Transjugular intrahepatic portosystemic shunt in refractory chylothorax due to liver cirrhosis

A pleural effusion containing chylomicrons is termed chylothorax and results from leakage of lymph fluid into the pleural cavity.We report on the case of a 59-year-old woman with severe dyspnea due to a large chylothorax.She was known to have liver cirrhosis but no ascites.There was no history of trauma,cardiac function was normal and thorough diagnostic work-up did not reveal any signs of malignancy.In summary,no other etiology of the chylothorax than portal hypertension could be found.Therapy with diuretics as well as parenteral feeding failed to relieve symptoms.After a transjugular intrahepatic portosystemic shunt(TIPS) had successfully been placed,pleural effusion decreased considerably.Eight months later,TIPS revision had to be performed because of stenosis,resulting in remission from chylothorax.This case shows that even in the absence of ascites,chylothorax might be caused by portal hypertension and that TIPS can be an effective treatment option.     

Transjugular liver biopsy is safe and diagnostic for patients with congenital bleeding disorders and hepatitis C infection

Summary.  The prevalence of chronic hepatitis C virus (HCV) infection among patients with severe congenital bleeding disorders is as high as 98%. Advances in HCV treatment currently result in sustained virological response rates of ≥50%. Recent recommendations have reaffirmed that liver biopsy, which provides a direct histological assessment of liver inflammation and fibrosis, is still important for accurate diagnosis and therapeutic decision making. Percutaneous liver biopsy is a simple, standardized procedure that can be performed rapidly and relatively inexpensively, and has been safely performed in patients with congenital coagulopathies. However, the safety and efficacy of the transjugular approach (transjugular liver biopsy, TJLB), recommended for patients with acquired coagulopathies, has only been minimally studied in the congenital bleeding diathesis population.
We now report our institutional experience with TJLB in 13 such adult patients (mean age 33 years) with severe/mild haemophilia A/B (10); von Willebrand disease (1); factor V deficiency (1) and factor XIII deficiency (1). Data were collected by retrospective chart review and the TJLB was performed according to institutional protocol as described. Haemostasis prophylaxis was given for 1–5 days. Patients were hospitalized for ≤48 h and all tolerated the procedure without bleeding. Three patients experienced self-limited abdominal discomfort; one episode was accompanied by transient transaminaemia. Diagnostic specimens were obtained from all patients and were instrumental in the therapeutic decision-making process. We suggest that with a co-ordinated multidisciplinary approach to care, TJLB is a safe, effective and potentially cost-effective alternative to the percutaneous approach in the congenital bleeding disorders population.     

Transjugular liver biopsy: a comparison of aspiration and trucut techniques

Abstract: The results of 67 transjugular liver biopsies are described. Two failures were encountered due to inability to pass the needle into acutely angulated hepatic veins. Thirty-four patients underwent a liver aspiration biopsy using a Colapinto needle, while the remainder were biopsied using a trucut needle. The success rate with the Colapinto needle was 68% and with the trucut model, 97%. Capsular perforation occurred in three cases, but without significant morbidity or mortality. It is concluded that the trucut needle biopsy is more reliable than aspiration biopsy, when the transjugular approach is mandated, in obtaining optimal liver tissue for histopathological diagnosis.     

经颈内静脉肝内门体分流术治疗肝硬化门脉高压症56例

目的:探讨经颈内静脉肝内门体分流术 (transjugular intrahepatic portosystemic shunt, TIPS)治疗肝硬化门脉高压症的临床疗效及并发症．方法:肝硬化并发门脉高压症患者56例,行 TIPS术,支架置入前后测量门静脉主干压力, 测定门静脉、脾静脉内径,测定门静脉、分流道血流速度;检测外周血象．结果:TIPS术成功率100％,门脉主干压 (cmH2O)24．5±4．2(P<0．01 vs 术前的45．8± 3．6),门静脉内径及脾静脉内径(cm)分别为 1．28±0．095和0．96±0．064(P<0．01 vs术前的 1．62±0．074和1．28±0．032),门脉主干血流速度(cm／s)48．0±17．6(P<0．01 vs术前的13．2± 3．5),分流道血流速度(cm／s)164．0±58．8．脾功能亢进患者30例,WBC(×109／L),PLT(× 109/L),HGB(×g／L)分别为3．92±0．76,80．74± 16．82,118．20±10．24(P<0．01 vs术前的2．65± 0．58,42．56±12．34,86．52±12．68)．食道胃底静脉曲张,腹水等临床症状明显好转．常见并发症有肝性脑病,支架狭窄及闭塞．结论:TIPS是一种治疗肝硬化门脉高压症的有效方法,他能有效地降低门脉压,控制食道、胃底静脉曲张破裂出血．     

