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     

死亡结构域相关蛋白及其在宫颈病变中的研究进展

宫颈癌对妇女健康构成严重威胁,人乳头瘤病毒感染与宫颈病变及宫颈癌的发生密切相关。关于宫颈癌发生发展的机制仍在研究中。近年研究发现一种多功能核蛋白,即死亡结构域相关蛋白(death domain associated protein,Daxx),其与细胞内蛋白或病毒蛋白相互作用,参与调节细胞凋亡、转录调控、抗病毒等细胞活动,在不同途径中发挥不同的生理或病理作用。通过对Daxx功能及其作用机制的研究有助于进一步阐明宫颈癌发生发展的机制,有助于发现新的预防和治疗方法。综述Daxx的一般特性和研究现况及其在宫颈病变的研究进展。     

Immune Checkpoint Inhibitor Toxicities

Immune checkpoint inhibitors are molecules that increase the endogenous immune response against tumors. They have revolutionized the field of oncology. Since their initial approval for the treatment of advanced melanoma, their use has expanded to the treatment of several other advanced cancers. Unfortunately, immune checkpoint inhibitors have also been associated with the emergence of a new subset of autoimmune-like toxicities, known as immune-related adverse events. These toxicities differ depending on the agent, malignancy, and individual susceptibilities. Although the skin and colon are most commonly involved, any organ may be affected, including the liver, lungs, kidneys, and heart. Most of these toxicities are diagnosed by excluding other secondary infectious or inflammatory causes. Corticosteroids are commonly used for treatment of moderate and severe immune-related adverse events, although additional immunosuppressive therapy may occasionally be required. The occurrence of immune-related toxicities may require discontinuation of immunotherapy, depending on the specific toxicity and its severity. In this article, we provide a focused review to familiarize practicing clinicians with this important topic given that the use of immune checkpoint inhibitors continues to increase.     

Inter-ictal assay of peripheral circulating inflammatory mediators in migraine patients under adjunctive cervical non-invasive vagus nerve stimulation (nVNS): A proof-of-concept study

Objective

To assay peripheral inter-ictal cytokine serum levels and possible relations with non-invasive vagus nerve stimulation (nVNS) responsiveness in migraineurs.

The relationships among monocyte subsets,miRNAs and inflammatory cytokines in patients with acute myocardial infarction

Background

Acute myocardial infarction (AMI) causes irreversible myocardial damage and release of inflammatory mediators, including cytokines, chemokines and miRNAs. We aimed to investigate changes in the levels of cytokines (IL-6, TNF-α and IL-10), miRNAs profiles (miR-146 and miR-155) and distribution of different monocyte subsets (CD14++CD16-, CD14++CD16+, CD14+CD16++) in the acute and post-healing phases of AMI.

Intracranial Non-Hodgkin’s MALT lymphoma mimicking a large convexity meningioma

Summary Primary presentation of an intradural Non-Hodgkin’s lymphoma is rare. Recently these B cell lymphomas of mucosa associated lymphoid tissue (MALT) have gained acceptance as an important pathological subtype and are distinguishable from other primary CNS lymphomas that exhibit aggressive behaviour. Over the past decade a number of these lesions have been reported to resemble a meningioma both intra-operatively and radiologically. The authors outline such a case of marginal zone B cell lymphoma that clinically and radiologically resembled a meningioma. This case illustrates the rare occurrence of low grade dural B cell lymphoma and the need to consider this entity in the differential diagnosis of CNS lesions, if appropriate targeted therapy is to be administered.     

快速眼动睡眠剥夺后大鼠脑内神经元骨架蛋白及超微结构的变化

目的探讨经历不同时间快速眼动（REM）睡眠剥夺对大鼠皮质及海马各区神经元形态结构的影响。方法选择微管相关蛋白（MAP2）和神经丝（NF）作为正常神经元结构的标识物，利用免疫组织化学法和Western blot技术观察REM睡眠剥夺1、3、5、7 d4个时间点大鼠皮质及海马MAP2和NF表达的时空变化规律。同时运用电镜技术观察睡眠剥夺后神经元超微结构的变化。我们的实验是用改良的多平台睡眠剥夺模型进行REM睡眠剥夺，结合免疫组织化学染色技术和蛋白质电泳以及电镜超微结构分析。结果REM睡眠剥夺后5d大鼠皮质、海马CA1及齿状回神经元结构蛋白MAP2和NF表达较对照组明显减少（P〈0．05）；电镜神经元核仁偏位，胞质中出现少量肿胀的线粒体和内质网；部分神经轴突的髓鞘溶解与浓集。环境对照组、REM睡眠剥夺5d和7d组，皮质中超微结构改变的神经元所占比例分别为1．2％、3．6％和5．8％。结论REM睡眠剥夺能够导致大鼠脑内神经元的超微结构发生异常变化。