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??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.     

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??Abstract??Objective To evaluate the clinical effect of transcatheter closure for ventricular septal defect in infants and young children. Methods Clinical data from 184 children under 3 years old who underwent the transcatheter VSD closure from Jan. 2011 to Dec. 2012 were analyzed. Results Procedure was successful in 176 cases ??95.7%??. No death occurred?? residual shunt in 13 cases?? tricuspid inflow accelerated in 2 cases?? late-onset complete atriovenous block in one case ??6 months after procedure??. All cases had no vascular complications. Conclusion Transcatheter closures for ventricular septal defects are effective in small children. Long-term follow-up is needed to evaluate the safety of this operation. The keys to the success of operation are the proper indications,suitable apparatus,skillful vascular punction and good anaesthetic management.     

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??Abstract?? Objective To evaluate effectiveness and safety of treatment for children’s acute bronchiolitis by nebulized recombinant human interferon α 1b in a multicenter clinical study??and to recommend a reasonable clinical dosage for children. Methods Withthe design of a randomized, controlled, multi-center study, totally 330 children who were hospitalized from December 2012 to May 2013 for acute bronchiolitis were randomly divided into three groups. The control group had routine and symptomatic treatment. Therapy groups inhaled human recombinant interferon α1b 2 μg/kg and 4 μg/kg respectively, twice a day plus routine treatment, for 5 to 7 days. We used four-level-scoring method to evaluate disease conditions and performed pathogen detections. Results ??1??The total improvement rates of low and high dose group were 92.3% and 95.0%, respectively, which were significantly higher than control group ??85.3%, P<0.05??. High dose group had obviously improved primary index ??wheezing, wheezing rale and three depression sign?? compared with low dose group. The disappearance time of symptoms was shortened significantly ??P<0.05??, and the improvement of three depression signs was obvious ??P<0.01??.??2??Treatments within and after 72 h were both effective, but the total improvement rate within 72 h was better.??3??The effectiveness of interferon α1b in RSV positive children was significantly better than in RSV negative children. Effectiveness of treatment group was superior to that of control group.??4??All children did not show irritation symptoms in local respiratory tracts, and no serious adverse reactions was observed. Conclusion The treatment for acute bronchiolitis in young children by nebulized human recombinant interferon α1b is effective, which shortened the duration of symptoms, with no safety problem. The effect is better when used in the early stage.     

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??Fulminant hepatic failure refers to the severe liver function damage?? caused by multiple factors in the onset of 8 weeks of hepatic encephalopathy and severe blood coagulation dysfunction?? with onset nasty?? bad prognosis?? and high fatality rate. For the past many years,there have been no effective treatment methods. In recent years?? artificial liver support system and liver transplantation in the treatment of fulminant liver failure have more and more been taken seriously. In this paper?? the etiology?? diagnosis and treatment of FHF in recent research will be summarized.     

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??Abstracts?? Objective To investigate the changes of ventricular late potentials ??VLP?? in children with vasovagal syncope of vasodepressor response ??VVS-V??. Methods Totally 184 children ??aged from 4 to 14 years old??the average age being ??10.54±2.15?? years old?? had been diagnosed with VVS-V by head-up tilt table test ??HUTT?? ??study group?? from July 2006 to January 2013 in the Second Xiangya Hospital of Central South University?? male 85 cases?? the average age of ??10.85±2.01?? years old?? female 99 cases?? mean age ??10.27±2.24?? years old. Totally 105 healthy children were matched as controls. All subjects underwent VLP examination. Investigate the difference in HR?? TQRS?? LAS40 and RMS40 between the study group and the control group. Results Compared with the control group?? in the study group heart rate ??HR?? decreased ???83.96±12.27?? beats/min vs??87.28±13.75?? beats/min?? t = -2.113?? P??0.05???the total QRS time??TQRS?? ???84.89±12.05?? ms vs ??81.21±8.23?? ms?? t = 3.070?? P??0.05?? and high frequency and low amplitude limit??LAS40?? ???62.43±19.17?? ms vs ??56.79±1.83?? ms?? t = 2.442?? P??0.05?? were prolonged??and the root mean square plant??RMS40?? increased ???28.73±7.23?? μV vs ??26.89±7.36?? μV?? t = 2.059?? P??0.05??. Conclusions Compared to healthy controls??in VVS-V children HR reduces??TQRS and LAS40 prolong??and RMS40 increases. It suggests that VVS-V childrenare more likely to have cardiovascular incidents.     

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??The true incidence of fulminant hepatic failure in the pediatric population is unknown?? though fulminant hepatic failure is a rare but devastating syndrome??such as hepatic encephalopathy??DIC??MODS?? that results in the death of most children affected. The mainstay of the treatment is liver transplantation??however?? organ shortage limits its use.The purpose of this review is to introduce the current situation of fulminant liver failure in children??focusing on assessing the application of artificial liver??which is considered to play a pivotal role in the treatment of fulminant liver failure.According to its classification??we mainly discussed the application of non-biological artificial liver?? including its indications??advantages and disadvantages??especially the MARS. At present?? the artificial liver treatment also faces many problems. None of the ELS techniques has yet been evaluated systematically in children??and survival benefits have not yet been demonstrated.     

