Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatic venous outflow obstruction also called the Budd-Chiari syndrome is increasingly being recognized as a cause of portal hypertension. In western countries the obstruction is usually in the hepatic veins while in reports from South Africa, Japan and India the predominant cause is a block in the IVC at the level of the diaphragm above the entry of the hepatic veins. A hypercoagulable state caused by myeloproliferative haematological disorders, clonal defects in haemopoietic stem cells, lupus anticoagulant, contraceptive pills and postpartum state are some of the aetiological conditions described. However in 25% to 75% cases no cause can be identified. The predominant presenting features in patients with hepatic vein obstruction are hepatomegaly and ascites while those with IVC obstruction show prominent veins on the trunk and back. Ultrasound examination should be the first investigative step. However a liver biopsy is the gold standard of diagnosis. To confirm the site of obstruction inferior vena cavography or functional hepatography may be required. In the acute phase thrombolytic therapy may be useful but for established cases either surgical intervention in the form of shunts or recently balloon angioplasty may be helpful. For patients with established cirrhosis and end-stage liver failure the only alternative is liver transplantation. All these patients however should be put on long term anticoagulants to prevent rethrombosis. Some series have reported that upto 45% of patients may develop hepatocellular carcinoma on long term followup. With proper management a larger proportion of patients can be returned to a useful productive life.
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PMID:Hepatic venous outflow obstruction. 982 3

The hypothesis tested that mechanisms other than retrograde transsinusoidal fluid wave transfer reported in patients with right heart failure are responsible for the ultrasonographic sign of advanced portal vein pulsatility (APP). Within a time-period of 3 years we have seen 13 patients with APP, defined as temporary portal flow reversal in the face of a normal echocardiogram. Nine of these patients had biopsy-proven liver cirrhosis and four with liver disease were without cirrhosis or cardiac pathology. A randomly selected control group of 18 healthy subjects was studied. Doppler ultrasound evaluation of the hepatic veins as well as the intra and extrahepatic portal vein territories was performed in both groups. Hepatopetal portal flow with APP reversed to hepatofugal flow in follow up studies in two patients. In another two hepatopetal flow with APP in the main portal vein and hepatofugal flow in the intrahepatic portal radicles was recorded during the same examination. The remaining group displayed APP in the intra and extrahepatic portal vein territories. None of the normal subjects presented with APP. Hepatic venous outflow obstruction associated with excessive arterioportal shunting is likely to account for APP of all of our patients. Based on a causal link between angiographic 'to-and-fro' flow pattern and the sonographic APP sign in patients with sinusoidal outflow obstruction we suggest, that APP expresses a short, transitional period of portal hypertension just before the occurence of flow reversal.
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PMID:Doppler ultrasound evaluation of advanced portal vein pulsatility in patients with normal echocardiograms. 984 93

Hepatic venous outflow obstruction after piggyback liver transplantation is a very rare complication. An unusual mechanism aggravating it is reported. A 33-year-old man with end-stage hepatitis B liver cirrhosis underwent a piggyback orthotopic liver transplantation using a full-size cadaveric graft. Two months after transplantation, he developed gross ascites refractory to maximal diuretic therapy. Doppler ultrasound showed patent portal and hepatic veins. Serial computed tomography scans revealed a hypoperfused right posterior segment of the liver which subsequently underwent atrophy. Hepatic venography demonstrated a high-grade stenosis with an element of torsion of venous drainage at the anastomosis. The stenosis was successfully treated with repeated percutaneous balloon angioplasty. The patient remained asymptomatic six months afterwards with complete resolution of ascites and peripheral edema. We postulate that liver allograft segmental hypoperfusion and atrophy may aggravate or result in a hepatic venous outflow problem by the mechanism of torsion effect. Percutaneous balloon angioplasty is a safe and effective treatment modality for anastomotic stenosis.
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PMID:Hepatic venous outflow obstruction after piggyback liver transplantation by an unusual mechanism: report of a case. 1698 Dec 82

Hepatic venous outflow obstruction is a relatively uncommon but important and devastating complication occurring after liver transplantation. Recently, right lateral sector liver grafts have sometimes been used in living-donor liver transplantation (LDLT), but, to our knowledge, early hepatic venous outflow obstruction has never been reported in right lateral sector LDLT. A 58-year-old woman was diagnosed with liver cirrhosis and hepatocellular carcinoma and underwent right lateral sector LDLT. Postoperatively, she developed liver dysfunction. Doppler ultrasound examination revealed flat waveforms and low-flow velocity in the right hepatic vein (RHV). A computed tomography (CT) scan revealed a ventrally distorted RHV due to hypertrophy of the liver graft. Hepatic venous obstruction was suspected and a hepatic venogram was performed. The venogram revealed stenosis of the RHV due to the distortion of the vein. We performed percutaneous transfemoral balloon dilatation, but this was not effective. We then inserted an expandable metallic stent (EMS) into the RHV. After the EMS placement, the condition of the patient improved. Venogram and CT data suggested that the obstruction of the RHV developed because of distortion of the RHV to the ventral side during liver regeneration.
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PMID:Hepatic venous outflow obstruction after right lateral sector living-donor liver transplantation, treated by insertion of an expandable metallic stent. 1839 21