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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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Even if the relationships between nutrition and inflammatory bowel disease (IBD) remain underexplored, the current literature is providing, day by day, much more evidence on the effects of various diets in both prevention and treatment of such illnesses. Wrong dietary habits, together with other environmental factors such as pollution, breastfeeding, smoke, and/or antibiotics, are among the theoretical pathogenetic causes of IBD, whose multifactorial aetiology has been already confirmed. While some of these risk factors are potentially reversible, some others cannot be avoided, and efficient treatments become necessary to prevent IBD spread or recurrence. Furthermore, the drugs currently available for treatment of such disease provide low-to-no effect against the symptoms, making the illnesses still strongly disabling. Whether nutrition and specific diets will prove to effectively interrupt the course of IBD has still to be clarified and, in this sense, further research concerning the applications of such dietary interventions is still needed.  相似文献   
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This study aimed to observe the therapeutic effects of magnesium lithospermate B on acute and chronic colitis induced by dextran sodiumsulfate (DSS) and the role of inflammasome complex (NOD-like receptor protein, NLRP; apoptosis-associated speck-like protein containing, ASC; caspase-1). Establishment of acute and chronic colitis models were by using 5% DSS oral administration in BALB/C male mice. Magnesium lithospermate B (240 mg/kg body weight) was given by subcutaneous injection. Samples were collected for biomarker assay, histological examination, immunohistochemical evaluation and western blot. There was obvious increase in TNF-α level and NLPR3, ASC, and caspase-1 expressions in acute and chronic colitis groups compared with the normal control. Significant decrease of the tumor necrosis factor-α level and the expressions of NLPR3, ASC, and caspase-1 were observed after treatment with magnesium lithospermate B. This study showed that magnesium lithospermate B could be used to treat acute and chronic colitis by inhibiting the activation of the NLRP3/ASC/Caspase-1 pathway.  相似文献   
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BackgroundManagement of inflammatory bowel diseases (IBD) in the emergency department is often suboptimal.AimsTo develop a national consensus checklist of indicators to facilitate decision-making in emergency departments concerning hospitalisation and referral for abdominopelvic computed tomography (CT).MethodsA Delphi survey was used to obtain consensus on a checklist of clinical and biological variables. 119 healthcare professionals experienced in treating IBD were invited to participate. Panellists were provided with a literature survey and invited to agree or disagree with items on a prototype checklist. Two successive rounds of voting were organised.ResultsThe prototype checklist included fifteen clinical or laboratory indicators for hospitalisation or CT. Four indicators were not retained in the Delphi process and four additional indicators added. The final indicators retained were: abdominal signs/symptoms of disease exacerbation, intravenous morphine titration, fever, vomiting, dehydration, recent intestinal surgery, ano-perineal abscess, bowel obstruction, haemodynamic instability, anaemia, acute kidney failure and elevated C-reactive protein. Consensus for the retained indicators was >88%.ConclusionsUse of this consensus checklist for the management of IBD in the emergency department may help improve standards of care and thus reduce the burden of these diseases.  相似文献   
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目的探讨化浊解毒方治疗溃疡性结肠炎(UC)的治疗作用及机制。方法120例UC患者按照随机数字表法分为观察组、对照组各60例。观察组予中药化浊解毒方口服,每日1剂,早晚2次温服;对照组予美沙拉嗪肠溶片口服,1.0 g/次,3次/d。2组疗程均4周。对比2组治疗前后Geboes指数、结肠镜下黏膜表现、生活质量评分、疾病活动指数及血清炎性因子IL-8、IL-35水平,凝血指标血清FIB水平,统计治疗后1年内复发情况。结果治疗后,观察组Geboes指数、疾病活动指数及血清炎性因子IL-8水平、凝血指标血清FIB水平均较本组治疗前降低,生活质量评分、血清炎性因子IL-35水平升高(P<0.05);且观察组治疗后疾病活动指数及血清炎性因子IL-8水平、凝血指标血清FIB水平均低于对照组,生活质量评分、血清炎性因子IL-35水平均高于对照组(P<0.05)。与对照组相比,治疗组糜烂、溃疡改善不明显(P>0.05),充血水肿、颗粒样变等肠镜表现改善情况均优于对照组(P<0.05)。治疗结束1年观察组复发率为10.64%,对照组为23.53%,2组差异有统计学意义(P<0.05)。结论化浊解毒方能改善UC患者临床症状,修复肠黏膜病理损伤,降低复发率;其机制可能与调节血清炎性因子IL-8、IL-35和凝血因子FIB水平有关。  相似文献   
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溃疡性结肠炎内镜、病理特点及其临床意义   总被引:3,自引:0,他引:3  
目的探讨溃疡性结肠炎内镜及病理组织学检查的临床特点及其意义。方法采用分级的方法描述219例活动期溃疡性结肠炎以及53例治疗后临床症状完全缓解者的内镜、病理组织学特点。运用Spearman等级相关系数进行相关分析。结果本组219例活动期溃疡性结肠炎内镜分级主要分布在Ⅱ~Ⅲ级,占59.8%。病理组织学分级主要分布在Ⅲ~Ⅳ级,占79.9%(r=0.1692,P=0.0122)。经治疗后4周~8个月间,53例临床症状完全消失。内镜分级由治疗前的Ⅲ~Ⅳ级向Ⅰ~Ⅱ级转归,而病理组织学分级Ⅳ级为22.7%(r=0.3007,P=0.0287)。内镜分级与病理组织学分级两者间均无相关性。结论本组内镜及病理组织学分级描述溃疡性结肠炎病情以及疗效有不一致性。早期诊断以及近期疗效的判断不仅应依靠临床症状及内镜检查所见,更应结合病理组织学检查。  相似文献   
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目的探讨全结肠切除后三环形回肠贮袋-肛管吻合加选择性截流术的价值。方法1994年9月至2004年9月对18例全结肠切除患者应用三环形回肠贮袋加选择性截流术,其中家族性大肠腺瘤性息肉病14例,溃疡性结肠炎4例。结果18例获12月~5年随访,无手术死亡。术后发生并发症3例(16.7%),贮袋阴道瘘、早期炎性肠梗阻、切口感染各1例,均治愈。术后平均排便频率:术后2周内4.9次/d,1个月3.8次/d,6个月3.1次/d,1年1.8次/d。至6个月时都能控制干便,其中控制正常者15例(83.3%),控制欠佳者3例(16.7%),无大便失禁。但稀便控制能力较差,至1年时仍有1例(5.6%)患者稀便失禁。无一例须插管排空。结论全结肠切除后三环形回肠贮袋肛管吻合加选择性截流术操作简单,手术并发症少,术后排便功能好,是家族性大肠腺瘤性息肉病和严重溃疡性结肠炎全结肠切除后较为合适的消化道重建方式。  相似文献   
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