肾移植术后肝脏弥散性动脉-门静脉瘘的诊治——附1例报告及文献复习

目的探讨少见的肝脏弥散性动脉-门静脉瘘的诊断和治疗。 方法报告本院收治的1例尿毒症患者在肾移植术后出现顽固性腹水、门静脉高压,最后确诊为肝脏弥散性动脉-门静脉瘘的诊治过程,并对该病的诊治进行探讨。结果DSA发现肝动脉及源于胃十二指肠动脉的一分支动脉进入肝脏,与门静脉在肝内形成弥散性动脉-门静脉瘘,栓塞治疗无显效,胆总管及门静脉骨骼化手术后腹水消失。结论肝脏弥散性动脉-门静脉瘘可突然发病,临床表现缺乏特异性,确诊须作DSA检查,治疗方法有待于探讨。

非肝病性门脉高压合并门静脉海绵样变性影像学诊断及临床

门静脉海绵样变(CTPV),特别是成人原发性较少见。本文通过介绍典型的1例原发性门静脉海绵样变病例,结合文献资料复习本病的病因,病理及其影像学及临床诊断方面的理论知识,并通过本例的几个特点:①老年女性;②病史40年以上,无肝病病史;③门静脉扩张呈海绵状血管瘤样改变;④伴有肺循环紊乱及肺动脉高压(门静脉性-肺动脉高压);⑤管壁大量钙化;⑥巨脾并大量钙化;⑦累及邻近器官(左肾);⑧病变范围较广泛。更加进一步提高对门静脉海绵样变特别是巨大海绵状血管瘤样改变和伴有肺循环紊乱及肺动脉高压(门脉性-肺动脉高压)的认识,并结合文献了解临床对门静脉海绵样变的治疗方法及进展。

Recurrent thrombotic occlusion of a transjugular intrahepatic portosystemic stent-shunt due to activated protein C resistance

The transjugular intrahepatic portosystemic stent-shunt (TIPS) has successfully been used in the management of refractory variceal bleeding and ascites in patients with portal hypertension. Major drawbacks are the induction of hepatic encephalopathy and shunt dysfunction. We present a 59-year-old woman with alcoholic liver cirrhosis who received a TIPS because of recurrent bleeding from esophageal varices. Stent occlusion occurred 4 mo after placement of the TIPS. Laboratory testing revealed resistance to activated protein C (APC). Combination therapy with low-dose enoxaparin and clopidogrel could not prevent her recurrent stent occlusion. Finally, therapy with high-dose enoxaparin was sufficient to prevent further shunt complications up to now (follow-up period of 1 year). In conclusion, early occlusion of a TIPS warrants testing for thrombophilia. If risk factors are confirmed,anticoagulation should be intensified. There are currently no evidence-based recommendations regarding the best available anticoagulant therapy and surveillance protocol for patients with TIPS.

Extensive hepatic-portal and mesenteric venous gas due to sigmoid diverticulitis

Hepatic portal venous gas is most often associated with extensive bowel necrosis due to mesenteric infarction.Mortality exceeds 75% with this condition.The most common precipitating factors include ischemia,intra-abdominal abscesses and inflammatory bowel disease.In this report,we present a 75-year-old woman with extensive hepatic portal and mesenteric venous gas due to colonic diverticulitis.She had a 10-year history of type diabetes mellitus and hypertension.She was treated by sigmoid resection and Hartmann's procedure and discharged from the hospital without any complications.

Septic thrombophlebitis of the porto-mesenteric veins as a complication of acute appendicitis

Pylephlebitis, a rare complication of acute appendicitis, is defined as thrombophlebitis of the portal venous system. Pylephlebitis usually occurs due to secondary infection in the region drained into the portal system. We report a case of pylephlebitis caused by acute appendicitis. The patient was transferred from a private clinic 1 wk after appendectomy with the chief complaints of high fever and abdominal pain. He was diagnosed with pylephlebitis of the portal vein and superior mesenteric vein by CT-scan. The patient was treated with antibiotics and anticoagulation therapy, and discharged on the 25th day and follow-up CT scan showed a cavernous transformation of portal thrombosis.