Transjugular liver biopsy is a safe and effective intervention to guide management for patients with a congenital bleeding disorder infected with hepatitis C

The prevalence of hepatitis C virus (HCV) infection in adult patients with a congenital bleeding disorder (CBD) approaches 95% and is a major cause of morbidity and mortality. Histological examination of the liver remains the cornerstone of management decisions in patients without a CBD. The reluctance to perform liver biopsies in patients with a CBD has been a major limitation in the management of these patients. We are currently the only haemophilia centre in Australasia performing liver biopsies in patients with a CBD for the purpose of guiding prognostic and therapeutic decisions. We report here the results of our centre's experience with transjugular liver biopsy (TJLB) in patients with a CBD. An adequate specimen for histological assessment was attained from all of the patients. There were no major complications recorded. Patients were hospitalized for < or = 48 h for haemostasis prophylaxis. The diagnostic specimen obtained from patients was integral in guiding their future management. We suggest that with a coordinated multidisciplinary approach, TJLB can be performed in patients with a CBD.     

Transjugular Intrahepatic Portosystemic Shunt does not affect the efficacy and safety of direct-acting antivirals in patients with advanced cirrhosis: A real-life,case-control study

BackgroundTransjugular Intrahepatic Portosystemic Shunt (TIPS) is a well-established treatment for complications of portal hypertension.AimsTo analyze the impact of TIPS on virologic response and safety profile in patients treated with direct-acting antivirals (DAAs).MethodsWe analyzed data from HCV-positive cirrhotic patients treated with DAAs. Twenty-one patients with previous TIPS placement were compared with 42 matched subjects without TIPS. Logistic regression was used to identify predictors of hepatic function worsening and adverse events.ResultsNo differences were found between the two groups in particular regarding sustained virologic response (92.5 and 97.6% in TIPS vs no-TIPS, p = 0.559). Model for End-stage Liver Disease (MELD) of both TIPS and no-TIPS groups declined from baseline to week 24 of follow-up (from 12.5 ± 3.5 to 10.8 ± 3.4 and from 11.1 ± 3.5 to 10.3 ± 3.4, p = 0.044 and 0.025). There were no differences in adverse event rates. At univariate analysis, age was associated with MELD increase from baseline to week 24 (OR 1.111, 95% CI 1.019-1.211, p = 0.017), and patients with higher baseline MELD developed serious adverse events more frequently (OR 0.815, 95% CI 0.658–1.010, p = 0.062). Patients with or without TIPS did not show differences in transplant-free survival.ConclusionTIPS placement does not affect virologic response and clinical outcome of patients receiving DAAs.     

超声血流参数对肝硬化门脉高压症的诊断价值

目的:研究超声参数对门脉高压的诊断价值.方法:以经颈静脉门体静脉分流术(TIPS) 中导管测量的门体静脉压差≥6.8 cmH2O为诊断PHT(PHT) 的金标准,将25 例肝硬化患者分为试验组(18 例)和对照组(7 例),利用随机双盲法研究两组的超声血流参数,使用单因素方差分析组间差异,利用受试者工作曲线ROC 曲线...     

Outpatient liver biopsy using ultrasound guidance and the Biopty™ gun is safe and cost effective

Background : The need for liver biopsy is increasing with the increasing availability of treatments for liver disease, in particular interferon. For this reason the procedure must be efficient and economical, as well as safe.
Aims : Firstly to assess the safety of outpatient liver biopsy when performed with ultrasound guidance and the 'Biopty™ gun. Secondly, to compare the cost of an outpatient and inpatient procedure.
Methods : Two hundred and fifty consecutive liver biopsies performed over a two year period at St Vincent's Hospital, Melbourne were reviewed retrospectively. Indications, histological findings and complications were recorded.
Results : Seventy per cent of the biopsies were performed as an outpatient procedure. There were no major complications. Four patients experienced prolonged pain post biopsy. The average cost for an outpatient biopsy was 351, the cost of an inpatient (overnight stay) was 690.
Conclusion : Outpatient liver biopsy is safe and cost effective, with minor complications in 1.6%. The routine use of ultrasound guidance and the Biopty™ gun is likely to contribute to these favourable results.     

