本文選題：隱源性多灶性潰瘍性狹窄性小腸炎　切入點：慢性非特異性多灶性小腸潰瘍　出處：《河北醫(yī)科大學》2014年碩士論文

【摘要】：隱源性多灶性潰瘍性狹窄性小腸炎(cryptogenic multifocal ulcerousstenosing enteritis, CMUSE)是一種臨床罕見的小腸慢性疾病，目前病因尚不明確。以反復發(fā)作的小腸不全性梗阻為主要表現(xiàn)，常伴隨腹痛、黑便、缺鐵性貧血、低蛋白血癥、水腫、生長發(fā)育遲緩等癥狀，病理表現(xiàn)為小腸多發(fā)淺表潰瘍，可見慢性炎性細胞浸潤伴少量嗜酸性粒細胞，潰瘍僅侵犯粘膜及粘膜下層，不累及肌層等深層組織，可伴有纖維組織增生。臨床易誤診為克羅恩病、腸結核及非甾體抗炎藥相關性小腸病。糖皮質(zhì)激素治療有效，但是容易產(chǎn)生激素依賴，目前仍未找到促進粘膜愈合的有效治療，針對腸道狹窄，多需要借助外科手術。 CMUSE在全球范圍內(nèi)尚屬少見，見諸報道的確診病例僅60余例。目前對該病沒有統(tǒng)一的診斷治療意見。本研究主要通過對CMUSE病例進行薈萃分析，總結歸納其主要臨床表現(xiàn)、影像學及內(nèi)鏡下特點及病理特點，以期為該病的診斷與治療提供依據(jù)。同時結合我院確診的CMUSE病例，研究TNF-α在病變腸組織中的表達，探討該病的發(fā)病機制，以期為該病的治療提供新思路。 第一部分隱源性多灶性潰瘍性狹窄性小腸炎1例報道 目的：分析我院1例CMUSE患者病例特點，并檢測TNF-α在其腸組織中的表達。 方法：選擇河北醫(yī)科大學第二醫(yī)院經(jīng)病理證實的CMUSE患者1例，搜集臨床資料及術后標本，回顧性分析其臨床特點及病理特征，應用組織染色技術觀察病變腸組織炎癥程度及纖維組織增生情況，應用免疫組織化學染色技術觀察病變腸組織TNF-α的表達情況。 結果：該例CMUSE患者為中年女性，以反復缺鐵性貧血、腸梗阻、黑便及低蛋白血癥為主要癥狀，腹部CT表現(xiàn)為臍水平腹腔右側(cè)及左下腹腸管壁環(huán)形增厚，腸腔狹窄伴明顯強化。膠囊內(nèi)鏡顯示小腸節(jié)段性粘膜水腫及潰瘍，潰瘍大小不等，較表淺，多為縱行，表面覆黃白苔。剖腹探查術可見回腸節(jié)段性狹窄，內(nèi)有膠囊狀物（滯留于腸內(nèi)的膠囊內(nèi)鏡）1枚，遂行小腸部分切除，小腸-小腸端側(cè)吻合術，術后病理示小腸粘膜部分滲出、壞死及肉芽，符合潰瘍，腸系膜淋巴結13枚反應性增生。小腸病變部位HE染色可見粘膜及粘膜下層炎性細胞浸潤，Masson染色及天狼星紅染色可見纖維組織明顯增生，免疫組織化學染色可見粘膜及粘膜下層TNF-α表達增加。 結論：本例小腸病變患者診斷為CMUSE，符合目前國內(nèi)外CMUSE診斷要點；病變腸組織TNF-α表達增加。 第二部分隱源性多灶性潰瘍性狹窄性小腸炎臨床特征薈萃分析 目的：通過分析國內(nèi)外文獻報道病例，總結CMUSE臨床特征，，并分析亞洲與歐洲CMUSE病例臨床特征的異同。 方法：通過中國生物醫(yī)學文獻服務系統(tǒng)、萬方數(shù)據(jù)庫、維普中文科技期刊數(shù)據(jù)庫、中國知網(wǎng)及PubMed檢索國內(nèi)外公開發(fā)表的CMUSE病例報道文獻，對符合入選標準的病例的臨床表現(xiàn)、實驗室檢查、影像學檢查、內(nèi)鏡學檢查、病理組織學檢查結果、治療及預后情況進行歸納總結。 結果：查閱發(fā)表于1996年-2013年間的入選文章，入選的CMUSE病例共18例，其中男性占38.9%，女性占61.1%，男女比為1:1.6。確診時平均年齡為36歲。臨床癥狀以腹痛、缺鐵性貧血、體重下降、黑便為主。實驗室檢查以血紅蛋白降低、白蛋白降低、大便潛血陽性為特征，CRP及ESR等炎癥指標多正常，自身抗體多為陰性，1例患者檢測纖維化4項明顯升高，1例血漿補體C2降低。3例患者可經(jīng)影像學檢查發(fā)現(xiàn)腸道梗阻及潰瘍性病變表現(xiàn)，全消化道造影較腹部CT及X線更易發(fā)現(xiàn)病變。胃鏡及腸鏡檢查多不能發(fā)現(xiàn)病變，除非病變位于十二指腸或回腸末端。小腸鏡及膠囊內(nèi)鏡檢出率較高。病變部位多位于回腸，呈多灶性，亦可見單發(fā)潰瘍或狹窄，病理標本均表現(xiàn)為淺表潰瘍，僅侵及粘膜及粘膜下層，不向肌層及深層組織浸潤，而纖維化和炎性浸潤可達到深層組織，粘膜下層可因纖維化增厚，以輕-中度炎癥改變?yōu)橹�，可見中性粒細胞、單核細胞、嗜酸性粒細胞及漿細胞浸潤，無肉芽腫形成，無絨毛萎縮、淋巴增殖、巨細胞肉芽腫及阿弗他或裂隙潰瘍表現(xiàn)。部分病例可見小靜脈增厚、炎性浸潤、血栓成形或靜脈內(nèi)膜炎�；颊叨鄶�(shù)誤診為克羅恩病，部分患者難以與潰瘍性回腸炎或腸結核鑒別。7例患者接受激素治療有效，有效率53.8%。3例患者出現(xiàn)激素依賴，3例激素抵抗。9例患者接受手術治療，術后4例患者復發(fā)，復發(fā)率44.4%。1例患者伴有雷諾氏綜合癥、干燥綜合癥、哮喘、關節(jié)腫痛等多器官損傷。1例患者伴有消化道腫瘤。 結論：CMUSE女性多發(fā)，臨床以腹痛、缺鐵性貧血、體重下降、黑便為主為主要特點，部分患者可有乏力、腹瀉、低熱、水腫、嘔吐、便秘等癥狀；病理特點為累及小腸的多灶性淺表潰瘍，潰瘍僅侵及粘膜及粘膜下層，不向肌層及深層組織浸潤，而纖維化和炎性浸潤可達到深層組織，粘膜下層可因纖維化增厚，以輕-中度炎癥改變?yōu)橹�，無肉芽腫形成，無絨毛萎縮、淋巴增殖、巨細胞肉芽腫及阿弗他或裂隙潰瘍表現(xiàn)；歐洲及亞洲患者CMUSE臨床特征基本相似。
[Abstract]:Cryptogenic multifocal ulcerative colitis ( CMUSE ) is a rare small intestinal chronic disease . It is a rare small intestinal chronic disease . It is characterized by recurrent small intestinal obstruction . It can be seen as chronic inflammatory cell infiltration with a small amount of eosinophils . It is easy to be misdiagnosed as Crohn ' s disease , intestinal tuberculosis and non - steroidal anti - inflammatory drug - related small bowel disease . It is easy to be misdiagnosed as Crohn ' s disease , intestinal tuberculosis and non - steroidal anti - inflammatory drug - related small bowel disease .

