Introduction: To date, there is not generally accepted and universal indicator of activity, and functional integrity of the small intestine in patients with coeliac disease. The aim of our study was to investigate whether serum concentrations of the non-essential amino acids citrulline and ornithine might have this function. Methods: We examined serum citrulline and ornithine concentrations in a subgroup of patients with proven coeliac disease and healthy controls (blood donors). Results: A total of 94 patients with coeliac disease (29 men, mean age 53 ± 18 years; 65 women, mean age 44 ± 14 years) and 35 healthy controls (blood donors) in whom coeliac disease was serologically excluded (10 men, mean age 51 ± 14 years; 25 women, mean age 46 ± 12 years) were included in the study. Significantly lower concentrations of serum ornithine were found in patients with coeliac disease (mean 65 ± 3 μmol/L; median 63 μmol/L, IQR 34 μmol/L, p < 0.001). No statistically nor clinically significant differences were found in the citrulline concentrations between the study and control group. Conclusions: Serum ornithine (but not citrulline) may be useful for assessing the functional status of the small intestine in uncomplicated coeliac disease. Further studies involving more detailed analysis of dietary and metabolic changes in patients will be needed to reach definitive conclusions.

Functional hyposplenism is a condition accompanying many diseases including autoimmune disorders and lymphomas. Hyposplenism is also commonly found in adult coeliac disease (up to 20 % of non-complicated and up to 80 % of complicated disease). Hyposplenism is associated with an increased risk of severe infections ( Streptococcus pneumoniae , Neisseria meningitidis and Haemophilus influenzae ). The aim of this prospective study was to investigate memory B lymphocytes as an indirect biomarker of functional hyposplenism. A total of 42 patients with coeliac disease (11 men, 31 women; mean age 49±14 years) and 10 healthy controls, blood donors (2 men, 8 women; mean age 39±7 years) were enrolled into the study. Nobody underwent previous splenectomy and no individual suffered from immunodeficiency. The DuraClone IM panel was used to identify B lymphocytes subpopulations in peripheral blood samples by flow cytometry Navios (Beckman Coulter) with software analysis using Kaluza version 1.2. Patients with coeliac disease and controls did not differ in basic parameters of leukocyte and total lymphocyte blood count. Switched memory B lymphocytes (CD19+CD27+IgD-), non-switched memory / marginal-zone-like B lymphocytes (CD19+CD27+IgD+) and IgM memory B lymphocytes (CD19+CD27+IgM++) were significantly lower in coeliac disease compared to controls. Follicular (naive) B lymphocytes were not significantly different between coeliac disease and controls. In conclusion, dysfunction of memory B lymphocytes can be responsible for an increased risk of severe bacterial infections in coeliac disease. Patients with coeliac disease with dysfunction of memory B lymphocytes are clearly indicated for anti-pneumococcal vaccination.

Variceal bleeding is the most severe life threatening complication of portal hypertension. Now, clear therapeutic algorithms are used, which include initial general management, fluid replacement and hemosubstitution, antibiotic prophylaxis, vasoactive medication and endoscopic treatment. Transjugular intrahepatic portosystemic shunt (TIPS) created using PTFE covered stent is indicated in case of failure. Dedicated oesophageal metal stent or balloon tamponade could be used as a bridge to the TIPS or in cease of TIPS contraindication. Non selective beta-blockers and endoscopic therapy are used in prophylaxis.

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.MATERIALS 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, Chi-square, 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.

Přehledový clanek diskutujici problematiku dispenzarizace nemocných po zavedeni intrahepatalni portosystemove spojky (TIPS) hlavně z hlediska diagnostiky dysfunkce zkratu a možnosti jejiho řeseni.

