Objective To analyze the clinical and pathological characteristics of ANCA associated systemic vasculitis(AASV) with immune complex deposition in kidney and compare with that of pauci-immune AASV. Methods Patients with AASV, admitted in our hospital in last 5 years, were retrospectively studied. The clinical and pathological characteristics were compared between patients with immune complex deposition and patients with pauci-immune deposition. Results There were eitht patients with immune complex deposition (five with IgM deposition, two with IgA deposition and one with IgG deposition) and 32 patients with pauci-immune deposition. There was no significant difference in age, gender, type of ANCA, interval between onset of vasculitis and renal biopsy, clinical manifestations and short-term renal survival rate between the two groups. Patients with immune complex deposition had a higher predromal infection rate ( P

ObjectiveTo investigate the clinical and pathological features of patients with anti-glomerular basement membrane(GBM) disease lacking linear IgG deposition along GBM on renal biopsy.Method Ninety-three patients with anti-GBM disease were collected in our hospital from 1991 to 2008,with 40 patients presenting negative linear IgG deposition along GBM on renal biopsy by direct immunofluorescence(group A) and 53 patients presenting classical linear IgG deposition along GBM(group B).The clinical manifestation,pathological presentation and prognosis were compared between two groups.Results Between two groups,there were no significant differences in gender,age,hemoptysis,oliguria or anuria,gross hematuria,proteinuria,anemia,ANCA positivity,level of circulating anti-GBM antibodies,the percentage of crescent formation in glomeruli and patient outcomes(P＞0.05).Patients in group A were diagnosed significantly later than patients in group B(68 d vs 36 d,P=0.013) and serum creatinine was significantly lower at diagnosis(716.0 μmol/L vs 896.8 μmol/L,P=0.027).Direct immunofluorescence was performed on the paraffin-embedded renal sections from four patients in group A,and all of them revealed positive linear IgG deposition along GBM.Conclusions Patients with circulating anti-GBM antibodies but withont IgG deposition along GBM present slower progress of renal injury,but same clinical,pathological and prognostic features as those with classical anti-GBM disease.Serum anti-GBM antibodies should be prescribed earlier to the suspected patients,and the diagnosed patients should be treated with plasmapheresis and extensive immunosuppression to improve prognosis.

Objective To analyze the epidemiologieal features and treatment of brain injury in Chinese Wenchuan earthquake so as to provide the experiences for improving treatment level. Methods A total of 685 patients with earthquake-induced brain injury in the City of Deyang were collected to ana-lyze age, injury type, injury localization and treatment characteristics. Results Of large number of earthquake victims, the number of the patients with brain injury ranked the second place, mainly minor and moderate brain injuries involving scalp laceration for the most and skull fracture and extradural hema-toma for the next. As for the age distribution of the patients, young and middie-aged patients accounted for the most, for they were at school or at work when the earthquake happened. Obvious position distribu-tion features were found in depressed fracture of skull and extredural hematoma but not in the scalp lacera-tion. Depressed fracture of skull occurred mostly at forehead and occiput while extradural hematoma could be most seen at froutotemple. Most brain injuries were combined with other injuries. Mainly extremity and pelvic fractures. Conclusions Self-protection sense and measures during earthquake are very important for decreasing incidence and severity of brain injuries. The management of rospimtory tract in prehospital care should be strengthened to avoid asphyxia and hence raise the success rate of treatment.

Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.

Objective To review the outcomes of atrioventricular valve replacement in patients with functional single ventricle and evaluate risk factors for mortality.Methods Retrospectively reviewed our experience with atrioventricular valve (AVV replacement) in patients with functional single ventricle from January 2008 to December 2014.Outcome included mortality and valve-related complications.Results Thirteen patients required AVV replacement.Prosthetic valve type was St.Jude bileaflet mechanical mitral valve.Valve size ranged from 27 to 31 mm,including 27 mm in 2,29 mm in 7,and 31 mm in 4 patients.Median age at valve replacement was 4.5 years(range:1.2 years to 18.0 years).Concomitant procedures included bidirectional Glenn shunt in 3,pulmonary artery banding in 1,and repair of pulmonary vein stenosis in 1 patient.There were four early deaths with a perioperative mortality of 30.8％.Complications after AAV replacement included complete atrioventricuiar block in 1,intracerebral hemorrhage in 1 patient due to valve-related anticoagulation.No patient had perivalvular leakage.There was one late death during a mean follow-up of 3 years (range:0.5-5.0 years).Five-year Kaplan-Meier survival was 61.5％.Fisher exact probability test showed that lower weight(＜ 10 kg) at operation and prosthetic size/weight ratio ＞ 2.0 were risk factors for overall mortality.Of the survival patients,functional status is NYHA class Ⅰ in 3,class Ⅱ in 5.No patient developed valve-related complications.Conclusion Atrioventricular valve replacement can be performed in patients with functional single ventricle with acceptable mortality.Lower weight and increased prosthetic size/weight ratio at operation were significantly associated with worse survival.

