成人自身免疫性肠病1例

自身免疫性肠病(AIE)是一种以顽固性腹泻、严重营养不良、小肠绒毛萎缩、血清中存在抗肠上皮细胞抗体或抗杯状细胞抗体为特点的自身免疫病, 主要见于儿童, 成人罕见。部分患者病情进展迅速, 短时间内出现重度营养不良表现, 一旦被延误诊断将导致不良预后。现报告1例40岁女性AIE病例, 通过及时诊断和恰当治疗获得良好的预后, 随访3年未见复发。     

1例成人自身免疫性肠病伴严重萎缩性胃炎及结肠炎:建议称为全消化道自身免疫病

自身免疫性肠病是一种在成人中极为罕见的蛋白丢失病。诊断标准为:(1)表现为迁延性腹泻和严重肠病;(2)选择性饮食或全肠外营养治疗无效;(3)自身免疫病证据(查见循环自身抗体或其他自身免疫病);(4)无严重免疫缺陷。现报道1例成人自身免疫肠病伴有严重萎缩性胃炎及隐原性结肠炎。     

自身免疫性肠病

自身免疫性肠病(autoimmune enteropathy,AIE)是一种病因不明、临床罕见的疾病,以往报道多见于婴幼儿,近年来成人病例报道逐渐增多.AIE的临床表现主要为顽固性腹泻和营养吸收不良,病理检查有小肠黏膜萎缩、黏膜固有层淋巴细胞和隐窝上皮内凋亡小体增多.AIE的治疗首选皮质类固醇激素,部分患者可能需给予免疫抑制剂治疗.     

胸腺瘤伴随的消化系统异常

目的探讨胸腺瘤中消化系统异常的发生率及可能的发生机制。方法结合文献,对北京协和医院1985年1月至2002年6月138例胸腺瘤的临床资料进行回顾性分析。结果138例胸腺瘤患者中消化系统症状总发生率12．3％（17／138）,其中以慢性腹泻最多（7．2％,10／138）,大多数腹泻原因不明确。胸腺瘤患者具有突出的自身免疫倾向,可检测到多种自身抗体;10例慢性腹泻患者中7例检测出自身抗体。胸腺瘤切除后有6例慢性腹泻症状基本缓解。结论胸腺瘤伴随消化系统异常比较常见,其中原因不明的慢性腹泻最多。胸腺瘤患者具有突出的自身免疫倾向,推测自身免疫性肠病可能是此类慢性腹泻的主要病因之一。     

肠上皮内淋巴细胞研究进展

肠上皮内淋巴细胞（IEL）是肠黏膜免疫系统的前沿守护者。肠黏膜上皮与肠腔大量微生物、食物抗原接触后,可通过调节IEL以预防肠腔致病微生物侵袭和对无害抗原产生耐受。IEL的表型和功能呈多样性,包括常规和非常规亚型,其功能失调不仅可引发肠道疾病,且与自身免疫病关系密切,而自身免疫病亦可致IEL发生相应变化。本文就IEL的分类、起源、在消化道中的分布情况及其与自身免疫性肠病、免疫缺陷性肠病、小肠恶性肿瘤的相关研究进展作一综述。     

自身免疫性胆管炎合并溃疡性结肠炎1例

自身免疫性胆管炎合并炎症性肠病临床上较罕见.本文报道的1例自身免疫性胆管炎合并溃疡性结肠炎患者以尿黄伴皮肤瘙痒为首发症状,经实验室检查及肝穿刺活检诊断为自身免疫性胆管炎.随诊2年后出现腹泻,经肠镜病理活检确诊合并溃疡性结肠炎.本例无典型的溃疡性结肠炎的临床表现,便常规、肠镜及病理检查提高了诊断率.     

脑源性神经营养因子在肠易激综合征患者腹痛中的作用

目的 探讨肠易激综合征(IBS)患者肠黏膜脑源性神经营养因子(BDNF)的表达水平改变及其与腹痛症状的相关性.观察IBS患者肠黏膜神经纤维密度和神经纤维超微结构的异常.方法 实验组选取2008年9月至2010年1月来本院消化科就诊且符合罗马Ⅲ诊断标准的IBS患者,根据其临床特点分为腹泻型IBS(D-IBS)和便秘型IB...     

