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Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4  相似文献   
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BackgroundCoronary artery bypass grafting (CABG) improves survival in patients with heart failure and severely reduced left ventricular systolic function (LVEF). Limited data exist regarding adverse cardiovascular event rates after CABG in patients with heart failure with midrange ejection fraction (HFmrEF; LVEF > 40% and < 55%).MethodsWe analyzed data on isolated CABG patients from the Veterans Affairs national database (2010-2019). We stratified patients into control (normal LVEF and no heart failure), HFmrEF, and heart failure with reduced LVEF (HFrEF) groups. We compared all-cause mortality and heart failure hospitalization rates between groups with a Cox model and recurrent events analysis, respectively.ResultsIn 6533 veterans, HFmrEF and HFrEF was present in 1715 (26.3%) and 566 (8.6%) respectively; the control group had 4252 (65.1%) patients. HFrEF patients were more likely to have diabetes mellitus (59%), insulin therapy (36%), and previous myocardial infarction (31%). Anemia was more prevalent in patients with HFrEF (49%) as was a lower serum albumin (mean, 3.6 mg/dL). Compared with the control group, a higher risk of death was observed in the HFmrEF (hazard ratio [HR], 1.3 [1.2-1.5)] and HFrEF (HR, 1.5 [1.2-1.7]) groups. HFmrEF patients had the higher risk of myocardial infarction (subdistribution HR, 1.2 [1-1.6]; P = .04). Risk of heart failure hospitalization was higher in patients with HFmrEF (HR, 4.1 [3.5-4.7]) and patients with HFrEF (HR, 7.2 [6.2-8.5]).ConclusionsHeart failure with midrange ejection fraction negatively affects survival after CABG. These patients also experience higher rates myocardial infarction and heart failure hospitalization.  相似文献   
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目的:探究原发性肝癌患者手术切除术后早期复发影响因素分析。方法:对广州市番禺区中医院2017年2月-2019年2月收治的原发性肝癌患者98例进行回顾性分析,依据各项临床指标分析手术切除术后早期复发的影响因素。结果:原发性肝癌患者经由手术切除后出现复发的可能性较高,同时手术切缘有残留、包膜不完整、肿瘤结节、心理因素、甲胎蛋白、血管侵犯、肝硬化及肿瘤直径等均是影响原发性肝癌手术期后再复发的高危因素。结论:原发性肝癌通常具有术后复发的生物学特性,依据对手术切除术之后早期复发因素的分析,对手术风险与治疗方案进行充分评估,同时要求患者进行定期复查及随访等工作,可对患者手术成功以及并发症的防治等均具有积极意义。  相似文献   
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Choledochojejunostomy (CJS) is commonly used for biliary reconstruction in liver transplantation for primary sclerosing cholangitis (PSC). We alternatively performed choledochoduodenostomy (CDS) and side-to-side choledochodocholedochstomy in a large cohort of patients. Fifty-one patients with PSC, transplanted between 1988 and 2000, were analyzed retrospectively. Biliary reconstruction was CDS in 25 (49%), CJS in 20 (39%) and CC in 6 transplantations (12%). Biliary leaks occurred in the early follow-up (< or =41 days) only in CDS patients (20%). However, in the late follow-up (>4 months), stricturing of anastomosis was found once in CDS (4%) and CJS (5%). Later (>9 months), intrahepatic bile duct strictures were diagnosed in four CDS (16%), one CJS (5%) and one CC (17%) patient(s). In 48% of CDS (12/25), 60% of CJS (12/20) and 17% of CC (1/6) at least one