Kawasaki disease has been the major etiology of aquired heart problems in children. Mecha-nisms of Kawasaki disease are still unclear in the past years. Some Kawasaki disease patients were not responsive to intravenous immunoglobulin therapy. And corticosteroid therapy remains controversial in the treatment of Ka-wasaki disease. A few patients with Kawasaki disease presented severe systemic symptoms,and even died for it. It is difficult to evaluate the severity of coronary artery lesions and prognosis of Kawasaki disease. In this article, we reviewed some new concepts on diagnosis and treatments on Kawasaki disease.

Marfan's syndrome(MFS) is a relatively common autosomal dominant hereditary disorder with prominent manifestations in the skeletal, ocular, and cardiovascular systems. MFS has a high penetrance. Recent research on the pathogenesis and diagnosis and therapy of MFS has made a lot of progress. So we reviewed the advance on the relation of molecular genetics and phenotype of MFS.We discussed the molecular hasis, gene mutation and location, diagnosis, clinical features and therapy. Now the detection on the molecular level has been a very important method in diagnose MFS very early or before bom.

Objective To study the value of clinical application for X-ray diagnosis in myodystrophy followed by osteoarticular change such as scapula,etc. Methods Different osteoarticular position,size,structure were observed on X-ray films.X-ray features of dysostosis,malformation and dislocation of joint caused by myodsytrophy were analysed in three cases.Results X-ray findings of myodsytrophy in 3 cases included:revolving and displacement of scapula,scapula getting shorterand smaller,dislocation of acromioc cleido joint and shoulder joint and bending and malformation of shoulder joint.Conclusion It can be certain that myodsytrophy directly causes the maltormation of osteoartthritis to make a diagnosis for one who suffers from a myodystrophy with obvious osteodysplasty by X-ray and find out the harm of this kind of disease.Finally we can predict how it goes in anaphase.

Objective To study the effect of active ingredients(saikosaponin) of bupleurum chinensis D C.on GABA of hippocampus of chronic kindling induced by pentylenetetrazol(PTZ) in rats.Methods Forty-eight healthy Sprague-Dawley rats were randomly divided into six groups with eight in each group: normal control(Group A), normal saline(NS) group(Group B),sodium valproate(VPA) group(Group C),group of large-dose saikosaponin(Group D),group of medium-dose saikosaponin(Group E),and group of small-dose saikosaponin(Group F).Except Group A to be controlled,the other groups were given different treatments when chronically kindled by PTZ.The process of the test lasted for 4 weeks.After 4 weeks' administration the brain tissues were sampled,sliced and stained by enzyme immunohistochemistry(EIH).The results were analyzed according to the positive cell population and gray value.Results The positive cell population of CA1,CA2 and DG in group B was significantly different from that in the other groups(P

AIM: To observe the ability of dendritic cells (DC) vaccine transfected with human hepatocellular carcinoma (HCC) total RNA induce specific cytotoxic T lymphocyte(CTL) response in vitro. METHODS: DCs generated from HCC patient's peripheral blood mononuclear cells (PBMC) were incubated with recombinant human granulocyte macrophage colony-stimulationg factor (GM-CSF) and human interleukin (IL-4). Tumor total RNA was isolated from Hep G-2 cells and HCC cells. DCs transfected with tumor total RNA were used to induce specific CTL proliferation. Specific cytotoxicity was measured using MTT method. RESULTS: DC transfected with HepG-2 cell RNA and HCC RNA exhibited increased expression of CD83, CD86 and HLA-DR. The CTL from DCs transfected with HepG-2 cell RNA killed 5.84%, 14.26%, 25.19%, or 35.78% of HepG-2 cells, and 5.26%, 11.67%, 14.68%, or 23.24% of HCC cells, respectively, at an E/T ratio of 2.5, 5, 10, or 20. The CTL from DCs transfected with HCC cells RNA killed 4.65%, 12.23%, 15.61%, or 19.15% of HepG-2 cells, and 7.20%, 12.83%, 27.21%, or 31.15% of HCC cells,respectively, at an E/T ratio of 2.5, 5, 10, and 20. These CTL did not kill allogeneic malignant cells as human gastric carcinoma cells SGC-7901. CONCLUSION: DC transfected with tumor-derived total RNA could induce specific antitumor immune CTL response. These results suggest that CTL generation is applicable to adoptive immunotherapy of HCC.