Needle liver biopsy in thalassaemia: analyses of diagnostic accuracy and safety in 1184 consecutive biopsies

We report the reliability and safety of percutaneous liver biopsy in the evaluation of hepatic iron loading and histology in patients with homozygous β-thalassaemia prior to and in serial biopsies following allogeneic bone marrow transplantation for this disorder. 501 thalassaemic patients aged 11 ± 4.5 years (range 1-32 years) underwent 1184 consecutive percutaneous liver biopsies without ultrasound guidance. Overall, 81% of biopsies were evaluable for histological examination and grading of iron. The adequacy of liver biopsy specimens increased with patient age: evaluable specimens were obtained in 73% of patients <5 years of age and in 86% of samples in patients aged >15 years. The degree of iron overload and fibrosis in each biopsy was reported separately by at least two pathologists who did not know the clinical status of each patient. In 103 biopsies, iron grade by light microscopy corresponded to an iron concentration varying between a mean of 32.46 ± 14 μmol/g dry weight liver tissue for iron stores graded by light microscopy as absent to 417.6 ± 150μmol/g dry weight liver tissue for stores graded as severe. The fibrosis score of multiple samples of liver obtained at autopsy within 100 d of the percutaneous biopsy in 41 patients who died following BMT correlated perfectly with that of the first sample in >60% biopsies; in most of the discordant cases fibrosis had been underestimated in the percutaneous biopsy. Liver biopsy demonstrated evidence of chronic hepatitis in 30% of patients with normal transaminase and in 57% of patients with transaminase within twice the normal range. Liver biopsy was complicated in six patients (0.5%) by haemoperitoneum, periocholecystic haematoma, kidney haematoma, or bile peritonitis; no complication was fatal. These data demonstrate that percutaneous liver biopsy provides reliable information regarding liver iron and histology in homozygous β-thalassaemia with an extremely low risk of complications.     

The effect of endothelin and its antagonist Bosentan on hemodynamics and microvascular exchange in cirrhotic rat liver

Background/Aims: To characterize the effects of endothelin-1 and of Bosentan, a mixed endothelin antagonist, on hepatic hemodynamics in cirrhotic animals in vivo and on hepatic microvascular exchange in the perfused rat liver.Methods: Biliary cirrhosis was induced by bile duct ligation, and micronodular cirrhosis by chronic exposure to phenobarbital/CCl₄ in male rats. Hepatic hemodynamics were studied under basal conditions and after administration of Bosentan (3–30 mg/kg) by the microsphere technique. Microvascular exchange was assessed in the in situ perfused rat liver by the multiple indicator dilution technique.Results: Bosentan lowered portal pressure in a dose-dependent fashion; at the highest dose tested, this decrease averaged −29±11 and −26±8% in biliary and micronodular cirrhosis, respectively (p<0.01). This was achieved mainly via a marked decrease in hepatic arterial flow. In the perfused liver, endothelin-1 induced a dose-dependent vasoconstriction; up to 10⁻⁹ mol/l; this was not associated with any effect on viability. At this dose, endothelin-1 markedly decreased extravascular albumin space in both controls and micronodular cirrhosis; this could be antagonized by Bosentan 10⁻⁵ mol/l.Conclusions: Endothelin-1 affects hepatic microvascular exchange, presumably by a direct effect on hepatic sinusoidal endothelial cells. A mixed endothelin antagonist lowers portal pressure in vivo, presumably by acting on hepatic stellate cells, and counteracts the microvascular effects of endothelin-1 in vitro. These properties could prove useful in treatment of portal hypertension.     

