主动脉弓阻塞合并心脏畸形18例的一期矫治

目的总结一期矫治主动脉弓阻塞合并心脏畸形的诊治经验。方法2005年1月至2006年6月一期矫治18例主动脉弓阻塞合并心脏畸形。其中主动脉弓中断(IAA)6例,主动脉缩窄(COA)12例。手术均为正中开胸低温体外循环下一期畸形矫治术。结果手术采用降主动脉与主动脉弓端端吻合11例,端侧吻合6例,Gore-Tex片加宽弓成形1例。围术期死亡1例,总死亡率5.56%。喉返神经损伤2例,术后左主支气管受压1例。随访1～11个月,1例上下肢压差25mmHg,6例压差小于20mmHg,7例端侧吻合无压差。存活者无症状,生长发育良好。结论正中开胸一期矫治COA及IAA合并心脏畸形可获得满意的近期疗效。手术成功的关键是充分游离降主动脉,尽可能行端侧吻合术。     

婴幼儿主动脉缩窄合并心内畸形的Ⅰ期手术纠治

目的:总结主动脉缩窄合并心内畸形的外科治疗经验。方法:回顾分析2007年1月至2011年6月我院收治的婴幼儿主动脉缩窄合并心内畸形Ⅰ期手术治疗的临床病例。共计42例,其中男性26例,女性16例。平均年龄(1.6±0.9)岁(6 d～3岁),平均体质量(7.2±2.5)kg(2.8～12kg)。主动脉缩窄合并心内畸形:37例合并室间隔缺损,5例合并房间隔缺损,7例同时合并主动脉瓣二瓣畸形,手术方法采用双切口I期纠治5例,正中切口I期纠治37例,其中锁骨下动脉翻转法(8例)、人工补片法(6例)、端端吻合法(15例)、端端吻合+补片法(13例)。结果:本组42例中,死亡1例,术后9 d死于肺部感染,病死率2.4%。术后均复查心脏彩超显示降主动脉血流通畅,无明显狭窄,3例提示降主动脉内仍存在20～40 mmHg(1 mmHg=0.133 kPa)压差,心脏彩超示吻合口处狭窄,血流速度增快。结论:婴幼儿主动脉缩窄合并心内畸形采用深低温选择性脑灌注方法经胸骨正中切口I期手术治疗可以达到满意疗效。彻底切除缩窄段及导管组织是手术成功的关键。     

婴幼儿主动脉缩窄合并心内复杂畸形的一期手术治疗

目的:探讨婴幼儿主动脉缩窄合并心内复杂畸形一期手术的治疗方法及手术效果.方法:回顾分析2002-11至2008-09收治的婴幼儿主动脉缩窄合并心内复杂畸形一期手术治疗的临床病例.共计18例,其中男12例,女6例.年龄平均(7.7±8.8)个月,体重(6.1±2.4)kg.主动脉缩窄包括合并主动脉弓发育不良5例,单纯主动脉缩窄13例.不合并主动脉弓发育不良者,切除缩窄段,端端吻合降主动脉;合并主动脉弓发育不良者,切除缩窄段,用自体心包或自体肺动脉片加宽主动脉弓.心内复杂畸形的矫治包括完全性大动脉转位大动脉调转术8例,右心室双出口根治术6例,右心室双出口陶西平畸形大动脉调转术3例,完全性心内膜垫缺损矫治术1例.结果:所有患儿出院前超声心动图检查均显示主动脉弓降部缩窄解除,吻合口通畅,血流正常.无中枢神经系统并发症.术后早期上、下肢收缩压基本相等者4例,上肢高于下肢15～20mmHg者8例,上肢低于下肢收缩压5～10mmHg者6例.死亡3例,死亡率16.7%.1例术后当天死于低心排综合征,1例术后9天死于肺部感染,1例术后7天死于突发恶性心律失常.术后另3例出现右侧膈肌麻痹行膈肌折叠术.结论:婴幼儿主动脉缩窄合并心内复杂畸形经胸骨正中切口一期手术治疗,主动脉弓及降主动脉上段显露良好.同期矫治心内复杂畸形手术效果满意.深低温停循环区域性脑灌注可有效降低神经系统并发症的发生.     