Objectives To explore the clinical features and pathogenesis of Kawasaki disease (KD) combined with sterile pyuria. Methods A total of 420 patients diagnosed of KD were recruited and divided into pyuria group ( 95 patients) and control group ( 325 patients) according to urine routine examination on admission. The clinical data between the two groups were compared. Results There was no difference in gender, age, and the incidence of atypical KD (P all?>?0 . 05 ). The levels of C-reactive protein, D-dimer concentrations, fibrinogen degradation products, alanine aminotransferase, aspartate aminotransferase, and urine retinol binding protein were higher in pyuria group than those in control group (all P>?0 . 05 ). No difference was found in the duration of fever before admission between two groups (P>?0 . 05 ). However, pyuria group had longer duration of fever after treatment with immunoglobulin (P?0.05). Conclusion The morbidity of sterile pyuria in KD patients was 22 . 6%. KD patients with sterile pyuria have more intense inlfammatory response, markedly high coagulation condition, and mild or subclinical renal damage.

Objective To improve the knowledge of paediatric pulmonary arterial hypertension(PAH) and to elevate the level of early diagnosis and treatment.Methods The clinical data of 45 PAH patients admitted in Shanghai Children's Hospital from Jan.2006 to Dec.2012 were reviewed,including clinical manifestation,laboratory examination,diagnosis,treatment and prognosis.Results Of the 45 PAH patients,21 cases (46.7％) were male and 24 cases (53.3 ％) were female,with an average age of 2.5 years old.Among them idiopathic PAH was in 24 cases (53.3 ％) and secondary PAH was in 21 cases [including interstitial lung disease in 4 cases,upper airway obstruction in 3 cases,systemic lupus erythematosus in 3 cases,hepatic disease in 3 cases,including hepatic hemangioma 1 case,liver cirrhosis portal hypertension 1 case and autoimmune hepatic sarcoidosis 1 case,tachycardia induced cardiomyopathy in 2 cases,extensive pulmonary small artery stenosis in 2 cases,human immunodeficiency virus (HIV) infection in 1 case,hypothyroidism in 1 case,and familial PAH in 2 cases].Main clinical manifestations were anhelation after exercise (71.1％),fatigue (68.9％),cough (48.9％),chest tightness (26.7％),chest pain (33.3％),syncope (8.9％),et al.The most common physical signs were splitting of the second sound in pulmonary valve area (93.3％),followed by tricuspid murmur (77.8％),cyanosis (53.3％),hepatomegaly (42.2％),hydropericardium (28.9％),and oedema (11.1％),et al.Twenty-seven cases did cardiac catheterization,compared with idiopathic PAH and secondary PAH,pulmonary artery systolic pressure,mean pulmonary artery pressure,pulmonary capillary wedge pressure,pulmonary arteriolar resistance index had significant difference(P ＜ 0.05).Thirty-one cases' condition improved after treatment,11 cases without any improvement and 3 cases died during follow-up visit.Conclusions PAH is a rare disease with no specific symptom and can be easily misdiagnosed in children.Ultrasonic cardiogram and cardiac catheterization are helpful in diagnosis.Actively looking for the cause can improve the prognosis.

