Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group

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     

Diet and nutrition against inflammatory bowel disease: Trick or treat(ment)?

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.     

The management of emergency hospital visits for inflammatory bowel diseases: A French national expert consensus report

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.     

Crohn病的CT表现

目的探讨Crohn病的CT表现特点。方法回顾性分析经病理证实的18例Crohn病的CT征象，大多数病例采用螺旋CT或多层螺旋CT扫描。结果所有病例均有节段性肠壁增厚，CT增强有7例表现为肠壁均匀增厚，缺乏层次，同时肠壁强化不明显；有11例表现为黏膜增厚和分层形成“靶征”和“双环征”，肠壁厚度多在5～10mm；有9例表现为病变段肠管周围纤维脂肪成分增多，蜂窝织炎及肠周或肠系膜血管增多增粗。有5例CT首先考虑为其他疾病，1例考虑为闭攀性肠梗阻，1例首先考虑为肠结核，2例回盲部病变怀疑为癌，1例肠穿孔形成脓肿仅诊为化脓性感染。结论cT不但能准确显示Crohn病肠壁、肠系膜及周围结构的异常，增强CT还能区分活动性与非活动性病变，有助于临床采取合理而积极的治疗方案。     

Indikation für eine immunsuppressive Therapie bei chronisch entzündlichen Darmerkrankungen

Zusammenfassung Der immunsuppressiven Therapie kommt bei chronischen Darmerkrankungen gro?e Bedeutung zu, insbesondere dort, wo die Standardtherapie nicht den gewünschten Erfolg zeigt. Als Therapie der ersten Wahl gelten bei Colitis ulcerosa 5-Aminosalizyls?ure-freisetzende Medikamente, bei Morbus Crohn Kortikosteroide. Innerhalb der Gruppe immunsuppressiver Pr?parate gibt es erhebliche Wirkungsunterschiede. Darüber hinaus ist die Immunsuppression auch mit ernstzunehmenden Nebenwirkungen belastet. über den Stellenwert der Immunsuppression als Erg?nzung, m?gliche oder bei Unvertr?glichkeit notwendige Substitution der Standardtherapien von Colitis ulcerosa und Morbus Crohn wird hier eingehend referiert. Auch die Behandlung dieser Erkrankungen in der Schwangerschaft wird dabei erl?utert.     

Growth failure in Crohn’s disease children: may the first treatment have a role?

Introduction: Growth failure in children is a frequent feature of childhood-onset Crohn’s disease (CD), and stunting can persist into adulthood. Growth is an important outcome by which to judge the effectiveness of therapies in children; currently available studies in CD children have focused on the short-term impact of treatments on growth, and there are limited data regarding the long-term effects of treatments upon growth.

Areas covered: We designed the present article to review whether the first treatment performed in newly diagnosed CD children may have a role on the future growth course. We conducted a systematic literature search to identify relevant studies published on the PubMed database from January 2002 up to now. We found only six surveys that documented mid-term growth course in newly diagnosed CD patients.

Expert commentary: In the last years there have been relevant advances in the clinical management of CD children; however, there is a lack of knowledge about the best strategy to reverse growth failure. Children treated with enteral nutrition have appropriate height and weight gain but do not reverse the growth course. Further surveys are required to better explore not only clinical outcomes but also long-term growth course following each therapeutic strategy.     

Altered expression of cell adhesion molecules in uninvolved gut in inflammatory bowel disease.

Adhesion of circulating cells to vascular endothelium occurs in the early phase of inflammation, and is mediated by specific cell adhesion molecules. Many such adhesion molecules are increased in inflamed regions of ulcerative colitis (UC) and Crohn's disease (CD) but there is limited knowledge of their expression in the uninvolved gut, adjacent to inflammation. We investigated immunohistochemically the expression of platelet endothelial cell adhesion molecule-1 (PECAM-1), intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function-associated antigen-1 (LFA-1) on resected specimens taken at a distance of 2-4 cm from the inflamed area and without histological signs of inflammation. Compared with normal gut, we found (i) a significant increase of PECAM-1-positive vessels in the mucosa of uninvolved UC (149.0 +/- 24.1 vessels/mm2 (mean +/- s.d.); normal colon = 123.1 +/- 21.6; P = 0.004); (ii) a significant decrease of ICAM-1-positive vessels in uninvolved CD (111.9 +/- 22.6 vessels/mm2; normal ileum = 136.9 +/- 27.6; P = 0.04); and (iii) a moderate but statistically insignificant increase of LFA-1-positive cells in the mucosa of uninvolved UC and Crohn's ileitis. This altered expression of cell adhesion molecules may contribute to the early lesion in inflammatory bowel disease and provide new therapeutic opportunities.     

The Binding of Different Lectins on Peripheral Blood Mononuclear Cells from Patients with Chronic Inflammatory and Malignant Diseases

Peripheral blood mononuclear cells from patients with multiple myeloma, gastrointestinal tumors, and inflammatory bowel disease were analyzed for binding of various lectins. The results demonstrated that in most of the patients with multiple myeloma a significantly increased percentage of cells positive for Lotus tetragonolobus agglutinin (LTA), peanut agglutinin (PNA), soybean agglutinin (SBA), and wheat germ agglutinin (WGA), and a decreased number of Agaricus bisporus agglutinin (ABA) positive cells were present as compared to a normal control group. This could not be shown in malignant or inflammatory disorders of the gastrointestinal tract where only some patients exhibited an increased PNA and LTA binding, respectively. Patients with the systemic malignant disease differed from patients with solid localized tumors by a significantly altered number of ABA, LTA and SBA-positive peripheral blood mononuclear cells. Double fluorescence studies using monoclonal antibodies and lectins revealed that most of the cells expressing receptors for ABA had also receptors for OKT3, whereas most of the cells with receptors for LTA, PNA SBA, and WGA were found to be positive for OKM.