Left ventricular noncompaction(LVNC) is a relatively rare cardiomyopathy due to the cessa tion of endocardial and myocardial cells in the early embryo development.The disease mainly is involved in the left ventricle.LVNC is sporadic or familial genetic disease,which has obvious genetic heterogeneity.It is X linkage and autosomal dominant inheritance.The genetic characteristics of LVNC are not single inheritance,and its pathogenic gene and mutation site are diverse.It has been shown that LVNC has a close correlation with multiple gene mutations,and it is also overlap with the genes that caused other cardiomyopathy.In this paper,we will review the research progress of LVNC and related gene mutations.

Objective To investigate the differences of clinical characteristics and disease severity between respiratory syncytial virus (RSV) and non-RSV bronchiolitis.Methods Ninety-six bronchiolitis inpatients of Shengjing Hospital from November 2012 to March 2014 were enrolled.RSV detection was performed at enrollment by direct immunofluorescence assay and the patients were divided into RSV group and non-RSV group.Clinical characteristics were collected and compared, including patients' demographic data, allergy, etc.Clinical scores were assessed by modified-Tal score system, and the associations between clinical scores and duration of onset or length of stay were analyzed to assess disease severity.Results In all 96 patients, forty (46.17％) were determined to be infected with RSV.Patients with RSV bronchiolitis were younger at hospitalization (t =-2.706,P =0.008) and the weight was lower (t =-2.070, P =0.041).There was no significant differences in sex, birth way, birth weight, height, ete (P ＞ 0.05).There was no significant differences in eczema, history of family allergy and total serum IgE (P ＞ 0.05).For patients with RSV bronchiolitis, the clinical scores were higher (t =-0.405 ,P ＜0.001) and the length of stay trended to be longer (t =0.480 ,P =0.081).The clinical score had a positive relation with length of stay (r =0.305, P =0.003), but not with the duration of onset (P ＞ 0.05).Conclusion Compared with non-RSV bronchiolitis, the clinical characteristics of patients with RSV bronchiolitis are younger age and lower weight, and have higher clinical scores, which indicates the clinical conditions may be more severe.For bronchiolitis, there is a positive correlation between clinical score and length of stay.The combination of etiological detection and modified-Tal score system may help to recognize severe cases, which should be observed carefully and treated immediately.

Objective To investigate the effects of mycoplasma pneumoniae(MP)infection on the exhaled nitric oxide level and lung function in asthmatic children,analyze the correlation between exhaled nitric oxide level and lung function and to provide reference for the treatment and monitoring of asthmatic children.Methods Sixty-eight children aged from 5 to 13 years old with mild or moderate asthma during June 2011 to January 2013 were collected.MP-IgG antibody,MP-IgM antibody,MP-DNA,total serum IgE,FeNO measurement and spirometry were examined on the next day morning.They were divided into two groups according to the pathogen:ashtma with MP group and ashtma without MP group.Then the levels of nitric oxide level and lung function indicators between the two groups were analyzed.Results The FeNO level in ashtma with MP group was significantly higher(P<0.05).There were no differences between two groups in lung function parameters such as FVC,FEV1,FEV1 / Vcmax,MEF25 and MEF50(P>0.05),but differnces were found in MEF75 and PEF(P<0.05).No correlation was found between FeNO levels and lung function parameters(FVC,FEV1,FEV1/Vcmax,MEF50,MEF25,MEF75 and PEF).Conclusion In asthma children with MP infection,the FeNO level was significantly increased and no correlation was found between FeNO levels and lung function.