Objectives To explore the effect of mibefradil, a kind of novel calcium channel antagonists, on proliferation of human pulmonary artery smooth muscle cells (HPASMCs) induced by platelet-derived growth factor (PDGF). MethodsHPASMCs were culturedin vitro, and randomly divided into control group, PDGF group, Mib group, and PDGF+Mib group. The PDGF group was stimulated by 25 ng/ml of PDGF. Mib group was intervented by 10 μmol/L of Mib. PDGF+Mib group was treated by PDGF and Mib. The reproduction rate in 48 hours and 72 hours were detected by MMT. Cell cycle was detected by lfow cytometry. The expression of proliferating cell nuclear antigen (PCNA) was observed by immunolfuorescence staining (IFS).ResultsThere were statistical differences among four groups in both 48 hours and 72 hours (P all??0.05). There were statistically differences of G0/G1 phase and S phase among four groups (P?

Objective To explore the clinical characteristics and treatment of paroxysmal supraventricular tachycardia (PSVT) in children. Methods The clinical data of 67 children with PSVT were analyzed retrospectively, and the therapeutic effects of different treatments were compared. Results The clinical manifestations of infants were paleness, shortness of breath, irritability and sweating, and children showed chest tightness, palpitations, abdominal discomfort and fatigue. The curative effect of electric cardioversion, transesophageal atrial pacing, physical therapy, and drug therapy was statistically different (P<0.05), The different cardioversion rates of them were observed for the treatment of paroxysmal supraventricular tachycardia. The cardioversion rate of transesophageal atrial pacing, was the highest, and the rate of physical therapy was the lowest. There was no significant difference in the cardioversion rate between propafenone, digoxin and amiodarone. Conclusion The clinical manifestations of PSVT in infants are atypical and easily to be ignored. There are many methods for treatment of PSVT. The vagus nerve can be stimulated first, and, if no response, either drugs or electric cardioversion and transesophageal atrial pacing can be used. The cardioversion rate of transesophageal atrial pacing is higher. The drug effectiveness for the treatment of PSVT depends on many factors, and our choice of medication varies from person to person.

ObjectiveTo investigate the effects of losartan on angiotensin (Ang)Ⅱ-induced the generation of oxidative stress and expression of transforming growth factor β1(TGF-β1) in rat proximal tubular epithelial cells and to explore its underlying mechanism. MethodsNRK-52E cells, a rat proximal tubular epithelial cell line, were applied to explore the antioxidationand antifibrosis of losartan. The expression of three subunits of nicotinamide-adenine dinucleotide phosphate (NADPH) oxidase, including p47phox, Nox-4, p22phox, and TGF-β1 were determined by real-time RT-PCR and/or Western blot. The generation of reactive oxygen species (ROS) was measured by DCF fluorescence analysis. Superoxide dismutase (SOD) in the supernatant was measured by colorimetric method. Results10-7 mol/L Ang Ⅱ up-regulated p22prox, p47phox and Nox-4 mRNA and protein expression, and the mRNA increased by 5.57-fold, 5.55-fold and 9.41-fold at 24 h (P<0.01, respectively) and the protein increased by 4.53-fold, 4.17-fold and 6.50-fold at 24 h (P<0.01, respectively) as compared with control. Losartan greatly reduced the mRNA elevation of p22prox, p47phox and Nox-4 by 2.71-fold, 2.18-fold and 5.23-fold (P<0.01, respectively) and reduced the protein elevation by 3.20-fold, 2.30-fold and 4.30-fold (P<0.01, respectively) as compared with control. Losartan also inhibited ROS generation induced by Ang Ⅱ in rat proximal tubular epithelial cells. SOD level in the supernatant was markedly decreased after Ang Ⅱ stimulation, while losartan could increase SOD levels (P<0.01). Furthermore, losartan signficantly inhibited Ang Ⅱ-induced TGF-β1 mRNA up-regulation by 64% (P<0.01). ConclusionsLosartan acts as an anti-oxidative and anti-fibrotic agent via the mechanisms of blocking NADPH oxidase-dependent oxidative stress and inhibiting TGF-β1 expression.