首页 理论教育 门脉高压性肠病

门脉高压性肠病

时间:2024-05-13 理论教育 版权反馈
【摘要】:门脉高压所致的肠黏膜病变,95%位于右半结肠和直肠、乙状结肠。PHE治疗原则主要是治疗引起门脉高压症。由于门脉高压症85%~90%是由肝硬化引起,所以基本上以药物治疗为主。近年来有人提出受体阻滞剂可减少因门脉高压症引起的消化道出血,并能改善肠黏膜的病变。

第十节 门脉高压性肠病

门脉高压性肠病(portal hypertensive enteropathy,PHE)系指在门脉高压的基础上发生的肠黏膜下毛细血管扩张、淤血及血流量增加,动静脉短路以及毛细血管内皮和黏膜上皮细胞超微结构的改变,其范围仅限于由门脉高压引起的以血管改变为特征的肠道改变。PHE临床表现除有门脉高压表现,如脾大、脾功能亢进、侧支循环开放、消化道出血及腹水外,肠镜检查可见肠黏膜充血、水肿、糜烂及溃疡,有些尚可见蜘蛛痣样小动脉扩张。门脉高压所致的肠黏膜病变,95%位于右半结肠和直肠、乙状结肠。

一、诊断

1.检查方法 ①内镜检查可观察到肠黏膜下静脉曲张和(或)血管扩张。黏膜活检可见毛细血管扩张、黏膜萎缩等;②其他,如内镜超声(EUS)、钡剂灌肠检查、经肝门静脉造影、放射性核素显影、CT扫描及MRI检查等有助于PHE的诊断。

2.诊断依据 ①有门脉高压症及引起门脉高压的相应原发性疾病,如肝硬化等;②有肠道黏膜下静脉曲张、血管扩张的证据。

二、治疗原则

PHE治疗原则主要是治疗引起门脉高压症。由于门脉高压症85%~90%是由肝硬化引起,所以基本上以药物治疗为主。近年来有人提出受体阻滞剂可减少因门脉高压症引起的消化道出血,并能改善肠黏膜的病变。也有人提出应用放射介入疗法可降低门脉压力。这些方法的治疗效果有待进一步的临床论证。

(郑晓风)

参考文献

1.王国品.Whipple病及其进展.临床荟萃,2001,16(4):190~191

2.王真真,王镇淘,陈文明.Whipple病.临床荟萃,2003,18(9):533~534

3.钱大可.Cronkhite-Canada综合征.中华内科杂志,2000,20(2):80~81

4.八重坚宽治,宇都宫让二.Peutz-Jeghers征候群.消化器外科,1982,5:942~946

5.大浜用克,山本弘,新开真人,他.Peutz-Jeghers征候群の病态と治疗.小儿外科,2001,33:765~769

6.牛尾恭辅,石川勉,福富隆志,他.Cowden病(multiple hamartoma syndrome).癌の临床,1998,44:1024~1032

7.宇都宫让二,牛场广树,宫永忠彦,他.Peutz-Jeghers征候群.外科诊疗,1975,17:413~425

8.饭田三雄.家族性大肠ポリポシ-スとGardner征候群の大肠外肿疡状病变に关する研究.福冈医志,1978,69:169~200

9.松本主之,饭田三雄.遗传性消化管ポリポシ-スの临床像と遗传子异常.日消志,2000,97:1007~1016

10.须田武保,渡边英伸,富山胜义,他.特殊な消化管ポリ-プ,Peutz-Jeghers征候群.临床科学,1988,24:332~340

11.韩少良,中村恭一.消化道息肉的病理学.日本医学介绍,1992,13(11):487~489

12.韩少良,武滕彻一.消化道息肉和息肉病.日本医学介绍,1992,13(11):483~484

13.韩少良,柳树林,春日井邦夫.结直肠息肉的预防性摘除.日本医学介绍,1997,18(9):404~407

14.Afolabi OO.Oral melanosis:a case report of Peutz-Jegher’s syndrome in Suva,Fiji.Pac Health Dialog,2003,10(1):55~56

15.Alimoglu O,Sahin M,Cefle K,et al.Peutz-Jeghers syndrome:report of 6cases in a family and management of polyps with intraoperative endoscopy.Turk J Gastroenterol,2004,15(3):164~168

16.Assimakopoulos E,Zafrakas M,Athanasiades A,et al.Klippel-Trenaunay-Weber syndrome with abdominal he-mangiomata appearing on ultrasound examination as intestinal obstruction.Ultrasound Obstet Gynecol,2003,22(5):549~550

