关键词： XIAP deficiency   X-linked lymphoproliferative syndrome   Hematopoietic cell transplantation   Hemophagocytic lymphohistiocytosis   Inflammatory bowel disease   Reduced-intensity conditioning   Posttransplantation cyclophosphamide   Microbiome   Dysbiosis of the gut microbiota  

摘要： BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is an infrequent inborn error of immunity that is often associated with refractory inflammatory bowel disease (IBD). The natural course of XIAP deficiency is typically associated with poor prognosis, and hematopoietic cell transplantation (HCT) is the only curative treatment. OBJECTIVE: To study (1) the effect of HCT on patients with XIAP deficiency undergoing HCT, (2) the status of XIAP deficiency-associated IBD after HCT, and (3) the gut micro-biota of XIAP deficiency-associated IBD before and after HCT. METHODS: A nationwide survey of patients with XIAP deficiency was conducted. A spreadsheet questionnaire was collected from the physicians. Feces samples collected from the patients before and after HCT and their healthy family members were analyzed. RESULTS: Twenty-six patients with XIAP deficiency underwent HCT by the end of March 2020, and 22 patients (84.6%) survived. All the survivors underwent a fludarabine-based reduced-intensity condition regimen. Acute graft-versus-host disease was observed in 17 patients (65.4%). Nineteen patients experienced refractory IBD before undergoing HCT. IBD improved remarkably after HCT. After HCT, the colonoscopic and pathological symptoms were restored to normal, and the pediatric ulcerative colitis activity index improved significantly. Gut microbiota indicated dysbiosis before HCT;however, it was improved to resemble that of the healthy family members after HCT. CONCLUSIONS: This study revealed that HCT has a favorable outcome for XIAP deficiency. HCT rescues gut inflammation and dysbiosis in patients with XIAP deficiency. (C) 2021 American Academy of Allergy, Asthma & Immunology

关键词： CD   Crohn's Disease   CDEIS   Crohn's Disease Endoscopic Index of Severity   DSS   Dextran Sulfate Sodium   EGF   Epidermal Growth Factor   ERRFI1   ErbB Receptor Feedback Inhibitor 1   FGF10   Fibroblast Growth Factor 10   GI   Gastrointestinal   HGF   Hepatocyte Growth Factor   HIF-1α   Hypoxia Inducible Factor 1-alpha   HNF4A   Hepatocyte Nuclear Factor 4 alpha   IBD   Inflammatory Bowel Disease   IgA   Immunoglobulin A   IGF-1   Insulin-like Growth Factor 1   IL-   Interleukin-   iPSC   Induced Pluripotent Stem Cell   JAK   Janus Kinase   KGF-2   Keratinocyte Growth Factor 2   LGR5   Leucine Rich Repeat Containing G Protein-Coupled Receptor 5   miR   microRNA   NF-κB   Nuclear Factor kappa-Light-Chain-Enhancer of Activated B Cells   PTGER4   Prostaglandin E Receptor 4   SES-CD   Simple Endoscopic Score for Crohn's Disease   SCFA   Short Chain Fatty Acid   SMAD7   Mothers Against Decapentaplegic Homolog 7   STAT   Signal Transducer and Activator of Transcription   TCRα   T-Cell Receptor alpha   TFF3   Trefoil Factor 3   TGFβ   Transforming Growth Factor beta   TLR   Toll-like Receptor   TNBS   2,4,6-Trinitrobenzenesulfonic Acid   TNF   Tumor Necrosis Factor   UC   Ulcerative Colitis   WAE   Wound-Associated Epithelium   WNT5A   Wnt Family Member 5A  