Nodular regenerative hyperplasia of the liver diagnosed by needle biopsy of the liver: Report of a case and review of the literature

The authors report the case of a 45 year old woman in whom the diagnosis of nodular regenerative hyperplasia of the liver was made by needle biopsy of the liver, laparoscopy and hepatic venography. The patient did not have any underlying disorders and had not ingested any of the many drugs and chemicals which may produce the lesion. Clinical manifestations were hepatosplenomegaly, portal hypertension and iron deficiency anaemia, and her condition remains unchanged 5 years after diagnosis.     

Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries

Background and Aim:  A major challenge in neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non-atretic causes. In developing countries there are considerable problems of late referral of NC cases and performing surgery without prelaparotomy liver biopsy that contributes to a high proportion of negative laparotomy and increased morbidity. We evaluated the hepatic histopathology for presence of features that correlate best with the diagnosis of BA and assessed the accuracy of percutaneous liver biopsy.
Methods:  Fifty-five cases of NC that fulfilled the selection criteria and had liver biopsy available were analyzed. Among the 49 adequate liver biopsies, 28 cases were diagnosed as BA, 15 neonatal hepatitis (NH) and 6 were due to other causes. Validity of percutaneous liver biopsy diagnoses was compared with confirmed cases by laparotomy findings and 1-year follow up. Twelve histological parameters of confirmed cases of BA and NH were evaluated by logistic regression analyses.
Results:  Ductular proliferation ( P  = 0.0002), bile duct and ductular bile plugs ( P  = 0.009), and portal fibrosis ( P  = 0.002) were the best indicators of BA and among them ductular proliferation was the most important in distinguishing BA from NH. Ductal plate malformation was observed in 17.9% cases of BA. Sensitivity and specificity of percutaneous liver biopsy for diagnosing BA was 88.2% each.
Conclusion:  Percutaneous liver biopsy is highly accurate (88.2%) in diagnosing BA. In developing countries. This investigation should be done to decrease the frequency of negative laparotomy and to achieve cost–benefit with reduced morbidity.     

Role of polymerase chain reaction and liver biopsy in the evaluation of patients with asymptomatic transaminitis: implications in diagnostic approach

BACKGROUND AND AIM: Detection of an asymptomatic rise in the hepatic aminotransferase (ARHA) value has become a distinct and frequent clinical problem. We evaluated a three-step diagnostic algorithm in such patients for maximum yield. METHODS: Consecutive patients with an ARHA value 1.5-fold the upper limit of normal for at least 4 weeks and who were apparently healthy were included in the study. Each patient underwent standard biochemical investigations and a stepwise investigative protocol. In the first step, serological markers for hepatitis viruses, serum ferritin, 24-h urinary copper, alpha-1-antitrypsin phenotyping, and autoimmune markers were carried out. In step two, patients who tested negative for all the above markers had polymerase chain reaction (PCR) analysis for hepatitis B virus (HBV)-DNA and hepatitis C virus (HCV)-RNA. Patients without a diagnosis despite the above investigations underwent a liver biopsy as part of step three. RESULTS: Of 105 patients with ARHA, 38 were excluded for various reasons and 67 were included for the final analysis. The mean age was 35.11 +/- 11.96 years and 56 patients were men. The mean body mass index was 24.17 +/- 3.2 kg/m(2). The stepwise diagnostic algorithm achieved a diagnosis in 65/67 (97%) patients. Non-alcoholic steatohepatitis (NASH) and chronic viral hepatitis were the most common diagnoses, in 24 (36%) patients each. Using the diagnostic algorithm a diagnosis was reached in 34% of patients with only serological and biochemical investigations, whereas PCR for HBV and HCV could further detect the presence of active HBV or HCV viremia in 21% (14/97) and a liver biopsy was necessary to establish the diagnosis in 28/67 (42%) patients. CONCLUSIONS: A stepwise diagnostic algorithm in patients with ARHA resulted in an optimal use of PCR and invasive tests such as liver biopsy. Cryptic HBV and HCV infection was frequent among these patients and PCR was necessary in such cases. NASH and chronic viral hepatitis were the most frequent causes of ARHA.     