UVOD: Buddův-Chiariho sy (BCS) ma mnohdy akutni až fulminantni průběh vedouci k ischemii až nekroze jaterniho parenchymu v důsledku venostazy. Obnova drenaže jaternich sinusoid je v takove situaci klicova. Při chronickem průběhu dominuje spise riziko komplikaci portalni hypertenze. TIPS jako portosystemova spojka zajisti jak venozni drenaž, tak korekci portalni hypertenzi. SOUBOR A METODIKA: TIPS je u nas dostupný již 21 let. S jeho pomoci jsme osetřili tež 52 nemocných s BCS na podkladě trombozy jaternich žil. Median věku je 37 let (13-82 let), 10% souboru tvoři děti, 16 nemocných (31%) jsou muži, 31% byl urgentni zakrok. Myeloproliferativni syndrom byl přicinou u 60%, jiný znamý prokoagulacni stav byl identifikovan u 15% a u necele 1/4 se přicinu nepodařilo objasnit. Prvnich 5 let se použivaly nepotažene stenty, od r. 1997 se zacaly uplatňovat různe ePTFE potažene stenty a od r. 2001 jsou standardem dedikovane ePTFE potažene stenty. VÝSLEDKY: Zaznamenali jsme 13 umrti: 3 fulminantni jaterni selhani, 1 extenzivni tumorozni trombozu, 3 casne septicke komplikace, 2 jaterni selhani při pozdějsi akutni okluzi zkratu, 2 v důsledku hematoonkologickeho onemocněni, jedno urazem a jedno z nezname přiciny. Dva nemocni podstoupili transplantaci jater. U 39 nemocných zkrat zůstava při antikoagulacni terapii a obcasných revizich funkcni, jaterni funkce stabilizovane a komplikace portalni hypertenze se neobjevuji. Nezaznamenali klinicky významnou jaterni encefalopatii po TIPS. Použiti potažených stentů výrazně snižilo předevsim výskyt casných okluzi zkratu. Při zobrazovacich vysetřenich lze casto pozorovat nodularni přestavbu. ZAVĚR: TIPS považujeme za velmi výhodnou terapeutickou volbu u BCS na podkladě trombozy jaternich žil. Za předpokladu důsledne nasledne pece TIPS obvykle zajisti potřebnou perfuzi jaterni tkaně a zabrani komplikacim portalni hypertenze, cimž velkou větsinu nemocných usetři transplantace jater.

Ascites a refrakterni ascites je jednou z nejzavažnějsich komplikaci portalni hypertenze a jeho rozvoj je spojen s vysokou umrtnosti. V připadě refrakterity na lecbu zůstava jako lecebna modalita jaterni transplantace, provaděni velkoobjemových paracentez se substituci albuminu, nebo nechirurgicke vytvořeni portokavalniho zkratu - transjugularni intrahepatalni portosystemove spojky. Misto v indikaci TIPS je stale diskutovaným tematem. Autoři přinaseji soucasný nahled na problematiku terapie refrakterniho ascitu i vlastni zkusenosti s touto terapii u 361 nemocných v obdobi polednich dvaceti dvou let.

INTRODUCTION:Liver cirrhosis is associated with hyperdynamic circulation which can result in heart failure. Transjugular intrahepatic portosystemic shunt (TIPS) due to increase of cardiac output is a stressful stimulus for cardiovascular system. Therefore, new methods for early detection of heart failure are needed. Transmitral flow is a marker of diastolic dysfunction. AIM:To analyze short- and long-term effect of TIPS procedure on transmitral flow. MATERIAL AND METHODS:55 patients (38 men and 17 women, 55.6 ± 8.9 years) with liver cirrhosis treated with TIPS were enrolled in the study. Echocardiography was performed before, 24 h, 7, 30 and 180 days after the procedure. During 6 month follow up 22 patients died. Results. Left ventricle end-diastolic diameter was increasing during the follow-up [baseline: 47 (44.7-51.2) mm, day 7: 50 (46.5-51.3) mm, p < 0.05; day 30: 49.5 (46.7-55.2) mm, p < 0.01; 6 months: 52.5 (48.3-55.2) mm, p < 0.01)]. The peak early filling velocity (E) was significantly increasing [before: 75.5 (60.5-87.3) cm/s, 24 h: 88 (74.3-109.7), p < 0.01; day 7: 89 (81.5-105) p < 0.01; 1 month: 94 (82.7-108.5) p < 0.01; 6 month: 91 (80.1-120.2) p < 0.01]. Peak late atrial filling velocity (A) significantly increased within 24 h after the procedure: 85.1 (76.2-99.5) vs. 91.2 (81.5-104.5) cm/s, p < 0.05. The E/A ratio was increasing during the follow up (baseline: 0.88, 24 h after: 0.89, 1 week: 1.0, 30 days: 1.13, 6 month: 1.06 p < 0.01). CONCLUSION:Hemodynamic changes following TIPS procedure can be monitored using echocardiography. Transmitral flow analysis can serve as a useful tool for evaluating of diastolic function in these patients.