17.Baisden BL,Lepidi H,Raoult D,et al.Diagnosis of Wihipple disease by immunohistochemical analysis:a sensi-tive and specific method for detection of Tropheryma Whipplei(the Whipple bacillus)in paraffin-embedded tissue.Am H Clin Pathol,2002,118:742~748

18.Boni R,Capasso A,de Nucci C,et al.Whipple disease.Report of a case with unusual therapeutic aspects.Re-centi Prog Med,1995,86(5):201~203

19.Cha SH,Romeo MA,Neutze JA.Visceral manifestations of Klippel-Trenaunay syndrome.Radiographics,2005,25(6):1694~1697

20.Cronkhite LW,Canada WJ.Generalized gastrointestinal polyposis-an unusual syndrome of polyposis,pigmenta-tion,alopecia,and onychatrophia.N Engl J Med,1955,252:1011~1015

21.Darwish K,Bleau BL.Extensive small bowel varices as a cause of severe anemia in Klippel-Trenaunay-Weber syndrome.Am J Gastroenterol,1998,93(11):2274~2275

22.Dong K,Li B,Li BH,et al.Clinical analysis of Peutz-Jeghers syndrome:a report of 6cases.Zhonghua Wei Chang Wai Ke Za Zhi,2005,8(4):336~338

23.Erdas E,Licheri S,Pisano G,et al.Peutz-Jeghers syndrome:an account of 3cases in the same family and a re-view of the literature.Chir Ital,2005,57(4):425~436

24.Gobbi S,Sarli L,Violi V,et al.Laparoscopically assisted treatment of acute abdomen in systemic lupus erythe-matosus.Surg Endosc,2000,14(11):1085~1086

25.Hanzawa,Yoshikawa N,Tezuka T,et al.Surgical treatment of Cronkhite-Canada syndrome associated protein-losing enteropathy:report of a case.Dis Colon Rectum,1998,41:932~934

26.Hemminki A,Markie D,Tomlinson I,et al.A serine threonine kinase gene defective in Peutz-Jeghers syn-drome.Gastroenterology,2000,119:1447~1453

27.Hinds R,Philp C,Hyer W,et al.Complications of childhood Peutz-Jeghers syndrome:implications for pediatric screening.J Pediatr Gastroenterol Nutr,2004,39(2):219~220

28.Hizawa K,Iida M,Matsumoto T,et al.Cancer in Peutz-Jeghers syndrome.Cancer,1993,72:2777~2781

29.Kohno T,Takahashi M,Fukutomi T,et al.Germline mutation of the PTEN/MMAC1gene in Japanese patients with Cowden disease.Jpn J Cancer Res,1998,89:471~474

30.Lantz H,Doos WG,Affi A.Peutz-Jeghers-type hamartomatous polyp in a patient without Peutz-Jeghers syn-drome.Gastrointest Endosc,2004,60(2):316~317

31.Lee NH,Choi EH,Choi WK,et al.Maffucci’s syndrome with oral and intestinal haemangioma.Br J Dermatol.1999,140(5):968~969

32.Lehmann TG,Dux M,von Herbay A,et al.Klippel-Trenaunay syndrome with involvement of the rectum.Sur-gical therapy after interventional-radiologic preparation.Chirurg,2000,71(2):228~233

33.Luman W,Chua KB,Cheong WK,et al.Gastrointestinal manifestations of systemic lupus erythematosus.Sin-gapore Med J,2001,42(8):380~384

34.Mako EF.Small-bowel hemangiomatosis in a patient with Maffucci’s and blue-rubber-bleb-nevus syndromes.AJR Am J Roentgenol,1996,166(6):1499~1500

35.Marinella MA,Chey W.The syndrome of inappropriate antidiuretic hormone secretion in a patient with Whipple’s disease.Am J Med Sci,1997,313(4):247~248

36.Marsh DJ,Kum JB,Lunetta KL,et al.PTEN mutation spectrum and genotype correlations in Bannayan-Riley-Ruvalcaba syndromes suggest a single entity with Cowden’s syndrome.Hum Mol Genet,1999,8:1461~1472

37.Mehta MV,Porecha MM,Mehta PJ.Small intestinal adenocarcinoma in Peutz-Jeghers syndrome.Indian J Gas-troenterol,2006,25(1):38~39

38.Miyahara S,Ito S,Soeda A,et al.Two cases of systemic lupus erythematosus complicated with colonic ulcers.Intern Med,2005,44(12):1298~1306