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??Abstract?? Objective To analyze ALDH3A2 mutation in four Chinese patients with Sjögren-Larsson syndrome ??SLS??. Methods Four patients were clinically diagnosed with SLS. Respectively take 3 ml of peripheral blood. All 11 exons and exon-intron boundaries of ALDH3A2 gene were amplified by polymerase chain reaction ??PCR?? and directly sequenced for genomic DNA. Results 1. All four patients had congenital ichthyosis?? mental retardation??and spastic diplegia or tetraplegia. Patient 1 had a compound heterozygote??c.1157A??G inherited from her father?? IVS5-1del G inherited from her mother. Both her parents had normal phenotype. Patient 2 and Patient 3 were siblings?? they were both homozygotes??a A-to-G transition at nucleotide 1157 in exon 8. The heterozygosity was demonstrated in their mother. Both her parents had normal phenotype. Conclusion Two different mutations were examined in these 4 Chinese patients?? and the SLS cases were confirmed by ALDH3A2 mutation analysis.     

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??Abstract?? Objective To identify the clinical features and the prognosis of anti-NMDAR encephalitis in children??so as to improve the pediatrician’s awareness about this disease. Methods The clinical features??therapeutic regimen and prognosis of 17 children with anti-NMDAR encephalitis were summarized by a retrospective and follow-up study. Results Among the 17 patients with anti-NMDAR encephalitis??12 were males and 5 were females.Their age varied from 2 to 12 years??and the average age was 5 years 8 months.The most common initial symptoms were convulsions ??7 cases?? and psychiatric symptoms ??7 cases??.The common symptoms in the course of the disease were psychiatric symptoms??17 cases????sleep disorders ??16 cases????extrapyramidal symptoms ??14 cases????and convulsion ??11 cases??.Only 4 patients had autonomic symptoms.The anti-NMDAR antibody were found in all the patients’CSF.The EEG test of 16 patients showed diffused slow background.Tumors were not found in any patient. Fourteen cases were treated with IVIg and methylprednisolone??2 cases only received IVIg therapy and 1 case gave up.Rituximab ??2 cases?? and cyclophosphamide ??1 cases?? were administrated in 3 cases with no improvement after the above treatment.The patients had been followed up for 3 to 15 months.Twelve of them recovered completely??2 cases died??2 cases had epilepsy??and 1 case had speech disorder. Conclusion Psychiatric symptoms??sleep disorders??extrapyramidal symptoms and convulsion are the common symptoms of anti-NMDAR encephalitis in children.Tumors and autonomic nervous system dysfunction rarely happen. Immunotherapy is effective in the majority of the patients.The prognosis of anti-NMDAR encephalitis in children is optimistic.     

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??Abstract?? Objective Toinvestigatethe correlation among exhalednitric oxide??lung function and Asthma Control Test inasthmatic childrenand the clinicalsignificance. Methods A total of 136 cases of asthmatic children aged 5 to 15 years old were recruited from Shengjing Hospital of China Medical University.The fraction of exhaled nitric oxide?? lung function and ACTscore were measured??and the correlation among them was analyzed. Results Negative correlation was found between FeNOand ACT??r= -0.251??P<0.005??.Negative correlation was found between FeNOandFEV1/FVC of lung function??r=-0.206??P<0.05????but not FEV1or PEF.Positive correlation was found betweenACT scoreandFEV1/FVC of lung function ??r=0.242??P<0.01???? but not FEV1or PEF. Conclusion For all subjects??FeNO is negatively correlated with ACT score and FEV1/FVC.ACT is positively correlated with FEV1/FVC.When the subjects are grouped??the three don’t have stable correlations.FeNO is a good biomarker to evaluate the airway inflammation of asthmatic children.ACT score evaluates the level of asthma control in children.Lung function evaluates airway obstruction.FeNO??lung function andACTare different measurements that evaluate asthma in different ways.None of them can be used instead of another in clinical practice??and they are complementary.Combinedapplicationscan effectivelyimprove the diagnosis??treatment and asthmacontrol inasthmatic children.     

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??Abstract??Allogeneic hematopoietic stem cell transplantation ??HSCT?? is the only curative therapy for severe beta-thalassemia major. Patients with an available human leukocyte antigen ??HLA?? identical sibling donor should be offered HSCT as soon as possible before development of iron overload and transfusion associated complications?? and the cure rate was up to 80%??90%. High-resolution HLA typing matched unrelated donor transplants also achieved good results. HSCT from HLA-mismatched relatives or HLA-phenotypically-identical donor is an option to be performed in expert centers. Optimization of conditioning regimen and techniques to control transplant-related complications will further improve outcomes.