ObjectiveTo collect and analyze the clinical data and quality of life of hemophilic children, understand the present condition of these patients in Sichuan Province, and analyze related influencing factors. MethodWe retrospectively analyzed the clinical data of hemophilic children treated in our hospital from January 1, 2008 to May 30, 2015. ResultsThere were 92 child patients from Sichuan Province with a median age of 9.6 years old (ranging from 3.6 to 18.0). There were 87 cases (94.6%) of hemophilia A and 5 (5.4%) of hemophilia B; the number of light cases was 4 (4.3%), of moderate cases was 67 (72.8%), and of severe cases was 21 (22.9%); eighteen (19.6) of the patients had family history. First bleeding episode occurred at a median age of 11 months (0-48 months). Mild bleeding occurred in 23 cases (25.0%), moderate bleeding in 31 cases (33.7%), and severe bleeding in 38 cases (41.3%). First bleeding site was mainly the mucous membrane of the skin, followed by the joint muscles. Fist joint bleeding occurred at a median age of 18 months (2-107 months). Forty-six patients (59.0%) had severe joint damage during the course of the disease. Intracranial hemorrhage occurred in 4 patients (4.3%), among whom 3 were cured and discharged from the hospital, and 1 had neurological sequelae. Median diagnostic age was 12 months (0-120 months). Sixty-two (67.4%) were diagnosed in a short period of time, 9 (9.8%) in a mediate period of time, and 21 (22.8%) in a long period of time. Forty (43.5%) of the patients had been given sufficient coagulation factors, while all the others had not received sufficient replacement therapy. Fifty-six (60.9%) children had received prophylactic treatment. First prophylaxis was administrated at a median age of 36 months (1-199 months), but 27 (48.2%) discontinued. The median score of the 29 retreated Disease Burden Scale was 22.7±11.6 (4-43), and among them, 11 (37.9%) could not care for themselves. Twenty-three participated in the assessment of social activity ability, among whom, 2 did not attend school, and 6 could not take part in the assessment because of school learning. Correlation analysis showed that there was no significant relationship between diagnostic timing and family history (P=0.795) or between diagnostic timing and areas they came from (P=0.495). However, significant association was found between diagnostic timing and the severity of first bleeding (r=0.392, P=0.035). Disease burden of family was significantly correlated with the number of target joints (r=0.370, P=0.048), and was not closely related with area, severity of bleeding, frequency of hemorrhage, medical insurance, or physical and social activities. ConclusionsThe general diagnosis and treatment condition of child hemophilia in Sichuan is relatively under-developed with a high prevalence of joint damage, poor quality of life, and high disease burden to the family. Improvement in the care of hemophilia children is urgently needed.
ObjectiveTo compare the effectiveness of antecolic duodenojejunostomy (ADJ) and retrocolic duodenojejunostomy (RDJ) after pylorus-preserving pancreaticoduodenectomy (PPPD). MethodsRandomized controlled trials (RCTs) of ADJ versus RDJ after PPPD were searched in Cochrane Library, PubMed database, Embase database, Web of Science, Chinese biomedicine database, CNKI database, VIP database, and Wanfang database from inception to April 2014, as well as Google. After quality assessment of RCTs according to the Cochrane Handbook for Systematic Reviews of Interventions Version, Meta analysis was performed by RevMan 5.1 software. ResultsFour RCTs of 462 patients in total were included in this Meta-analysis. The results of Meta-analysis showed that, there were no significant differences in the operation time (MD=14.02, 95% CI:-41.42-69.46, P=0.62), incidence of postoperative complications (RR=1.09, 95% CI:0.81-1.48, P=0.56), incidence of delayed gastric emptying (RR=0.63, 95% CI:0.31-1.28, P=0.20), incidence of pancreatic fistula (RR=1.13, 95% CI:0.72-1.75, P=0.60), incidence of abdominal abscess (RR=0.92, 95% CI:0.54-1.58, P=0.77), and mortality (RR=0.61, 95% CI:0.24-1.60, P=0.32) between ADJ group and RDJ group. ConclusionsThe effectiveness of ADJ is similar with RDJ after PPPD, so the reconstruction way after PPPD can be routed according to the surgeon's preference.
ObjectiveTo investigate the impact of deep hypothermic circulatory arrest (DHCA) with antegrade cerebral perfusion (ACP) on cognitive function of patients undergoing surgical therapy for acute Stanford type A aortic dissection (AD). MethodsBetween January 2009 and March 2012, 48 patients with acute Stanford type A AD underwent Sun's procedure (aortic arch replacement combined with stented elephant trunk implantation) under DHCA with ACP in Nanjing Hospital affiliated to Nanjing Medical University. There were 40 males and 8 females with their age of 51.3±13.6 years. Circulatory arrest time and time for postoperative consciousness recovery were recorded. Preoperative and postoperative cognitive functions of each patient were evaluated by mini-mental status examination (MMSE). ResultsMean cardiopulmonary bypass time of the 48 patients was 237.3±58.5 minutes, and mean circulatory arrest time was 37.3 ±6.9 minutes. Four patients died postoperatively with the causes of death including lung infection, multiple organ dysfunction syndrome, myocardial infarction and acute respiratory distress syndrome. Forty-one patients recovered their consciousness within 24 hours postoperatively, and the mean time for postoperative consciousness recovery was 15.3±6.5 hours. Preoperative MMSE score was 28.6±1.1 points, and MMSE score at 1 week postoperatively was 23.6±4.5 points. Thirty-one patients were followed up for 6 months with the follow-up rate of 70.45%. The average MMSE score of the 31 patients at 6 months after surgery was 27.6±2.1 points which was significantly higher than postoperative average MMSE score (P < 0.05), but not statistically different from preoperative average MMSE score (P > 0.05). ConclusionsDHCA with ACP can provide satisfactory cerebral protection for patients undergoing surgical therapy for acute Stanford type A AD, but patients' cognitive function may be adversely affected in the short term. As long as cerebral infarction or hemorrhage is excluded in CT scan of the brain, such adverse impact may generally disappear automatically within 6 months after surgery.
