[Liver transplant indication and waiting list inclusion]. / Indikace k transplantaci jater a zarazování na cekací listinu.

The authors have presented an overview of indications and inclusion of patients to the liver transplant waiting list in the Brno transplant centre over a period of 30 years from the first liver transplant performed in the Czech Republic (the then Czechoslovakia). The first clinical liver transplant in the Czech Republic was performed on 2 February 1983. Indication for the transplant was a malignant liver tumour. For a period of 30 years (from 2 February 1983 to 15 January 2013) a total of 592 patients were placed on the waiting list and a total of 453 liver transplants were performed. An urgent liver transplant was carried out in 32 patients over the same period of time.

[Liver transplant outcomes in Brno]. / Výsledky transplantací jater v Brne

The first liver transplant in the former Czechoslovakia took place in Brno on 2 February 1983; the patient concerned has lived for more than 30 years with a normally functioning liver and is one of the longest surviving patients after a liver transplant in Europe. The Cardiovascular and Transplantation Surgery Centre (CTSC) in Brno has experienced an increased development in the area of liver transplants since the mid  1990s. At present, about 30 patients a year undergo a transplantation, with 451 liver transplants in total as of 31 December 2012. The primary indication concerns liver cirrhosis of various etiologies, with an increasing number of cases of cirrhosis resulting from hepatitis C. Urgent liver transplants (for acute liver failure or primary dysfunction of first liver graft) amount to 11% of cases. There were 18 retransplants performed as of 31 December 2012, with 50% five year survival. The primary graft dysfunction was present in 7 patients (i.e. 1.5%). The 1 year survival rate of all patients after a liver transplant performed in CTSC is 92%, 5 year survival rate is 80%, and 10 year survival rate is 71%. Currently the parameters such as recipients age, donors age, and transplant waiting time has been statistically increasing; small recipients with a body weight below 70 kg especially have to wait for a significantly longer period of time (waiting median of 178 days). In CTSC Brno 14 combined simultaneous transplants (13 transplants of liver + kidney and one combined simultaneous transplants of liver + heart + kidney in 2005) have been performed as of 31 December 2012.

[Interventional radiology options to address certain complications of the orthotopic liver transplant]. / Moznosti intervencní radiologie pri resení ne-kte-rých komplikací ortotopické transplantace jater.

At the clinic of imaging method St. Annas University Hospital solved complications arising after ortotopic liver transplantation. For more than 15 years of cooperation with CKTCH Brno intervention was performed on both the arterial and venous system, but most on the biliary tract. The order was a unit patients, which correlates with other comparable work. In the years 1998- 2013 we conducted one intervention on arterial bed, 3× intervention in hepatic venous system and we solved biliary complications in 7 transplant. .

[Liver transplants in viral hepatitides]. / Transplantace jater u virových hepatitid.

The authors present their own results of liver transplants over the last 30 years (from 2 February 1983 to 31 December 2012). For the respective period, 451 patients underwent a transplant in the Brno Transplantation Centre. Ten out of 451 patients (2.2%) underwent transplants for the indication of cirrhosis caused by viral hepatitis B in the Brno Transplantation Centre. The first liver transplant indicated due to viral hepatitis B was performed in 1994. Out of the ten patients, 3 died (30%). Forty  one out of 451 patients (9%) underwent liver transplant for cirrhosis caused by viral hepatitis C. The first liver transplant indicated due to viral hepatitis B was performed in 1991. A total of 21 patients out of 41 (51%) were treated with antiviral therapy; 27 out of 41 (66%) developed a recurrence after the liver transplant. Out of the 41 patients who underwent a liver transplant for viral hepatitis C indication, a total of 15 (36.6%) died.

[Liver transplants in tumours and alcoholic cirrhoses]. / Transplantace jater u tumoru a alkoholických cirhóz.