Quantitative Assessment of Hepatic Fibrosis by Contrast-enhanced Ultrasonography

Objective To explore the contrast-enhanced ultrasonographic features for quantitative assessment of hepatic fibrosis. Methods 86 patients with chronic viral hepatitis B were enrolled in this study from March 2007 to August 2009. The patients were classified into 5 groups (S0-S4) according to fibrosis stage evaluated with ultrasound guided liver biopsy. New contrast-enhanced ultrasonography (CEUS) features including area under the time-intensity curve (TIC) of portal venous phase/hepatic arterial phase (Qp/Qa) and intensity of portal venous phase/hepatic arterial phase (Ip/Ia) were used to detect the blood supply ratio (portal vein/hepatic artery) in each group. Arrival time of portal vein trunk (Tp) and decreasing rate of TIC (β ) were also analyzed. Results Qp/Qa and Ip/Ia decreased from S0 to S4, while Tp and β increased. These 4 features were significantly correlated with the degree of fibrosis (P<0.001) and were significantly different among the five groups (P<0.001). Sensitivity and specificity of Ip/Ia were 80% and 86% for groups ≥S1, 75% and 86% for groups ≥ S2, 71% and 84% for groups ≥ S3, and 76% and 80% for group S4, respectively. Sensitivity and specificity of Qp/Qa were 70% and 88% for groups ≥ S1, 80% and 76% for groups ≥ S2, 74% and 70% for groups ≥ S3, and 81% and 95% for group S4, respectively. Conclusion Ip/Ia and Qp/Qa could be adopted as reliable, non-invasive features for quantitative assessment of hepatic fibrosis.

Unrecognized pylephlebitis causing life-threatening septic shock: A case report

A man who developed profound septic shock was treated for Escherichia colisepsis of unknown origin. Following stabilisation, a diagnosis of pylephlebitis (infection and thrombosis in the portal vein) was made at computed tomography. A review of the condition, its primary causes,typical features, investigation and management was presented.

Phosphate-activated glutaminase activity is enhanced in brain, intestine and kidneys of rats following portacaval anastomosis

AIM： To assess whether portacaval anastomosis （PCA） in rats affects the protein expression and/or activity of glutaminase in kidneys, intestines and in three brain areas of cortex, basal ganglia and cerebellum and to explain the neurological alterations found in hepatic encephalopathy （HE）. METHODS： Sixteen male Wistar rats weighing 250-350 g were grouped into sham-operation control （n=8） or portacaval shunt （n = 8）. Twenty-eight days after the procedure, the animals were sacrificed. The duodenum, kidney and brain were removed, homogenised and mitochondria were isolated. Ammonia was measured in brain and blood. Phosphate-activated glutaminase （PAG） activity was determined by measuring ammonia production following incubation for one hour at 37 ℃ with O-phthalaldehyde （OPA） and specific activity expressed in units per gram of protein （pkat/g of protein）. Protein expression was measured by immunoblotting. RESULTS： Duodenal and kidney PAG activities together with protein content were significantly higher in PCA group than in control or sham-operated rats （duodenum PAG activity was 976.95±268.87μkat/g of protein in PCA rats vs 429.19±126.92.μkat/g of protein in shamoperated rats; kidneys PAG activity was 1259.18±228.79 μkat/g protein in PCA rats vs 669.67±400.8 μkat/g of protein in controls, P〈0.05; duodenal protein content： 173% in PCA vs sham-operated rats; in kidneys the content of protein was 152% in PCA vs sham-operated rats）. PAG activity and protein expression in PCA rats were higher in cortex and basal ganglia than those in shamoperated rats （cortex： 6646.6 ±1870.4 μkat/g of protein vs 3573.8± 2037.4 μkat/g of protein in control rats, P〈 0.01; basal ganglia, PAG activity was 3657.3± 1469.6 μkat/g of protein in PCA rats vs 2271.2±384 μkat/g of protein in sham operated rats, P〈0.05; In the cerebellum, the PAG activity was 2471.6±701.4 μkat/g of protein vs 1452.9 ±567.8 μkat/g of protein in the PCA and sham rats, respectively, P〈0.05; content of protein： cerebral cortex： 162% ±40% vs 100% ± 26%, P〈 0.009; and basal ganglia： 140% ±39% vs 100% ±14%, P〈0.05; but not in cerebellum： 100% ±25% vs 100% ± 16%, P= ns）. CONCLUSION： Increased PAG activity in kidney and duodenum could contribute significantly to the hyperammonaemia in PCA rats, animal model of encephalopathy. PAG is increased in non-synaptic mitochondria from the cortex and basal ganglia and could be implicated in the pathogenesis of hepatic encephalopathy. Therefore, PAG could be a possible target for the treatment of HE or liver dysfunction.

