short gut syndrome

短肠综合征
  • 文章类型: Journal Article
    目的:在因短肠综合征引起的肠衰竭(IF)的儿科患者中,我们假设年幼的孩子,残余小肠较短的患者和先天性肠旋转不良的患者更有可能接受泛小肠镜检查.我们的目的是确定该队列中泛肠镜检查的可行性和诊断率。
    方法:我们执行了单中心,回顾性研究2018年1月1日至2023年1月1日期间接受至少一次胃肠道内镜评估的短肠综合征(SBS)导致IF的儿科患者.
    结果:在所审查的431例手术(206例患者)中,有381例可能进行了全肠镜检查。44例(21%)患者接受了54例泛肠镜检查。残余肠长<35厘米的儿童接受全肠镜检查的几率更高(比值比[OR]3.72,95%置信区间[CI][1.32,10.48],p=0.01),围手术期使用胰高血糖素样肽2(GLP-2)类似物的患者也是如此(OR4.30,95%CI[1.24,14.95],p=0.02)。诊断为坏死性小肠结肠炎(NEC)以外的患者往往更有可能获得泛小肠镜检查(OR2.73,95%CI[0.95,7.88],p=0.06)。在77.8%和78%的手术中发现了总体和组织病理学异常的证据,分别。未发现并发症。
    结论:在一大群SBS患儿中,在14.2%的手术中成功进行了泛小肠镜检查,显微镜异常很常见.较短的残余肠长度,非NEC的潜在诊断,GLP-2类似物的使用通常与成功的全肠镜检查相关,独立于年龄和其他几个因素。这些数据表明,在SBS患者的一部分中,泛小肠镜检查是可行的,并且是高产量的。
    OBJECTIVE: In pediatric patients with intestinal failure (IF) due to short bowel syndrome, we hypothesized that young children, those with shorter residual small bowel and those with congenital malrotation of the bowel would be more likely to undergo pan-enteroscopy. We aimed to determine the feasibility and diagnostic yield of pan-enteroscopy in this cohort.
    METHODS: We performed a single-center, retrospective study of pediatric patients with IF due to short bowel syndrome (SBS) who had undergone at least one GI endoscopic evaluation between January 1, 2018 and January 1, 2023.
    RESULTS: A pan-enteroscopy might have been possible in 381 of the 431 procedures (206 patients) reviewed. 44 (21%) patients underwent 54 pan-enteroscopies. Children with a residual bowel length <35 cm had higher odds of undergoing pan-enteroscopy (odds ratio [OR] 3.72, 95% confidence interval [CI] [1.32, 10.48], p = 0.01), as did patients with periprocedural glucagon-like peptide 2 (GLP-2) analog use (OR 4.30, 95% CI [1.24, 14.95], p = 0.02). Patients with diagnoses other than necrotizing enterocolitis (NEC) tended to be more likely to achieve a pan-enteroscopy (OR 2.73, 95% CI [0.95,7.88], p = 0.06). Evidence of gross and histopathologic abnormalities were found in 77.8% and 78% of the procedures, respectively. No complications were identified.
    CONCLUSIONS: In a large cohort of children with SBS, pan-enteroscopy was successfully performed in 14.2% of the procedures and microscopic abnormalities were common. Shorter residual bowel length, underlying diagnoses of non-NEC, and GLP-2 analog use were generally associated with successful pan-enteroscopy, independent of age and several other factors. These data suggest that pan-enteroscopy is feasible and of high-yield in a subset of patients with SBS.
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  • 文章类型: Journal Article
    目的:肠衰竭(IF)患者肠道解剖结构异常,分泌,和运动障碍,这会损害肠道稳态机制,并可能导致小肠细菌过度生长(SIBO)。我们进行了系统评价和荟萃分析,以确定IF患者中SIBO的患病率,并确定SIBO的危险因素。
    方法:MEDLINE(PubMed)和Embase电子数据库从开始到2023年12月进行了搜索,以获得报告在IF中SIBO患病率的研究。患病率,比值比(OR),使用随机效应模型计算IF中SIBO的95%置信区间和IF中SIBO的危险因素。
    结果:最终数据集包括9项报告407例IF患者的研究。SIBO在IF中的患病率为57.5%(95%CI44.6-69.4),在本分析中具有实质性异质性(I2=80.9,P=0.0001)。接受肠外营养(PN)的IF患者的SIBO患病率比未接受PN的IF患者高6倍(OR=6.0,95%CI3.0-11.9,P=0.0001)。总的来说,使用PPI/酸抑制剂的IF患者中SIBO的患病率(72.0%,与不使用这些药物的IF患者相比,95%CI57.5-83.8)在数值上较高(47.6%,95%CI25.7-70.2)。
    结论:本系统综述和荟萃分析提示,在IF和PN患者中,SIBO的风险增加,潜在的,PPI/抑酸剂的使用是IF患者发生SIBO的危险因素.然而,证据质量较低,可归因于缺乏病例对照研究和研究中的临床异质性.
