Efficacy and Feasibility of Allogeneic Hematopoietic Stem-Cell Transplantation in the Treatment of Refractory Acute Myeloid Leukemia

Background

Refractory acute myeloid leukemia (AML) includes AML includes failure of disease to respond to standard induction chemotherapy, relapse within 6 months after first CR, and 2 or more relapses. The outcome of these patients is usually very poor; only a small proportion can be rescued by allogenic hematopoietic stem-cell transplantation (allo-HSCT). The aim of this study was to evaluate the efficacy and feasibility of allo-HSCT in patients with refractory AML.

完全型雄激素不敏感综合征2例临床特点及处理

雄激素不敏感综合征(androgen insensitivity syndrome,AIS)又称为睾丸女性化综合征(testicular feminization syndrome,TFS),是一种X连锁遗传病,是男性假两性畸形中较常见的类型,可分为完全型AIS和不完全型AIS,其原因主要是雄激素受体(androgen receptor,AR)基因的突变导致其对雄激素产生抵抗和不应答。本文回顾南京医科大学附属妇产医院2例CAIS患者的临床资料及诊疗过程,以期能进一步提高对该病的认知及诊治水平。     

Voluntary ambulation using voluntary upper limb muscle activity and Hybrid Assistive Limb® (HAL®) in a patient with complete paraplegia due to chronic spinal cord injury: A case report

Context: We sought to describe our experience with the Hybrid Assistive Limb® (HAL®) for active knee extension and voluntary ambulation with remaining muscle activity in a patient with complete paraplegia after spinal cord injury.

Findings: A 30-year-old man with complete paraplegia used the HAL® for 1 month (10 sessions) using his remaining muscle activity, including hip flexor and upper limb activity. Electromyography was used to evaluate muscle activity of the gluteus maximus, tensor fascia lata, quadriceps femoris, and hamstring muscles in synchronization with the Vicon motion capture system. A HAL® session included a knee extension session with the hip flexor and voluntary gait with upper limb activity. After using the HAL® for one month, the patient’s manual muscle hip flexor scores improved from 1/5 to 2/5 for the right and from 2/5 to 3/5 for the left knee, and from 0/5 to 1/5 for the extension of both knees.

Conclusion/clinical relevance: Knee extension sessions with HAL®, and hip flexor and upper-limb-triggered HAL® ambulation seem a safe and feasible option in a patient with complete paraplegia due to spinal cord injury.     

经皮激光心肌血运重建术治疗心绞痛的临床疗效观察

【目的】观察经皮激光打孔心肌血运重建术治疗顽固性心绞痛的临床疗效。【方法】应用经皮激光 (HO :YAGlaser)心内膜心肌打孔治疗 2 7例顽固性心绞痛患者 ,以加拿大心绞痛标准分级和Naughton运动试验ST段下移至 1mm所需时间作为激光心肌血运重建术疗效的临床观察指标。【结果】每例打孔 5～ 15个 ,平均 (8± 4)个。打孔前和打孔后 1月、3月、6月按加拿大心绞痛标准分级 ,分别为 (3 5± 0 5 )、(2 2± 0 8)、(2 0± 0 6 )、(2 1± 0 5 )级 ;Naughton运动试验ST段下移至1mm所需时间则分别为 (36 0± 16 0 )s、(4 0 6± 2 6 0 )s、(4 2 0± 2 90 )s、(4 5 0± 312 )s。【结论】经皮激光打孔心肌血运重建术能降低患者心绞痛的严重程度 ,对不能常规施行经皮冠状动脉腔内成形术 (PTCA)或冠状动脉旁路搭桥术 (CABG)的严重冠心病心绞痛的患者 ,经皮激光心肌血运重建术是一种有效治疗方法。     

完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭的抢救

目的总结完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭的治疗经验,以提高抢救成功率.方法对经抢救治疗的完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭9例(16次)患者进行治疗方面的回顾分析.结果9例(16次)患者14次抢救成功,成功率87.0%,但复发率较高,预后较差,有5例患者在抢救时或出院后1～2年内猝死.结论该组患者经传统"强心、利尿、扩血管",控制心房纤颤、心室率等治疗是远远不够的.应在未发生肺泡性肺水肿及心源性休克之前,尽早尽快地应用血管扩张剂及联合应用小剂量非洋地黄类正性肌力药物,并维持治疗24～72 h.其中以酚妥拉明加多巴胺和(或)多巴酚丁胺效果可能较佳,血管扩张剂硝酸酯类不及α-受体阻滞剂酚妥拉明疗效明显.     

