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《Journal of pediatric surgery》2023,58(5):856-861
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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Jesus K Yamamoto-Furusho Norma N Parra-Holgu n 《World journal of gastroenterology : WJG》2021,27(48):8242-8261
Inflammatory bowel disease (IBD) is a chronic disease that requires chronic treatment throughout the evolution of the disease, with a complex physiopathology that entails great challenges for the development of new and specific treatments for ulcerative colitis and Crohn´s disease. The anti-tumor necrosis factor alpha therapy has impacted the clinical course of IBD in those patients who do not respond to conventional treatment, so there is a need to develop new therapies and markers of treatment response. Various pathways involved in the development of the disease are known and the new therapies have focused on blocking the inflammatory process at the gastrointestinal level by oral, intravenous, subcutaneous, and topical route. All these new therapies can lead to more personalized treatments with higher success rates and fewer relapses. These treatments have not only focused on clinical remission, but also on achieving macroscopic changes at the endoscopic level and microscopic changes by achieving mucosal healing. These treatments are mainly based on modifying signaling pathways, by blocking receptors or ligands, reducing cell migration and maintaining the integrity of the epithelial barrier. Therefore, this review presents the efficacy and safety of the new treatments that are currently under study and the advances that have been made in this area in recent years. 相似文献
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目的:观察翁舌汤灌肠治疗大肠湿热型溃疡性结肠炎的疗效及其对患者血清炎症因子水平的影响。方法:将80例大肠湿热型溃疡性结肠炎患者按随机数字表法分为观察组和对照组各40例。观察组给予翁舌汤灌肠治疗,对照组给予美沙拉嗪栓肛门塞入治疗,两组均连续治疗8周。比较两组患者的临床疗效、肠黏膜征象积分和血清炎症因子水平。结果:治疗后,观察组和对照组总有效率分别为95.00%、70.00%,两组疗效比较,差异有统计学意义(P<0.05)。治疗后,观察组患者炎症增生、黏膜溃疡、黏膜糜烂、黏膜水肿、黏膜充血积分均下降,与治疗前比较,差异有统计学意义(P<0.05);且观察组各项积分均低于对照组,差异有统计学意义(P<0.05)。治疗后,两组血清白细胞介素-8(IL-8)、肿瘤坏死因子-β(TNF-β)水平均降低,血清白细胞介素-10(IL-10)水平均升高,与治疗前比较,差异均有统计学意义(P<0.05);且观察组血清IL-8、TNF-β水平明显低于对照组,血清IL-10水平明显高于对照组,差异有统计学意义(P<0.05)。结论:翁舌汤灌肠治疗大肠湿热型溃疡性结肠炎疗效较好,可改善患者肠黏膜征象积分,调节血清炎症因子水平。 相似文献
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Biologics and immunomodulators (IMM) are generally considered the most effective therapies for the treatment of ulcerative colitis and Crohn’s disease. However, despite the efficacy of these therapies, many patients either have a primary lack of response or a secondary loss of response to these medications. Therapeutic drug monitoring (TDM) is a systematic approach to managing such patients. In this review, we summarize the latest data on TDM, including reactive and proactive TDM, in patients with inflammatory bowel disease on biologics and/or IMM. 相似文献
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Dong-Hui Xu Bo Zhou Zhi-Peng Li Lian-Ping He Xin-Juan Wang 《World journal of gastrointestinal oncology》2022,14(8):1597-1599
The original article by Yuichi et al explored whether the Japan Narrow-Band Imaging Expert Team classification and the pit pattern classification are suitable for diagnosing neoplastic lesions in patients with ulcerative colitis. In this letter, we offer some other perspectives. Risk factors for colorectal tumors include type 2 diabetes. Among genetic factors, the deletion or mutation of some genes, such as the p53 gene, can lead to colorectal tumors. There are significant gender differences in the occurrence and development of colorectal tumors. Some non-genetic factors, such as smoking, are also associated with the development of colorectal tumors. These all suggest that colorectal tumors are not only caused by ulcerative colitis, and we suggest further exploration and differentiation between colitis and colorectal tumors. 相似文献
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Salvatore Greco Beatrice Bonsi Nicol Fabbri 《World Journal of Experimental Medicine》2022,12(5):104-107
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