Clinical Observation on the Long-Term Therapeutic Effects of Traditional Chinese Medicine for Treatment of Liver Fibrosis

48 patients with liver fibrosis due to hepatitis B were treated for 2 years with the drugs for tonifying the kidney, supplementing qi, cooling and invigorating the blood and detoxification. The symptoms were markedly improved, and serum ALT and bilirubin were recovered and kept normal in most of the cases. The mean levels of serum hyaluronic acid, procollagen peptide III and circulating immune complex were decreased and returned to normal after the treatment. B-ultrasonography showed that the portal vein kept in normal size in 82% of the patients, the enlarged portal vein diminished in diameter, and the enlarged spleen reduced.

Clinical characteristics of idiopathic portal hypertension

Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

Stability of a rat model of prehepatic portal hypertension caused by partial ligation of the portal vein

AIM： To study the stability of portal hypertension （PHT） caused by partial ligation of the portal vein ligation （PVL） in a rat model.METHODS： Thirty male adult Wistar rats were divided into two groups： 10 in Group Ⅰ received a sham operation; and 20 in Group Ⅱreceived partial PVL. Portal vein pressure （PVP） was measured at four time periods： before ligation, 2 wk, 6 wk and 10 wk postsurgery. Portal venography, blood sampling and liver and spleen pathological examinations were conducted at 10 wk after surgery.RESULTS： The PVP was 9.15± 0.58 cmH2O before ligation, and increased to 17.32 ±0.63 cmH2O 2 wk after PVL. By repeat measurement of the PVP in each rat, it was shown to remain elevated for 10 wk. There were no significant differences in the pressure measurements at 2 wk, 6 wk and 10 wk. Varices were found mainly in the mesenteric vein 2 wk after PVL, which were more obvious later, while these manifestations were similar at week 6 and week 10. Portal venography demonstrated the varices and collaterals. There was no significant change in liver pathology. The volume of the spleen was enlarged 2-fold after ligation, and the sinus of the spleen was enlarged due to congestion. Significant sinus endothelial cell proliferation was observed, but no evidence of hypersplenia was found on hemogram and biochemical examination.CONCLUSION： These findings suggest that a satisfactory prehepatic PHT rat model can be obtained by partial ligation of the portal vein, and this PHT rat model was stable for at least 10 wk.

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?

Biliary disease in the setting of non-cirrhotic portal vein thrombosis(and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease.This is mostly due to portal biliary ductopathy.There are several mechanisms that play a role in the development of portal biliary ductopathy,such as induction of fibrosis in the biliary tract(due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis),loss of biliary motility,chronic cholestasis(due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis(due to various factors).The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention.Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy,diagnosis and therapy can become very complicated.Due to increased incidence and complications of cholelithiasis,standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems.Cholangitis,biliary strictures and hemobilia are the most common complications that occur during management of these patients.In this review,we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.

Acute extensive portal and mesenteric venous thrombosis after splenectomy:Treated by interventional thrombolysis with transjugular approach

AIM:To present a series of cases with symptomatic acute extensive portal vein(PV)and superior mesenteric vein(SMV)thrombosis after splenectomy treated by transjugular intrahepatic approach catheter-directed thrombolysis. METHODS:A total of 6 patients with acute extensive PV-SMV thrombosis after splenectomy were treated by transjugular approach catheter-directed thrombolysis. The mean age of the patients was 41.2 years.After access to the portal system via the transjugular approach,pigtail catheter fragmentation of clots, local urokinase injection,and manual aspiration thrombectomy were used for the initial treatment of PV-SMV thrombosis,followed by continuous thrombolytic therapy via an indwelling infusion catheter in the SMV,which was performed for three to six days. Adequate anticoagulation was given during treatment, throughout hospitalization,and after discharge. RESULTS:Technical success was achieved in all 6 patients.Clinical improvement was seen in these patients within 12-24 h of the procedure.No complications were observed.The 6 patients were discharged 6-14 d(8±2.5 d)after admission.The mean duration of follow-up after hospital discharge was 40±16.5 mo.Ultrasound and contrast-enhanced computed tomography confirmed patency of the PV and SMV,and no recurrent episodes of PV-SMV thrombosis developed during the follow-up period. CONCLUSION:Catheter-directed thrombolysis via transjugular intrahepatic access is a safe and effective therapy for the management of patients with symptomatic acute extensive PV-SMV thrombosis.

