門脈高壓

門脈高壓
	The portal vein and its tributaries
症状	腹水、黄疸、脾腫大[*]、微血管擴張
类型	hepatic vascular disease[]、hypertension[]、疾病
肇因	門靜脈血栓[]、血吸虫病、肝硬化、限制型心肌病[]、心包炎
分类和外部资源
醫學專科	Gastroenterology
ICD-11	DB98.7
ICD-9-CM	572.3
DiseasesDB	10388
eMedicine	182098
	[编辑此条目的维基数据]

門脈高壓（英語：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]}。

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
食道靜脈曲張（英语：Oesophageal varices），可能破裂導致呕血^{[來源請求]}。
前腹壁靜脈曲张（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）或脾靜脈（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

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

^ Portal hypertension | Disease | Overview | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. rarediseases.info.nih.gov. [2016-01-08]. （原始内容存档于2016-01-27）.
^ ^2.0 ^2.1 ^2.2 ^2.3 ^2.4 Portal Hypertension. Learn about Portal Hypertension | Patient. Patient. [2016-01-08]. （原始内容存档于2021-01-19）（英国英语）.
^ Spontaneous Bacterial Peritonitis. www.karger.com. [2016-01-08]. （原始内容存档于2020-05-10）.
^ 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.
^ Bloom, S.; Kemp, W.; Lubel, J. Portal hypertension: pathophysiology, diagnosis and management. Internal Medicine Journal. 2015-01-01, 45 (1): 16–26 [2017-01-03]. ISSN 1445-5994. doi:10.1111/imj.12590. （原始内容存档于2017-09-14）.
^ 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
^ Portal Hypertension: Practice Essentials, Background, Anatomy. [2017-01-03]. （原始内容存档于2021-05-06）.

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]. （原始内容存档于2019-07-10）.
Rossi, Plinio. Portal Hypertension: Diagnostic Imaging and Imaging-Guided Therapy. Springer Science & Business Media. 2012-12-06 [2017-01-03]. ISBN 9783642571169. （原始内容存档于2020-08-31）.
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 （页面存档备份，存于互联网档案馆）

[1] Portal hypertension | Disease | Overview | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. rarediseases.info.nih.gov. [2016-01-08]. （原始内容存档于2016-01-27）.

[patient-2] 2.0 ^2.1 ^2.2 ^2.3 ^2.4 Portal Hypertension. Learn about Portal Hypertension | Patient. Patient. [2016-01-08]. （原始内容存档于2021-01-19）（英国英语）.

[3] Spontaneous Bacterial Peritonitis. www.karger.com. [2016-01-08]. （原始内容存档于2020-05-10）.

[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 [2017-01-03]. ISSN 1445-5994. doi:10.1111/imj.12590. （原始内容存档于2017-09-14）.

[Robbins_9th_Edition-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. [2017-01-03]. （原始内容存档于2021-05-06）.

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