    OBJECTIVE: Patients with intestinal failure (IF) have abnormal intestinal anatomy, secretion, and dysmotility, which impairs intestinal homeostatic mechanisms and may lead to small intestinal bacterial overgrowth (SIBO). We conducted a systematic review and meta-analysis to determine the prevalence of SIBO in patients with IF and to identify risk factors for SIBO.
    METHODS: MEDLINE (PubMed) and Embase electronic databases were searched from inception to December 2023 for studies that reported the prevalence of SIBO in IF. The prevalence rates, odds ratio (OR), and 95% confidence intervals of SIBO in IF and the risk factors for SIBO in IF were calculated using random effects model.
    RESULTS: Final dataset included nine studies reporting on 407 patients with IF. The prevalence of SIBO in IF was 57.5% (95% CI 44.6-69.4), with substantial heterogeneity in this analysis (I2 = 80.9, P = 0.0001). SIBO prevalence was sixfold higher in patients with IF who received parenteral nutrition (PN) compared with IF patients not on PN (OR = 6.0, 95% CI 3.0-11.9, P = 0.0001). Overall, the prevalence of SIBO in patients with IF using PPI/acid-suppressing agents (72.0%, 95% CI 57.5-83.8) was numerically higher compared with IF patients not using these agents (47.6%, 95% CI 25.7-70.2).
    CONCLUSIONS: This systematic review and meta-analysis suggests that there is an increased risk of SIBO in patients with IF and that PN, and potentially, the use of PPI/acid-suppressing agents is risk factors for SIBO development in patients with IF. However, the quality of evidence is low and can be attributed to lack of case-control studies and clinical heterogeneity seen in the studies.
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  • 文章类型: English Abstract
    背景:腹裂和脐膨出是最常见的先天性腹壁缺损。这项研究的主要目的是调查发病率,冰岛其他相关异常和这些疾病的病程。
    方法:本研究为回顾性研究。人口是1991-2020年因胃裂或脐膨出而入院冰岛儿童医院NICU的所有新生儿。此外,所有产前或死后诊断为自然流产或人工流产的胎儿,包括在内。
    结果:在研究期间,54名婴儿出生时患有腹裂,5名患有脐膨出。腹裂的发生率为4.11,脐膨出的发生率为0.38/10,000。在研究期间,疾病的发生率没有显着变化。此外,5例胎儿诊断为腹裂,31例胎儿诊断为脐膨出,终止妊娠.除了活产婴儿和胎儿的腹裂外,最常见的相关异常是胃肠道或泌尿道,但在患有心脏脐膨出异常的婴儿和胎儿中,中枢神经或骨骼系统是最常见的。16例诊断为脐膨出的胎儿患有18三体。与年龄较大的母亲相比,16-20岁的母亲更有可能生下患有胃裂的婴儿(p<0.001)。86%的婴儿成功了初次闭合。那些明显更早地达到完全喂养,并更早地排出。总生存率为95%。由于短肠综合征,三名儿童在出院时仍在接受肠外营养。
    结论:冰岛腹裂的发生率与其他国家的研究一致,但脐膨出的发生率较低,这可以部分解释为自发或人工流产。与脐膨出相关的其他异常比与腹裂相关的异常更严重。原发性闭合与更良性的病程相关。患有腹裂的儿童可能需要长时间的肠外营养,因为他们的肠道缩短。
    BACKGROUND: Gastroschisis and omphalocele are the most common congenital abdominal wall defects. The main purpose of this study was to investigate the incidence, other associated anomalies and the course of these diseases in Iceland.
    METHODS: The study was retrospective. The population was all newborns who were admitted to the NICU of Children\'s Hospital Iceland due to gastroschisis or omphalocele in 1991-2020. Furthermore, all fetuses diagnosed prenatally or post mortem where the pregnancy ended in spontaneous or induced abortion, were included.