摘要： Patients with one of the many chronic inflammatory disorders broadly classified as inflammatory bowel disease (IBD) now have a diverse set of immunomodulatory therapies at their disposal. Despite these recent medical advances, complete sustained remission of disease remains elusive for most patients. The full healing of the damaged intestinal mucosa is the primary goal of all therapies. Achieving this requires not just a reduction of the aberrant immunological response, but also wound healing of the epithelium. No currently approved therapy directly targets the epithelium. Epithelial repair is compromised in IBD and normally facilitates re-establishment of the homeostatic barrier between the host and the microbiome. In this review, we summarize the evidence that epithelial wound healing represents an important yet underdeveloped therapeutic modality for IBD. We highlight 3 general approaches that are promising for developing a new class of epithelium-targeted therapies: epithelial stem cells, cytokines, and microbiome engineering. We also provide a frank discussion of some of the challenges that must be overcome for epithelial repair to be therapeutically leveraged. A concerted approach by the field to develop new therapies targeting epithelial wound healing will offer patients a game-changing, complementary class of medications and could dramatically improve outcomes. (Translational Research 2021;236:35-51)

关键词： 磁共振小肠造影   小肠疾病   诊断  

摘要： 探究分析磁共振小肠造影在小肠疾病诊断中的意义。方法:本次研究起止时间为2018年1月至2021年1月,在该时间段内选取40例疑似小肠疾病患者作为观察对象,依次对患者进行磁共振小肠造影检查,以手术病理结果作为“金标准”,比较磁共振小肠造影诊断的准确性。结果:40例疑似小肠疾病患者中,共20例患者最终经病理结果确诊,经治疗,其中2名患者为克罗恩病,4名患者为小肠平滑肌瘤,2名患者为非霍奇金淋巴瘤,10例为十二指肠结肠癌,2例为癌性肠梗阻,在磁共振小肠造影诊断中,2名患者为克罗恩病,4名患者为小肠平滑肌瘤,2名患者为非霍奇金淋巴瘤,10例为十二指肠结肠癌,2例为癌性肠梗阻,20名患者无小肠病变显现。手术病理结果与磁共振小肠造影诊断的符合率100.00%。结论:磁共振小肠造影技术具有安全、有效的特点,对于小肠疾病诊断具有良好的临床效果,方案值得推广。

关键词： 肛肠疾病   术后尿潴留   原因分析   护理  

摘要： 探讨肛肠疾病术后尿潴留的原因分析及护理。方法:选取我院肛肠疾病术后尿潴留患者43例作为研究对象,结合尿潴留致病诱因的分析,开展相应的对症护理方案,比较护理前后患者生活质量。结果:43例患者中,2例患者因诱发前列腺增生,转为导尿治疗;7例患者通过开塞露刺激的手段,达到顺利排尿的目的;34例患者经临床护理后,均表现为排尿正常,且出院。护理后,患者生活质量明显优于护理前,即护理前后患者生活质量数据对比,差异有统计学意义(P<0.05)。结论:心理因素和术后疼痛、麻醉及前列腺增生等因素,均是诱发肛肠疾病患者术后尿潴留的关键,适当且针对性护理方案的开展。

关键词： Vitamins  

摘要： Vitamin D (VitD) is a fat-soluble micronutrient that plays a critical role in inflammatory bowel disease (IBD). Although the effective properties of VitD in anti-inflammatory and immune moderation were reviewed, some important issues still remain uncovered. Considering the practicability and high bioavailability, as a more recommended therapeutic approach, the effects of oral VitD supplementation on IBD remain inconclusive. This study aims to investigate the effect and safety of oral VitD supplementation on IBD patients, which has already been registered on PROSPERO (no. CRD42020165045). A pooled analysis of 17 trials with 1127 patients revealed that as a safety therapeutic strategy, oral VitD supplementation effectively increased the concentration of serum 25-hydroxyvitamin D [weighted mean difference 12.15 ng mL(-1);95% confidence interval (CI) 9.26, 15.03;I-2 = 90%] and decreased serum C-reactive protein levels [standard mean difference (SMD) -0.33;95% CI -0.61, -0.05;I-2 = 55%], but it did not decrease erythrocyte sedimentation rate levels (SMD 0.35;95% CI -4.33, 5.03;I-2 = 57%), disease activity index (SMD -0.13;95% CI -0.66, 0.39;I-2 = 84%) and relapse rate (RR 0.59;95% CI 0.19, 1.86;I-2 = 79%). These findings suggest that oral VitD supplementation has a role to play in the therapeutic management of IBD. These findings may contribute to public health and clinical dietary guidelines and improve the health of IBD patients.