Transmyocardial revascularization ameliorates ischemia by attenuating paradoxical catecholamine-induced vasoconstriction

Background  The mechanism by which transmyocardial revascularization (TMR) offers clinical benefit is controversial. We hypothesized that TMR ameliorates ischemia by reversing paradoxical catecholamine-induced vasoconstriction. Methods and Results  Chronic ischemic cardiomyopathy was created in 11 dogs by placing ameroid constrictors on the proximal coronary arteries and their major branches. Six weeks later, 35 channels were created percutaneously in the left circumflex artery region, with the left anterior descending artery region serving as control. At rest, wall thickening and myocardial blood flow did not change in the treated region, whereas they deteriorated in the control bed. Contractile and myocardial blood flow reserve increased in the treated region but deteriorated in the control region. There was diminished iodine 123 metaiodobenzylguanidine uptake and a significant reduction in noradrenergic nerves in the treated region compared with the control region, with a corresponding reduction in tissue tyrosine hydroxylase activity. Conclusions  We conclude that the absence of a catecholamine-induced reduction in MBF reserve and contractile reserve in the TMR-treated region with associated evidence of neuronal injury indicates that the relief of exercise-induced ischemia after TMR most likely results from reversal of paradoxical catecholamine-induced vasoconstriction. These findings may have implications in selecting patients who would benefit from TMR. Supported in part by grants from the National Institutes of Health (R01-HL66034 and K-08-HL074290-01). Bethesda. Md. The radio-labeled microspheres were provided by DuPont Pharmaceuticals, North Billerica. Mass, and the ultrasound equipment was supplied by Philips. Andover, Mass. Dr Leong-Poi was the recipient of a Fellowship Training Grant from the Canadian Institute of Health Research and the Heart and Stroke Foundation of Canada.     

Changes in the basal ganglia and thalamus following reperfusion after complete cerebral ischaemia

We report specific changes bilaterally in the basal ganglia and thalamus following reperfusion after complete cerebral ischaemia. A 69-year-old man, resuscitated after cardiac arrest, showed symmetrical lowdensity lesions in the head of the caudate nucleus and lentiform nucleus on CT. MRI revealed methaemoglobin derived from minor haemorrhage in the basal ganglia and thalamus, not evident on CT. We suggest that this haemorrhage results from diapedesis of red blood cells through the damaged capillary endothelium following reperfusion.     

Myocardial cooling and right ventricular function in patients with right coronary artery disease: antegrade vs. retrograde cardioplegia

Background: Protection of the right ventricular (RV) myocardium during ischaemia in cardiac surgery is difficult, especially in patients with severe right coronary artery (RCA) disease. Retrograde coronary sinus cardioplegia is thought to distribute uniformly, but doubts still remain as to its adequacy in RV preservation. This study evaluated distribution of antegrade vs. exclusively retrograde coronary sinus cold blood cardioplegia by assessing myocardial cooling and compared the effects on RV function. Methods: Fifty-eight patients scheduled for elective coronary artery surgery - 29 patients with significant RCA disease and another 29 with no significant RCA stenosis (controls) - were randomised to receive either antegrade or retrograde cold blood cardioplegia through either aortic root or conventional self-inflating coronary sinus catheter (RCA-ante, RCA-retro, C-ante and C-retro groups). RV function was assessed by fast-response thermodilution. Myocardial temperatures were measured in the anterior and posterior wall of the right and left ventricle. Results: Cooling of the posterior wall of the RV was effective only in the control patients given antegrade cardioplegia (14.7°C), whereas in the other groups the lowest myocardial temperatures there remained above 20°C (RO.001). In patients with obstructed RCA both antegrade and retrograde cold cardioplegia led to uneven cooling of the myocardium. After cardiopulmonary bypass the RV ejection fraction (RVEF), RV stroke work index (RVSWI) and cardiac index (CI) were significantly reduced in the RCA-retro group, and RVSWI and CI in the C-retro group, too. Regression analysis showed an inverse relationship between the temperatures of the posterior walls of the ventricles and changes in the RVEF and CI. Conclusions: Retrograde and antegrade cardioplegia alone were not effective in reducing the temperature of the posterior wall of the RV in the patients with obstructed RCA, in whom with retrograde cardioplegia RV haemodynamics were impaired for 1 hour following bypass. Neither retrograde nor antegrade cardioplegia alone can be relied on to protect the posterior wall of the RV in the patients with obstructed RCA.     

1405例冠状动脉旁路移植、激光心肌血运重建术的临床分析

目的总结冠心病患者行冠状动脉旁路移植术(CABG)和激光心肌血运重建术(TMLR)中的治疗难点、围术期处理要点,以提高冠心病患者的外科治疗效果。方法1997年5月~2006年1月,1405例冠心病患者中在体外循环下行CABG825例,其中单纯CABG666例,CABG+心瓣膜手术98例,CABG+室壁瘤手术55例,CABG+左心房粘液瘤摘除术2例,CABG+室间隔穿孔修补术2例,CABG+升主动脉成形术1例,CABG+纵隔内肿瘤切除术1例;非体外循环下CABG(OPCAB)500例;单纯TMLR30例,CABG+TMLR50例。结果每例移植旁路血管2.9±1.0支。住院死亡42例(3.0%),死亡原因包括出血、心肌梗死、低心排血量综合征、肾功能衰竭、多器官功能衰竭等。术后发生并发症70例,包括出血、低心排血量综合征、心肌梗死、肾功能不全等,均经积极的对症处理后治愈或好转。术前心绞痛(CCS)为~级的1177例患者中,术后1154例(98.0%)改善为0~级。术后随访857例(62.9%),随访时间8.3±2.9个月。随访6个月时788例(91.9%)无心绞痛发作,复查超声心动图提示:左心室射血分数0.66±0.10,较术前提高7.9%,生活质量较术前大为提高。结论CABG已成为治疗冠心病最有效的常规手术,只要能正确掌握适应证,有效地加强围术期管理,便可以扩大手术适应证范围,降低手术死亡率和并发症发生率,提高手术疗效。