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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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目的研究新生儿肺炎(NP)患儿血清高敏C反应蛋白(hs-CRP)、降钙素原(PCT)、白细胞介素-6(IL-6)水平表达及其意义。方法选取2017年1月-2018年2月韶关市曲江区妇幼保健计划生育服务中心诊治的NP患儿80例,按细菌感染情况分为观察1组和观察2组,各40例。选择同期40例健康新生儿作为对照组。测定并比较三组的hs-CRP、PCT和IL-6水平。结果观察1组血清PCT、hs-CRP和IL-6水平明显高于观察2组和对照组,差异均有统计学意义(P<0.05)。观察1组血清PCT、hs-CRP和1L-6检测阳性率明显高于观察2组和对照组,差异均有统计学意义(P<0.05),且观察1组血清PCT、hs-CRP和1L-6诊断NP的敏感度均明显高于观察2组,差异均有统计学意义(P<0.05);观察组各指标特异度与观察2组比较差异无统计学意义(P>0.05)。结论血清hs-CRP、PCT、IL-6水平可作为NP患儿的辅助鉴别指标,为抗生素的合理使用和疗效提供依据。  相似文献   
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Bowel diseases of prematurity, including necrotizing enterocolitis, are dreaded ailments of neonates. Early diagnosis is difficult, with clinical and radiographic findings often inconclusive. We present a novel use of contrast-enhanced ultrasound in detection of pediatric bowel disease. Early identification of compromised blood flow or an at-risk bowel can be quantitatively detected and monitored. This ability has implications for guidance of emerging therapies, allowing targeting of inflammation. These findings represent an advancement in detection of bowel disease in neonates.  相似文献   
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目的分析成人血液系统恶性肿瘤患者接受强烈化疗后中性粒细胞减少性肠炎(NE)的发生率、危险因素及预后情况。方法收集2004至2013年接受化疗的1804例血液系统恶性肿瘤患者,记录患者血常规、凝血检测和血液生化检测结果,并记录患者年龄、性别、原发病、既往化疗次数、既往化疗方案中是否使用阿糖胞苷、临床症状、肠壁厚度、中性粒细胞最低计数、中性粒细胞缺乏持续时间、NE的治疗方法和预后等,探讨NE起病诱因、临床特征、腹部B超特点、症状的预后意义及化疗药物对发病的影响等。结果1804例患者中226例(12.5%)化疗后合并NE,化疗后10~19d起病,中位起病时间为化疗后第14天。发生NE后26例患者死亡,病死率11.5%。化疗药物包括阿糖胞苷、临床症状≥4项、中性粒细胞缺乏持续超过7d以及B超下肠壁厚度≥10mm的患者病死率相对较高。结论NE是接受强烈化疗的血液系统肿瘤患者的严重的并发症,发生NE后患者病死率较高。  相似文献   
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ObjectivesThis study aimed to determine the effect of lower leg compression during cesarean section (CS) on post-spinal hypotension (PSH) and neonatal hemodynamic parameters.MethodsThis study is a nonrandomized controlled clinical trial conducted in the cesarean delivery unit of the National Medical institute, Damanhour, Egypt. The sample included 120 parturients (60 intervention and 60 control). The researchers developed three tools for data collection: sociodemographic data and reproductive history interview schedule, electronic monitoring of maternal hemodynamic parameters, and neonatal hemodynamic assessment sheet. All parturients received ordinary pre-operative care. For the intervention group, a long elastic stocking (ordinary pressure 20–30 mmHg, 1 mmHg = 0.133 kPa) was applied on both legs during cesarean section. The control group received the same care without the elastic stocking.ResultsSystolic blood pressure, diastolic blood pressure, and mean arterial blood pressure were significantly higher in the intervention group throughout the entire operation period except in the last 5–15 min. Heart rate was significantly lower in the intervention group. Only 13.3% of the intervention group took ephedrine compared with 45% of the control group. Apgar score was higher among neonates of intervention group compared with the control group at 1 min. Neonatal acidosis was significantly higher in the control group than in the contral group.ConclusionLower leg compression technique can effectively reduce PSH and neonatal acidosis.  相似文献   
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Aeromonas species can cause gastroenteritis, soft tissue infections, hepatobiliary tract and other infections. While most reported soft tissue infections have been attributed to Aeromonas hydrophila, we report a case of necrotizing fasciitis caused by Aeromonas veronii biovar sobria. Prompt and accurate diagnosis cum treatment could help save children with Aeromonas soft tissue infections from developing a bacteremia with extended morbidity and mortality.  相似文献   
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