Objective To study the effect and toxicity ofgefitinib combined with selected radiotherapy in the treatment of patients with advanced non-small-cell lung cancer (NSCLC). Methods From March 2006 to February 2009,10 of 13 advanced NSCLC patients who got benefit from gefitinib were enrolled to treatment group (gefitinib concurrent selected radiotherapy) and control group (gefitinib only), with 5 cases in each group. The response was evaluated as progression free survival (PFS) and overall survival (OS).Results No patient got complete remission (CR). Ten of 13 patients got partial remission (PR) and stable disease (SD). The 1 year and 2 years survival rate was 53.8%(7/13) and 46.2%(6/13) respectively. The median PFS in treatment group and control group was 24 months and 8 months respectively(P= 0.0019). The median OS was 32 months and 10 months respectively (P= 0.0062). The main toxicities were reversible skin rash and diarrhea,and 3 patients developed asymptomatic radiation pulmonary fibrosis. Conclusions Gefitinib combining with selected radiotherapy is effective and tolerated in patients with advanced NSCLC. It may prolong PFS and OS. It may be a rational choice for the standard and individualized treatment of NSCLC.

Objective To analyze the relationship between clinical and pathological features in children with Henoche-Sch?nelin purpura nephritis (HSPN). Methods Clinical and pathological data of 89 children with HSPN from April 2009 to April 2013 in our hospital were retrospectively analyzed. Results A majority of patients (94.83%) suffered nephritis within two month after purpura. Clinically, hematuria and proteinuria (41.6%), nephrotic syndrome (27.0%), and isolated protein-uria (21.3%) are the most common type in children with HSPN. The majority of pathological changes of these three types HSPN are gradeⅢ, which accounts for 67.6%, 70.8%, and 73.7%, respectively. The clinical manifestations are closely associated with pathologic classiifcation (H=26.88, P=0.000). IgA plus IgM deposit was the most common type (40.45%) in histological and immunopathologic changes.There was no statistical difference between the pathologic classification and immunopathologic types. Gastrointestinal bleeding was associated with pathologic classiifcation. Conclusions The pathological changes of HPSN patients are mainly of gradesⅡandⅢ, and the clinical manifestations, gastrointestinal bleeding in particular, are closely associated with pathological classiifcation.

Objective To measure the concentrations of antibodies against outer membrane protein P6 and its T-and B-combined antigenic epitopes of nontypeable Haemophilus influenzae ( NTHi) in children and adults of different ages and to evaluate the differences among different subjects for further investigation on NTHi multiple antigenic peptide vaccine .Methods A prokaryotic expression system was established to ex-press the recombinant outer membrane protein P 6 of NTHi.The expressed protein was purified by using Ni-NTA affinity chromatography .T-and B-cell epitopes in protein P6 were predicted with Epitope prediction software 1.0 and ANTIGENIC program and were used to synthesize T-and B-combined antigenic epitopes .A total of 605 subjects aged from 1 day to 103 years old were recruited from October 2013 to March 2014 .Ser-um concentrations of antibodies against protein P 6 and its T-and B-combined antigenic epitopes were meas-ured by using ELISA .Mann-Whitney U test was used to analyze the differences between groups .Pearson product-moment correlation coefficient was used for correlation analysis .Results Four T-and B-combined antigenic epitopes including P 6-2, P6-61, P6-95 and P6-122 were predicted and synthesized .The levels of antibodies against NTHi P6 and P6-2, P6-61, P6-95 and P6-122 were significant lower in the <1 months group than those in the 1-6 months group (all P<0.001) and 7 months-3 years group (all P<0.001).Three groups including 7 months-3 years group , 4-6 years group and 7-14 years group showed significant differ-ences regarding to the antibodies levels , among the 7 months-3 years group showed the highest levels , fol-lowed by the 4-6 years group and the 7-14 years group.However, no significant difference was found be-tween other adjacent groups .Concentrations of antibodies against P 6-2, P6-61, P6-95 and P6-122 were pos-itively correlated with the level of antibody against P 6 (P<0.0001).Conclusion The distribution of anti-bodies against T-and B-combined antigenic epitopes in P6 was highly in accord with those against P6, which indicated good immunogenicity of those epitopes .The highest antibodies levels were found in subjects aged 7 months to 3 years old , which might correlate with the high risk of NTHi infection at that stage .

