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A prospective study of hepatic encephalopathy (HE) including neuropsychiatric and psychometric evaluation, electroencephalography, and determination of arterial ammonia levels was performed in 55 cirrhotic patients treated consecutively by transjugular intrahepatic portosystemic shunt (TIPS). The cumulative HE rate increased from 23.6% within the 3-month interval before TIPS to 50. 9% within the first 3-month interval post-TIPS (P = .003). Significant and independent predictors of HE post-TIPS were the presence of HE pre-TIPS and reduced liver function. The cumulative HE rate declined in the second 3-month interval post-TIPS and reached the pre-TIPS level. Chronic forms of HE exceeding grade I were not observed. In a subgroup of 22 nonencephalopathic TIPS patients, the prevalence of subclinical HE did not change after TIPS. Among individual psychometric tests, the block design test gave the highest proportion of pathological results (about 50%), whereas selective reminding gave the lowest (10%-25%). Electroencephalography (EEG) showed a temporary increase of pathological results at 1 month after TIPS, when patients with overt HE (grade I) were included (proportion of 21.1% before vs. 57.1%, P = .005). Arterial ammonia concentration increased from a mean of 94 +/- 26 microgram/dL to 140 +/- 28 microgram/dL at 3 months after TIPS (P < .001). Elevated ammonia levels persisted. TIPS led to a temporary increase of HE incidence within 3 months. The decline of the HE rate beyond 3 months despite a sustained increase of arterial ammonia levels could not entirely be explained by reduction of shunt flow, nor by alteration of liver function. Instead, cerebral adaptation to gut-derived neurotoxins might be anticipated.

Shunt insufficiency due to shunt occlusion or stenosis is frequent after TIPS (about 50% after one year). Controversially discussed is whether Doppler sonography is effective in detecting shunt stenosis or whether regular angiographies are required. The experience with a noninvasive method of surveillance primarily based on Doppler sonography is reported here. 58 patients (35 men, 23 women, mean age 55 years, range 33-82 years) were treated by TIPS because of complications of portal hypertension (43 x gastroesophageal bleeding, 14 x refractory ascites, 1 x venoocclusive disease). Liver cirrhosis (alcoholtoxic etiology in 63%) was present in 55 cases, according to Child-Pugh's classification 23 patients = A, 19 patients = B and 13 patients = C. Within a mean observation period of 14 months, Doppler sonography was performed in three months intervals, endoscopy in six months intervals and angiography only when shunt insufficiency was suspected by Doppler sonography and/or because of clinical events, e.g. recurrent bleeding. Immediately after TIPS, maximal flow velocity and flow volume in the portal vein increased by 116% and 115%, respectively. Three months later, a significant increase of portal vein diameter of about 15% was measured. Shunt flow was initially 2.700 ml/min (one week after TIPS) and decreased progressively by about 30% within the first nine months of follow-up. Correspondingly, angiographically proven shunt insufficiency was present in 22 patients (33 episodes). A total of twelve bleeding episodes recurred in seven patients (rebleeding rate of 16% after one year and 19% after two years). In the remaining 15 patients (68%; 21 episodes) shunt insufficiency could be corrected prior to complications because of detection by Doppler sonography (19x) and endoscopy (2x). Therefore, Doppler sonography is an effective diagnostic tool for the detection of shunt insufficiency and should be performed at three months intervals for at least 18 months. In this context it appears allowable to avoid routine angiographies.

A 65-year-old italian patient developed complicated portal hypertension immediately after orthtopic liver transplantation (OLT) necessitating shunt creation. One to five weeks after OLT, massive ascitic fluid losses of up to 121/day developed. Vascular and major hepatic-parenchymal abnormalities were excluded by duplexsonography, angiography and initial histology, respectively. A peritoneovenous shunt (Denver-shunt) on day 31 after OLT reduced (by about 50%) but did not stop ascitic fluid losses. Furthermore, three variceal bleedings occurred after implantation of the Denver-shunt. Direct portography on day 45 after OLT revealed portal hypertension (pressure gradient of 26 mmHg) requiring the implantation of a transjugular intrahepatic portosystemic stent-shunt (TIPS) leading to a reduction of the pressure gradient to 13 mmHg. Subsequently, ascites resolved within ten days and esophageal varices improved. Liver function parameters normalized inspite of recurrence of HCV infection with detection of HCV RNA in serum already in the fifth week after OLT. During follow-up, histological findings deteriorated from mild changes to extended fibrosis at day 61 after OLT, which might have contributed to the maintenance of portal hypertension. The deterioration of liver histology was accompanied by an improvement/normalization of liver graft function. There was no evidence for additional viral liver infections, e.g. hepatitis B or cytomegalovirus infection. This case illustrates an etiologically unclear syndrome developing directly after OLT and reaffirms the effectiveness of TIPS in the treatment of complicated portal hypertension even after liver transplantation.