AIM:Atherosclerotic calcification is highly linked with plaque instability and cardiovascular events .Adenosine monophosphate-activated protein kinase ( AMPK) has been involved in the pathogenesis of various cardiovascular disease .The contributions of AMPKαsubunits to the development of atherosclerotic calcification in vivo remained unknown .We hypothesized that AMPKαsubunits may play a role in the development of atherosclerotic calcification .METHODS: Atherosclerotic calcification was generated by 24-week fed of western diet in ApoE-/-background mice .Calcification was evaluated in aortic roots and innominate arteries of ApoE-/-mice or in mice with dual deficiencies of ApoE and AMPKαsubunits globally ( AMPKα1 and AMPKα2 ) , or vascular smooth muscle cell ( VSMC)-specific or macrophage-specific knockout of AMPKα1 with atherosclerotic calcification pone diet . The mechanism of AMPKα1 in regulating Runx2 was further explored in human aortic VSMC .RESULTS: Ablation of AMPKα1 but not AMPKα2 in ApoE-/-background promoted atherosclerotic calcification with increased Runt -related transcription factor ( Runx2 ) expression in VSMC compared with ApoE-/-mice.Conversely, chronic administration of metformin, which activated AMPK, markedly reduced ath-erosclerotic calcification and Runx2 expression in ApoE-/-mice but had less effects in ApoE-/-/AMPKα1 -/-mice.Furthermore, VSMC-but not macrophage-specific deficiency of AMPKα1 in ApoE-/-background promoted atherosclerotic calcification in vivo com-pared with the controls .AMPKα1 silencing in human aortic VSMC prevented Runx 2 from proteasome degradation to trigger osteoblastic differentiation of VSMC .Conversely , activation of AMPK led to Runx 2 instability by inducing its small ubiquitin-like modifier modifi-cation (SUMOylation).Protein inhibitor of activated STAT-1 (PIAS1), the SUMO E3-ligase of Runx2, was directly phosphorylated by
AMPKα1 at serine 510, to enhance its SUMO E3-ligase activity.Ablation of PIAS1 serine 510 phosphorylation inhibited metformin-in-duced Runx2 SUMOylation, and subsequently prevented the effect of metformin on reducing oxLDL-triggered Runx2 expression in hu-man aortic VSMC.CONCLUSION:Deficiency of AMPKα1 in VSMC increases Runx2 expression and promotes atherosclerotic calcifi-cation in vivo.AMPKα1 phosphorylates PIAS1 to enhance Runx2 SUMOyalation and subsequent degradation .

Objective To learn the current capacity building of emergency response teams in Tianjin for the purpose of goals setting.Methods Health Emergency Capacity Questionnaire was issued to 89 secondary and above medical institutions and 19 CDCs in Tianjin.The form covered such items as basic institutional information, workforce makeup, emergency preparations, detection and early warning, emergency response, and summary/assessment.Data collected in the questionnaire were subject to descriptive and correlation analysis.Results Tianjin has scored an initial success in emergency medicine as evidenced in its emergency response mechanisms in place, elevated capacity in emergency medical rescue and disposal, and enhanced competence of emergency teams.Rooms of improvement however include insufficient professionalism and independence of health emergency, inadequate emergency commanding and decision making system functions, insufficient laboratory test capacity at district/county levels, and insufficient social involvement in health emergency.Conclusions Top-down design should be emphasized, health emergency response should be enhanced in terms of management and response planning system, while capacity building of the teams and long-term primary care emergency mechanism deserve higher attention.

OBJECTIVETo discuss the experience of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in infants.