Is portal vein cavernous transformation a component of congenital hepatic fibrosis?

Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein (CTPV) is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices.We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.

Portal hypertensive colopathy is associated with portal hypertension severity in cirrhotic patients

AIM： To assess the prevalence of portal hypertension （PH） related colorectal lesions in liver transplant candidates, and to evaluate its association with the severity of PH. METHODS： Between October 2004 and December 2005, colonoscopy was performed in 92 cirrhotic liver transplant candidates. We described the lesions resulting from colorectal PH and their association with the grade of PH in 77 patients who underwent measurement of hepatic venous pressure gradient （HVPG）. RESULTS： Mean age was 55 years and 80.7% of patients were men. The main etiology of cirrhosis wasalcoholism （45.5%）. Portal hypertensive colopathy （PHC） was found in 23.9%, colonic varices in 7.6% and polyps in 38% of patients （adenomatous type 65.2%）. One asymptomatic patient had a well-differentiated adenocarcinoma. The manifestations of colorectal PH were not associated with the etiology of liver disease or with the Child-Pugh grade. Ninety percent of patients with colopathy presented with gastroesophageal varices （GEV）, and 27.5% of patients with GEV presented with colopathy （P = 0.12）. A relationship between higher values of HVPG and presence of colopathy was observed （19.9：1：6.2 mmHg vs 16.8 ± 5.4 mmHg, P = 0.045）, but not with the grade of colopathy （P = 0.13）. Preneoplastic polyps and neoplasm （P = 0.02） and spontaneous bacterial peritonitis （P = 0.006） were more prevalent in patients with colopathy. We did not observe any association between previous β-blocker therapy and the presence of colorectal portal hypertensive vasculopathy. CONCLUSION： PHC is common in cirrhotic liver transplant candidates and is associated with higher portal pressure.

Side effects of budesonide in liver cirrhosis due to chronic autoimmune hepatitis: Influence of hepatic metabolism versus portosystemic shunts on a patient complicated with HCC

AIM:To investigate the systemic availability of budesonide in a patient with Child A cirrhosis due to autoimmune hepatitis (AIH) and primary hepatocellular carcinoma,who developed serious side effects. METHODS:Serum levels of budesonide,6β-OH-budesonide and 16α-OH-prednisolon were measured by HPLC/MS/MS; portosystemic shunt-index (SI) was determined by 99mTc nuclear imaging.All values were compared with a matched control patient without side effects. RESULTS:Serum levels of budesonide were 13-fold increased in the index patient.The ratio between serum levels of the metabolites 6β-OH-budesonide and 16α-OH- prednisolone,respectively,and serum levels of budesonide was diminished (1.0 vs.4.0 for 6β-OH-budesonide,4.2 vs. 10.7 for 16α-OH-prednisolone).Both patients had portosystemic SI (5.7 % and 3.1%) within the range of healthy subjects.CONCLUSION:Serum levels of budesonide Vary uP to 13-fold in AIH Patients with Child A eirrhosis in the absenee ofrelevant Portosystemic shunting.Redueed hePatiemetabolism,as indicated by redueed metabolite-to-drugratio,rather than Portosystemie shunting may explainsystemic side effects of this drug in cirrhosis

Stent placement is effective on both postoperative hepatic arterial pseudoaneurysm and subsequent portal vein stricture:A case report

To treat postoperative bleeding after hepato-pancreato-biliary surgery, interventional radiology has become essential. We report a case of coincidental pseudoan-eurysm and jejunal varices that were both successfully treated by stent-grafts. After a pancreaticoduodenec-tomy, the patient developed a pseudoaneurysm in the hepatic artery and a stenosis in its periphery. After establishing hepatic arterial flow by placing stent-grafts over both the pseudoaneurysm and the stenosis, the pseudoaneurysm was embolized with microcoils. Nine months later, the patient developed jejunal varices caused by a severe stricture in the main trunk of the portal vein. Percutaneous transhepatic portography was performed and stent-grafts were placed over the stenotic segment. A venoplasty using stent-grafts nor-malized the portal blood flow and the jejunal varices vanished. Although stenosis occurred due to scarred tissues from leakage after pancreaticoduodenectomy, stent-grafts were useful for managing jejunal bleeding post-operatively.