慢性腹泻肠黏膜结构和屏障功能的变化

目的 探讨慢性腹泻患者肠黏膜结构和屏障功能的变化.方法 对33例慢性腹泻患者和30名健康志愿者行结肠镜检查,并在镜下取回肠末端和回盲部黏膜各3块,光镜下观察肠黏膜的组织结构,同时测定血浆内毒素水平,以评价肠黏膜屏障功能.结果 慢性腹泻患者光镜下可见回肠末端有绒毛顶端上皮细胞水肿、脱落,但与健康志愿者无明显差异(P>0.05);回盲部可见被覆上皮细胞明显水肿、脱落和破溃,甚至被覆上皮细胞完全脱落,与健康志愿者有明显差异(P<0.05).慢性腹泻患者和健康志愿者血中内毒素含量无统计学差异(P>0.05).结论 慢性腹泻患者可能存在结肠黏膜屏障损害.     

肠型白塞氏病临床病理特征分析3例

目的: 探讨肠型白塞氏病的临床特点、病理学特征及鉴别诊断要点.方法: 收集肠型白塞氏病确诊患者3例, 进行临床资料复习和组织病理学检查. 患者均以消化系症状入院, 同时患者伴有白塞氏病的主要临床症状.结果: 患者溃疡病变多发生于肠系膜附着的对侧, 溃疡呈圆形, 较深; 镜下见肠黏膜组织溃疡形成, 周围黏膜下淋巴组织增生显著, 主要表现以静脉为主的血管炎症. 治疗上, 糖皮质激素及免疫抑制剂是基础用药, 其预后不令人满意.结论: 肠型白塞氏病早期诊断有赖于对病史及临床症状的综合分析. 肠型白塞氏病无特异性治疗方法, 除非合并严重并发症, 一般不主张手术治疗.     

麦胶性肠病实验诊断的进展

麦胶性肠病又名乳糜泻、非热带口炎性腹泻,是慢性腹泻不可忽视的一种病因。其临床表现包括腹痛、腹泻、体重减轻,以及由于营养物质吸收不良所引起的各种临床表现。它在北美、欧洲、澳大利亚的发病率较高,我国目前尚无明确的流行病学资料,估计发病率较低。近来越来越多的资料显示麦胶性肠病与许多疾病的关系非常密切,其中包括疱疹样皮炎,并且该病患者肿瘤发生率较普通人群有所增加。因此其诊断非常重要,本文就其实验诊断方法研究的进展作一综述。传统的诊断方法主要涉及诊断小肠吸收不良综合征的各种检查方法,如粪便苏丹Ⅲ染色,72ｈ粪脂定量…     

Diarrhoea due to small bowel diseases

Small intestinal diseases are a common, though often overlooked cause of diarrhoeal illness. Fully 1% of the Caucasian population are affected by coeliac disease and a substantial portion of children living in poverty in the developing world are affected by environmental enteropathy. These are but two examples of the many diseases that cause mucosal injury to the primary digestive and absorptive organ in our body. While diarrhoea may be a common, though not universally seen symptom of small bowel mucosal disease, the consequent malabsorption can lead to substantial malnutrition and nutrient deficiencies. The small intestine, unlike the colon, has been relatively inaccessible, and systematic evaluation is often necessary to identify and treat small intestinal mucosal diseases that lead to diarrhoea. Immunodeficiency states, including HIV enteropathy, adult autoimmune enteropathy, drug-associated enteropathy, and tropical sprue continue to occur and require specific therapy. All patients with severe diarrhoea or diarrhoea associated with features suggestive of malabsorption may have a disease of the small intestinal mucosa that requires careful evaluation and targeted management.     

嗜酸细胞性胃肠炎的临床诊治

目的探讨嗜酸细胞性胃肠炎(enosinophilic gastroenteritis,EG)的临床特点和诊断治疗。方法对40例EG患者的临床特点、诊断和治疗情况进行分析。结果 1黏膜型27例,肌型5例,浆膜型8例。2黏膜型EG患者常以腹痛、腹泻和体质量减轻为主要症状,肌型以肠梗阻表现为主,浆膜型以腹水为首发症状。3外周血中嗜酸细胞计数明显增高,三型分别为4949.6±2075.0(27/27),2659.2±1344.0(4/5)和5876.0±1515.9(6/8),各型间差异无统计学意义(P0.05)。4内镜下活检阴性者,胃窦10例次(10/22),十二指肠7例次(7/30),结直肠7例次(7/16),反复做胃肠镜检查确诊者3例。5治疗以激素为主,可迅速缓解症状,但病情可有反复。结论诊断时尽可能内镜多次、多灶取活检,以提高诊断率,治疗仍以激素为主,长期小剂量激素治疗是预防病情复发值得推荐的治疗方案。     