一期升主动脉-降主动脉转流治疗主动脉缩窄合并心脏畸形

目的:主动脉缩窄合并其他心脏病手术的方法仍然存在争议。本研究的目的是评估经胸骨正中切口一期升主动脉-降主动脉转流+心脏畸形矫治术治疗主动脉缩窄合并其他心脏畸形的疗效。方法:选择我院于2009年4月至2017年6月,应用经胸部正中切口行升主动脉-降主动脉心包内转流术,同期行合并心脏畸形矫治手术的患者13例,女性3例,男性10例,平均年龄35岁(19～59岁)。其中3例bentall术,8例主动脉瓣置换,1例二尖瓣置换,1例二尖瓣成形术。结果:随访期间无死亡病例,主动脉阻断时间和体外循环时间分别是(81±33) min、(123±47) min。术后上肢血压明显改善(P0.001),有术前的(159±34) mmHg(1 mmHg=0.133 kPa)将至术后(122±17) mmHg,截止最后一次随访,患者上下肢血压无明显压差。结论:一期升主动脉-降主动脉转流治疗主动脉缩窄合并心脏畸形远期效果显著,可以作为主动脉缩窄合并其他心脏畸形的患者选择此手术方式。     

新生儿主动脉缩窄的外科治疗

目的:回顾总结新生儿主动脉缩窄(Co A)的外科治疗经验,探讨新生儿期Co A的最佳手术时机及手术方式。方法:2010年7月至2014年3月,共收治新生儿Co A 31例,男性20例,女性11例。手术年龄9~30天,平均(24.8±6.1)天,体质量2~4.1kg,平均体质量(93.2±0.7)kg。合并心内畸形者23例,合并PDA者4例,单纯Co A 4例。一期手术21例;分期手术10例,首先于非体外循环下行Co A矫治+肺动脉环缩术,随访25天~12个月后再行心内畸形矫治术。Co A矫治的方法包括:缩窄段切除行端端吻合、扩大的端端吻合或端侧吻合术、扩大的端端吻合+补片成形术、左锁骨下动脉翻转主动脉成形术。结果:1例死于出血,病死率3.2%;1例术后心肺功能较差,无法撤离呼吸机,放弃治疗;术后无肾衰竭、左上肢缺血及神经系统并发症发生。全组术毕有创动脉监测上、下肢动脉平均压差7.2mm Hg(1mm Hg=0.133k Pa)较术前39.1mm Hg明显下降(P<0.05)。结论:严重的新生儿Co A,病情危重,需早期诊断、及时治疗;对于合并心内畸形者,若导致心功能不全的主要原因为Co A、不能耐受体外循环的患儿行分期手术安全可靠;Co A合并主动脉弓发育不良者,采应扩大端端吻合或端侧吻合术可获得满意疗效。     

主动脉缩窄患儿围手术期处理——附40例临床资料分析

目的：总结主动脉缩窄围手术期情况。方法：整理了１９８８年至１９９６年收治的主动脉缩窄患儿４０例，其中合并动脉导管未闭（ＰＤＡ）２６例，心室间隔缺损（ＶＳＤ）１４例，心房间隔缺损（ＡＳＤ）２例。手术方法包括：Ｇｏｒｅ－ｔｅｘ补片扩大２４例，左锁骨下动脉瓣作主动脉成形术１０例，人造血管移植，缩窄端切除端端吻合，狭窄部锲形切除直接缝合各２例。结果：死亡１例，术后高血压３５例，乳糜胸１例，喉返神经麻痹２例，肾功能不全１０例，未发现下肢活动障碍。结论：术前充分估计侧支循环情况，术中选择合适的手术方法，对术后并发症的防治至关重要。     