History and Clinical Findings: A 55-year-old woman with known primary biliary cirrhosis (PBC) was hospitalized because of increasing dyspnoea. A year before she had for the first time experienced a right-sided pleural effusion which had to be drained every 4 weeks. Physical examination revealed dullness on percussion and greatly decreased breath sounds on auscultation over the entire right thorax. In addition there were signs of moderate ascites and leg oedema., Investigations: Chest radiograph showed a homogeneous shadowing of the right thorax without mediastinal shift. Diagnostic thoracocentesis produced a serous effusion, a transudate on chemical analysis, comparable to the composition of the ascitic fluid. Bacteriological and cytological tests on both fluids were unremarkable., Treatment and Course: The right pleural effusion was presumed to be due to a hydrothorax from the ascites caused by portal hypertension associated with the PBC. Despite continuous diuretic treatment and thoracocentesis with albumin substitution every 3 days there was no improvement and implantation of a transjugular intrahepatic portosystemic stent shunt (TIPSS) was performed. This effectively lowered portal pressure and markedly improved the patient's condition so that further thoracocentesis were no longer necessary. 3 weeks after TIPSS implantation she was discharged in good condition. Radiography 3 weeks later demonstrated continued reduction in the hydrothorax., Conclusion: Hydrothorax is a rare complication of liver cirrhosis. TIPSS implantation can provide lasting resolution and corresponding clinical improvement of a hydrothorax, especially in those conditions which are refractory to diuretic treatment and thoracocentesis.

History and Clinical Findings: A 41-year-old woman, known for 10 month to have polycythaemia vera, developed severe right upper abdominal pain. The abdomen was tense from marked ascites and the liver enlarged by 18 cm in the mid-clavicular line., Investigations: Serum bilirubin was slightly elevated to 2.2mg/dl, liver synthesis being much reduced (recalcifying time minimally 23%, albumin minimally 2.8 g/dl). Doppler sonography detected no flow in the right and middle hepatic veins, indicating Budd-Chiari syndrome. Portal vein flow was diminished., Treatment and Course: Heparin treatment had to be stopped because of heparin-associated type II thrombocytopenia and hirudin was substituted. Attempted lysis with a total of 100 mg r-tPA failed. As the patient's condition deteriorated a TIPSS was implanted to provide portal decompression. Incomplete portal vein thrombosis was demonstrated and worsened during the procedure until nearly complete occlusion. Local lysis treatment for 2 days with urokinase, 50,000-60,000 U/h, and two shunt revisions finally succeeded in completely dissolving the thrombus. Portocaval pressure fell from 32 to 21 mm Hg, and the size and function of the liver became almost normal and the ascites disappeared. Anticoagulation with a coumarin derivative was started and hydrocarbamide again given for recurrent thrombocytosis. The patient remained largely symptom-free one year after TIPSS., Conclusion: This case demonstrates the effectiveness of TIPSS in Budd-Chiari syndrome, even in complicated portal vein thrombosis.

A 69-yr-old man with known carcinoid syndrome treated with octreotide and interferon-alpha 2b developed diarrhea, with six to eight watery to semiliquid stools per day. Diminished stool frequency and increased stool consistency were obtained by treatment with the 5-hydroxytryptamine-3 receptor antagonists ondansetron and tropisetron. Successful alleviation of the diarrhea was also observed with the alpha 2-receptor agonist clonidine. These observations indicate that these classes of drugs should be evaluated in a controlled trial in patients with carcinoid-associated diarrhea.