Refractory celiac disease

Celiac disease is a gluten-sensitive enteropathy, characterized by villous atrophy, which is reversed by gluten withdrawal. A minority of patients with celiac-like enteropathy are resistant to gluten-free diet, so-called refractory sprue, or unclassified sprue. Refractory sprue is a diagnosis of exclusion; all other causes of a celiac-like enteropathy must be eliminated before a diagnosis of refractory sprue can be made. Recent evidence suggests that refractory sprue comprises a heterogenous group of patients with diverse underlying causes. A small proportion of these patients seem to have an adult form of autoimmune enteropathy, characterized by the presence of antienterocyte antibodies. However, a larger group of patients with refractory sprue now seem to have a cryptic intestinal T-cell lymphoma, characterized by the presence of phenotypically abnormal, monoclonal intraepithelial lymphocytes, despite benign cytology. Current therapeutic options include nutritional support and immunosuppressive therapy, but response is variable. The prognosis of refractory sprue may be poor; patients may die of severe malabsorption, or through synchronous or metachronous development of an enteropathy-associated T-cell lymphoma. Based on this recent evidence, patients with refractory sprue should be screened for antienterocyte antibodies and have T-cell receptor and monoclonal antibody studies performed; this could facilitate identification of cases of adult-onset autoimmune enteropathy and those of cryptic T-cell lymphoma. Moreover, early recognition of the malignant nature of the intestinal infiltrate in some cases of refractory sprue could permit the development of novel chemotherapeutic regimens for this condition.     

Autoimmune Enteropathy: A Review and Update of Clinical Management

Autoimmune enteropathy (AIE) is a rare condition characterized by intractable diarrhea, histologic changes on small intestinal biopsy, and failed response to dietary manipulation that also may present with extraintestinal manifestations. In many patients, immunosuppressive therapies are necessary. Although AIE is more common in infants, adult involvement has also been documented. Much of what is known about AIE has been gathered from case reports and small case series; therefore, more research in this evolving field is needed. IPEX (immunodysregulation polyendocrinopathy enteropathy X-linked syndrome) and APECED (autoimmune phenomena, polyendocrinopathy, candidiasis, and ectodermal dystrophy) are systemic forms of AIE.     

Giardiasis. Functional, immunological and histological study of the small bowel. Therapeutic trial with a single dose of tinidazole

Fourteen patients between the ages of 9 months and 5 years with chronic diarrhea and giardiasis were studied. Ten were eutrophic and 4 undernourished. The parasitological diagnosis was based on stool examination, a trophozoite search in duodenal aspiration, mucus adhered to mucosa and parasite identification in the intestinal biopsy material. Functional intestinal absorption studies, IgA determination in intestinal secretions and immunofluorescence studies were made. After the tests, tinidazole in suspension was administered at 60-70 mg/kg in one single oral dose. Patients were clinically re-evaluated and tests were done again after 30 days. The purpose of this paper was to evaluate the changes in the functional morphologic and immunologic studies and the therapeutic efficacy of the drug in a single dose. Nine patients had good clinical results, 2 fair and 3 were not evaluated due to celiac disease. All had negative results on the parasitological tests after treatment. There was no relationship between the number of parasites and the severity of symptoms. There was no significant difference between stool fat and d-xylose at the time of diagnosis and 30 days after the administration of tinidazole. The lactose tolerance test presented a significant difference (p less than 0.05) in the disaccharide absorption after treatment. The secretory IgA revealed significantly lower value (p less than 0.01) with respect to the normal values. The immunofluorescence showed productive IgA cells in all cases. The histologic changes were: mild enteropathy (grade I) in 6 patients; moderate (grade II) in 5; and severe (grade III-IV) in 3. Improvement of the mucosa was seen in 6 patients.     