扩大端端或端侧吻合术矫治主动脉缩窄伴弓发育不良

目的:分析本中心采用扩大端端或端侧吻合术矫治主动脉缩窄(CoA)伴弓发育不良(HAA)的早中期效果,并总结将该术式应用于临床的相关经验。方法:回顾性分析2011-06-2016-10于本中心采用扩大端端或端侧吻合术矫治CoA伴HAA的45例患儿临床资料,其中男29例,女16例;年龄0.1～9.0岁,平均(1.6±3.8)岁;体质量2.5～17.3kg,平均(6.8±5.7)kg。按照手术年龄将患儿分为低龄组(≤1岁)和高龄组(1岁)。手术采用正中开胸选择性脑灌注下行扩大端端或端侧吻合术,总结并分析2组患儿手术前后缩窄段压差、肺动脉压力及主动脉Z值等变化。结果:所有手术均顺利完成,1例患儿于术后24h再次手术;术后早期死亡1例,11例发生严重并发症的患儿经积极治疗后均顺利恢复。全组随访40例(90.9%),随访9～75个月,平均(25.0±13.5)个月,随访期间无死亡。发生高血压的患儿10例,全组主动脉缩窄处平均压差为(10.7±3.4)mmHg,3例缩窄处压差25mmHg,2例已行球囊扩张术,1例继续随访观察。组内比较:2组患儿术后早期及随访时CoA患处压差及平均肺动脉压均显著低于术前(P0.05),主动脉Z值较术前均明显增大(P0.05)。组间比较:高龄组患儿术前及随访时肺动脉压力均较高(P0.05),术中选择性脑灌注时间、主动脉阻闭时间、体外循环时间及停机后止血时间均较长(P0.05),术后引流较多(P0.05),呼吸机时间、ICU时间及住院天数均较长(P0.05),但术后低龄组患儿CoA患处压差较高(P0.05),且主动脉横弓Z值及峡部Z值均较低(P0.05)。结论:对于CoA伴HAA患儿明确诊断后应尽早手术,采用扩大端端或端侧吻合术矫治具有较理想的早中期效果。     

新法矫治主动脉缩窄

目的:探讨不同方法治疗主动脉弓缩窄的疗效。方法:从2017年1月到2018年6月我们尝试一种新的方法矫治主动脉缩窄15例。在非体外循环下阻断主动脉进行矫治手术期间,将监测上、下肢动脉血压的通路通过侧支管路连通,使缩窄段近端高压的血流可以通过侧支管路分流到低压的缩窄段远端,增加主动脉阻断期间缩窄段远端的血供,使手术安全时限延长。关闭侧支通路时可以检验缩窄矫形效果。取同期未采用此方法,但诊断、年龄和缩窄程度等特征匹配的18例患儿做对比。结果:分别采用新旧两种方法的两组患儿术后均没有残余压差,主动脉阻断时间和术后上下肢压差差异无统计学意义(P≥0. 05)。但吻合期间缩窄远端的血压差异有统计学意义(P≤0. 01)。结论:新法使手术操作更加从容,虽然与前期方法相比,矫治效果无显著的差异,这可能是病例数较少的缘故。但此法优势明显,值得推广。     

自体肺动脉补片和扩大端端/端侧术矫治婴幼儿主动脉缩窄疗效分析

目的:比较分析本中心主动脉弓肺动脉补片成形术和扩大端端或端侧吻合术治疗主动脉缩窄(CoA)伴主动脉弓发育不良(HAA)的矫治效果,为临床提供更合理的手术方法。方法:回顾性分析手术方法为肺动脉补片成形和扩大端端/端侧矫治的87例CoA伴HAA患儿临床资料,其中42例患儿采用自体肺动脉补片加宽术为肺动脉补片组,45例患儿采用扩大端端/端侧吻合术为扩大端端组。比较分析2组患儿手术前后缩窄段压差及主动脉Z值变化,并进行随访。结果:术后早期扩大端端组死亡患者1例;余86例中发生严重并发症19例,其中肺动脉补片组8例,扩大端端组11例,19例患者经积极治疗后均顺利康复,全组未发现明显脑部并发症。肺动脉补片组体外循环时间、主动脉阻断时间及呼吸机辅助时间与扩大端端组相比,差异无统计学意义(P0.05),但肺动脉补片组选择性脑灌注时间及ICU时间明显延长(P0.05),超声所测肺动脉补片组术后早期平均缩窄段压差较术前显著降低[(9.7±3.5)mmHg∶(36.8±12.5)mmHg,P0.05],横弓及峡部Z值较术前明显增高[(-0.50±0.21)、(-0.45±0.24)∶(-3.38±0.64)、(-2.22±0.43),P0.05)];扩大端端组术后早期平均缩窄段压差较术前显著降低[(9.5±3.3)mmHg∶(37.2±8.9)mmHg,P0.05],横弓及峡部Z值较术前明显增高(-0.52±0.29、-0.48±0.22∶-3.89±0.61、-2.45±0.33,P0.05)。随访75例,随访4~106(32±12.8)个月,肺动脉补片组发生再缩窄5例,2例已行主动脉球囊扩张术,其余3例继续随访;扩大端端组发生再缩窄3例(6.98%),2例已行球囊扩张术,1例继续随访。Kaplan-Meier曲线分析表明,扩大端端补片组免于再狭窄率稍优于肺动脉补片组,但差异无统计学意义(P0.05)。结论:自体肺动脉成形及扩大端端/端侧吻合术均为矫治CoA伴HAA较为理想手术方式,早期矫治效果无明显差异,中期随访表明扩大端端组矫治效果稍优于肺动脉补片组。     