39.Nagata J,Kijima H,Hasumi K,et al.Adenocarcinoma and multiple adenomas of the large intestine,associated with Cronkhite-Canada syndrome.Dig Liver Dis,2003,35:434~438

40.Nakama T,Matsui T.Iwashita A.Characteristic clinical findings and pathological findings in the small intestinal lessions of the Satoyoshi Syndrome.Stomach Intestine(Tokyo),2008,43(4):713~718

41.Narvaez J,Perez-Vega C,Castro-Bohorquez FJ,et al.Intestinal pseudo-obstruction in systemic lupus erythema-tosus.Scand J Rheumatol,2003,32(3):191~195

42.Orchahard TR,Chua CN,Ahmad T,et al.Uveitis and erythema nodosum in inflammatory bowel disease:clini-cal features and the role of HLA genes.Gastroenterology,2002,123:442~444

43.Oshia T,et al.Extraintestinal manifestations in gastrointestinal disease.Stomach Intestine(Tokyo),2003,38(4):415~426

44.Pardos-Gea J,Ordi-Ros J,Selva A,et al.Chronic intestinal pseudo-obstruction associated with biliary tract dila-tation in a patient with systemic lupus erythematosus.Lupus,2005,14(4):328~330

45.Qualman SJ,Bowen J,Erdman SH.Molecular basis of the brain tumor-polyposis(Turcot)syndrome.Pediatr Dev Pathol,2003,6(6):574~576

46.Schmitt B,Posselt HG,Waag KL,et al.Severe hemorrhage from intestinal hemangiomatosis in Klippel-Trenau-nay syndrome:pitfalls in diagnosis and management.J Pediatr Gastroenterol Nutr,1986;5(1):155~158

47.Schmitt BP,Richardson H,Smith E,et al.Encephalopathy complicating Whipple’s disease:failure to respond to antibiotics.Ann Intern Med,1981,94:51~52

48.Schreibman IR,Baker M,Amos C,et al.The hamartomatous polyposis syndrome:a clinical and molecular re-view.Am J Gastroenterol,2005,100(2):476~490

49.Schulmann K,Schmiegel W.Capsule endoscopy for small bowel surveillance in hereditary intestinal polyposis and non-polyposis syndromes.Gastrointest Endosc Clin N Am,2004,4(1):149~158

50.Shibata C,Sasaki I,Naito H,et al.Turcot syndrome with colonic obstruction and small intestinal invagination:report of a case.Surg Today,1999,29(8):785~788

51.Sunahara M,Nakagawara A.Turcot syndrome.Nippon Rinsho,2000,58(7):1484~1489

52.Takhashi H,Ohara M,Imai K.Collagen diseases with gastrointestinal manifestations.Nihon Rinsho Meneki

Gakkai Kaishi,2004;27(3):145~155

53.Thomas JM,Howard EK,Richard JZ.Peutz-Jeghers syndrome.Am J Gastroenterol,2000,95:596~604

54.Vasen HFA.Clinical diagnosis and management of hereditary colorectal cancer syndrome.J Clin Oncol,2000,18:81S~92S

55.Vazquez-Sequeiros E,Sorbi D,Kamath PS,et al.Klippel-Trenaunay-Weber syndrome:role of EUS.Gastroin-test Endosc,2001,54(5):660~661

56.Verhagen WI,Huygen PL,Dalman JE,et al.Whipple’s disease and the central nervous system.A case report and a review of the literature.Clin Neurol Neurosurg,1996,98(4):299~304

57.Wang ZJ,Ellis I,Zauber P,et al.Allelic imbalance at the LKB1(STK11)locus in tumors from patients with Peutz-Jeghers’syndrome provides evidence for a hamartoma-(adenoma)-carcinoma sequence.J Pathol,1999,188:9~13

58.Westerman AM,Entius MM,de Baar E,et al.Peutz-Jeghers syndrome:78-year follow-up of the original fami-ly.Lancet,1999,353:1211~1215

59.Wu JS,Paul P,McGannon EA,et al.APC genotype,polyp number,and surgical options in familial adenoma-tous polyposis.Ann Surg,1998,227:57~62

60.Zanca P.Multiple hereditary cartilaginous exostosis with polyposis of the colon.US Armed Forces Med,1956,7:116~120

免责声明:以上内容源自网络,版权归原作者所有,如有侵犯您的原创版权请告知,我们将尽快删除相关内容。

我要反馈