The treatment of hepatocellular carcinoma requires a multidisciplinary approach; liver transplant is suitable only in the minority of strictly selected patients. The CSTC Brno applies the so  called Milan criteria. As of 31 December 2012 the CSTC Brno performed liver transplants in 16 patients with hepatocellular carcinoma in cirrhosis, with a five year survival rate of 40% and a 10 year survival after transplant of 20%. It is a paradox that the longest living (30 years) patient of CSTC Brno underwent the transplant for a large fibrolamellar hepatocellular carcinoma, which emphasises the prognostic significance of the tumour histology -  criterion taken into account for practical reasons only in some indication schemes. Liver transplant for cholangiocarcinoma is no longer being carried out in CSTC. Five patients underwent this operation in the past and the longest living one survived for four years after the transplant. Benign liver tumours (adenomatosis, cystadenoma, hemangioma with compression symptoms) are rather rare indications and transplant results are positive. Four patients were transplanted for liver carcinoid, with one patient experiencing recurrence. The most frequent compact indication for liver transplant in CSTC Brno concerns alcoholic cirrhosis (24%, 108 patients); the survival rate of these patients after transplant is very good (81% 5 year survival and 68% 10 year survival). Likewise, efforts are made to select patients with a low risk of alcohol abuse after the transplant, which is, however, not very successful. The recurrence of some kind of alcohol abuse occurred in 26% transplanted patients, while seven died of alcoholic cirrhosis of the graft.

[Liver function assessment in oncology practice]. / Hodnocení jaterní funkce v onkologické praxi.

The overall condition and prognosis of a patient can be affected by impaired liver function. It applies to anticancer pharmacotherapy, liver surgery and radiological interventions. The liver condition is usually assessed by common laboratory tests and clinical examination in daily practice. Liver tests consist of aminotransferases - alanine transaminase, aspartate transaminase, bilirubin, alkaline phosphatase, gamma glutamyl transpeptidase, lactate dehydrogenase, albumin and prothrombin time, less frequently prealbumin and cholinesterase. The alkaline phosphatase and aspartate transaminase are markers of a liver damage, the alkaline phosphatase and gamma glutamyl transpeptidase are most useful as markers for cholestatic liver injury. Albumin, prealbumin, cholinesterase and prothrombin time are the markers of synthetic liver function. Bilirubin and bile acids are related to the liver transport and excretory capacity. The Child-Pugh score is used to assess prognosis of chronic liver disease, mainly cirrhosis. The examination of liver function using indocyanine green helps to determinate the extent of possible liver resection. A mathematical analysis of dynamic cholescintigraphy and a calculation of hepatic extraction fraction enables quantification of liver function. Other liver function tests are of little use in oncology.

[The influence of some factors on presence of varices and variceal bleeding in liver cirrhosis patients]. / Faktory ovlivnující pritomnost varixu a varikóznnho krvácení u pacientu s jaterní cirhózou.

STUDY AIM: The aim of the study was to identify non-invasive investigations that would provide a sufficiently reliable prediction of the presence of varices in patients with liver cirrhosis (LC) and identification of patients in a risk of variceal bleeding. PATIENT SAMPLE AND METHODOLOGY: As part of a prospective evaluation, 165 patients with alcoholic LC admitted to the Department of Internal Hepatology and Gastroenterology of the Medical School, Masaryk University in Brno between 2007-2009 were divided according to the presence of varices and variceal bleeding into three groups: I. patients with LC and varices with no variceal bleeding (N = 50), II. patients with LC with no varices and thus no variceal bleeding (N = 51), III. patients with LC and varices with confirmed variceal bleeding (N = 64). A statistical evaluation was performed of a range of haematological and biochemical parameters, spleen size and a thrombocyte, leucocyte and erythrocyte count to spleen size ratio. RESULTS: Patients with varices (groups I and III) had, compared to patients with no varices (group II), a significantly larger spleen, lower thrombocyte count, lower plasma fibrinogen level, extended prothrombin time (higher INR), lower plasma GGT activity and lower erythrocyte, leucocyte and thrombocyte count to spleen size ratio. In the present cohort, statistically significant factors for the presence of variceal bleeding included lower erythrocyte count and lower level of haemoglobin, i.e. the expected effect of bleeding, lower fibrinogen serum levels, lower serum ALP activity, lower total serum protein and serum albumin, higher plasma levels of uric acid and lower leucocyte or erythrocyte count to spleen size ratio. CONCLUSION: Using common haematological and biochemical parameters together with spleen size enables, even without endoscopic examination, highly reliable prediction of the presence of varices and identification of patients in a high risk of variceal bleeding. When these non-invasive methods are used, it is possible to eliminate the need for the invasive endoscopic examination that is frequently poorly tolerated by the patients and economically demanding.