Background and Aim: Behcet's disease is rare and can cause inflammation in blood vessels throughout the body. Although various studies have been conducted on the possible association between BD and various pathogens such as viruses, the major cause of this disease is still unknown. Our study aimed to evaluate the presence of B19 in Behcet patients and healthy carriers. Materials and Methods: For the current case-control study, we examined 103 samples including 54 males and 49 females, and 40 healthy control samples. At first, all samples were checked by ELISA technique and then, the level of B19 DNA was confirmed by Realtime PCR. Finally, the results of patients were compared to healthy control samples. Results: A wide range of clinical manifestations was observed in the BD patient group. We found statistical differences in the prevalence of B19 IgG between patients and healthy populations (84.46% vs. 55%, respectively). However, the prevalence of B19 IgM was similar between patients and healthy control groups (4.58% vs. 2.5%, respectively). We couldn’t observe any detectable levels of B19 DNA in the patient and healthy carrier groups. Conclusion: Our results failed to establish a relationship between B19 infection and BD development, but such a correlation has been reported. However, there may be an indirect association between genetically susceptible people after a viral infection. [ABSTRACT FROM AUTHOR]

Introduction and aim. Hepatic encephalopathy (HE) is a common complication of transjugular intrahepatic portosystemic shunting (TIPS). It is associated with a reduced quality of life and poor prognosis. The aim of this study was to compare two groups of patients who did and did not develop overt HE after TIPS. We looked for differences between these groups before TIPS. Material and methods. A study of 895 patients was conducted based on a retrospective analysis of clinical data. Data was analyzed using Fisher's exact test, x2, Mann Whitney test, unpaired t-test and logistic regression. After the initial analyses, we have looked at a regression models for the factors associated with development of HE after TIPS. Results. 257 (37.9%) patients developed HE after TIPS. Patients' age, pre-TIPS portal venous pressure, serum creatinine, aspartate transaminase, albumin, presence of diabetes mellitus and etiology of portal hypertension were statistically significantly associated with the occurrence of HE after TIPS (p < 0.01). However, only the age, pre-TIPS portal venous pressure, serum creatinine, presence of diabetes mellitus and etiology of portal hypertension contributed to the regression model. Patients age, serum creatinine, presence of diabetes mellitus and portal vein pressure formed the model describing development of HE after TIPS for a subgroup of patients with refractory ascites. Conclusion. We have identified, using a substantial sample, several factors associated with the development of HE after TIPS. This could be helpful in further research. [ABSTRACT FROM AUTHOR]

Purpose: Hepatic encephalopathy (HE) is a common complication in patients undergoing transjugular intrahepatic portosystemic shunt (TIPS). The objective of this study was to assess the prognostic factors and make risk stratification of post-TIPS HE., Methods: This was a retrospective cohort study consisting of cirrhotic patients who had undergone TIPS creation at our center from November 2015 to August 2020. The baseline characteristics including spleen volume (SVol) and other markers were collected. The univariate and multivariate Cox regression analyses were used to identify independent predictors of post-TIPS overt HE (OHE)., Results: Higher Child-Pugh (CP) score (HR 1.334, 95% CI 1.090-1.632, P = 0.005) and smaller SVol (HR 0.999, 95% CI 0.997-1.000, P = 0.004) were identified as the independent risk factors for post-TIPS OHE. And a time-dependent ROC analysis was used to determine the cutoff values of CP score and SVol, which were respectively 6.5 and 773 cm 3 . Subsequently, the CP-SVol grading system was developed to divide patients into three risk grades according to the above two cutoff values. Kaplan-Meier analysis showed that the cumulative rates of patients free of OHE in Grade 1, 2 and 3 were respectively 96.4% ± 3.5%, 82.1 ± 4.7%, and 59.3% ± 6.4%, which were in descending order (Log rank P < 0.001)., Conclusion: SVol might be a novel marker to predict the prognosis of post-TIPS OHE, and the proposed CP-SVol grading system composed of CP score and SVol achieved a superior predictive performance.

