Abstract: Background/Aims: Postparacentesis circulatory dysfunction is the most severe complication of ascites paracentesis. The aim of our study was to compare the standard treatment with the administration of a vasoconstrictor terlipressin. Methodology: Forty-nine patients treated by paracentesis due to tense ascites were randomized for the treatment with albumin (8g/L of removed ascites) or terlipressin (ling every four hours for 48 hours). The blood pressure, heart rate, diuresis, electrocardiograph, standard biochemical and hematological parameters, sodium, potassium and nitrogen urinary excretion, aldosterone and renin activity in the blood plasma were monitored for a period of 72 hours. Results: In any parameter of hemodynamic changes, no statistically significant difference was demonstrated between randomized groups, in particular measurements as well as in the development in the course of the first three days after the intervention. The result suggests similar efficacy of the circulatory dysfunction prevention after the paracentesis in both treatment procedures. In both groups, on the first three days, there was a tendency to improve hemodynamics reflected by the renin-angiotensin-aldosteron system activity. In the terlipressin group, this tendency approached statistically significant levels. Conclusions: The administration of terlipressin in a dose of 1mg every fourth hour performed for a period of 48 hours was as effective as intravenous albumin in preventing hemodynamic changes in patients with tense ascites treated by paracentesis. The treatment was well tolerated.
Abstract: Background & Aims: The clinical symptoms and age at onset of Wilson's disease (WD) are highly variable. This study investigated patients who became symptomatic at >40 years of age. Methods: Clinical features, laboratory data, and mutation analysis were evaluated in 46 (3.8%) of 1223 patients who were investigated in a multinational study on genotype-phenotype correlations (1053 index patients, 170 siblings) who were >40 years of age at onset of symptoms and, in 2 asymptomatic siblings, diagnosed at >40 years of age. Results: Thirty-one patients presented with neurologic symptoms (mean age, 44.5 years; range, 40-52; male/.female, 14/17), 15 presented with liver disease (mean age, 47.1 years; range, 40-58; male/female, 6/9), and 2 were asymptomatic siblings. Hepatic copper content was measured in 17 patients and was above 250 mu g/g dry weight in 13. One patient with hepatic presentation had "fulminant" WD, the remaining 14 abnormal liver function tests and/or hepatomegaly. Liver biopsy specimens were available in 13 patients presenting with liver disease (cirrhosis, 10; chronic hepatitis, 2; steatosis, 1; no abnormalities, 1) and in 14 neurologic patients (cirrhosis, 9; advanced fibrosis, 1; chronic hepatitis, 2; no abnormalities, 2). Twenty-seven of the 46 index cases had mutations on both chromosomes (including 13 H1069Q/H1069Q), 13 on just 1 chromosome. Conclusions: Late-onset WD is a frequently overlooked condition. The diagnostic features and the frequency of late-onset WD gene mutations were not different than in patients with an earlier onset of disease. Factors other than ATP7B mutations may modify the phenotypic presentation of WD.
Abstract: Objective. Bleeding from esophageal varices is a major complication of liver cirrhosis. Non-selective beta-blockers exert an influence on the functional part of portal hypertension, thereby reducing the risk of bleeding. Direct measurement of this functional part is not possible; nevertheless, pro-inflammatory markers as well as parameters of endothelial dysfunction might serve as surrogate markers. The aim of study was to assess the correlation between the therapeutic efficacy of carvedilol and markers of endothelial dysfunction and systemic inflammation in patients with liver cirrhosis and portal hypertension. Material and methods. Thirty-six patients with cirrhosis and portal hypertension were given carvedilol, 25 mg q.i.d. for 30 days. Hepatic venous pressure gradient (HVPG) and biochemical determinations were performed prior to and after the treatment. Eight healthy individuals served as controls for comparison of biochemical markers. Results. In the whole group of cirrhotic patients, HVPG decreased from 17.7 +/- 3.8 to 14.9 +/- 4.8 mmHg (p < 0.001). Complete response was seen in 15 patients (42%). Baseline serum levels of E-selectin were significantly higher in responders than in non-responders (119.89 +/- 70.6 versus 52.69 +/- 25.7 ng/ml; p = 0.023) and in controls (28.89 +/- 22.2 ng/ml; p = 0.004). Furthermore, baseline TNF-alpha a levels were significantly higher in responders than in non-responders (22.8 +/- 15.7 versus 7 +/- 8.9; p = 0.047) and in controls (5.5 +/- 5.9 pg/ml; p = 0.005). Serum levels of ICAM-1 showed the same trend (4360 +/- 2870 versus 2861 +/- 1577 versus 651 +/- 196 ng/ml), although differences did not reach statistical significance. Conclusions. Markers of systemic inflammation and endothelial dysfunction seem to predict the hypotensive effect of carvedilol on portal hypertension in patients with liver cirrhosis and may be useful in the assessment of the efficacy of the therapy.
Abstract: The case is presented of a patient with stenosis of the intrahepatic portion of the inferior vena cava and the terminal part of hepatic veins, a state manifesting itself in ascites resistant to treatment. The case was originally seen as decompensated cirrhosis of the liver. Once properly diagnosed, it was successfully treated with angioplasty and subsequent anticoagulation. After all the relevant hypercoagulation states potentially leading to IVC and hepatic vein stenosis were ruled out, the cause of the patient's stenosis could be traced to: 1. secondary polycythemia concomitant to severe pulmonary obstruction disease and 2. simultaneous corticosteroid therapy. This cause has not been described in literature before.