[Immunoglobulin G4-associated cholangitis]. / Imunoglobulin G4 pozitivní cholangitida.

Autoimmune LgG4- associated cholangitis is a new entity among the liver and biliary tree disorders, classified among the so-called IgG4-related diseases. Even though prognosis of this disease is unclear, this type of sclerosing cholangitis is not being linked to a carcinoma. Clinical and laboratory data differ slightly from the findings associated with the usual primary sclerosing cholangitis and it is mainly the high IgG4 level and hyperbilirubinaemia that supports the diagnosis ofautoimmune disease. Unlike primary sclerosing cholangitis, this disease is not associated with a malignant prognosis and steroids represent an effective treatment. Combination of steroids with azathioprin is a possible alternative in case of a relapse. Patient's response to steroid therapy is a diagnosis-supporting criterion. This disease should always be considered as part of differential diagnosis of primary sclerosing cholangitis, especially when autoimmune aberrations or other autoimmune diseases are present. Long-term evaluations of these patients are so far lacking and thus studies on larger patient samples are required.

[The case of familial adenomatous polyposis and a proposal for the system of dispensarisation]. / Prípad familiární adenomatózni polypózy a návrh systému dispenzarizace.

We present a case of a 46 years old female with familial adenomatous polyposis of the colon. The adenocarcinoma had been treated using all available oncology therapeutic modalities. Late post-radiation changes caused ileus that required acute surgical revision, and formation of difficult-to-define enterovesical fistula that led to recurring urosepsis. When the conservative methods, including enteral and, subsequently, home total parenteral nutrition, were unsuccessful, the patient underwent successful surgery with resection of the affected intestinal loops, part of the bladder, evacuation of an abscess in the small pelvis and terminal jejunostomy. As a result of this procedure, the patient is now able to take food per os without infectious complications. The resulting short bowel syndrome is managed by administration of additional parenteral nutrition and registration of the patient for intestinal transplantation is being considered.

[Ascites in Mulibrey syndrome]. / Ascites u Mulibrev syndromu.

The authors describe a case of 22 years old adult male with Mulibrey syndrome. This is an autosomal recessive hereditary disease that manifests through multiple malformations. Diagnosis of Mulibrey syndrome in the present case was first based on clinical signs (facial dysmorphia, growth disorder, muscle hypotrophy) and was later confirmed by genetic examination. At the age of 18 months, the patient underwent surgery for Wilms' tumour followed by cytostatic therapy. Facial, neck and lower extremities oedemas started to occur from the age of 11 years when diastolic ventricular dysfunction was also diagnosed. Pericardiectomy was performed at the age of 13 with no significant clinical effect. Significant ascites dominated the clinical picture and required repeated paracentesis at the age of 15 years. Subjective complaints improved when adequate diuretic therapy was introduced and ascites was managed with conservative therapy without the need for further paracentesis.

[Bacterial infection and its relation to the genesis and course of varicose hemorrhage]. / Bakteriální infekce ve vztahu ke vzniku a prubehu varikózního krvácení.

Acute hemorrhage from esophageal varices due to portal hypertension is a frequent and serious complication of liver cirrhosis. Bacterial infection may be one of the factors influencing such hemorrhage. Endotoxins may increase portal tension and at the same time result in primary hemostasis disorder, thus becoming one of the causes of hemorrhage. The authors of the paper compared the incidence of bacterial infection in 53 patients with varicose hemorrhage due to portal hypertension with 62 patients with liver cirrhosis and portal hypertension without varicose hemorrhage. At least one pathogen was found in considerable 61.1% of the total of patients in the liver cirrhosis group, while the difference between the two groups was but insignificant. No statistically significant difference was found between the group of patients with hemorrhage and those without hemorrhage in terms of presence of bacterial infection in hemoculture, urine, throat, faeces and ascites, nor was there a difference in the etiology of the G+ bacteria, G- bacteria or fungi and yeast infectious agents in the hemoculture, urine, throat, faeces and ascites in either of the groups. No statistically significant difference was found in comparing the patients with a recurrence of hemorrhage (or with mortality) and with infection with those without recurrence of hemorrhage. Bacterial infection was more often found in patients with a recurrence of hemorrhage (75%) as compared with those without any recurrence (52%), and also in patients who died bacterial infection was proven more often than in those who survived (61.9% vs. 58.1%, respectively). There was no difference in morbidity or recurrence of hemorrhage between the patients treated with norfloxacin and ampicilin/sulbactam. No statistically significant difference was recorded between the 1st and 5th day in terms of decrease in bacterial infection. A significant difference was found in the urine etiological agent, where a significant increase in the share of fungal and yeast urine infection (p = 0.011) was recorded after the application of the therapy, as well as a drop in urine infection caused by the G- bacterial agent (p = 0.057).

