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 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.

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.

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:To study the drug resistance patterns of Bordetella pertussis in vitro, and to know the clinical characteristics of pediatric pertussis and evaluation the treatment outcomes, which may provide references for experiential diagnosis and treatment of this disease. Methods:Nasopharyngeal swabs of the hospitalized children with suspected pertussis in Children′s Hospital, Zhejiang University School of Medicine in 2017 were collected for culture. And the clinical data of the children were collected. The strains were identified by pertussis-specific antiserum agglutination and finally confirmed by mass spectrometry. The drug sensitivity test was performed using the E-test method. The efficacy of therapy with antibiotic was evaluated after two weeks of treatment. Statistical analysis was performed with Mann-Whitney U test and chi-square test. Results:Of 1 029 children, 211 (20.5%) nasopharyngeal swabs were positive for Bordetella pertussis culture, and the isolation rate of the specimens was highest (31.2%, 45/144) in July. Of the 211 pertussis patients, 105 (49.8%) were male and the age were 3.8 (2.2, 6.9) months, 114 (54.0%) were not vaccinated with pertussis diphtheria tetanus mixed vaccine and 192 (91.0%) were prescribed with previous antibiotics. There were 142 (67.3%) children from families with two or more than two children, and 136 (95.8%) of which were the youngest siblings. One hundred and fifty-nine (75.4%) patients had paroxysmal cough and 61 (28.9%) had whooping. The white blood cell counts were higher than 20×10 9/L in 94 (44.5%) patients, and the lymphocyte counts were higher than 10×10 9/L in 97 (46.0%) of patients. The drug susceptibility results showed that 138 (65.4%) strains were against erythromycin, azithromycin and clindamycin with minimum inhibitory concentration (MIC)>256.000 mg/L. The MIC 90 of the isolates to ampicillin, ceftriaxone, cefoperazone/sulbactam, meropenem and trimethoprim/sulfamethoxazole were 0.190 mg/L, 0.190 mg/L, 0.094 mg/L, 0.094 mg/L and 0.750 mg/L, respectively. All strains had a MIC of <0.016 mg/L for piperacillin/tazobactam. After treatment, symptoms were improved in 195(92.4%) patients when they were discharged from hospital. Seventy-six (57.1%) children whose symptoms did not improve after seven-day treatment with macrolides, were prescribed with other antibiotics or other antibiotic with macrolides in combination. Compared with the patients treated with macrolides, more patients treated with cefoperazone/sulbactam or piperacillin/tazobactam had negative nasopharyngeal culture results after two weeks of therapy (46/48(95.8%) vs 46/57(80.7%)), or on day seven (45/46(97.8%) vs 39/47(83.0%)) and on day 14 (45/45(100.0%) vs 41/47(87.2%)) since discharged. The differences were all statistically significant ( χ2=5.50, 5.86 and 6.15, respectively, P=0.019, 0.015 and 0.013, respectively). Conclusions:The majority of children with pertussis do not have whooping, and the resistant rate of Bordetella pertussis to macrolides is high. Further study is needed to evaluate the feasibility and reasonability of cefoperazone/sulbactam and piperacillin/tazobactam in treating pediatric pertussis caused by macrolides-resistant Bordetella pertussis.

Objective The study aimed to evaluate the short and middle term results in the patients with tetralogy of Fallot(TOF) after one-stage repair and staged repair.Methods A total of 459 TOF younger children less than five-year-old between January 2009 and December 2013 had received surgical repair,including 416 patients by one-stage repair(group Ⅰ)and 43 patients by staged repair(group Ⅱ).Among them,245 were male and 214 were female.The average repair surgical age was 27.8 ranged from 4 to 60 months,average palliation age was 15.4 ranged from 3 to 40 months.23 perioperative and follow-up parameters were assessed including sex,age,weight,preoperative clinic symptom,ratio of McGoon,pulmonary artery index,Z score of pulmonary annulus,cardiopulmonary bypass time,aortic cross-clamping time,type of VSD repair,type of RVOT procedure,Peak RV/LV pressure radio,RV-PA pressure grade,mortality,severely low cardiac output syndrome,hypoxemia,extubation time,ICU time,fellow-up time,left ventricular eject faction,RV index of myocardial performance (MPI),tricuspid annular plane systolic excursion(TAPSE) and pulmonary regurgitation.Results Compared with group Ⅰ,age and weight were significantly lower in patients in group Ⅱ[(19.1 ± 16.4) months vs.(21.1 ± 11.2) months,P ＜ 0.05) and(19.1 ±16.4) kgvs.(21.1 ±11.2) kg,P ＜0.01].The Z score of pulmonary annulus of patients in group Ⅱ were significantly less than those in group Ⅰ (-3.69 ± 2.36 vs.-2.50 ± 1.95,P ＜ 0.01).The ratio of repairing VSD by RV incision and using TAP in patient of group Ⅱ were significantly more than those in group(27/43 vs.71/413,P ＜0.01),(41/43 vs.221/413,P ＜0.01).There was no difference of mortality,complication,extubation time and ICU time bewteen two groups.All patients followed up 12-52 months,there was no difference of LVEF,MPI and TAPSE between two groups.However,the severity of pulmonary regurgitation in patients of group Ⅱ was significant more than those of group Ⅰ (47.6％ vs.32.1％,P ＜ 0.01).Conclusion The early and mid-term results in the the young children patients with TOF after one-stage repair or staged repair were good.Although the prior palliative shunt could promote the development of the hypoplasia pulmonary arteries in the young children patient,it may related to the technique of repairing operations and the postoperative pulmonary regurgitation.

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.