complication of hemoptysis related to vascular malformations in children,and bronchial-pulmonary artery fistula is the most common type with hemoptysis recurrence. The main causes of hemoptysis recurrence include mycoplasma pneumonia infection,vessels without occlusion enlargement and recanalization.

The effect of melatonin on juveniles with cardio fibrosis is poorly understood. We investigated whether HDACs participate in the anti-fibrotic processes regulated by melatonin during hypertrophic remodeling. Abdominal aortic constriction (AAC) was employed in juvenile rats resulting in pressure overload-induced ventricular hypertrophy and melatonin was subsequently decreased via continuous light exposure for 5 weeks after surgery. AAC rats displayed an increased cross-sectional area of myocardial fibers and significantly elevated collagen deposition compared to sham-operated rats, as measured by HE and Masson Trichrome staining. Continuous light exposure following surgery exacerbated the increase in the cross-sectional area of myocardial fibers. The expression of HDAC1, HDAC2, HDAC3, HDAC4 and HDAC6 genes were all significantly enhanced in AAC rats with light exposure relative to the other rats. Moreover, the protein level of TNF-α was also upregulated in the AAC light exposure groups when compared with the sham. However, Smad4 protein expression was unchanged in the juveniles' hearts. In contrast, beginning 5 weeks after the operation, the AAC rats were treated with melatonin (10 mg/kg, intraperitoneal injection every evening) or vehicle 4 weeks, and sham rats were given vehicle. The changes in the histological measures of cardio fibrosis and the gene expressions of HDAC1, HDAC2, HDAC3, HDAC4 and HDAC6 were attenuated by melatonin administration. The results reveal that melatonin plays a role in the development of cardio fibrosis and the expression of HDAC1, HDAC2, HDAC3, HDAC4 and HDAC6 in cardiomyocytes.
Animals ; Collagen ; Constriction ; Fibrosis* ; Gene Expression ; Heart ; Histone Deacetylases ; Hypertrophy ; Injections, Intraperitoneal ; Melatonin* ; Myocytes, Cardiac ; Rats* ; Smad4 Protein

Kawasaki disease,also known as multiple mucocutaneous lymph node syndrome,is a self-limiting and acute systemic vasculitis.Kawasaki disease is often complicated by coronary artery lesions.Kawasa-ki disease is a leading cause of coronary artery lesions in childhood in developed countries.The mechanisms of coronary artery lesions and new therapeutic regimens have been the hotpots in research of Kawasaki disease.This paper reviews current research on mechanism of coronary artery lesions and development of new therapeutic regi-mens.

ObjectiveTo investigate the relationship between microtubule-associated protein l light chain 3B (LC3-II) and the inlfammatory response of Kawasaki disease (KD).MethodsThirty-nine cases of acute KD before intravenous admin-istration of immunoglobulin were enrolled. According to the results of echocardiography, the 39 cases were furtherly divided into KD with coronary artery lesion (CAL, 20 cases) group and KD with non-CAL (NCAL, 19 cases) group. At the same time, 12 healthy children were selected as controls. Serum samples were collected and cultured in vitro by human coronary artery endothe-lial cells (HCAEC) for 12 h. The LC3-II protein and mRNA expression of HCAEC were detected by Western-blotting and Q-PCR respectively.ResultsThe LC3-II protein and mRNA expression in CAL group and NCAL group were signiifcantly higher than those in control group, the LC3-II protein and mRNA expression in CAL group was higher than those in NCAL group, and both differences were statistically signiifcant (P<0.05).ConclusionsAutophagy may be involved in the inlfammatory response of KD in acute phase, which may be related to endothelial cell lesions of coronary artery in children with KD.

Incidence of dilated cardiomyopathy of children is increasing in recent years. But, the pathogenesis remains obscure. Genetic factor. viral infection, autoimmunity and apoptosis may play some important roles in the pathogenesis of dilated cardiomyopathy. We will summarize the progress in pathogenesis of dilated cardiomyopathy of children.

Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a cardiac muscle disease characterized by peculiar right ventricular involvement that precipitates ventricular arrhythmias and sudden death.ARVC is a major cause of sudden death in the young and athletes.It is familial origin in 50% to 70% of cases,which follows an autosomal in heritance pattern.The pathology consists of a genetically determined dystrophy of the right ventricular myocardium with fibrofatty replacement.Clinical diagnosis can be achieved by demonstrating functional and structural alterations of the right ventricle,depolarization and repolarization abnormalities,arrhythmias with the left bundle branch block and fibro-fatty replacement through endomyocardial biopsy.Clinical research has indicated that successful treatment of ARVC is based on lifestyle modifications,prophylactic therapy(antiarrhythmic drugs,catheter ablation and implantable cardioverter defibrillator)to prevent sudden death.

Objective:To explore the effect of 5-hydroxytryptamine(5-HT) on proliferation of bone marrow stromal cells(BMSCs) in vitro.Methods:Murine BMSCs colony-forming unit assay and human bone marrow mononuclear cells count assay were used respectively to determine the effect on proliferation of BMSCs with different dose 5-HT(0,50,100,200,500nM) and 5-HT combined with basic fibroblast growth factor(bFGF),platelet-derived growth factor(PDGF),and ventricular endothelial growth factor(VEGF).Reversal transcript polymerase chain reaction(RT-PCR) was used to detect expression of mRNA of 5-HT2 receptor subtypes.Results:The results showed that 5-HT significantly stimulated murine colony-forming unit formation(CFU-F) proliferation in a dose-dependent manner(50 to 500nM) using murine bone marrow cells( n =3).Maxmum stimulation was obtained with 200nM of 5-HT( P =0.03).The effect of 5-HT on murine CFU-F proliferation was compared with that of bFGF,PDGF and VEGF at their optimal dose.The stimulating activity of 5-HT was the same as that of PDGF and VEGF,but lower than that of bFGF.A significantly enhancing effect was observed in 5-HT plus bFGF,PDGF,and VEGF,respectively.We also found that 5-HT,or 5-HT plus bFGF,PDGF,and VEGF had similar effect on human bone marrow stromal cells proliferation.There were expression of mRNA of 5-HT 2A,2B and 2C receptor subtypes on human bone marrow stromal cells.Conclusion:These data indicate that 5-HT has a significant mitogenic effect on BMSCs in vitro and enhance proliferating effect of bFGF,PDGF,and VEGF on BMSCs.This effect of 5-HT on BMSCs appears to be mediated via 5-HT2 receptor subtypes.