Objective This retrospective study was based on 1 415 cases that had been done the flex-ible bronchoscopy examination.The data were analysed to investigate the value of flexible bronchoscope in the children's respiratory system diseases diagnosis,treatment and etiological study.Methods A total of 1 415 cases who admitted from June 2012 to December 2013 were included in the study and they were all met the inclusion criteria,had complete clinical data,done bronchoscope examinations,abnormal in the broncho-scope and diagnosed definitely.The endoscopic manifestation,clinical symptoms,X-ray film,laboratory data were analysed.Results In 1 415 cases,55.4% were boy,and 55.5% were younger than 5 years.Two cases (0.14%)were laryngeal cartilage soften,one case(0.07%)was epiglottic cyst,3 cases(0.21 %)were tra-cheomalacia,25 cases(1.8%)were bronchial foreign bodies,20 cases(1.4%)were tracheal bronchus de-formity,8 cases(5.7%)were tracheal stenosis,two cases(0.14%)were bronchial bridge,5 cases(0.35%) were bronchiolitis obliterans,6 cases (0.42%)were bronchiectasis,one case(0.07%)was immotile cilia syndrome,10 cases (0.71%)were bronchial tuberculosis,one case (0.07%)was aspergillosis,one case (0.07%)was pulmonary hemosiderosis,2 cases (0.14%)were pulmonary arteriovenous fistula,9 cases (0.63%)were plastic bronchitis,1 316 cases(93%)were founded tracheal intima inflammation,including the 350 cases(24.7%)of edema,mucosal folds form,279 cases(19.7%)of mucus plug obstruction,176 cases(12.4%)of suppurative obstruction,355 cases(25.1 %)of tracheal mucosal erosion necrosis,156 ca-ses(1 1.1 %)of wall fibrosis,stenosis,occlusion.Mycoplasma pneumoniae was the most common pathogen dectected in alveolar lavage.We also found that mycoplasma pneumonia easily combined the infection of bac-teria.A total of 1 19(22.7%)cases were no pathogens detected.In 1 415 cases,the main adverse reaction in the operations was hypoxemia caused by airway obstruction.Conclusion Flexible bronchoscopy examination is a very safe and reliable operation in diagnosis and treatment of respiratory diseases in pediat-rics,and plays an important role in the diagnosis of congenital developmental airway diseases,detection of pneumonia patho-gens and the treatment of lobe pneumonia.

Objective To investigate the advantages of pediatric electronic fiber bronchoscope ( FBO) in the infant bronchial foreign body,discuss the clinical features of infant bronchial foreign body,lung imaging characteristics and the kinds of microscopically position,the change of airway mucosa after stimulation by for-eign body under local anesthesia in 30 cases of infant bronchial foreign body. Methods Thirty cases,aged 0 to 3 years,were collected from September to December,2014. All of them were with foreign bodies examined by FBO in pediatric bronchoscopy room in Shengjing Hospital of China Medical University. Results In all infants, 6 cases (20. 0%) without history of inhaled foreign bodies and 24 cases (80. 0%) with a record history of in-haled. In the aspects of signs:normal breath sounds with a history of no choking cough in children were 2 cases (6. 7%) ,wheezing sounds were 3 patients (10. 0%) and weakened side breath sound was 1 case (3. 3%);with a history of choking cough in children,6 cases(20. 0%) with normal breath sounds,12 cases(40. 0%) with wheez-ing,6 cases(20. 0%) with lateral breath sounds less. Lung imaging characteristics was lack of specific perform-ance:only a case of all(n=30)show foreign body directly. Otherwise,other 29 cases had no specificity. Lung em-physema in 13 cases (43. 3%) is the main characteristic,while normal imaging findings in 2 cases (6. 7%). For-eign bodies in 19 cases were in the left lung (63. 4%) and 21 cases(70. 0%) of foreign body stimulated granula-tion inside airway,necrosis sputum bolt in distal obstruction of airway occured in 5 cases (16. 7%). Inhalled time of foreign body in airway was 4. 5 [2. 8,12. 5] day and inhalled time of foreign body in airway correlation coefficient with granulation hyperplasia(r=0. 688,P=0. 000),there was a significant correlation. Main adverse reaction was low oxygen in 6 cases (16. 7%). Conclusion The diagnostic accuracy of FBO under local anesthesia on children is high-er than other methods,and the FBO bronchial foreign bodies under local anesthesia is a safe and effective method.