incidence of cholangitis was observed. Overall, biliary complication rates were significantly higher in CDS (40%) than CJS (10%) and CC (17%); of those none in CC and 12% in CDS were anastomosis-related. Graft/patient survival showed no significant differences among groups. Based on our results we consider CJS the standard method for biliary reconstruction in PSC; however, in selected cases where CJS is difficult to accomplish because of previous surgery or for retransplantation, CDS may present an alternative technique.  相似文献   
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The severity of illness in transplant patients and the complexity of transplant operations results in significant postoperative morbidity and mortality. Remarkable efforts have been made by transplant physicians to study and improve organ allocation, graft and patient survival, immunosuppression and the long-term management of post-transplant complications. Less effort has been spent studying the actual transplant operation and systems of acute transplant care. The National Surgical Quality Improvement Program (NSQIP) has provided a standardized approach to quality improvement and has demonstrated significant potential for a reduction in postoperative morbidity and mortality in other surgical disciplines. Medical centers are under increasing pressure to measure surgical quality and the nexus of transplant surgical quality improvement should not lie in the hands of CMS or JACHO, but rather it should be created and developed within the transplant community. The time has come for a national transplant surgical quality improvement program based on the NSQIP infrastructure. Such a proactive approach toward quality improvement from the transplant community is an excellent investment for patients, providers and health care payers.  相似文献   
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目的 总结下肢动脉硬化性闭塞症介入治疗围手术期相关并发症的防治经验.方法 对207例下肢动脉硬化件闭塞症行介入治疗,统计围手术期各种并发症的发生情况,分析并发症的发生率与泛大西洋介入协会(transAtlantic inter-society consensus,TASC)分型分级之间的关系以及糖尿病和冠心病对重要脏器并发症发生率的影响.结果 本组207例,介入手术成功190例.17例失败,其中13例为介入操作失败、4例死于术后重要脏器并发症.围手术期并发症包括穿刺点出血12例、假性动脉瘤4例、消化道出血2例、动脉破裂6例、脑梗塞8例、急性心衰9例、呼衰13例、肾衰6例、支架内血栓形成5例和蓝趾综合征1例.在本组患者的TASC分型中,股腘动脉病变介入治疗并发症的发生率为39.84%(51/128),高于主髂型的18.99%(15/79)(P<0.05);患有糖尿病以及合并糖尿病和冠心病患者重要脏器并发症的发生率分别为27.66%(13/47)和24.49%(12/49),高于无合并症患者的5%(2/40)(P<0.05).结论 下肢动脉硬化性闭塞症介入治疗围手术期并发症发生率较高可能与术中操作不当、病变类型复杂及合并糖尿病和冠心病有关,术前注意合并症的处理及术中选择合理治疗方式、缩短手术时间等可减少并发症的发生.  相似文献   
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在颈椎前路减压、植骨、带锁钢板内固定技术日渐成熟的今天,越来越多的医院开展了该项手术。自1998年2月-2005年8月应用该方法治疗颈椎病215例,发生与内置物钢板及钛网相关的并发症14例,为此积累了一定的经验,现总结如下。1临床资料本组215例,男126例,女89例;年龄42~82岁,平均  相似文献   
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我国大陆恶性高热病例的分析   总被引:16,自引:4,他引:12  
目的 分析我国大陆恶性高热(MH)病例的临床资料,探讨诊断和治疗中应注意的问题。方法 检索中国生物医学文献数据库,收集1978年1月至2004年3月报道的MH病例34例,分析其流行病学特征、发病特点、临床表现、治疗及转归。结果 我国大陆报道的MH病例以青少年居多,男性多于女性,集中在口腔科手术(尤其是唇腭裂修补术)、骨科手术和小儿外科手术(尤其是先天畸形矫治术),典型的临床表现为突然发生的高碳酸血症、体温急剧升高和骨骼肌僵直等。诊断上主要是根据临床表现,尚未建立骨骼肌体外收缩试验方法,治疗上主要是对症处理,缺乏特效药物(丹曲林)。结论 近几年我国大陆MH病例逐渐增加,应加强对MH发病机制的研究,尽快建立MH的实验诊断标准,增强预防、诊断和治疗MH的能力。  相似文献   
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