ObjectiveTo compare early outcomes of the minimally invasive mitral valve surgery (MIMVS) through right anterolateral mini-thoracotomy (ALMT) with conventional mitral valve surgery (MVS), and evaluate feasibility and safety of MIMVS. MethodsFrom January 2011 to December 2013, 120 patients undergoing elective MVS in Nanjing First Hospital were prospectively enrolled in this study. There were 72 male and 48 female patients with their age of 22-71 (42.4±11.0) years. Using a random number table, all the patients were randomly divided into a portaccess MIMVS group (MIMVS group, n=60) and a conventional MVS group (conventional group, n=60). MIMVS group patients received port-access cardiopulmonary bypass (CPB) establishment via femoral artery, femoral vein and right internal jugular vein cannulation through right ALMT 5-6 cm in length. Special MIMVS operative instruments were used for mitral valve repair or replacement. Conventional group patients received mitral valve repair or replacement under conventional CPB through median sternotomy. Perioperative clinical data, morbidity and mortality were compared between the 2 groups. ResultsThere was no death in-hospital or shortly after discharge in this study. CPB time (98.0±26.0 minutes vs. 63.0±21.0 minutes) and aortic cross-clamping time (68.0±9.0 minutes vs.37.0±6.0 minutes) of MIMVS group were significantly longer than those of conventional group (P<0.05). Postoperative mechanical ventilation time (6.0±3.9 hours vs. 11.2±5.6 hours), length of ICU stay (18.5±3.0 hours vs. 28.6±9.5 hours) and postoperative hospital stay (8.0±2.0 days vs. 13.5±2.5 days) of MIMVS group were significantly shorter than those of conventional group (P<0.05). Chest drainage volume within postoperative 12 hours (110.0±30.0 ml vs. 385.0±95.0 ml) and the percentage of patients receiving blood transfusion (25.0% vs. 58.3%) of MIMVS group were significantly lower than those of conventional group (P<0.05). Patients were followed up for 1-24 months, and the follow-up rate was 94.2%. There was no statistical difference in postoperative morbidity or mortality between the 2 groups (P>0.05). ConclusionMIMVS through right ALMT is a safe and feasible procedure for surgical treatment of mitral valve diseases. MIMVS can achieve similar clinical outcomes as conventional MVS, but can significantly shorten postoperative ICU stay and hospital stay, reduce blood transfusion, and is a good alternative to conventional MVS.
ObjectiveTo summarize the clinical experience in the treatment of Carpentier's type Ⅲb ischemic mitral regurgitation through the mitral valve repair versus mitral valve replacement, and to evaluate the early and midlong term effects. MethodsWe retrospectively analyzed the clinical data of 308 consecutive patients with type Ⅲb ischemic mitral regurgitation undergoing coronary artery bypass grafting (CABG) with mitral valve repair (a repair group, n=172) or with mitral valve replacement (a replacement group, n=136) in our hospital between January 2000 and March 2014. Among the 308 patients, 215 were males and 93 were females with mean age of 62.7±11.5 years(ranged 30-78 years). In the repair group, 170 patients underwent restrictive mitral annuloplasty (128 patients with total ring, 42 patients with C ring), and 2 patients underwent commissural constriction. In the replacement group, 11 patients underwent mechanical valve prosthesis and 125 patients underwent biological valve prosthesis. ResultsThe time of total aortic cross-clamp was 81.9±21.5 min. The time of total extracorporeal circulation was 122.0±31.3 min. Six patients died during the perioperative period. No significant differences were observed between the two groups in general information (P>0.05). There were no significant differences between the two groups in aortic cross-clamp time, total extracorporeal circulation time, numbers of bypass grafts and the usage rate of left internal mammary artery. The early result after the surgery showed that the incidence rates of low cardiac output and ventricular arrhythmia were significantly higher in the replacement group compared with those in the repair group. The patients were followed up for 1-85 months. No significant difference was revealed in the mid-long term survival rate between the two groups. The severity of mitral regurgitation and the rate of redo mitral valve replacement were significantly lower in the replacement group compared with those in the repair group (P<0.05). ConclusionThe early-term curative effect of valve repair is better than valve replacement for the treatment of Carpentier's type Ⅲb ischemic mitral regurgitation. In mid-long term, Chordal-sparing mitral valve replacement remains a low incidence of valve-related complications compared with mitral valve repair.