克隆氏症
克隆氏症(英语:Crohn's disease),又称克罗恩病、克隆氏症候群或局部性肠炎,是一种发炎性肠道疾病,可能影响肠胃道从口腔至肛门的任何部分[3]。症状通常包含:腹痛、腹泻(如果发炎严重可能会呈血性)、发烧和体重减轻[3][4]。其他合并症可能发生于肠胃道之外且包括:贫血、皮疹、关节炎、葡萄膜炎和倦怠。可能会因为感染出现皮疹、坏疽性脓皮症或结节性红斑。肠梗阻也常发生,那些具有此疾病的人罹患肠癌的风险更大[4]。
克隆氏症 (克罗恩病) | |
---|---|
克隆氏症最常影响的3个部位[1][2] | |
症状 | 腹泻、减肥、肚痛、疲倦 |
类型 | 炎症性肠病、疾病 |
风险因素 | 一级亲属、吸烟、抗菌药、城市风格[*]、阑尾炎、扁桃体切除术[*] |
诊断方法 | 活体组织切片、医学影像 |
分类和外部资源 | |
医学专科 | 胃肠学 |
ICD-11 | DD70 |
ICD-10 | K50 |
ICD-9-CM | 555 |
OMIM | 266600 |
DiseasesDB | 3178 |
MedlinePlus | 000249 |
eMedicine | med/477 ped/507 radio/197 |
克隆氏症是由环境、免疫和细菌等因素在遗传易感个体上的组合所引起[5][6][7]。它会导致慢性发炎疾病,其中身体的免疫系统可能针对肠胃道的微生物抗原进行攻击[6][8]。虽然克隆氏症是一种免疫相关疾病,但它不会以自体免疫疾病的形式表现(免疫系统不被身体本身所触发)[9]。确切的潜在性免疫问题尚未明确;然而它可能是免疫缺陷的状态[8][10][11]。整体风险中约有一半和超过70个的基因相关[4][12]。吸烟者罹患克隆氏症的几率是没吸烟者的两倍[13]。它也时常发生于肠胃炎后。诊断是基于许多发现结果包括:组织切片和肠壁外观、医学影像和疾病描述。可以呈现其他的类似病症包含:肠躁症和贝赛特氏症[4]。
目前并无任何药物治疗和手术能根治克隆氏症。治疗选项的目的在于改善症状,使病情缓解以及预防复发。新诊断为克隆氏症的病人通常会接受短暂的类固醇治疗,使病情有显著的改善,之后并合并灭杀除癌锭或硫嘌呤等药物防止复发。对于有烟瘾的病患来说,在治疗过程中最重要的一部分就是戒烟。患有克隆氏症的病患中,每五位就有一位需要每年定期回医院覆诊,其中约有一半的病患在发病后十年左右需要接受手术治疗。虽然手术并非必要的治疗方式,但适用于脓疡、某些情况下的肠阻塞以及癌症。同时也建议患者在发病的八年后,需要隔几年接受大肠镜,以检查是否有罹患大肠癌[4]。
在欧洲以及北美等地区,克隆氏症的盛行率约为每1000人中有3.2人罹患克隆氏症[14]。在亚洲以及非洲等地区较为罕见[15][16]。就历史的发展情形来说,克隆氏症在发达国家较为常见[17]。但罹患克隆氏症的人口比例自1970年以来,在发展中国家已经不断窜升[16][17]。发炎性肠道疾病在2010年造成35000例死亡案例[18],而罹患克隆氏症的病患的预期寿命有轻微的减少[4]。克隆氏症好发的年龄层位于十多岁至二十多岁,但依然会在各种年龄层中出现[3][4]。且不因性别有所不同[3]。克隆氏症的名称是用来纪念布里尔·伯纳德·克罗恩,这位肠胃科医师和另外两位同事在1932年于纽约西乃山医院描述许多在回肠末端以及小肠等容易受感染的部分有发炎状况,病患的病情[19]。 治疗克隆氏症的方法有二:
- 利用TNF-α的抗体
- 利用可调节T细胞过敏反应的interleukin-10
参看
参考
- ^ Baumgart DC, Sandborn WJ. Inflammatory bowel disease: clinical aspects and established and evolving therapies.. The Lancet. 2007, 369 (9573): 1641–57. PMID 17499606. doi:10.1016/S0140-6736(07)60751-X.
- ^ Hanauer, Stephen B.; William Sandborn. Management of Crohn's Disease in Adults (PDF). American Journal of Gastroenterology. 2001-03-01, 96 (3): 635–43 [2006-11-08]. PMID 11280528. doi:10.1111/j.1572-0241.2001.03671.x. (原始内容存档 (PDF)于2011-09-27).
- ^ 3.0 3.1 3.2 3.3 Crohn's Disease. National Digestive Diseases Information Clearinghouse (NDDIC). July 10, 2013 [12 June 2014]. (原始内容存档于2014-06-09).
- ^ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Baumgart DC, Sandborn WJ; Sandborn. Crohn's disease. The Lancet. 2012, 380 (9853): 1590–605. PMID 22914295. doi:10.1016/S0140-6736(12)60026-9.
- ^ Cho JH, Brant SR. Recent Insights into the Genetics of Inflammatory Bowel Disease. Gastroenterology. 2011, 140 (6): 1704–12. PMID 21530736. doi:10.1053/j.gastro.2011.02.046.