Taussig-Bing畸形合并主动脉弓病变的外科治疗

目的：总结Taussig-Bing畸形合并主动脉弓病变的手术治疗经验。方法：2012年3月至2017年5月手术治疗5例Taussig-Bing畸形合并主动脉弓病变的患儿,其中主动脉弓中断（Interrupted aortic arch,IAA）3例,主动脉缩窄（Coarctation of aorta,COA）2例。3例患儿采用一期大动脉调转术(Arterial switch operation,ASO)+IAA矫治术（降主动脉与主动脉弓端侧吻合）;1例患儿分期手术（一期行COA矫治术,二期行ASO手术）;1例患儿行ASO手术+COA矫治术。结果：全组患儿均顺利出院。气管插管时间平均（158.20±55.64）h;ICU停留时间平均（13.20±3.42）d;住院时间平均（42.00±11.04）d;术后早期并发症包括低心排血量综合征1例、术后出血1例、少尿和腹膜透析1例、肺不张1例。术后随访患儿无因残余解剖问题,无再次手术者。结论：Taussig-Bing畸形合并主动脉弓病变病理解剖比较复杂,手术方式与合并主动脉弓病变畸形的类型对治疗效果有影响,合并IAA患儿应一期矫治,合并COA的个别患儿可选择二期手术矫治。     

A patient with rectal ulcer with severe stenosis presenting with perforated peritonitis

We report a patient with rectal ulcer with severe stenosis, who underwent urgent surgical treatment for perforated peritonitis. The 54-year-old man suddenly developed cramping abdominal pain and fever while hospitalized, with signs of peritoneal irritation. An emergency laparotomy was performed, and severe stenosis of the rectum and a perforated lesion on the oral side approximately 10 cm distant from the stenosis were found, with massive abdominal purulent fluid. He was treated by rectosigmoid colon resection with transverse colon loop colostomy. Histopathologically, the stenosis was caused by ulceration extending to all muscular layers of the rectum, with inflammatory changes. Benign rectal stenosis is so rare that differential diagnosis from malignancy may be difficult when there are inflammatory changes in the surrounding tissues. However, it is necessary to keep in mind the likelihood of this disease in differentiation from rectal cancer. Received: December 21, 1998 / Accepted: May 28, 1999     

Evaluation of atrial vulnerability with transoesophageal stimulation in patients with atrioventricular junctional: Comparison with patients with ventricular pre-excitation and with normal subjects