    RESULTS: During the study period, 54 infants were born with gastroschisis and five with omphalocele. The incidence of gastroschisis was 4.11 and omphalocele 0,38/10,000 births. There was no significant change in the incidence of the diseases during the study period. In addition, five fetuses were diagnosed with gastroschisis and 31 with omphalocele where the pregnancy was terminated. In addition to gastroschisis in the live born infants and fetuses the most common associated anomalies were in the gastrointestinal or urinary tract but in infants and fetuses with omphalocele anomalies of the cardiac, central nervous or skeletal systems were the most common. Sixteen fetuses diagnosed with omphalocele had trisomy 18. Mothers aged 16-20 were more likely to give birth to an infant with gastroschisis than older mothers (p< 0.001). Primary closure was successful in 86% of the infants. Those reached full feedings significantly earlier and were discharged earlier. Overall survival rate was 95%. Three children were still receiving parenteral nutrition at discharge due to short bowel syndrome.
    CONCLUSIONS: The incidence of gastroschisis in Iceland is in accordance with studies in other countries but but the incidence of omphalocele is lower, which can be partly explained by spontaneous or induced abortions. Other anomalies associated with omphalocele are more severe than those associated with gastroschisis. Primary closure was associated with more benign course. Children with gastroschisis may need prolonged parenteral nutrition due to shortening of their intestines.
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  • 文章类型: Case Reports
    已知严重急性呼吸道综合征冠状病毒2(SARS-CoV-2)会引起高凝状态,可能导致破坏性后果。自2019年冠状病毒大流行爆发以来,这一点已经确立;然而,COVID-19与血栓形成之间的具体关系仍然知之甚少。越来越多的文献记载了感染该病毒的患者的胃肠道(GI)并发症,包括潜在致死性急性肠系膜缺血(AMI),与胃肠道疾病的既往史或高凝状态的危险因素无关。肠系膜缺血不仅难以诊断,而且与高发病率和死亡率有关。保证及时识别和治疗以改善临床结果。我们在此介绍一例先前健康的女性在其COVID-19症状缓解五天后,继发于肠系膜血栓形成的弥漫性肠坏死。与AMI相关的高发病率和死亡率支撑了临床医生需要保持对COVID血栓并发症的高怀疑指数,即使是健康的病人。这个案例强调了彻底了解历史的重要性,体检,甚至在没有当前COVID-19感染或易感血栓危险因素的患者中进行实验室检查。此外,这提示与COVID-19感染相关的高凝状态可能在COVID-19的主要症状缓解后持续存在.
    Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is known to provoke a state of hypercoagulability that may lead to devastating consequences. This has been well established since the onset of the coronavirus pandemic in 2019; however, the specific relationship between COVID-19 and thrombus formation remains poorly understood. There has been increasing documentation of gastrointestinal (GI) complications in patients infected with the virus, including potentially lethal acute mesenteric ischemia (AMI), regardless of prior history of GI disease or risk factors for hypercoagulable states. Not only is mesenteric ischemia difficult to diagnose but it is also associated with high rates of morbidity and mortality, warranting prompt identification and treatment to improve clinical outcomes. We herein present a case of diffuse intestinal necrosis secondary to mesenteric thrombus formation in a previously healthy female five days after the resolution of her COVID-19 symptoms. The high rates of morbidity and mortality linked to AMI underpin the need for clinicians to maintain a high index of suspicion for thrombotic complications of COVID, even in healthy patients. This case emphasizes the importance of a thorough history-taking, physical examination, and laboratory workup even in patients without a current COVID-19 infection or predisposing thrombotic risk factors. Additionally, it suggests that the hypercoagulable state associated with a COVID-19 infection may persist after the primary COVID-19 symptoms have resolved.