Objective To analyze the causes and treatment methods of early complications after central systemic-pulmonary shunt in complex cyanotic congenital heart diseases.Methods Two hundred and twelve cases of central systemic-pulmonary shunt in complex cyanotic congenital heart diseases were retro-spectively analyzed in order to explore the early postoperative complications and related treatment measures. Results There were 61 cases(28.77%)of the early postoperative complications,including severe low car-diac output syndrome in 27 cases,acute pulmonary edema in 14 cases,24 h shunt pipe blockage in 12 cases, and supraventricular tachycardia in 8 cases.All patients got followed up,average for(2.49 ±1.21 )years.Af-ter the systemic-to-pulmonary artery shunts,pulmonary vascular had significant growth,8 patients(3.77%) of them who pulmonary hypoplasia were promoted by transcatheter aortopulmonary collateral vessels.At the end of the follow-up,77 patients(36.32%)achieved the standard of radical surgery.Conclusion The factors affecting surgical survival rate include:enhancement of patients cardiac function and strictly handle operation indication before operation a clear operational view;rational surgical methods;treatment of complication with-out delay;strict,intensive care and synthesized treatment.

Objective To evaluate the indication and surgical technique for treating tetralogy of F allot with pulmonary atresia (TOF-PA).Methods From June 1984 to June 2009,66 patients with TOF-PA underwent 69 operations.Among them,34 were males and 32 females.Their age ranged from 6 months to 29 years.The anatomic characteristics of TOF-PA included 31 cases of Type Ⅰ,14 Type Ⅱ,12 Type Ⅲ and 9 Type Ⅳ.The operations included palliative aorto-pulmonary shunts in 11 cases,one-stage unifocalization with unpatched VSD in 2 cases,one stage complete repair in 40 cases,one-stage unifocalization with VSD repair in 13 cases,and delayed intracardiac repair after shunt procedures in 3 cases.Results There were 6 early deaths,including 1 death happened after aorta-pulmonary shunt and 5 after complete repair.The causes of death were severe low cardiac output in 3 cases,respiratory failure in 1,multiorgan function failure in 1 and severe wound infection with endocarditis in 1 after aorta-pulmonary shunt.The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization with unpatched VSD increased to 82％ ～ 91％.The postoperative ratio of right ventricular pressure/left ventricular pressure after complete repair was ＜ 0.5 in 31 cases,18 cases were between 0.5 and 7 cases ＞ 0.75.47 patients were followed up from 3 months to 15.5 years.The heart function(NYHA) of 44 patients were in class Ⅰ or Ⅱ and 3 in class Ⅲ or Ⅳafter operation.Conclusion The surgical strategy for TOF-PA mainly depends on the anatomic characteristics of the pulmonary and aortopulmonary collateral arteries.An individualized approach based on the anatomy of the pulmonary circuits permits a better result in the patients with TOF-PA.Patients with well developed pulmonary arteries should undergo one stage complete repair as early as possible.

Objective To investigate the chanegs and correla iton between IL-17 and MMP-9 in c hildren of henoch-schonlein purpura,and to explore the pathogenesis of teh IL-17 and MMP-9 in henoch-schonlein pur-pura /henoch-schonlein purpura nephritis.Methods The level of serum IL-17 andMMP-9 in 74 henoch-schon-lein purpura childr en in initial stage and 30 cases of healthy children( control group) were detetced by ELISA. Results (1) The level of plasma IL-17 was significantly higher in children of henoch-schonlein purpura thna tha t foh ealthy control group (86.59 ±35.50) fg/L vs.(62.38 ±14.65)fg/L,P<0.01].(2) The level of plasma MMP-9 was significantly higher in children of henoch-schonlein pupru ar than that o fhealt hy control group [(201.82 ±105.87) fg/L vs(89.27 ±27.99)fg/L,P <0.01].(3) Henoch-schonlein purpura children were followed up for6 months,dependin g on whether the kidney was involved,they were devided into non-he-noch-schonlein purpura nephritsi grou p nad henoch-s chonlein purpura nephritis group.In initial phase of HSP,the level of plasma IL-17 was significant lyhigher in henoch-schonlein purpura nephritis group than non-henoch-schonlein purpura nephrits group [(101.67 ±39.55) fg/L vs.(101.67 ±39.55)fg/L,P<0.05].The level of p lasma MMP-9 was significantly higher in henoch-schonlein purpura nephritsi group than no n-henoc h-schon-lein purpura nephritis group [(249.63 ±97.57) Pg/L vs.(185.30 ±104.39) pg/L,P<0.05 ].(4) IL-17 and MMP-9 correlation aanlysis didn't show sign ificant correlation(r=0.184,P>0.05).Conclusion IL -17 and MMP-9 are involved in the pathogenesis of henohc-schonlein purpura/henoch-schonlein pur pura nephritis.