关键词： Ramulus mori polysaccharides   poly(lactic-co-glycolic acid)   anti-inflammation therapy   IBD   intestinal immune cells  

摘要： Ramulus mori polysaccharide (RMP), one of the most important active components of R. mori, has been attracting increasing interest because of its potent bioactive properties, including anti-inflammatory, antitumor, and antidiabetic effects. Despite the great therapeutic potential of RMP, its inherent properties of low bioavailability and brief biological half-life have limited its applications to the clinic. Thus, RMP was packaged by poly(lactic-co-glycolic acid) (PLGA) nanoparticles to develop a novel anti-inflammatory nanomedicine (PLGA-RMP) in this study. The nanoparticles were synthesized via a double-emulsion solvent evaporation technique, and the average diameter of PLGA-RMP was about 202 nm. PLGA-RMP nanoparticles reduced the expression of inflammatory cytokines while promoting the production of IL-10, and boosted the phenotypic shift in macrophages in vitro. Furthermore, lipopolysaccharide (LPS)-induced inflammatory bowel disease (IBD) in mouse was used to examine the anti-inflammatory effect of PLGA-RMP in vivo. Oral administration of PLGA-RMP in LPS-induced IBD mice substantially mitigated the intestinal inflammation compared to treatment with LPS alone, as evidenced by attenuation of disease activity index scores and inflammatory damage in the intestine. Meanwhile, PLGA-RMP suppressed the expression and secretion of specific inflammatory cytokines including TNF-alpha, IL-6, IL-1 beta, and PGE2 in the inflamed intestine while inhibiting the activation of CD3(+)CD8(+) T-cells and increasing the number of activated T-regs in the intestine. These results indicated that PLGA-RMP deserves further consideration as a potential therapeutic nanomedicine to treat various inflammatory diseases, including IBD.

关键词： inflammatory bowel diseases (IBDs)   biomarkers   pediatrics  

摘要： Inflammatory bowel diseases (IBDs), including Crohn's disease (CD) and ulcerative colitis, are chronic diseases of the gastrointestinal tract, with an unknown etiology, that affect over 6.8 million people worldwide. To characterize disease pathogenesis, proteomic and bioinformatic analyses were performed on colon biopsies collected during diagnostic endoscopy from 119 treatment-naive pediatric patients, including from 78 IBD patients and 41 non-IBD patients who served as controls. Due to the presence of noninflamed and/or inflamed regions in IBD patients, up to two biopsies were obtained from IBD patients as compared to a single noninflamed biopsy from non-IBD pediatric control patients. Additional biopsies were obtained and analyzed from 33 of the IBD patients after IBD-directed therapeutic intervention for comparison of pre- and post-treatment proteomes. SuperSILAC was utilized to perform quantitative analysis of homogenized tissues, which were processed by filter-aided sample preparation. Hierarchical clustering and principal component analyses revealed proteomic patterns that distinguished inflamed from noninflamed tissues independent of therapy. Gene ontology revealed that proteins downregulated in inflammation are associated with metabolism, whereas upregulated proteins contribute to protein processing. A comparison of pre- and post-treatment proteomes from CD patients identified over 100 proteins that are significantly different between patients who responded and those who did not respond to therapy, including creatine kinase B and basigin.