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  • 文章类型: Case Reports
    吻合口溃疡是短肠综合征的已知并发症,这可能是并发缺铁和慢性出血的复杂性。通过内窥镜检查确诊,通常用于严重贫血的调查。先前已报道短肠综合征中的炎症性结肠炎;因此,治疗溃疡和炎症都很重要。目前,没有有效的治疗方法。在这里,我们描述了一个患有短肠综合征的儿童的病例,该儿童随后发展为吻合溃疡并反复严重出血,并通过内镜氩等离子体烧灼术成功治疗了环状溃疡。随后延长口服布地奈德肠用药疗程。这种干预措施可能是吻合口溃疡的潜在且有益的非手术治疗方法。
    Anastomotic ulceration is a known complication of short gut syndrome, which can be complicated by concurrent iron deficiency and chronic bleeding. Diagnosis is confirmed through endoscopy, typically performed for the investigation of severe anemia. Inflammatory colitis in short gut syndrome has been previously reported; therefore, it is important to treat both ulceration and inflammation. Currently, no effective therapies are available. Herein, we describe the case of a child with short gut syndrome who subsequently developed anastomotic ulcers with recurrent severe bleeding and was successfully treated with endoscopic argon plasma cauterization for circumferential ulcerations, followed by a prolonged course of oral enteric budesonide. This intervention could be a potential and beneficial nonsurgical treatment for anastomotic ulceration.
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  • 文章类型: Case Reports
    胃刺激器已被用作难以治疗的胃轻瘫患者的手术选择。文献中仅报道了先前关于胃起搏器导线继发的小肠坏疽的报道。我们的患者是一名38岁的女性,有系统性红斑狼疮病史和全结肠切除术史,她因特发性胃轻瘫接受了顺利的开放式胃起搏器放置。起搏器初次放置四个月后,她因急性腹痛到急诊科就诊,发现小肠140厘米坏疽,继发于小肠系膜周围的胃起搏器导线。她接受了顺利的小肠切除和吻合以及回肠直肠吻合。这种情况凸显了对胃起搏器患者的这种灾难性并发症的高度怀疑。
    Gastric stimulator has been used as a surgical option for patients with gastroparesis refractory to medical management. Only one previous report of small bowel gangrene secondary to gastric pacemaker wires has been reported in the literature. Our patient was a 38-year-old woman with a history of systemic lupus erythematosus and history of total colectomy who underwent an uneventful open gastric pacemaker placement for idiopathic gastroparesis. Four months after the initial placement of the pacemaker, she presented to the emergency department with acute abdominal pain and was found to have gangrene of 140 cm of small bowel secondary to looping of gastric pacemaker wires around small bowel mesentery. She underwent uneventful small bowel resection and anastomosis with an ileorectal anastomosis. This case highlights the need for a high index of suspicion for this catastrophic complication in patients with gastric pacemakers.
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  • 文章类型: Case Reports
    腹侧疝占内部疝病例的50%以上。它可以导致危险的后遗症,如肠缺血和穿孔。我们报告了一例患有急性肠梗阻和腹膜炎的患者,并在剖腹手术中偶然发现了一例复杂的十二指肠旁。一名26岁的男性患者有三天的连续严重失能的弥漫性腹痛病史。疼痛与多次胆汁性呕吐和绝对便秘有关。患者有休克的体征和症状。腹部检查示全身性腹膜炎。患者的实验室检查混乱。腹部X线片显示急性肠梗阻。患者被复苏并接受紧急剖腹手术。术中有一段长的坏疽小肠包裹在十二指肠旁囊中。坏疽肠从囊中释放,并通过不同的部位切除近端和远端形成为造口。对患者进行静脉输液和总的父母营养管理。患者逐渐开始口服饮食,并通过远端造口重新进行空肠造口输出。患者在术后第14天出院。患者在术后第45天有顺利的早期造口关闭,现在正在户外部门进行定期随访。十二指肠旁疝是肠梗阻的罕见原因之一,难以诊断。诸如腹部计算机断层扫描(CT)扫描之类的放射学检查可以帮助诊断十二指肠旁疝。外科医生应该清楚了解内部疝的异常解剖结构以及他们在手术中可能面临的并发症。
    Paraduonenal hernia constitutes more than 50% of internal hernia cases. It can result in perilous sequelae like gut ischemia and perforation. We report a case of a patient who presented with acute intestinal obstruction and peritonitis and was diagnosed as a case of complicated paraduodenal as an incidental finding on laparotomy.  A 26-year-old male patient presented with three days history of continuous severe incapacitating diffuse abdominal pain. The pain was associated with multiple episodes of bilious vomiting and absolute constipation. Patient had signs and symptoms of shock. Abdomen examination showed generalized peritonitis. Patient had deranged laboratory investigations. Abdominal X-ray showed acute intestinal obstruction. Patient was resuscitated and taken up for emergency laparotomy. Intraoperatively there was a long segment of gangrenous small bowel entrapped in the paraduodenal sac. Gangrenous gut was released from the sac and excised with proximal and distal ends fashioned as stoma through separate sites. Patient was managed with intravenous fluids with total parental nutrition. Patient gradually started on oral diet and jejunostomy output was refed through the distal stoma. Patient was discharged on postoperative day 14. Patient had uneventful early stoma closure at postoperative day 45 and now is on regular follow-up in the outdoor department. Paraduodenal hernias are one of the rare causes of intestinal obstruction that is difficult to diagnose. Radiologic investigation like abdominal computed tomography (CT) scan can aid in diagnosis of paraduodenal hernia. Surgeons should have clear knowledge about abnormal anatomy of internal hernias and complications they can face during surgery.