Minimal hepatic encephalopathy (mHE) is diagnosed in patients with severe liver disease but no clinical symptoms of encephalopathy if either neuropsychological or neurophysiological tests indicate cerebral dysfunction and other possible causes of brain dysfunction have been excluded. mHE is characterized by deficits in attention, visuospatial orientation, visuoconstructive abilities and motor function. Accordingly, mHE can be expected to interfere with a subject's working ability, especially in those occupations that require handiwork, and driving ability. Indeed, about 60% of blue-collar workers with mHE have been shown to be assessed as unfit for work compared to only 20% of white-collar workers, and about 50% of patients with mHE have been judged unfit to drive a car in several studies. mHE interferes with a patient's quality of life and is associated with an increased risk of developing overt HE as well as increased mortality. Whether mHE is of importance for cognitive function after liver transplantation has still to be clarified. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Short- and long-term effects of transjugular intrahepatic portosystemic shunt (TIPS) on cerebral blood flow (CBF) in patients with cirrhosis are still unclear. The purpose of this longitudinal study was to explore CBF alteration patterns in cirrhotic patients after TIPS. Thirteen cirrhotic patients (7 male, 6 female, mean age 50.0 ± 9.3 years) underwent arterial-spin labeling (ASL) MRI 1-9 days (median 1 days) before TIPS. Follow-up MR examinations were performed about 1 week (median 6 days), 3 months ( n = 6), 6-9 months ( n = 5) and 12-18 months ( n = 5) after TIPS. CBF, ammonia level, Child-Pugh score, number connection test type A (NCT-A) and digit symbol test (DST) scores were converted into relative values by dividing by his/her pre-TIPS values, and then, compared via one-way analysis of variance (ANOVA). Correlations between the pre- and post-TIPS changes of relative CBF (rCBF) and the changes of relative ammonia (rAmmonia), Child-Pugh (rChild-Pugh), and NCT-A/DST (rNCT-A/rDST) scores were calculated by crossing subjects. Compared with the pre-TIPS level, the global rCBF slightly increased by 10.9 % about 1 week later, then rapidly decreased by 14.2 % 3 months later, and flatly decreased by 17.2 % in 6-9 months and 18.0 % in 12-18 months following TIPS. The changes of 3-month rDST score were slightly correlated with 3-month rCBF rather than 1-week rCBF, ( P < 0.1, FDR-corrected) No difference was found between the pre- and post-TIPS rAmmonia levels, rChild-Pugh and rNCT-A/rDST scores (Post-hoc P > 0.05). CBF measured at different time points after TIPS insertion showed different patterns, indicating varying longitudinal effects of TIPS on CBF. A sharp decline of rCBF was found in the 1 week to 3 months period after insertion, indicating that high event rate of hepatic encephalopathy might relate with the unadaptable CBF in patients undergoing TIPS insertion. [ABSTRACT FROM AUTHOR]

Abstract Behçet’s disease (BD) is a chronic, inflammatory multisystemic condition of unknown aetiology. It is clinically characterized by recurrent orogenital ulcerations and skin eruptions; ocular manifestations; arthritis; vasculitis and in some cases neurological and large vessel involvement. Aetiology has not been defined, but genetic, environmental, viral, bacterial and immunological factors have been proposed as causative agents. Treatment includes colchicine, thalidomide, steroids and immunosuppressive agents and it is based on the severity of systemic manifestations, such as central nervous system involvement, arterial aneurysms and thrombosis of the major veins. Mortality is related to major system involvement. In this article the different clinical features, the multiple faces of BD and a list of currently suspected aetiological factors of the disease are discussed, and treatment modalities summarized. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate predictive parameters for the development of Hepatic Encephalopathy (HE) after Transjugular Intrahepatic Portosystemic Shunt (TIPS) placement and for success of shunt modification in the management of shunt-induced HE. Methods: A retrospective analysis of all patients with TIPS (n = 344) has been performed since 2011 in our university liver center. n = 45 patients with HE after TIPS were compared to n = 48 patients without HE after TIPS (case-control-matching). Of n = 45 patients with TIPS-induced HE, n = 20 patients received a reduction stent (n = 18) or TIPS occlusion (n = 2) and were differentiated into responders (improvement by at least one HE grade according to the West Haven classification) and non-responders (no improvement). Results: Older patient age, increased serum creatinine and elevated International Normalized Ratio (INR) immediately after TIPS placement were independent predictors for the development of HE. In 11/20 patients (responders, 55%) undergoing shunt modification, the HE grade was improved compared with nine non-responders (45%), with no relevant recurrence of refractory ascites or variceal bleeding. A high HE grade after TIPS insertion was the only positive predictor of treatment response (p = 0.019). A total of 10/11 responders (91%) survived the 6 months follow-up after modification but only 6/9 non-responders (67%) survived. Discussion: Older patient age as well as an increased serum creatinine and INR after TIPS are potential predictors for the development of HE. TIPS reduction for the treatment of TIPS-induced HE is safe, with particular benefit for patients with pronounced HE. [ABSTRACT FROM AUTHOR]