关键词： 中西医   无痛化   肛肠疾病   围手术期   应用  

摘要： 探讨中西医结合无痛化治疗模式对肛肠疾病围手术期镇痛的效果。方法:选取痔疮、肛裂、肛周脓肿及肛瘘患者324例,随机分为两组。观察组选用中西医结合的镇痛方法,对照组只选用西医镇痛方法,观察术后首次排气、首次排便时间,应用VAS评分评判术后4h、12h、1d、2d、3d、5d肛周疼痛,术前1h及术后4h、12h、1d、2d、3d、5d抽取肘静脉检测血清5-羟色胺(5-HT)浓度。结果:两组首次排便时间有统计学差异(P<0.05);术后4h、12h、1d、2d、3d、5d时间节点肛周疼痛VAS评分两组比较差异具有统计学意义(P均<0.05);术前1h及术后4h、12h、1d、2d、3d、5d时间节点血清5-HT浓度两组比较差异具有统计学意义(P均<0.05)。结论:采用中西医结合的无痛化治疗模式对肛肠疾病围手术期患者的镇痛效果良好。

关键词： 胃肠超声造影   胃十二指肠疾病   诊断符合率  

摘要： 目的探讨胃肠超声造影在胃十二指肠疾病中的应用价值。方法选取2018年5月至2020年5月湖南省脑科医院收治的60例胃十二指肠疾病患者作为研究对象,给予胃肠超声造影与上消化道钡餐造影检查,以胃镜下病理检查为金标准,比较胃肠超声造影与上消化道钡餐造影检出率的灵敏度、准确度及特异度。结果60例患者经胃镜下病理检查后确诊为恶性病变15例(25.00%),其中胃恶性淋巴瘤2例、胃癌13例;经胃肠超声造影检查后确诊为恶性胃十二指肠疾病17例,其中胃恶性淋巴瘤3例、胃癌14例;经上消化道钡餐造影检查后确诊为恶性病变25例,其中胃恶性淋巴瘤7例、胃癌18例。胃肠超声造影检查的灵敏度、准确度及特异度(93.33%、93.33%、93.33%)高于上消化道钡餐造影(46.67%、56.67%、60.00%),差异有统计学意义(P<0.05)。结论胃肠超声造影用于胃十二指肠疾病诊断中具有较高的临床价值,不仅操作简单、方便、无创,并且安全性高、可重复,适用于大规模的胃肠疾病普查,值得在基层医院推广。

关键词： 耳穴埋豆   穴位按摩   肛肠疾病   术后疼痛   不良反应   临床有效性  

摘要： 目的分析耳穴埋豆联合穴位按摩护理干预对肛肠疾病术后疼痛缓解状况的影响。方法方便选取2020年8—10月该院接受手术治疗的肛肠疾病患者74例进行研究,采用抽签法进行分组,对照组(37例)给予常规护理干预,观察组(37例)给予耳穴埋豆联合穴位按摩护理干预,分析两组护理后的疼痛程度、不良反应及临床有效性。结果护理前对照组的VAS评分(7.63±1.53)分与观察组(7.60±1.56)分比较,差异无统计学意义(t=0.084,P>0.05);护理后,观察组患者的VAS评分(1.13±0.26)分较对照组(2.07±0.72)分低,差异有统计学意义(t=7.469,P<0.05);观察组不良反应发生率13.51%(5/37)较对照组37.84%(14/37)低,差异有统计学意义(χ^(2)=5.736,P<0.05);对照组临床有效率64.86%(24/37)较观察组86.49%(32/37)低,差异有统计学意义(χ^(2)=4.698,P<0.05)。观察组患者的生理健康、心理状态、独立能力、社会信仰、社会关系、周围环境及总分分别(46.53±2.64)分、(67.76±6.54)分、(59.81±3.92)分、(9.98±1.82)分、(42.65±3.29)分、(86.51±2.54)分、(315.84±20.42)分,均较对照组的(42.65±2.58)分、(62.69±6.51)分、(40.62±4.35)分、(8.53±1.64)分、(36.59±4.63)分、(60.55±5.41)分、(285.96±18.05)分高,差异有统计学意义(t=6.394、3.342、19.934、3.600、6.490、26.421、6.669,P<0.05)。结论将耳穴埋豆联合穴位按摩护理模式应用于经手术治疗的肛肠疾病患者中,能降低患者术后痛感,减少不良反应发生。