Bacterial infection and acute bleeding from upper gastrointestinal tract in patients with liver cirrhosis.

BACKGROUND/AIMS: Acute bleeding from the upper gastrointestinal tract is a common and serious complication of liver cirrhosis. It is believed that bacterial infection may be the immediate cause of the bleeding and the latest meta-analyses show that bacterial infection is an independent predictive factor of the failure to stop bleeding. METHODOLOGY: The authors evaluated the presence of bacterial infection (blood, urine, throat and ascitic fluid) in 35 consecutive patients with liver cirrhosis and acute bleeding with portal hypertension and compared these results with a group of 35 patients with liver cirrhosis with portal hypertension without acute bleeding. RESULTS: According to the results obtained, there is a statistically higher incidence of bacterial infection among patients with acute bleeding with portal hypertension (25 of 35 patients, 71%) than among patients with liver cirrhosis and portal hypertension without acute bleeding (14 of 35 patients, 40%, p < 0.01). The incidence of bacteriological findings in blood and throat samples is statistically higher in patients with acute bleeding as opposed to the control group (p < 0.05). CONCLUSIONS: These results confirm the necessity of administering antibiotic prophylaxis to all cirrhotic patients with variceal bleeding, not just to those with confirmed infection or symptoms thereof.

Efficacy and safety of chronic hepatitis C treatment in hemophilic patients.

BACKGROUND/AIMS: Chronic hepatitis C infection is very common among hemophiliacs in the developed World. METHODOLOGY: Retrospective evaluation of the treatment results in hemophiliacs with chronic hepatitis C, all infected with genotype 1b. Twelve patients were treated with interferon-alpha monotherapy, 21 patients with interferon-alpha and ribavirin, and 3 patients with pegylated interferon and ribavirin, all for 48 weeks. RESULTS: Sustained virologic response (defined as an undetectable serum HCV RNA level 24 weeks after treatment was completed) was not achieved in any of 12 patients treated with interferon-alpha alone. Combination therapy with interferon-alpha and ribavirin was associated with better results: 4/10 (40%) patients still untreated with interferon-alpha, 2/4 (50%) relapsers, and 2/7 (29%) non-responders to previous interferon-alpha monotherapy achieved sustained virologic responses. Combination therapy with pegylated interferon and ribavirin has been used in 3 patients. Sustained response was achieved in one patient who had relapsed after treatment with interferon-alpha and ribavirin and in 1 of 2 non-responders to this combination therapy. There were no serious adverse events and it was not necessary to reduce dosages or even cease therapy prematurely. CONCLUSIONS: The efficacy and tolerability of antiviral treatment in hemophiliacs did not differ from other patients with chronic hepatitis C.

[Acute oesophageal necrosis]. / Akutní nekróza jícnu.

The case of acute oesophageal necrosis in 56-year-old patient with tumor dissemination is presented. Acute endoscopy was performed after several hours lasting haematemesis and melena. Endoscopy revealed acute oesophageal necrosis in more than 2/3 of the oesophagus. Patient was treated with proton pump blockers and the total parenteral sustenance was introduced. Three days after the hospital admission the patient died in the septical metabolic disorder resulting from a disseminated spinocellular carcinoma of cervix uteri. Autopsy confirmed necrosis of oesophageal mucosa penetrating into the muscularis mucosae. Acute oesophageal necrosis is a rare disease, which is characteristic by the endoscopic finding of "black oesophagus", histological image of mucous membrane necrosis and by unknown aetiology. The article gives an overview of contemporary knowledge on the clinical, endoscopic and histologic images. The finding of "black oesophagus", which has been reported by several endoscopic units, has highly heterogeneous origin and it represents 0.0125 % (Moreto) till 0.28 % (Augusto) of cases. Our department has recorded 2 cases from 24271 of endoscopies performed in the last 8 years.

