出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/08/28 13:02:32」(JST)
Portal hypertension | |
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The portal vein and its tributaries.
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Classification and external resources | |
Specialty |
Gastroenterology DiseasesDB = 10388 |
ICD-10 | K76.6 |
ICD-9-CM | 572.3 |
eMedicine | radio/570 med/1889 |
MeSH | D006975 |
Portal hypertension is hypertension (high blood pressure) in the portal vein system, which is composed of the portal vein, and its branches and tributaries.
Portal hypertension is defined as elevation of hepatic venous pressure gradient to >5mmHg. Generally, in clinical practice the pressure is not measured directly until the decision to place a transjugular intrahepatic portosystemic shunt (TIPS) has already been made. As part of that procedure, a hepatic vein wedge pressure is measured with the assumption of no pressure drop across the liver yielding portal vein pressure.
Consequences of portal hypertension are caused by blood being forced down alternate channels by the increased resistance to flow through the systemic venous system rather than the portal system. They include:
Causes can be divided into pre-hepatic, intra-hepatic, and post-hepatic.
HVPG (hepatic venous pressure gradient) measurement has been accepted as the gold standard for assessing the severity of portal hypertension, and replaced the old one - contrast angiography.[3] Portal hypertension is defined as HVPG greater than or equal to 5mm Hg and is considered to be clinically significant when HVPG exceeds 10 to 12 mm Hg.[4]
These can be categorized by several different concepts: selective vs non-selective, mesocaval vs portocaval, and the specific arrangement of vessels, e.g. end-to-side or side-to-side. Selective shunts select non-intestinal flow to be shunted to the systemic venous drainage while leaving the intestinal venous drainage to continue pass through the liver. The most well known of this type is the splenorenal, or Warren, shunt. This connects the splenic vein to the left renal vein thus reducing portal system pressure while minimizing any encephalopathy. In an H-shunt, which could be mesocaval (from the superior mesenteric vein to the inferior vena cava) or could be, unlikely, portocaval (from the portal vein to the inferior vena cava) a graft, either synthetic or the preferred vein harvested from somewhere else on the patient's body, is connected between the superior mesenteric vein and the inferior vena cava. The size of this shunt will determine how selective it is.
It should be noted that with the advent of transjugular intrahepatic portosystemic shunting (TIPS), portosystemic shunts are now very rarely performed. TIPS has the advantage of being much easier to perform and it doesn't disrupt any of the liver's vascularity, which will be needed if a given patient's hopes for liver transplant. In general, non-selective shunts are emergency surgeries that are done as quickly as possible to minimize intraoperative blood loss. On the contrary, a splenorenal shunt would be an elective procedure due to its great technical demands. Further contributing to their rare use today is the fact that few, if any, current general surgery residents are trained in how to carry out these surgeries
Both pharmacological (non-specific ß-blockers like Propranolol and the nitrate isosorbide mononitrate) and endoscopic (banding ligation) treatment have similar results. TIPS (transjugular intrahepatic portosystemic shunting) is superior to either of them at reducing rate of rebleeding. Disadvantages of TIPS include high cost and increased risk of hepatic encephalopathy, and it does not improve the mortality rate.
After resuscitation, which may require blood transfusion, the management of active variceal bleeding includes administering vasoactive drugs (somatostatin, octreotide or terlipressin), endoscopic banding ligation, balloon tamponade and TIPS(Transjugular intrahepatic portocaval shunt)
This should be gradual to avoid sudden changes in systemic volume status which can precipitate hepatic encephalopathy, renal failure and death. The management includes salt restriction, diuretics (spironolactone), paracentesis, transjugular intrahepatic portosystemic shunt (TIPS) and peritoneovenous shunt.
A standard treatment plan may involve lactulose, bowel enemas, and use of oral antibiotics such as rifaximin, neomycin, metronidazole, vancomycin, and the quinolones. Previously, restriction of dietary protein was recommended but this is now refuted by a clinical trial which showed no benefit.[5] Instead, the maintenance of adequate nutrition is now advocated.[6]
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リンク元 | 「門脈圧亢進症」 |
拡張検索 | 「idiopathic portal hypertension」「postsinusoidal portal hypertension」「extrahepatic portal hypertension」 |
関連記事 | 「port」「portal」「hypertension」 |
肝前性 | 肝内性 | 肝後性 | ||
肝外門脈閉塞症 | 特発性門脈圧亢進症 | 肝硬変 | Budd-Chiari症候群 | |
extrahepatic portal obstruction | idiopathic portal hypertension | liver cirrhosis | ||
門脈圧亢進症に占める割合 | 2-5% | 90%以上 | ||
閉塞肝静脈圧 vs 門脈圧 | 小 | 小 | 大 | 大 |
閉塞部位 | 肝外門脈 | 肝内門脈 類洞前 |
肝内肝静脈 類洞後 |
肝外肝静脈 |
疫学 | (一次性)小児期に発症 | 中年女性に多い | ||
病因 | (一次性)原因不明(新生児臍帯炎) (二次性)腫瘍病変 |
原因不明 | (一次性)原因不明 (二次性)血栓説 | |
症状 | 肝機能はほぼ正常 食道胃静脈瘤、脾腫、汎血球減少 |
肝機能は良好な事が多い 巨脾、食道胃静脈瘤、汎血球減少、脾機能亢進 |
進行例で肝機能低下 腹水、下腿浮腫、下肢静脈瘤、難治性下腿 胸腹壁の上行性皮下静脈怒張 食道胃静脈瘤、脾腫、汎血球減少 | |
肝臓の外観 | 表面平滑or波打ち状 | 再生結節により表面に凹凸を認める | ||
血管検査 | (肝静脈造影)肝静脈枝相互吻合、しだれ柳状 | (下大静脈造影)膜様閉塞 or 完全閉塞 | ||
病理 | 海綿状血管増生 | 肝内門脈末梢枝のつぶれ像 | 肝小葉構造の改築 | 肝静脈周囲のうっ血と壊死。進行すれば肝硬変 |
予後 | (一次性)10年生存率90% |
10年生存率70-80% 肝硬変より良好 |
(一次性)10年生存率40% |
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