Endomysial antibody-negative coeliac disease: clinical characteristics and intestinal autoantibody deposits

BACKGROUND: Some patients with untreated coeliac disease are negative for serum endomysial autoantibodies (EmA) targeted against transglutaminase 2 (TG2). AIMS: To evaluate the clinical and histological features of EmA-negative coeliac disease, and to examine whether EmA-equivalent autoantibodies against TG2 can be seen in the small-bowel mucosa when absent in serum. PATIENTS: Serum EmA was studied in 177 biopsy-proved specimens from adult patients with coeliac disease. 20 patients with intestinal diseases served as non-coeliac controls; three had autoimmune enteropathy with villous atrophy. METHODS: Clinical manifestations, small-bowel mucosal morphology, intraepithelial inflammation and TG2-specific extracellular immunoglobulin A (IgA) deposits were investigated in both serum EmA-negative and EmA-positive patients. RESULTS: 22 patients with IgA-competent coeliac disease were negative for serum EmA. Three of these had small-bowel lymphoma. Patients with EmA-negative coeliac disease were older, had abdominal symptoms more often, and the density of gammadelta+ intraepithelial lymphocytes in their intestinal mucosa was lower than in EmA-positive patients; otherwise the histology was similar. All serum EmA-negative patients with coeliac disease, but none of the disease controls, had gluten-dependent mucosal IgA deposits alongside TG2 in the small-bowel mucosal specimens. In vivo deposited IgA was shown to be TG2-specific by its ability to bind recombinant TG2. CONCLUSIONS: Negative serum EmA might be associated with advanced coeliac disease. TG2-targeted autoantibodies were deposited in the small-bowel mucosa even when absent in serum. This finding can be used in the diagnosis of seronegative coeliac disease when the histology is equivocal. It may also be helpful in the differential diagnosis between autoimmune enteropathy and coeliac disease.     

北京市6例输入性曼氏血吸虫病临床特点分析

目的分析6例输入性曼氏血吸虫病患者的临床特点,为提高临床医师的诊治水平提供资料依据。方法收集2009年1月-2016年7月确诊的6例输入性曼氏血吸虫病患者的临床资料,并进行分析。结果 6例输入性曼氏血吸虫病患者均有明确的流行病学史。主要临床表现为发热和嗜酸性粒细胞增高,50%患者伴有腹泻;肠黏膜组织压片均可以找到曼氏血吸虫卵,但日本血吸虫IgG抗体阳性率仅为33.3%。所有患者CD3~+CD8~+T细胞比例明显下降,B细胞比例明显上调,血免疫球蛋白以IgG抗体为主;16.6%的患者腹部超声见腹腔积液或脾脏增厚,16.6%患者磁共振检查见肝脏出现多发小结节或肠壁增厚,所有患者电子结肠镜检查均可见结肠炎,其中66.6%的患者合并多发溃疡,肠黏膜病理学均可见大量嗜酸性粒细胞浸润。6例患者经吡喹酮治疗后均达到临床治愈标准。结论本文通过系统总结6例输入性曼氏血吸虫病患者的流行病学史、临床和实验室多方面资料,为提高临床医师对该病的认知及诊治水平提供依据。     

Autoimmune Enteropathy with Severe Atrophic Gastritis and Colitis in an Adult: Proposal of a Generalized Autoimmune Disorder of the Alimentary Tract

Background: We describe the case of an adult with autoimmune enteropathy consistent with both severe atrophic gastritis accompanying antral stenosis and colitis. Methods and Results: The patient, positive for anti-intrinsic factor antibody, had intractable diarrhea and protein-losing enteropathy. In the ileum inflammatory cells were observed infiltrating the lamina propria along with villus atrophy, and similar inflammation was also found in the lamina propria of the colon and stomach, with complete loss of specialized glands. The myenteric ganglion cells of the hypertrophied muscularis propria in the stenosed antrum showed degeneration with surrounding T-lymphocyte infiltration. There were more CD8⁺ than CD4⁺ lymphocytes in the lamina propria of the stomach and colon. Conclusions: The CD8⁺ (suppressor-cytotoxic) T lymphocytes may have played an important role in the production of lesions in the stomach, small intestine, and colon, so we propose this case as an example of a generalized autoimmune disorder of the alimentary tract.     

Infectious enteritis

Enteric infections are a major cause of diarrhea in the United States. Pathogens can cause diarrhea by elaboration of toxins that affect the intestinal mucosa or by direct invasion of the intestinal wall. Clinical evaluation can provide important clues to aid in establishing a correct diagnosis in most patients with infectious enteritis. Appropriate cultures are necessary to confirm the diagnosis in most cases. Most types of infectious enteritis are self-limiting, but some pathogens can cause serious disease, requiring accurate diagnosis and suitable antibiotic therapy. Appropriate precautions are mandatory to prevent the spread of infectious diarrhea from occurring in the hospital environment. Dietary restrictions and appropriate hygiene should be observed during travel to foreign countries to reduce the chance of acquiring infectious enteritis.