METHODSThe clinic data of 84 cases with TAPVC under 6 months of age underwent surgical treatment at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center from January 2012 to October 2015 were analyzed retrospectively. There were 58 male and 26 female patients. The patients were aged 1 days to 6 months with a mean of (2.4±2.2) months at surgery, including 22 newborns. Body weight was 1.8 to 6.8 kg with a mean of (4.3±1.2) kg. There were 24 cases of intracardiac type, 46 cases of supracardiac type, 10 cases of infracardiac type and 4 cases of mixed type. There were 26 cases received emergent operation. There were 14 cases used Sutureless technique in operations and 46 cases used conventional methods in the no-intracardiac type cases, and 2 cases enlarged the anastomsis with autologous pericardium. According to the condition, corrective surgeries of other anomalies were performed in the meantime, including 3 Warden operations (right side), 3 bilateral bidirectional Gleen operation, 2 correction of unroofed coronary sinus syndrome, 1 coarctation of aorta correction with deep hypothermic circulation arrest, and 1 repair of ventricular septal defect.

RESULTSThe ratio of newborn was higher in Sutureless technique group than in conventional methods group (7/14 vs. 32.6%, χ(2)=4.927, P=0.043), and mean age was less ((1.8±0.4) months vs. (2.4±2.2) months, F=4.257, P=0.042), but there were no difference in body weight, cardiopulmonary bypass time and aorta clamped time between the two groups. Followed up for 1 to 46 months, 10 cases (11.9%) died overall and the mortality of intracardiac (3/10) and mixed (2/4) type were much higher than in intracardiac (4.2%) and supracardiac (13.0%) type. The mortality were no difference between newborn and infants, or whether emergent operation, or Sutureless technique and conventional methods. The maximal pulmonary venous flow velocity was abnormal speed-up >1.8 m/s at 1 week and 1 to 3 months post-operation mostly.

CONCLUSIONSThe mortality of TAPVC was differed by different types. Intrinsic pulmonary vein stenosis maybe the main cause of mortality. The high quality of anastomsis could reduce the operative mortality.

Aortic Coarctation ; Body Weight ; Cardiac Surgical Procedures ; methods ; Circulatory Arrest, Deep Hypothermia Induced ; Female ; Heart Defects, Congenital ; mortality ; surgery ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Period ; Pulmonary Veins ; pathology ; surgery ; Retrospective Studies

Objective: To review the early and mid-term results of end-to-side anastomosis technique for interrupted aortic arch in neonates and infants. Methods: Clinic data of 46 patients were diagnosed as interrupted aortic arch in Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center between January 2010 and December 2016 were analyzed retrospectively. Twenty-six cases were neonates. The median age underwent surgery was 23 days (range: 2 days to 8 years). Anatomical subtypes included 36 cases of type A and 10 cases of type B. There was no type C case. The reconstruction of the aortic arch was completed by an extended end-to-side anastomosis technique between the descending aorta and the undersurface of the proximal aortic arch. In 42 patients, all with intracardiac anomalies, had concomitant complete repair of intracardiac anomalies through a median sternotomy. The remaining 4 patients, all without intracardiac anomalies, an end-to-side anastomosis was constructed through a left thoracotomy. During follow-up, aortic arch recurrent obstruction, left ventricular outflow tract obstruction (LVOTO) and tracheal stenosis were focused. Results: There were 3 surgical deaths, with amortality rate of 6.5%. The remaining 43 patients survived after surgery. In 39 of these patients, deep hypothermic cardiac arrest (DHCA) strategy was used for brain protection, and the mean time of DHCA was (16±3) minutes. Eight patients underwent delayed sternal closure. The mean mechanical ventilation time and ICU stay time were (3.4±1.6) days (range: 2 to 12 days) and (6.4±2.7) days (range: 3 to 16 days) respectively. In 16 patients, all with tracheal or bronchial stenosis before surgery, mechanical ventilation was successfully evacuated, and no new airway stenosis occurred. There was no residual pressure difference between upper and lower extremity arterial blood pressure at discharge. Echocardiography showed normal arterial blood flow velocity in aortic arch. At a mean follow-up of (36.2±18.9) months (range: 6 months to 7 years), there were two patients lost and one late date. Four patients developed a recurrent stenosis at the aortic arch, of which two were severe, and the other two were mild. In 2 patients, both with mild LVOTO before surgery, no significant increase in the degree of obstruction was found during the follow-up. Two patients developed new mild to moderate LVOTO without clinical symptoms, and continued to follow up. In all patients, the tracheal or bronchial stenosis were extenuated, and there was no new progressive airway stenosis by regular bronchoscopy. Conclusions The end-to-side anastomosis technique for the reconstruction of the aortic arch achieved excellent early and mid-term results in neonates and infants suffered from interrupted aortic arch. Reducing the anastomotic tension by extensive mobilization is the key to prevent postoperative early complications and late recurrent arch obstruction.

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.