- ^ 6.0 6.1 Dessein R, Chamaillard M, Danese S. Innate Immunity in Crohnʼs Disease. Journal of Clinical Gastroenterology. 2008, 42: S144–7. PMID 18806708. doi:10.1097/MCG.0b013e3181662c90.
- ^ Stefanelli T, Malesci A, Repici A, Vetrano S, Danese S. New Insights into Inflammatory Bowel Disease Pathophysiology: Paving the Way for Novel Therapeutic Targets. Current Drug Targets. 2008, 9 (5): 413–8. PMID 18473770. doi:10.2174/138945008784221170.
- ^ 8.0 8.1 Marks DJ, Rahman FZ, Sewell GW, Segal AW. Crohn's disease: An immune deficiency state. Clinical reviews in allergy & immunology. 2010, 38 (1): 20–31. PMID 19437144. doi:10.1007/s12016-009-8133-2.
- ^ Casanova JL, Abel L. Revisiting Crohn's disease as a primary immunodeficiency of macrophages.. The Journal of experimental medicine. Aug 31, 2009, 206 (9): 1839–43. PMID 19687225. doi:10.1084/jem.20091683.
- ^ Lalande JD, Behr MA. Mycobacteria in Crohn's disease: How innate immune deficiency may result in chronic inflammation. Expert review of clinical immunology. 2010, 6 (4): 633–41. PMID 20594136. doi:10.1586/eci.10.29.
- ^ Yamamoto-Furusho JK, Korzenik JR. Crohn's disease: Innate immunodeficiency?. World Journal of Gastroenterology. 2006, 12 (42): 6751–5 [2016-01-18]. PMID 17106921. (原始内容存档于2013-06-06).
- ^ Barrett JC, Hansoul S, Nicolae DL, Cho JH, Duerr RH, Rioux JD, Brant SR, Silverberg MS, Taylor KD, Barmada MM, Bitton A, Dassopoulos T, Datta LW, Green T, Griffiths AM, Kistner EO, Murtha MT, Regueiro MD, Rotter JI, Schumm LP, Steinhart AH, Targan SR, Xavier RJ, Libioulle C, Sandor C, Lathrop M, Belaiche J, Dewit O, Gut I, Heath S, Laukens D, Mni M, Rutgeerts P, Van Gossum A, Zelenika D, Franchimont D, Hugot JP, de Vos M, Vermeire S, Louis E, Cardon LR, Anderson CA, Drummond H, Nimmo E, Ahmad T, Prescott NJ, Onnie CM, Fisher SA, Marchini J, Ghori J, Bumpstead S, Gwilliam R, Tremelling M, Deloukas P, Mansfield J, Jewell D, Satsangi J, Mathew CG, Parkes M, Georges M, Daly MJ. Genome-wide association defines more than 30 distinct susceptibility loci for Crohn's disease. Nature Genetics. 2008, 40 (8): 955–62. PMC 2574810 . PMID 18587394. doi:10.1038/ng.175.
- ^ Cosnes J. Tobacco and IBD: Relevance in the understanding of disease mechanisms and clinical practice. Best Practice & Research Clinical Gastroenterology. 2004, 18 (3): 481–96. PMID 15157822. doi:10.1016/j.bpg.2003.12.003.
- ^ Molodecky, NA; Soon, IS; Rabi, DM; Ghali, WA; Ferris, M; Chernoff, G; Benchimol, EI; Panaccione, R; Ghosh, S; Barkema, HW; Kaplan, GG. Increasing incidence and prevalence of the inflammatory bowel diseases with time, based on systematic review.. Gastroenterology. Jan 2012, 142 (1): 46–54.e42; quiz e30. PMID 22001864. doi:10.1053/j.gastro.2011.10.001.
- ^ Prideaux, L; Kamm, MA; De Cruz, PP; Chan, FK; Ng, SC. Inflammatory bowel disease in Asia: a systematic review.. Journal of Gastroenterology and Hepatology. Aug 2012, 27 (8): 1266–80. PMID 22497584. doi:10.1111/j.1440-1746.2012.07150.x.
- ^ 16.0 16.1 Hovde, Ø; Moum, BA. Epidemiology and clinical course of Crohn's disease: results from observational studies.. World journal of gastroenterology : WJG. Apr 21, 2012, 18 (15): 1723–31. PMC 3332285 . PMID 22553396. doi:10.3748/wjg.v18.i15.1723.
- ^ 17.0 17.1 Burisch, J; Munkholm, P. Inflammatory bowel disease epidemiology.. Current opinion in gastroenterology. Jul 2013, 29 (4): 357–62. PMID 23695429. doi:10.1097/MOG.0b013e32836229fb.
- ^ Lozano, R; Naghavi, M. Global and regional mortality from 235 causes of death for 20 age groups in 1990 and 2010: a systematic analysis for the Global Burden of Disease Study 2010.. Lancet. Dec 15, 2012, 380 (9859): 2095–128. PMID 23245604. doi:10.1016/S0140-6736(12)61728-0.
- ^ Crohn BB, Ginzburg L, Oppenheimer GD. Regional ileitis: A pathologic and clinical entity. 1932. The Mount Sinai journal of medicine, New York. 2000, 67 (3): 263–8. PMID 10828911.