Bile duct ligation in rats: A reliable model of hepatorenal syndrome?

The two most widely used experimental models of advanced liver disease are the administration of carbon tetrachloride, and common bile duct ligation (BDL), however, neither has been systematically evaluated as a model of hepatorenal syndrome (HRS). The BDL model in rats, studied at diverse time points, induced a progressive renal dysfunction without structural changes in the kidney. The authors concluded that BDL is a good model for further studies of HRS and its treatment. However, the renal impairment observed at the acute phase of the BDL model is based on a different pathophysiology than that of HRS. Specifi cally, in acute obstructive jaundice, cholemia predominates over parenchymal liver disease (reversible at this stage without portal hypertension or cirrhosis) and independently induces negative inotropic and chronotropic effects on the heart, impaired sympathetic vasoconstriction response and profound natriuresis and diuresis that might lead to volume depletion. In addition, systemic endotoxemia contributes to the prerenal etiology of renal impairment and promotes direct nephrotoxicity and acute tubular necrosis. On the other hand, the renal failure observed in the chronic BDL model (with development of biliary cirrhosis, portal hypertension and ascites) shares pathophysiological similarities with HRS, but the accordance of the chronic BDL model to the diagnostic criteria of HRS (e.g. absence of spontaneous bacterial peritonitis, no renal function improvement after plasma volume expansion) should have been confirmed. In conclusion, we think that the BDL model is not suitable for the study of the natural history of HRS, but the chronic BDL model might be valid for the study of established HRS and its potential therapies.

Evaluation and management of patients with refractory ascites

Some patients with ascites due to liver cirrhosis become no longer responsive to diuretics. Once other causes of ascites such as portal vein thrombosis, malignancy or infection and non-compliance with medications and low sodium diet have been excluded, the diagnosis of refractory ascites can be made based on strict criteria. Patients with refractory ascites have very poor prognosis and therefore referral for consideration for liver transplantation should be initiated. Search for reversible components of the underlying liver pathology should be undertaken and targeted therapy, when available, should be considered. Currently, serial large volume paracentesis （LVP） and transjugular intrahepatic portasystemic stent-shunt （TIPS） are the two mainstay treatment options for refractory ascites. Other treatment options are available but not widely used either because they carry high morlJidity and mortality （most surgical options） rates, or are new interventions that have shown promise but still need further evaluation. In this comprehensive review, we describe the evaluation and management of patients with refractory ascites from the prospective of the practicing physician.

Attenuation of portal hypertension by natural taurine in rats with liver cirrhosis

AIM： To investigate the inhibitory effect of natural taurine （NTau） on portal hypertension （PHT） in rats with experimentally-induced liver cirrhosis （LC）. METHODS： Experimentally-induced LC Wistar rats （20 rats/group） were treated with either oral saline or oral NTau for 6 consecutive weeks. Evaluation parameters included portal venous pressure （PVP）, portal venous resistance （PVR）, portal venous flow （PVF）, splanchnic vascular resistance （SVR） and mean arterial pressure （NAP）. Vasoactive substance levels including nitric oxide （NO）, nitric oxide synthase （NOS） and cyclic guanosine monophosphate （cGMP） were also measured. Histological investigation of type Ⅰ and Ⅲ collagen （COL Ⅰ and Ⅲ） and transforming growth factor-β1 （TGF-β1） was also performed. RESULTS： Treatment with NTau （1） significantly decreased PVP, PVR and PVF, and increased MAP and SVP; （2） markedly increased the vascular compliance and reduced the zero-stress of the portal vein; （3） markedly decreased the amount of NO and cGMP and activity of NOS; and （4） improved the pathological status of the liver tissue and reduced the expression of COL Ⅰ, COL Ⅲ and TGF-β1. CONCLUSION： NTau inhibited the LC-induced PHT by improving hyperdynamic circulation, morphology of liver and biomechanical properties of the portal vein in experimentally-induced LC rats.