門脈高壓

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門脈高壓

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The portal vein and its tributaries
分类和外部资源
醫學專科
Gastroenterology

ICD-10

K76.6

ICD-9-CM

572.3
DiseasesDB
10388
eMedicine
radio/570 med/1889
MeSH
D006975

[编辑此条目的维基数据]

門脈高壓(Portal hypertension)為描述肝門靜脈系統英语hepatic portal system高壓的狀態。目前的定義為肝門靜脈梯度英语hepatic venous pressure gradient高於或等於6mmHg[1]。門脈高壓最常見的原因就是肝硬化,其他較罕見的原因則稱為非肝硬化性門脈高壓(non-cirrhotic portal hypertension)。當門脈高壓已造成問題,則必須適當紓解門脈壓力[來源請求]




目录





  • 1 症狀和徵象


  • 2 病因


  • 3 病生理


  • 4 參考文獻


  • 5 延伸閱讀


  • 6 外部連結




症狀和徵象


門脈高壓的症狀和徵象包含:




  • 腹水[2]
    • 腹痛或腹脹[3],如果合併細菌感染則稱為自發性細菌性腹膜炎英语Spontaneous bacterial peritonitis


  • 脾腫大英语Splenomegaly[2],可能導致血小板數量降低


  • 食道靜脈屈張英语Oesophageal varices,可能破裂導致呕血[來源請求]

  • 前腹壁靜脈腫脹(有時又稱Caput medusae英语Caput medusae[2]


  • 痔疮[4]



病因


門脈高壓可藉由病灶發生的位置分為肝前性、肝內性、肝後性等。最常見的因素為肝硬化,其他原因則包含[2][5][6]















































非肝硬化性門脈高壓的相關因子
肝前性
門靜脈英语Portal vein thrombosis脾靜脈英语splenic vein血栓
動靜脈瘻管
脾腫大(增加門脈血流)
肝內性所有造成肝硬化的原因,包含酗酒、慢性病毒性肝炎、膽道閉鎖英语biliary atresia等等
Primary biliary cirrhosis
Primary sclerosing cholangitis
Chronic pancreatitis
Hereditary haemorrhagic telangiectasia
血吸蟲症
Congenital hepatic fibrosis
Nodular regenerative hyperplasia
Fibrosis of space of Disse
Granulomatous or infiltrative liver diseases (Gaucher, mucopolysaccharidosis, sarcoidosis, lymphoproliferative malignancies, amyloid deposition, …)
毒素(如砷、銅、氯乙烯、礦物油、維生素A、azathioprine、dacarbazine、methotrexate、amiodarone...)
病毒性肝炎
脂肪肝
Veno-occlusive disease
肝後性下腔靜脈阻塞
(Right-sided) heart failure, e.g. from constrictive pericarditis英语constrictive pericarditis

布加综合征
Budd-Chiari syndrome


病生理




Stellate cell


門脈高壓的直接原因主要是血管的阻力增加,但造成血管阻力增加的原因則相當多樣。一般來說還會合併星狀細胞及肌成纖維細胞英语myofibroblasts活化。內源性血管擴張劑也可能導致靜脈血流增加[2][7]



參考文獻




  1. ^ Portal hypertension | Disease | Overview | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. rarediseases.info.nih.gov. [2016-01-08]. 


  2. ^ 2.02.12.22.32.4 Portal Hypertension. Learn about Portal Hypertension | Patient. Patient. [2016-01-08] (英国英语). 


  3. ^ Spontaneous Bacterial Peritonitis. www.karger.com. [2016-01-08]. 


  4. ^ Sun, Z; Migaly, J. Review of Hemorrhoid Disease: Presentation and Management.. Clinics in colon and rectal surgery. March 2016, 29 (1): 22–9. PMID 26929748. doi:10.1055/s-0035-1568144. 


  5. ^ Bloom, S.; Kemp, W.; Lubel, J. Portal hypertension: pathophysiology, diagnosis and management. Internal Medicine Journal. 2015-01-01, 45 (1): 16–26. ISSN 1445-5994. doi:10.1111/imj.12590. 


  6. ^ Perkins, [edited by] Vinay Kumar, Abul K. Abbas, Jon C. Aster ; artist, James A. Robbins basic pathology 9th. Philadelphia, PA: Elsevier/Saunders. : 608. ISBN 978-1-4377-1781-5. 2013


  7. ^ Portal Hypertension: Practice Essentials, Background, Anatomy. 



延伸閱讀



  • Garbuzenko D.V., Arefyev N.O., Belov D.V. Mechanisms of adaptation of the hepatic vasculature to the deteriorating conditions of blood circulation in liver cirrhosis. World J. Hepatol. 2016; 8 (16): 665-672.

  • Garbuzenko D.V. Current approaches to the management of patients with liver cirrhosis who have acute esophageal variceal bleeding. Curr. Med. Res. Opin. 2016; 32 (3): 467-475.

  • Garbuzenko D.V. Contemporary concepts of the medical therapy of portal hypertension under liver cirrhosis. World J. Gastroenterol. 2015; 21 (20): 6117-6126.


  • Functional aspects on the pathophysiology of portal hypertension in cirrhosis – Journal of Hepatology. www.journal-of-hepatology.eu. [2016-01-08]. 


  • Rossi, Plinio. Portal Hypertension: Diagnostic Imaging and Imaging-Guided Therapy. Springer Science & Business Media. 2012-12-06. ISBN 9783642571169. 


  • Imanieh, Mohammad Hadi; Dehghani, Seyed Mohsen; Khoshkhui, Maryam; Malekpour, Abdorrasoul. Etiology of Portal Hypertension in Children:A Single Center’s Experiences. Middle East Journal of Digestive Diseases. 2012-10-01, 4 (4): 206–210. ISSN 2008-5230. PMC 3990125. PMID 24829658. 



外部連結



  • Ascites at Merck Manual of Diagnosis and Therapy英语Merck Manual of Diagnosis and Therapy Home Edition

  • Children's Liver Disease Foundation

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