Objective To investigate the clinical,laboratory and imaging characterstics of refractory Mycoplasma pneumoniae pneumonia(RMPP) with lobar pneumonia change and efficacy of the therapy with different doses of glucocorticoids in children.Methods The clinical data of 103 children with Mycoplasma pneumoniae pneumonia(MPP) between September 2015 and February 2016 were investigated.Among the 103 children,there were 52 cases of RMPP and 51 cases of non-refractory MPP.The clinical features,laboratory examination and imaging characteristics were compared between the two groups.For the children with RMPP,the change of clinical symptoms and imaging were observed after the treatment with routine dose and large dose of glucocorticoids.Then,for the children with poor imaging,fiber bronchoscope can be used,the differences of airway mucosa injury and immune cells in the bronchoavleolar lavage fluid were compared after the treatment with routine dose and large dose of glucocorticoids.Results The children in the RMPP group had longer febrile time and hospital stay and were more likely to suffer from extrapulmonary complications.Peripheral blood neutrophil count,CRP,PCT,LDH and D-dimers were higher than these in the MPP group.At the same time it was more common that two or more pulmonary lobes were involved synchronally or pleural effusion appeared,the differences were statistically significant(P ＜ 0.05);There were no statistical differences that the clinical symptoms,imaging change,airway mucosa injury and the proportion of immune cells in BALF between the children in the RMPP group after the treatment with routine dose and large dose of glucocorticoids (P ＞ 0.05).Conclusion It should be alert to the occurence of RMPP in children with MPP when there was a persistent fever,extrapulmonary complications,increased levels of inflammatory index significantly,pleural effusion or two or more pulmonary lobes involvement.Compared with the treatment with routine dose of glucocorticoids in children with RMPP,it does not show a clear advantage with large dose of glucocorticoids on the clinical symptoms and inhibition of airway mucosa injury.

Allergen immunotherapy(AIT)is considered the treatment capable of modifying the natural history of allergic respiratory disorders.The adverse reactions associated with AIT limit its clinical use in moderate to severe allergic asthma.Omalizumab is currently approved for the treatment of allergic asthma, chronic spontaneous urticaria, allergic rhinitis and other allergic diseases.A few trials have demonstrated the clinical efficacy of AIT and omalizumab combination therapy in children with moderate to severe allergic asthma.This review summarizes the research progress, mechanisma and application of omalizumab combined with AIT in children with moderate to severe allergic asthma.

Objective To analyze the effects of Hual qi huang granules on children with mycoplasma pneumoniae pneumonia.Methods A randomized,multicenter parallel controlled clinical trial was carried out.A total of 3 000 cases of hospitalized children with mycoplasma pneumoniae pneumonia were selected.All of them were given treatment for mycoplasma pneumoniae pneumonia with macrolide antibiotics and symptomatic treatment.They were randomly divided into 2 groups:research group and control group.The children of research group were give oral Huai qi huang granules for three months.According to the classification of pneumonia,these two groups were divided into:lobar pneumonia research group,lobar pneumonia control group,lobular pneumonia research group,lobular pneumonia control group.The hospitalization duration of fever,length of hospital stay,the absorption area of lung inflammation and pneumonia severity sores were observed.The frequency of upper respiratory infections,bronchitis,pneumonia were observed in 3 months after discharge.Results 2 378 cases were investigated.The hospitalization duration of fever,length of hospital stay of research group were significantly shorter than that of in control group (P ＜ 0.001).The children with lobar pneumonia,2 weeks after treatment,the absorption of consolidation of the lobar pneumonia research group is significantly better than lobar pneumonia control group (P ＜0.001).After two weeks treatment,the pneumonia scores of lobar pneumonia research group is lower than lobar pneumonia control group (P ＜ 0.05).Followup of 3 months after hospital discharge,frequency of upper respiratory infection and bronchitis of research group,were significantly lower than that of control.In addition,appetite increased significantly in research group than control (P ＜ 0.001).There are 21 cases with drug associated adverse reactions (mild diarrhea),including 12 cases of research group,9 cases of control group,and there was no statistical significance (P ＞0.05).Conclusion Standard treatment combined with oral Huai qi huang granules in the treatment of mycoplasma pneumoniae pneumonia,can significantly shorten hospitalization duration of fever,length of hospital stay and reduce the severity score of pneumonia.Three months oral Huai qi huang granules can significant reduce the frequency of respiratory infections and bronchitis,also can increase patients appetite,and be safe.