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  • 文章类型: Journal Article
    Short bowel syndrome (SBS) is a chronic disease whose natural history requires a changing array of management strategies over time. Chief amongst these is the chronic use of parenteral nutrition (PN) to ensure adequate nutritional intake. With time and appropriate management, approximately half of all SBS patients will successfully regain a functional, baseline level of intrinsic bowel function that will allow for them to achieve PN independence. However, the other half of SBS patients will progress into chronic intestinal failure which warrants a change in therapy to include more aggressive medical and potentially surgical measures. This review examines the evolving treatment strategies involved in the management of SBS as well as intestinal failure.
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  • 文章类型: Journal Article
    背景:研究在仔猪的实验性短肠综合征(SBS)模型中,远端小肠的逆转段改善体重增加的作用。
    方法:24只仔猪切除了70%的远端小肠。在一半的动物中进行了常规吻合,而另一半,在重建肠道连续性之前,残余空肠的远端25厘米被逆转。每天测量体重,直到第28天,此时对动物实施安乐死。在第28天在术前和术后测量胰高血糖素样肽-2(GLP-2)和葡萄糖依赖性促胰岛素肽(GIP)。
    结果:与对照组(11.14±3.83kg)相比,小肠逆转组在5.26±3.39kg(平均值±SD)时的体重增加显着降低(p<0.05)。在对照组中,远端发现了更大的绒毛高度和隐窝深度,并且在干预组中发现了更大的肌肉厚度。对照组GLP-2和GIP水平显著升高。
    结论:治疗空肠节段25厘米逆转的短肠综合征对体重增加有不利影响。
    BACKGROUND: To investigate the effects of a reversed segment of the distal small intestine to improve weight gain in an experimental short bowel syndrome (SBS) model in piglets.
    METHODS: Twenty-four piglets underwent resection of 70% of the distal small intestine. In half of the animals a conventional anastomosis was performed, and in the other half, the distal 25 cm of the remnant jejunum was reversed before the intestinal continuity was recreated. Weight was measured daily until day 28, where the animals were euthanized. Glucagon-Like Peptide-2 (GLP-2) and Glucose-dependent Insulinotropic Peptide (GIP) was measured pre- and postoperatively at day 28.
    RESULTS: The group with reversal of small intestine had a significant lower weight gain at 5.26 ± 3.39 kg (mean ± SD) compared to the control group with 11.14 ± 3.83 kg (p < 0.05). In the control group greater villus height and crypt depth was found distally, and greater muscular thickness was found proximally in the intervention group. GLP-2 and GIP levels increased significantly in the control group.
    CONCLUSIONS: Treatment of short bowel syndrome with a reversed jejunal segment of 25 cm had a detrimental effect on the weight gain.
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  • 文章类型: Case Reports
    由于短肠综合征引起的肠衰竭的婴儿和儿童的中心线相关血流感染(CLABSI)可能是由于肠道易位和皮肤污染引起的不同生物引起的。我们报告了我们认为第一例由环境酵母大豆念珠菌引起的短肠综合症婴儿的念珠菌菌血症,最初被误认为是热带念珠菌。我们讨论了其可能的来源,包括中心静脉导管(CVC)和肠道易位以及两种念珠菌之间的差异。
    Central line associated blood stream infections (CLABSIs) in infants and children with intestinal failure due to short bowel syndrome may be caused by different organisms due to intestinal translocation and skin contamination. We report what we believe the first case of candidemia in an infant with short bowel syndrome caused by the environmental yeast Candida sojae that was initially misidentified as Candida tropicalis. We discuss its possible sources including a central venous catheter (CVC) and gut translocation and the differences between the two Candida species.
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