Patients with liver cirrhosis have increased risk of diabetes mellitus development, especially when the underlying disease is hereditary hemochromatosis, autoimmune hepatitis, non-alcoholic steatohepatitis or chronic hepatitis C. Patients with associated diabetes according to liver cirrhosis complications have worse prognosis and the therapy is influenced by both diseases. The authors bring short review of particular diseases, diagnosis and treatment strategy.

AIM:To analyze survival of patients after TIPS (transjugular intrahepatic portosystemic shunt).PATIENT SAMPLE AND METHODOLOGY:Between September 1992 and August 2010, TIPS was created in 848 patients of the University Hospital Hradec Kralove. These patients were divided into groups. Survival was analyzed using Kaplan-Meier survival curves. Differences between groups were evaluated using log-rank test.RESULTS:Ten percent of patients do not survive one month after TIPS, 40% of patients survive 5 years and 20% of patients survive 10 years. There were statistically significant differences between groups divided according to Child-Pugh classification (A vs B p = 0.0053; B vs. C p < 0.0001), indication for surgery [prevention of bleeding recurrence differed from refractory ascites (p = 0.0001) and the indication to stop acute bleeding (p = 0.026)]; aetiology of the liver disease [patients with alcoholic cirrhosis differed from patients with Budd-Chiari syndrome (p < 0.0001) and from patients with chronic viral hepatitis (p = 0.024)].CONCLUSION:Survival of patients after TIPS is influenced by Child-Pugh score, indication and aetiology of the liver disease.

PURPOSE:To evaluate the effects of secondary deployment of expanded polytetrafluoroethylene (ePTFE)-covered stent grafts in the treatment of dysfunctional transjugular intrahepatic portosystemic shunts (TIPSs) in comparison with other common approaches (conventional angioplasty or implantation of bare metal stents).MATERIALS AND METHODS:A retrospective review of 121 dysfunctional bare metal TIPS presenting between 2000 and 2004 was conducted. The group was divided into four subgroups according to the type of intervention: conventional angioplasty (52 cases; 43%), bare metal stent deployment (35 cases; 28.9%), nondedicated ePTFE-covered stent-graft deployment (15 cases; 12.4%), and dedicated ePTFE-covered stent-graft deployment (19 cases; 15.7%). In all four groups, the primary patency after the specific intervention was calculated and mutually compared.RESULTS:Primary patency rates after 12 and 24 months were 49.7% and 25.3%, respectively, in conventional angioplasty; 74.9% and 64.9%, respectively, with bare metal stents; 75.2% and 64.5%, respectively, with nondedicated ePTFE-covered stent grafts; and 88.1% and 80.8%, respectively, with dedicated ePTFE-covered stent grafts.CONCLUSIONS:In the treatment of dysfunctional TIPS, better patency after the intervention was obtained by deploying dedicated ePTFE-covered stent grafts in comparison with conventional angioplasty, bare metal stents, and nondedicated ePTFE-covered stents.

