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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

「肝外門脈閉塞症」

　　[★]

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]

肝外門脈閉塞 : 約 156,000 件
肝外門脈閉塞症 : 約 13,900 件

肝外門脈閉塞症 EHO : 約 1,190 件
肝外門脈閉塞 EHO : 77 件

小児の門脈圧亢進症では肝外門脈閉塞が多い。肝外門脈閉塞は(門脈圧亢進症の)2/3を占める(QB.B-315)。

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。