The aim of our work was to evaluate the inducibility of atrialfibrillation in a group of patients with atrioventricular junctionalreentrant tachycardia and to compare it with that of patientswith a Kent-type ventricular pre-excitation (Wolff-Parkinson-Whitesyndrome) and a control group. One hundred and twenty-five subjects were separated into groups.Group 1 comprised 49 Wolff-Parkinson-White patients, with amean age of 26.4, range 10.66 years; group 2, 51 patients withatrioventricular junctional reentrant tachycardia inducibleby transoesophageal atrial stimulation andlor clinically documented,with a mean age of 43.4, range 16–78 years; group 3, 25control subjects with a mean age of2.64, range 13–76 years. Each subject underwent atrial transoesophageal stimulation withthe following protocol: programmed atrial stimulation with 1and 2 stimuli during atrial pacing of 100. min^–1 and 150.min^–1; atrial stimulation for 10 s at a rate of 200–300–400–500–600.min^–1 with intervals of 10 s between stimulations, fivesuccessive ‘ramp-up’ atrial stimulations for 9 swith the rate increasing from 100 to 800. min^–1 with intervalsof 10 s between stimulations. The end point was the completionof the protocol or induction of sustained atrial fibrillation(>1 min). The chi-square test was used for statistical analysis. Our resultsshowed that in group 1 atrial fibrillation was induced in 27149patients (55.1%); this was sustained in 13149 (26.5%) and non-sustainedin 14149 (28.5%); in group 2, atrial fibrillation was inducedin 22151 patients (43.0%); it was sustained in 7151 (13.7%)and non-sustained in 15151 (29.4%); in group 3, sustained atrialfibrillation was not induced in any subject and in only onesubject was a non-sustained atrial fibrillation (4 s) induced. The chi-square test showed that group 2 vs group 1 were non-significant,while group 2 vs group 3 and group 1 vs group 3 were significant(P<0.003 and P<0.0007, respectively). Therefore group 2 patients showed a greater atrial vulnerabilityin comparison to the control subjects and a similar vulnerabilityto group 1 patients. It is possible that the greater atrialvulnerability in the patients of group 2 was due to the doublenodal pathway.     

肿瘤病人弓形虫感染分析

在肿瘤的发生和发展进程中 ,多伴有免疫功能低下或缺陷 ,从而极易遭受各种感染。弓形虫是机会感染因子 ,当患者免疫功能受损时 ,易于感染 ,还会使隐性感染激活 ,引起低热不退、淋巴结肿和脑神经系统的反应 ,此现象尚未引起临床医师的重视。近年来 ,我们对 4 0 9例肿瘤病人进行了弓形虫感染及弓形虫病的分析观察 ,报告如下 :1　材料与方法1 1　材料　 30 4例病人血清取自江西省肿瘤医院住院或门诊病人 ,随机抽样后低温保存待检 ,10 5例取自其他医院送检样品 ,有急性症状者随到随检 ,以便及时做病原学检测。1 2　弓形虫病诊断方法1 2 1　免疫…     

Infection with Rhodococcus equi in a patient with sarcoidosis treated with corticosteroids

A 51-year-old female farmer was diagnosed as having sarcoidosis. During 4 years of observation, slow radiological progression was observed. Cough then developed, necessitating treatment with corticosteroids. After 28 months of continuous treatment with prednisolone in low doses (5-7.5 mg daily), she suffered fever episodes, recurrent haemoptyses, general malaise and loss of weight. A chest roentgenogram showed a left upper lobe infiltrate, which progressed and finally cavitated, and rib destruction. Despite efforts, including a thoracotomy, 22 months passed before a diagnosis could be made. Blood and sputum cultures and cultures from the destroyed rib showed growth of Rhodococcus equi, a common soil organism which can cause infections in foals and other animals. Treatment with rifampicin and erythromycin was successful. R. equi has been reported to cause infection in patients with neoplastic disease and/or immunosuppression, but the disease might be more common than is suggested by the sparse case reports in the literature, owing to lack of familiarity with the organism, which will tend to be overlooked as a contaminant.     

Treating patients with lupus with B-cell depletion

A new era in the treatment of systemic lupus erythematosus has dawned with the increasing introduction of monoclonal antibodies and other approaches, that target the key molecules involved in the pathogenesis of the disease. At present the ability to block the CD20 molecule on those B cells that carry this marker has proved the most effective way to treat patients resistant to conventional immunosuppressive drugs. However, these studies have all been open label and the results of double blind controlled studies are eagerly awaited.     

Myoclonic Epilepsy with Ragged-red Fibers with Intranuclear Inclusions

We herein report a case of myoclonic epilepsy with ragged-red fibers (MERRF) harboring a novel variant in mitochondrial cysteine transfer RNA (MT-TC). A 68-year-old woman presented with progressive myoclonic epilepsy with optic atrophy and peripheral neuropathy. A skin biopsy revealed p62-positive intranuclear inclusions. No mutations were found in the causative genes for diseases known to be related to intranuclear inclusions; however, a novel variant in MT-TC was found. The association between intranuclear inclusions and this newly identified MERRF-associated variant is unclear; however, the rare complication of intranuclear inclusions in a patient with typical MERRF symptoms should be noted for future studies.