CMUSE is rare in the global scope . There are only 60 cases of confirmed cases . There is no unified diagnosis and treatment for the disease . The main clinical manifestation , imaging and endoscopic features and pathological characteristics of CMUSE cases are summarized . The expression of TNF - 偽 in the intestinal tissue of the lesion is studied by combining CMUSE cases diagnosed by our hospital .

The first part of cryptogenic multifocal ulcerative colitis : a report of 1 case

Objective : To analyze the characteristics of one CMUSE patient in our hospital and to detect the expression of TNF - 偽 in its intestinal tissue .

Methods : 1 case of CMUSE patient confirmed by pathology was selected from the Second Affiliated Hospital of Hebei Medical University . Clinical data and postoperative specimens were collected . The clinical characteristics and pathological characteristics were analyzed retrospectively . The expression of TNF - 偽 was observed by immunohistochemical staining .

Results : The CMUSE patient was a middle - aged female with recurrent iron deficiency anemia , intestinal obstruction , black stool and hypoproteinaemia as the main symptoms . The abdominal CT findings were as follows : the small intestine segmental mucosa edema and ulcer , the intestinal canal stenosis accompanied by obvious enhancement .

Conclusion : The diagnosis of small intestinal lesion is CMUSE , which accords with the present diagnostic points of CMUSE at home and abroad .
The expression of TNF - 偽 in diseased intestinal tissue was increased .

Meta - analysis of clinical features of second partial cryptogenic multifocal ulcerative colitis

Objective : To summarize the clinical features of CMUSE and analyze the similarities and differences between the clinical features of CMUSE cases in Asia and Europe .

Methods : The clinical manifestation , laboratory examination , imaging examination , endoscopic examination , pathological histology examination result , treatment and prognosis of cases with inclusion criteria were summarized through the literature of CMUSE cases published at home and abroad through the Chinese Biomedical Literature Service System , Wanfang Database , Vip Chinese Sci - tech Periodical Database , China Knowledge Network and the Chinese Medicine .

Results : There were 18 cases of CMUSE published between 1996 and 2013 , including 38 . 9 % for men , 61 . 1 % for women , 1 鈭

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