奥曲肽联合普萘洛尔治疗肝细胞癌并发门脉高压症患者门静脉血流动力学的变化

目的 观察奥曲肽联合普萘洛尔对原发性肝癌（PLC）并发门脉高压（PHT）患者相关静脉血流量和门静脉血流动力学指标的影响。方法 将纳入研究的84例PLC并发PHT患者随机分为观察组44例和对照组40例,给予观察组患者奥曲肽和普萘洛尔,给予对照组普萘洛尔治疗,3天后观察脾静脉、门静脉、肝静脉血流量和门静脉血管内径、平均血流速度（TAV）、血流量（BF）]的变化。结果 治疗后,观察组门静脉血流量为（712.55±83.76）ml/min,显著低于对照组【（826.53±106.92）ml/min,P<0.05】,脾静脉血流量为（273.45±43.17） ml/min,显著低于对照组【（332.31±53.93）ml/min,P<0.05】,肝静脉血流量为（1232.17±792.63）ml/min,与对照组【（1195.65±801.37）ml/min,P>0.05】比,无统计学差异;治疗后,观察组门静脉直径为（10.88±0.31）mm,显著小于对照组【（11.48±0.59）mm,P<0.05】,PVP为（2.36±0.64）kPa,显著低于对照组【（2.89±0.66）kPa,P<0.05】,TAV为（14.93±2.56）cm/s,显著快于对照组【（13.75±2.35）cm/s,P<0.05】。结论 奥曲肽和普萘洛尔联合应用能更好地控制PLC并发PHT患者的门静脉高压症。     

Distinctive aspects of peptic ulcer disease,Dieulafoy's lesion,and Mallory-Weiss syndrome in patients with advanced alcoholic liver disease or cirrhosis

AIM: To systematically review the data on distinctive aspects of peptic ulcer disease (PUD), Dieulafoy’s lesion (DL), and Mallory-Weiss syndrome (MWS) in patients with advanced alcoholic liver disease (aALD), including alcoholic hepatitis or alcoholic cirrhosis. METHODS: Computerized literature search performed via PubMed using the following medical subject heading terms and keywords: “alcoholic liver disease”, “alcoholic hepatitis”,“ alcoholic cirrhosis”, “cirrhosis”, “liver disease”, “upper gastrointestinal bleeding”, “non-variceal upper gastrointestinal bleeding”, “PUD”, ‘‘DL’’, ‘‘Mallory-Weiss tear”, and “MWS’’. RESULTS: While the majority of acute gastrointestinal (GI) bleeding with aALD is related to portal hypertension, about 30%-40% of acute GI bleeding in patients with aALD is unrelated to portal hypertension. Such bleeding constitutes an important complication of aALD because of its frequency, severity, and associated mortality. Patients with cirrhosis have a markedly increased risk of PUD, which further increases with the progression of cirrhosis. Patients with cirrhosis or aALD and peptic ulcer bleeding (PUB) have worse clinical outcomes than other patients with PUB, including uncontrolled bleeding, rebleeding, and mortality. Alcohol consumption, nonsteroidal anti-inflammatory drug use, and portal hypertension may have a pathogenic role in the development of PUD in patients with aALD. Limited data suggest that Helicobacter pylori does not play a significant role in the pathogenesis of PUD in most cirrhotic patients. The frequency of bleeding from DL appears to be increased in patients with aALD. DL may be associated with an especially high mortality in these patients. MWS is strongly associated with heavy alcohol consumption from binge drinking or chronic alcoholism, and is associated with aALD. Patients with aALD have more severe MWS bleeding and are more likely to rebleed when compared to non-cirrhotics. Pre-endoscopic management of acute GI bleeding in patients with aALD unrelated to portal hypertension is similar to the management of aALD patients with GI bleeding from portal hypertension, because clinical distinction before endoscopy is difficult. Most patients require intensive care unit admission and attention to avoid over-transfusion, to correct electrolyte abnormalities and coagulopathies, and to administer antibiotic prophylaxis. Alcoholics should receive thiamine and be closely monitored for symptoms of alcohol withdrawal. Prompt endoscopy, after initial resuscitation, is essential to diagnose and appropriately treat these patients. Generally, the same endoscopic hemostatic techniques are used in patients bleeding from PUD, DL, or MWS in patients with aALD as in the general population. CONCLUSION: Nonvariceal upper GI bleeding in patients with aALD has clinically important differences from that in the general population without aALD, including: more frequent and more severe bleeding from PUD, DL, or MWS.     

Total area of spontaneous portosystemic shunts independently predicts hepatic encephalopathy and mortality in liver cirrhosis

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