肝外性門脈圧亢進症
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- 1. 非肝硬変性門脈圧亢進症noncirrhotic portal hypertension [show details]
… Idiopathic portal hypertension; Hepatoportal sclerosis; Incomplete septal cirrhosis; Obliterative portal venopathy; Benign intrahepatic portal hypertension; Idiopathic presinusoidal portal hypertension; Partial …
- 2. 成人における門脈圧亢進症portal hypertension in adults [show details]
… Portal hypertension often develops in the setting of cirrhosis, schistosomiasis, or extrahepatic portal vein thrombosis. It is the result of resistance to portal blood flow and may lead to complications …
- 3. 学会が公開する診療ガイドラインのリンク:門脈圧亢進症と腹水症society guideline links portal hypertension and ascites [show details]
…Consensus Workshop: Expanding consensus in portal hypertension – Stratifying risk and individualizing care for portal hypertension (2015) Portal hypertension in children – Expert pediatric opinion on the …
- 4. 脾腫を有する小児に対するアプローチapproach to the child with an enlarged spleen [show details]
…(cirrhosis). Cirrhosis-related splenomegaly results from congestion due to portal hypertension. Other sequelae of portal hypertension (eg, esophageal varices, gastroesophageal bleeding, ascites) may also be …
- 5. 学会が公開する診療ガイドラインのリンク:成人の消化管出血society guideline links gastrointestinal bleeding in adults [show details]
…Hepatitis C Program: Recommendations for management and treatment of patients with cirrhosis and portal hypertension (2009) ACG: Practice guideline for prevention and management of gastroesophageal varices…
English Journal
- Congenital extrahepatic portosystemic shunt with progressive myelopathy and encephalopathy.
- Lux D, Naito A, Harikrishnan S.
- Practical neurology. 2019 May;().
- Portosystemic encephalopathy commonly occurs in patients with portal hypertension caused by end-stage liver disease or portal vein thrombosis. Congenital extrahepatic portosystemic shunt (CEPS) is an underdiagnosed and treatable condition that can cause encephalopathy and various neuropsychiatric sy
- PMID 31048365
- PMID 29763077
- Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics.
- Ohfuji S, Furuichi Y, Akahoshi T, Kage M, Obara K, Hashizume M, Matsuura T, Fukushima W, Nakamura Y.
- Hepatology research : the official journal of the Japan Society of Hepatology. 2019 Apr;().
- Idiopathic portal hypertension (IPH), extrahepatic portal obstruction (EHO), and Budd-Chiari syndrome (BCS) are characterized by aberrant portal hemodynamics of unknown etiology. The aim of this study was to explore trends in the descriptive epidemiology of these diseases through periodical nationwi
- PMID 30945395
Japanese Journal
- Successful Balloon-Occluded Retrograde Obliteration of Duodenal Varices Due to Chronic Portal Vein Thrombosis: A Feasible Treatment Option for Duodenal Varices with Atrophic Intrahepatic and Extrahepatic Portal Veins
- Kamo Minobu,Horiuchi Saya,Morita Yuka,Ikeya Takashi,Fujita Yoshiyuki
- Interventional Radiology 4(1), 1-5, 2019
- … <p>A 61-year-old man with a history of septic portal vein thrombosis presented with an enlarging duodenal varix. … Prominent atrophic changes of the intrahepatic and extrahepatic portal veins were observed on computed tomography. … Gradual re-enlargement of the main trunk of the portal vein was also observed.</p> …
- NAID 130007614439
- Successful Balloon-Occluded Retrograde Obliteration of Duodenal Varices Due to Chronic Portal Vein Thrombosis: A Feasible Treatment Option for Duodenal Varices with Atrophic Intrahepatic and Extrahepatic Portal Veins
- Kamo Minobu,Horiuchi Saya,Morita Yuka,Ikeya Takashi,Fujita Yoshiyuki
- Interventional Radiology, 2019
- … <p>A 61-year-old man with a history of septic portal vein thrombosis presented with an enlarging duodenal varix. … Prominent atrophic changes of the intrahepatic and extrahepatic portal veins were observed on computed tomography. … Gradual re-enlargement of the main trunk of the portal vein was also observed.</p> …
- NAID 130007604278
- 症例報告 続発性肝外門脈閉塞症に伴う繰り返す異所性静脈瘤出血に対して外科的腸間膜静脈-体循環シャント造設術を施行した1例
- 千代田 武大,金子 順一,国土 典宏,長谷川 潔
- 日本門脈圧亢進症学会雑誌 = Japanese journal of portal
- NAID 40021806121
Related Links
- Abstract A retrospective analysis of 19 patients with esophagogastric or intestinal varices secondary to extrahepatic portal hypertension was conducted. Nine patients had onset of bleeding during childhood. In six of eight pediatric ...
- Noncirrhotic portal hypertension (NCPH), as it generally is termed, is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can ...
- Background Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are... Background Idiopathic portal ...
★リンクテーブル★
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- 英
- extrahepatic portal obstruction EHO
- 同
- 肝外性門脈圧亢進症 extrahepatic portal hypertension
- 関
- 肝外門脈閉塞
[show details]
- 小児の肝外門脈閉塞症(PED.1101):(病態)脾静脈、門脈本幹、または左右の太い門脈枝が閉塞し門脈圧が亢進。門脈本幹の閉塞と門脈海綿状血管新生(海綿状血管増生)をきたす。(病因)新生児期の臍炎、臍静脈カテーテルの使用、年長児では虫垂炎、腹膜炎、慢性炎症性腸疾患、胆管炎などによる炎症の門脈波及に伴う血栓症、プロテインS欠損・プロテインC欠損など血液凝固亢進が上げられるが、薬半数は原因不明。(症状)初発は突然の消化管出血、脾腫、鉄欠乏性貧血。
分類
- 小児期に好発。新生児臍帯炎が原因とされている。 (SSUR.645)
症状
- (一次性)食道胃静脈瘤、脾腫、汎血球減少。(SSUR.645)
- 腹水(-):肝前性門脈閉塞症では通常腹水を認めないが、閉塞の急性期における門脈上昇時には腹水が認められる。(QB.B-319)
検査
- (一次性)肝機能は保たれる。(SSUR.645)
- (一次性)腹部画像検査:求肝性側副血行路の発達(海綿状血管増生)
予後
- (一次性)10年生存率は約90% (SSUR.645)
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- 関
- gateway
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- 関
- extrahepatically、nonhepatic
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- 関
- portal vein
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高血圧, 高血圧症, HT