Objectives To review the clinical features in children with methylmalonic academia (MMA) having the primary clinical manifestations of microangiopathic hemolytic anemia and renal impairment. Methods The clinical data of 4 children diagnosed of MMA with the primary clinical manifestations of microangiopathic hemolytic anemia and renal impairment were retrospectively analyzed from August 2013 to present. Results In the four children (two boys and two girls) with the age from nine months to three years seven mouths, two children were diagnosed with MMA combined with homocysteine, and 2 children were diagnosed with MMA, but there was no homocysteine testing. All four children showed moderate to severe anemia, proteinuria, hematuria, and hypertension. One child had abnormal renal function and thrombocytopenia, and manifested as hemolytic uremic syndrome. Renal biopsy was performed in 2 children and they had glomerular sclerosis lesions with renal tubular necrosis and mesangial proliferative glomerulonephritis respectively. All children were treated with vitamin B12, and the indexes of microangiopathic hemolytic anemia and renal impairment were improved signiifcantly. Conclusions MMA may be combined with microangiopathic hemolytic anemia, renal impairment and even hemolytic uremic syndrome, and the early diagnosis and treatment is required.

Objective The aim was to evaluate the development of pulmonary arteries(PA) in patients with pulmonary atresia,ventricular septal defect and diminutive pulmonary arteries by using a central end-to-side shunt between the ascending aorta and pulmonary arteries and to identify the associated factors for the results.Methods 51 consecutive patients(37 male,14 female) with pulmonary atresia,ventricular septal defect and diminutive pulmonary arteries received a central end-to-side shunt between PA and the ascending aorta from May 2004 to June 2013.Ages and weight ranged between 2-86 months and 2.5-21.5 kg,respectively.39 patients with main PA diameters less than 4 mm received the central end-to-side shunt between the ascending aorta and PA,and 14 patients with main pulmonary arteries absence received a modified shunt.Results There were no deaths during operation and follow-up.Compared with preoperative measures,total pulmonary artery index increased from a mean value of(68.8 ± 11.4) mm2/m2 to(129.1 ± 24.9) mm2/m2 (P ＜ 0.001) at the time of six months or the final repair after shunt.The increased pulmonary artery index change was(87.7 ± 27.4) ％ (27.0％ to 150.0％).By multivariate regression analysis,age at shunt,shunt procedure and number of major aortopulmonary collateral arteries were correlated with increasing pulmonary artery index change.Conclusion The central end-to-side shunt between the ascending aorta and pulmonary arteries promoted sufficient growth of the diminutive central pulmonary arteries.Due to the risk of a distortion of pulmonary branches,we interposed a modified procedure for patients with main pulmonary arteries absence.It is technically easy to perform,warrants low risk of shunt thrombosis in the early postoperative period.

Objective To analyze the relation of right ventricular outflow tract (RVOT) procedures for repair of tetralogy of Fallot and postoperative right ventricular function in order to improve the postoperative results at mid-long term follow-up.Methods A total of 125 patients for data collection with repair of TOF between January 2003 and January 2006 were prospectively enrolled in the study.Among them,66 were male and 59 were female.The ages ranged from 1 to 5 (3.4 ± 1.1) years,weight from 7 to 26 (15.2 ± 5.7) kg.20 healthy controls with a similar age and body weight were included.The type of RVOT procedure included transannular patch repair (M1) in 67 patients,patch to infundibulum (M2) in 6 patients,tranright-ventricle (RV) no patch repair (M3) in 18 patients and transatrial-transpulmonary no patch repair (M4) in 34 patients.Doppler echocardiogram and tissue Doppler imaging were performed to evaluate RV function.RV systolic function was evaluatedusing tricuspid annular plane systolic excursion (TAPSE) and index of myocardial performance (MPI).RV diastolic function was evaluatedusing ratio of Em/Am and E/Em.Unvariate and logistic regression analysis were used to identify the affective factors.Results In five years fellow-up,MPI and E/Em were higher in patents with M1 and M2 than those in the patients with M3 and M4.Univariate analysis revealed Nakata index,previous shunt operation,operative techniques、postoperative pulmonary regurgitation and during of QRS as risk factors for MPI.In the multivariate analysis,operative techniques and during of QRS were risk factors for MPI.Univariate analysis revealed Nakata index,operative techniques,PR and during of QRS as risk factors for E/Em.In the multivariate analysis,PR was the risk factor for E/Em.Conclusion RV dysfunction is associated with the type of RVOT procedure,diastolic dysfunction is correlative with postoperative PR and systolic dysfunction is correlative with enlarge of RVOT with patch and during of QRS.Echocardiographic imaging is a simple and reliable method for assessment of RV function after repaired TOF.

Objectives To investigate the clinical effect of hemoperfusion in severe juvenile dermatomyositis. Meth-ods Two patients with severe juvenile dermatomyositis, who accepted hemoperfusion treatment, were retrospectively analyzed. Results In these 2 patients, the hemoperfusion process were smooth. The muscle pain was reduced and the muscle enzymes were decreased. After hemoperfusion, one patient refused to continue treatment and the disease recurrent. The other patient con-tinued to accept the immunosuppressive therapy and eventually improved. Conclusions For juvenile dermatomyositis, hemo-perfusion was a safe and effective auxiliary treatment. It could be applied when the general treatment was invalid.