Hepatitis D.

Hepatitis D virus (HDV) is a small, RNA-containing virus that requires the concomitant presence of Hepatitis B virus (HBV) in an obligate manner for its survival and pathogenicity. HDV infection is very uncommon in Czech Republic. The results of antiviral therapy of hepatitis D patients are not satisfactory. Alpha-interferon (alpha-IFN) in high doses (9-10 MU three times a week for 12 months) is usually recommended.

[Atypital localisation of pyoderma gangraenosum in patient with ulcerative colitis]. / Atypická lokalizace pyoderma gangraenosum u nemocného s ulcerózní kolitidou.

The authors present a case of 61-year-old man with ulcerative colitis and with extraintestinal manifestation of the disease in the form of pyoderma gangraenosum. Multiple skin defects, which developed in atypical localisation (extensive affection of facial and hairy parts of the head) in patient with chronically active form of ulcerative colitis were complicated with bacterial contamination of methicilin-resistant strains of Staphylococcus aureus. After application of the parenteral feeding, corticotherapy and targeted antibiotic therapy the subjective and objective status of the patient markedly improved, stool frequency was reduced, admixture of blood in the stool disappeared, temperatures fell back and there was a decrease in activity of non-specific bowel inflammation in laboratory findings. However endoscopic examination of the intestine confirmed the finding of chronically active ulcerative colitis with ulcerations and bridging polyps. Patient was indicated to total colectomy, but he refused it.

Treatment of chronic hepatitis C in hemophilic patients.

Chronic hepatitis C infection is common among hemophiliacs in all the developed countries. Since 1996, only alpha-interferon (alpha-IFN) in monotherapy has been used for the treatment of chronic hepatitis C in hemophiliacs (6 patients). In Czech Republic a combination therapy with alpha-IFN and ribavirin has been used since 1999 (13 patients). Finally, a combination therapy with pegylated alpha-IFN (PEG-alpha-IFN) and ribavirin is being used since 2001 (still 3 patients). In all cases, the treatment lasted 48 weeks. A sustained virological response (SVR, defined as an undetectable serum HCV RNA level 24 weeks after the treatment was completed) was not achieved in any of 6 patients treated with alpha-IFN alone. A combination therapy with alpha-IFN and ribavirin yielded better results: four of eight patients still untreated with alpha-IFN (naive patients), one of two relapsers, and one of three non-responders to previous alpha-IFN monotherapy achieved SVR. So far the combination therapy with PEG-alpha-IFN and ribavirin has been used only in 3 patients. SVR was achieved in one patient who had relapsed after the combination therapy with IFN-alpha and ribavirin, and in 1 of 2 non-responders to this therapy. We conclude that the efficacy and tolerability of the treatment of chronic hepatitis C in hemophiliacs did not differ from that of chronic hepatitis C in other patients.

[Large-droplet liver steatosis in celiac disease]. / Velkokapénková steatóza jater pri celiakii.

Authors monitored a case of a 25 years old woman who was admitted for swelling of lower limbs. Laboratory results showed hypoproteinemia, elevation of liver enzymes, and prolonged prothrombin time. Ultrasound examination proved hepatomegalia with diffusely hyperechogenic liver without central lesion. Computer tomography confirmed hepatomegalia with diffusely hyperechogenic liver and a suspicion of liver steatosis was expressed. Liver biopsy confirmed serious diffuse large droplet steatosis of unclear genesis. Carried out examinations excluded infectious and autoimmune liver diseases, metabolic diseases, and congenital liver diseases (Wilson's disease, porphyria, haemochromatosis etc.). Laboratory results showed gliadin, endomysin, and reticulin antibodies. An enteroscopy picture showed villi decrease. Histology examination of a biopsy specimen confirmed total villi atrophy with non-differentiated enterocytes and round-cell cellulisation of epithelium and proprium. Histology and histochemical findings were distinct proves of coeliac disease. A patient was prescribed a gluten free diet. Her metabolic parameters (normalisation of albumine levels, prothrombine time, and trace elements) and anino transferase levels gradually improved. This case documents development of a serious liver disorder as a result of malnutrition which developed in a young woman as a result of unrecognised coeliac disease.