Abstract: Background/Aims: 1) To compare the effect of 2-day application of 0.2mg terlipressin i.v. every 4 hours (group I) with that of 5-day application of ling ix. every 4 hours (group II)in the treatment of bleeding esophageal varices and portal gastropathy. 2) To assess the incidence of adverse events. Methodology: Eighty-six patients with liver cirrhosis (54 men and 32 women, average age 51 years) were randomized over a period of 2 years into 2 groups. Acute bleeding was diagnosed endoscopically within 24 hours of its onset. The two groups fully comparable;, treatment failure rated according to "Baveno II". Results: Success rate in group I was 78% at day 2 and 75% at day 5; in group II 89% and 79%, respectively (no statistical significance). Rebleeding had occurred by day 5 in 15% in group I, and in 16.3% in group II. Transfusion needs by day 2 were significantly lower in group II (2.4 units compare to 3.4 units in I). The 30-day mortality was 17.1% in group I and 20% in group II. No statistical difference between I and II in the occurrence of adverse events. Conclusions: At a dosage of 1 mg i.v. every 4 hours, the success rate at day 2 was as much as 90% while blood consumption was significantly lower compared with the lower dosage. Rebleeding during first 48 hours occurred almost exclusively at lower dosage. There was no increase in the rate of adverse events relative to the higher dosage.
Abstract: Background. Transjugular renal biopsy (TJRB) is still a novel technique of renal tissue sampling exploiting the transjugular route. TJRB should be performed particularly in situations when the percutaneous route is precluded, i.e. especially in patients with clotting disorders. In the past, only a few papers reported the experience with larger numbers of patients. The goal of this paper is to analyze our experience with TJRB. Methods and Results: From 1993 to 1999, 67 patients, mean age 49.8 years (SD +/- 10.2), male/female ratio 40/27, underwent TJRB. Fifty-two patients (78%) suffered from renal insufficiency and 19 of them (28%) were on dialysis treatment at the time of TJRB. Arterial hypertension was recorded in 42%. The combined kidney and liver biopsy (46%) and clotting disorders (39%) were the most frequent indications for performing TJRB. Renal tissue was yielded in 53 patients (79%) but a sample sufficient for histological diagnosis was taken in 49 (73%), reaching on average 10.8 glomeruli. Altogether 19 different histological entities were disclosed and out of them, vascular nephro-sclerosis (12%), necrotizing and crescentic glomerulonephritis, IgA nephropathy (IgAN) and amyloidosis (three latter per 10%) represented the most frequent diagnoses. TJRB was combined with liver biopsy in 31 patients (46%) and/or hepatic vein catheterization in 22 patients (33%) confirming portal hypertension in 8. The clinically significant liver histology was found in 20 patients, of them cirrhosis/fibrosis in 8, chronic hepatitis in 4 and steatosis in 5. Among those 20 patients, IgAN was disclosed as the most common renal diagnosis (6). Clinically symptomatic complications were recorded in 12 cases (18%) but 9 of them suffered from clotting disorders. Complications included development of subcapsular hematoma in 6 cases, macroscopic hematuria in 4 cases, and hypovolemic hemorrhagic shock in 2. One patient had to undergo surgical treatment. Dividing the patients into a subgroup with or without clotting disorders, the complication rate was 34 vs. 7%. Conclusions: TJRB is a new diagnostic method, which, looking at its indications, facilitates the diagnosis of glomerulopathies in patients who could not be considered for percutaneous renal biopsy, particularly due to clotting disorders. The technical aspect of this procedure plays a fundamental role in the final risk/benefit ratio but if done correctly it involves acceptable risk and is well tolerated. Copyright (C) 2001 S. Karger AG, Basel.
Abstract: Background/Aims: To evaluate the effect of a combination of intraluminal brachytherapy and metallic stent implantation in the treatment of patients with nonresectable biliary tumors. Methodology: Thirty-two patients aged 41-80 years with nonresectable biliary malignancies - Klatskin's tumor (n = 17), gallbladder carcinoma (n = 11) and carcinoma of papilla Vateri (n = 4) - were treated with a combination of intraluminal brachytherapy (source Ir192, high-dose radiation regimen, total dose 30Gy) and metallic stent implantation. Intraluminal brachytherapy and stent insertion (metallic, spiral-Z stent) were performed percutaneously in all patients. Results: The mean survival in patients with Klatskin's tumor was 457 days (range: 64-1186; median: 358 days), in patients with gallbladder carcioma 237 days (range: 92-609; median: 210 days) and in patients with carcinoma of papilla Vateri 850 days (range: 48-1518; median: 1277 days). The rate of 2-year survival in these groups was 27, 0 and 50%, respectively. The survival time differed significantly at the 5% level. The mean time of stent patency was 418, 220 and 850 days, respectively. No complications related directly to intraluminal brachytherapy were observed. Conclusions: Intraluminal brachytherapy combined with stent implantation is a safe method and appears to prolong survival in inoperable patients with Klatskin's tumor and carcinoma of papilla Vateri compared with nontreated patients in previous studies. In contrast no similar effect should be expected in patients with gallbladder carcinoma.
