目的：探讨蛋白酶体抑制剂——硼替佐米对初治多发性骨髓瘤的疗效及对移植造血干细胞采集的影响。方法：对一例初发的中年男性多发性骨髓瘤患者使用硼替佐米+地塞米松+反应停（VTD）的方案进行化疗，获得缓解后采集外周血造血干细胞。结果：应用以硼替佐米为基础的方案治疗3个疗程后，患者即获得完全缓解；完成4个疗程化疗后成功采集足够数量的外周血造血干细胞；完成6个疗程化疗后，进入维持治疗，至今已完全缓解17个月。治疗过程中除恶心、呕吐外无其他明显不良反应。结论：硼替佐米用于初治多发性骨髓瘤有良好的治疗效果，不良反应少，不影响造血干细胞采集。
Objective To analyze the clinical characteristics of dermatomyositis ( DM) and polymyositis ( PM) with pulmonary involvement. Methods A retrospective study was performed in 27 DM/PM patients with pulmonary involvement, who were admitted to the First People’s Hospital of Kunming fromJanuary2001 to December 2009. The clinical manifestation, laboratory examination, chest high resolution CT ( HRCT) , pulmonary function test, treatment efficacy and prognosis were analyzed. Results In 27 DM/PM patients with pulmonary involvement, pulmonary manifestations occurred in 23 cases, such as cough ( 44% ) , expectoration ( 30% ) , and dyspnea ( 11% ) . Erythrocyte sedimentation rate, creatine kinase, C-reactive protein, and lactic dehydrogenase were significantly increased in 63% , 67% , 56% , and 44% of patients. Anti-Jo-1 antibody was positive in eight cases ( 29% ) . The electromyogram ( EMG) revealed myogenic changes in all patients. Pulmonary interstitial changes were the predominant HRCT manifestations. Pulmonary function test revealed mainly restrictive ventilation dysfunction and decreased diffusion capacity. Most patients had a good prognosis by glucocorticoid treatment. Conclusions For patients with DM/PM, especially who present nonspecific pulmonary symptoms, chest HRCT and pulmonary function test should be recommended as early screening tools.
Objective To investigate surgical strategy for the treatment of muscular ventricular septal defect （MVSD） in infants with multiple ventricular septal defects（VSD）. Methods Clinical data of 46 infants with multiple VSD who underwent surgical repair in Shanghai Children’s Medical Center from January 2010 to April 2012 were retrospectively analyzed. There were 24 males and 22 females with their age of 8±6 months and body weight of 6.1±1.9 kg. All the patients received one-stage surgical repair，among whom MVSD of 10 patients was not found and repaired during the surgery. MVSD was repaired by surgical suture in 19 patients，hybrid repair under direct vision in 12 patients，and hybrid repair via the right ventricle in 5 patients. All the patients were regularly followed up after discharge by chest X-ray，ECG and color Doppler echocardiography to observe the closure of MVSD and the presence of residual shunt. Results All the 46 patients with multiple VSD survived their surgery without perioperative death. Three patients undergoing hybrid repair under direct vision received delayed sternal closure. One patient undergoing hybrid repair under direct vision had postoperative cardiac dysfunction. All the 46 patients were followed up for 1-17 months. Twenty-three patients had residual shunt of varying degrees during follow-up，and most of the MVSD of patients with residual shunt were ＜4 mm，who were receiving further follow-up and evaluation. Conclusion Appropriate surgical strategies can be applied according to specific surgical views during the operation for the treatment of MVSD in infants with multiple VSD，and satisfactory clinical outcomes can be achieved.
Objective To evaluate clinical efficacy of four-claw Ti-planes for internal fixation of multiple rib fractures and flail chest. Methods Clinical data of 93 patients with multiple rib fractures and flail chest who were admittedto Shanghai Pudong Hospital from December 2011 to November 2012 were retrospectively analyzed. There were 78 male and 15 female patients with their age of 20-80 years. All the patients received internal fixation of rib fractures using four-clawTi-planes. Finite element modeling and analysis were performed to investigate biomechanical behaviors of rib fractures after internal fixation with four-claw Ti-planes. Results The average number of rib fractures of the 93 patients was 5.9±2.1，and each patient received 3.8±1.3 four-claw Ti-planes for internal fixation. The operations were performed 6.3±3.2 days after admission. After the rib fractures were fixed with four-claw Ti-planes，rib dislocations and chest-wall collapse of flail chest were restored，and patients’ pain was relieved. Postoperative CT image reconstruction of the chest showed no dislocationor displacement at the fixation areas of the four-claw Ti-planes. Rib fractures were stabilized well，and normal contours of the chest were restored. Finite element analysis showed that the maximum bearable stress of the rib fractures after internal fixation with four-claw Ti-planes was twice as large as normal ribs. Conclusion Clinical outcomes of four-claw Ti-planesfor internal fixation of rib fractures are satisfactory with small incisions and less muscle injury of the chest wall，so this technique deserves wide clinical use.