PurposePortal hypertension (PH) without thrombosis of hepatic or spleno-portal veins occurs infrequently in patients with myeloproliferative diseases. In those patients PH is related to an increased intrahepatic resistance due to extramedullary hematopoiesis with the myeloid metaplasia and/or to marked portal flow increase, as a consequence of massive splenomegaly. Only a few cases with the use of TIPS for treatment of symptomatic (pre-) sinusoidal intrahepatic PH secondary to myelofibrosis have been published. We present a small series of 6 patients.Materials & Methods6 patients (4 males, age 56 to 82 years) with diagnose of myeloproliferative disease and extramedullary hematopoiesis (4 with idiopathic myelofibrosis, 1 with polycytemia vera, 1 with chronic myeloid leukemia), as a cause of symptomatic PH, were treated by TIPS in our center during last 15 years (from total of 694 TIPS in this period). Prevention of variceal rebleeding in 5 patients (all of them had also ascites) and refractory ascites in 1 patient were indications for TIPS placement. Shunt was created with the primary use of ePTFE stent-graft in 5 patients.ResultsPortosystemic gradient reduction after the TIPS creation from 18.5±3.3 mmHg to 6.4±1.8 mmHg on average was noted. The secondary deployment of ePTFE stent-graft was necessary in the patient with a non-covered stent due to early shunt thrombosis. During other follow up (range from 8 days to 47 months, median 6.5 months) no dysfunction with rebleeding was noted and ascites was completely reabsorbed except 1 patient, which died too early. In the follow up period 3 patients died. The oldest one died because of acute liver failure the 8th day after TIPS creation, with a large right liver lobe infarction and TIPS thrombosis at autopsy. The next two patients died 6 and 47 months after the TIPS due to hematologic disease progression.ConclusionSymptomatic intrahepatic (pre-) sinusoidal PH due to extramedullary hematopoiesis in patients with myelofibrosis secondary to myeloproliferative diseases is rare indication for TIPS creation with a very good effect on prevention of variceal rebleeding and to ascites absorption. PurposePortal hypertension (PH) without thrombosis of hepatic or spleno-portal veins occurs infrequently in patients with myeloproliferative diseases. In those patients PH is related to an increased intrahepatic resistance due to extramedullary hematopoiesis with the myeloid metaplasia and/or to marked portal flow increase, as a consequence of massive splenomegaly. Only a few cases with the use of TIPS for treatment of symptomatic (pre-) sinusoidal intrahepatic PH secondary to myelofibrosis have been published. We present a small series of 6 patients. Portal hypertension (PH) without thrombosis of hepatic or spleno-portal veins occurs infrequently in patients with myeloproliferative diseases. In those patients PH is related to an increased intrahepatic resistance due to extramedullary hematopoiesis with the myeloid metaplasia and/or to marked portal flow increase, as a consequence of massive splenomegaly. Only a few cases with the use of TIPS for treatment of symptomatic (pre-) sinusoidal intrahepatic PH secondary to myelofibrosis have been published. We present a small series of 6 patients. Materials & Methods6 patients (4 males, age 56 to 82 years) with diagnose of myeloproliferative disease and extramedullary hematopoiesis (4 with idiopathic myelofibrosis, 1 with polycytemia vera, 1 with chronic myeloid leukemia), as a cause of symptomatic PH, were treated by TIPS in our center during last 15 years (from total of 694 TIPS in this period). Prevention of variceal rebleeding in 5 patients (all of them had also ascites) and refractory ascites in 1 patient were indications for TIPS placement. Shunt was created with the primary use of ePTFE stent-graft in 5 patients. 6 patients (4 males, age 56 to 82 years) with diagnose of myeloproliferative disease and extramedullary hematopoiesis (4 with idiopathic myelofibrosis, 1 with polycytemia vera, 1 with chronic myeloid leukemia), as a cause of symptomatic PH, were treated by TIPS in our center during last 15 years (from total of 694 TIPS in this period). Prevention of variceal rebleeding in 5 patients (all of them had also ascites) and refractory ascites in 1 patient were indications for TIPS placement. Shunt was created with the primary use of ePTFE stent-graft in 5 patients. ResultsPortosystemic gradient reduction after the TIPS creation from 18.5±3.3 mmHg to 6.4±1.8 mmHg on average was noted. The secondary deployment of ePTFE stent-graft was necessary in the patient with a non-covered stent due to early shunt thrombosis. During other follow up (range from 8 days to 47 months, median 6.5 months) no dysfunction with rebleeding was noted and ascites was completely reabsorbed except 1 patient, which died too early. In the follow up period 3 patients died. The oldest one died because of acute liver failure the 8th day after TIPS creation, with a large right liver lobe infarction and TIPS thrombosis at autopsy. The next two patients died 6 and 47 months after the TIPS due to hematologic disease progression. Portosystemic gradient reduction after the TIPS creation from 18.5±3.3 mmHg to 6.4±1.8 mmHg on average was noted. The secondary deployment of ePTFE stent-graft was necessary in the patient with a non-covered stent due to early shunt thrombosis. During other follow up (range from 8 days to 47 months, median 6.5 months) no dysfunction with rebleeding was noted and ascites was completely reabsorbed except 1 patient, which died too early. In the follow up period 3 patients died. The oldest one died because of acute liver failure the 8th day after TIPS creation, with a large right liver lobe infarction and TIPS thrombosis at autopsy. The next two patients died 6 and 47 months after the TIPS due to hematologic disease progression. ConclusionSymptomatic intrahepatic (pre-) sinusoidal PH due to extramedullary hematopoiesis in patients with myelofibrosis secondary to myeloproliferative diseases is rare indication for TIPS creation with a very good effect on prevention of variceal rebleeding and to ascites absorption. Symptomatic intrahepatic (pre-) sinusoidal PH due to extramedullary hematopoiesis in patients with myelofibrosis secondary to myeloproliferative diseases is rare indication for TIPS creation with a very good effect on prevention of variceal rebleeding and to ascites absorption.