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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.

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.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.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%, p0.01). The incidence of bacteriological findings in blood and throat samples is statistically higher in patients with acute bleeding as opposed to the control group (p0.05).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.

An acute bleeding from oesophageal varices as a result of portal hypertension is a frequent and serious complication of liver cirrhosis. The development of oesophageal varices and their rupture depends on the portal pressure. However, a range of other factors can contribute to a development of bleeding and its negative prognosis. A sample of 46 patients admitted for the acute bleeding has been compared to 48 cirrhosis patients hospitalised for other conditions in this work. There were significantly higher levels of nitrogenous matters in bleeding patients (urea 14.1 mmol/l vs. 7.78 mmol/l, p0.01, creatinine 129.8 micromol/l vs. 106.04 micromol/l, p = 0.09). Perhaps it can't be said that impaired renal functions alone increase the risk of bleeding. From this point of view they could rather be seen as definite prognostic markers of the degree of portal hypertension. Moreover, there was a decreased level of total proteins in bleeding patients (60.7 g/l vs. 69.9 g/l, p0.01) at a mild nonsignificant decrease of albumin (26.64 g/l vs. 28.51 g/l). Cirrhotic patients are known to suffer from malnutrition and there is a possibility that malnutrition can contribute to development of bleeding. A prognostic marker of mortality was a considerable impairment of liver function (bilirubin 97.4 micromol/l vs. 57.4 micromol/l; p = 0.1 and prolonged prothrombin time 1.99 INR vs. 1.56 INR; p = 0.09) and impaired kidney function (creatinine 166.7 micromol/l vs. 114.9 micromol/l, p = 0.09). Therefore a care of a good renal function must be a part of the complex care of bleeding patients.

An acute bleeding from oesophageal varices as a result of portal hypertension is a frequent and at the same time serious complication of cirrhosis of the liver. One of factors influencing this bleeding can be a bacterial infection. Endotoxines can increase portal pressure and so participate in development of bleeding and simultaneously deteriorate a patient's prognosis. An antibiotic treatment is a part of a treatment algorithm, however what antibiotics to administer and in what manner is unclear. A group of 46 patients who were admitted to a hospital for an acute bleeding from varices has been compared in the study to 48 cirrhosis patients hospitalised for other reasons. An infection incidence was high in both groups (63.0 % vs. 54.2 %), bleeding patients had more often positive hemoculture (17.3 % vs. 8.6 %), and statistically significantly more often positive findings in throat swab culture (36.9 % vs. 17.3 %, p = 0.04) which is an evidence of an increased pathology colonisation of these patients. Bleeding patients were randomised for peroral norfloxacin administration (n = 25) or an intravenous administration of a combination of ampicilin and sulbactam (n = 21). There was no difference in survival of both groups. Due to a high number of bacterial infections antibiotics administration has been indicated in these patients. Intravenous administration is probably of the same effect as peroral administration.

The authors submit an uncommon case of a true leftsided posterolateral (Bochdalek) hernia with the stomach, transverse colon and spleen in the hernial sac in a 25-year-old patient. They assume that the large hernation developed as a result of rupture of an already congenitally preformed hernial opening as a result of intraabdominally increased pressure during repeated pregnancies. The authors describe effective surgical solution of the case by laparoscopy from a transabdominal approach.

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.

Acute bleeding from upper part of gastrointestinal tract is a frequent and serious complication affecting 20 to 60% of patients with liver cirrhosis and portal hypertension. It is associated with a high death rate of 30 to 50% and a frequent relapse of bleeding occurs in up to 40% of these patients. The most recent meta-analyses have shown that bacterial infection is an independent factor in the failure of blood hemostasis and significantly influenced mortality in these patients. The authors investigated 25 patients with acute bleeding from the upper part of gastrointestinal tract due to portal hypertension in patients with liver cirrhosis. Irrespective of the proved bacterial infection the patients were given antibiotic prophylaxis. In 13 patients the authors administered norfloxacin orally and 12 patients were treated intravenously with ampicilin/sulbactam. The prophylaxis of the bleeding cirrhotic patients by norfloxacin (orally) resulted in a statistically significant prevention of early relapse as compared with the therapy by ampicilin/sulbactam (intravenously). The death rate reached 40% in spite of the antibiotic prophylaxis. There was no significant difference in the death rate between the two groups with different treatments.

The authors present an unusual case of a true left-sided posterolateral (Bochdalek) hernia containing stomach, transverse colon and spleen within a hernial sac in a 25-year-old female patient. They presume this voluminous herniation took place due to a rupture of congenitally preformed hernial ostium on account of increased intraabdominal pressure during repeated pregnancy. They describe an effective surgical transabdominal laparoscopic treatment.

Acute haemorrhage from the upper gastrointestinal tract is a frequent and serious complication which affects 20-60% patients with cirrhosis of the liver and portal hypertension. It is assumed that bacterial infections can be the direct cause of haemorrhage but accurate data on the influence of infection on the development and course of haemorrhage are lacking. Acute haemorrhage as a result of portal hypertension has a very high mortality, 30-50%, and an early relapse of haemorrhage occurs in as many as 40% of these patients. Most recent meta-analyses indicate that bacterial infection is an independent prognostic factor in failure of haemostasis and has a significant impact on the mortality of these patients. The authors examined for the presence of bacterial infection (blood, urine, throat, ascites) 25 patients with cirrhosis of the liver and acute haemorrhage as a result of portal hypertension and compared the results with a group of 25 patients with cirrhosis of the liver and portal hypertension without acute haemorrhage. According to the results in patients with acute haemorrhage due to portal hypertension there is a significantly higher incidence of bacterial infections than in patients with cirrhosis of the liver and portal hypertension without acute haemorrhage. The results confirm the necessity to administer antibiotic prophylaxis to cirrhotic patients with varicose bleeding, not only to patients with symptoms and evidence of infection but also in their absence. Antibiotic prophylaxis extends the survival period of these patients.

The objective of the study was to assess the prevalence and more detailed data pertaining to the incidence of spontaneous bacterial peritonitis (SBP) in the Czech Republic. The authors examined 99 patients with cirrhosis of the liver and ascites. SBP was diagnosed in a high percentage--35 patients, i.e. 35.4%. It was found more frequently in patients with an alcoholic etiology of cirrhosis who had a history of subfebrile and febrile temperatures and increasing trend of ascites. For the diagnosis the increase of leucocytes in serum and C reactive protein levels may prove useful. Lower values of total protein and albumin in ascites predispose to the development of this infection. Reduction of the number of thrombocytes in the group of patients with SBP indicates the influence of portal hypertension in the etiology of this disease.

Gastrooesophageal reflux (GER) and asthma bronchiale are frequent diseases. Asthma affects some 3-10% of adults. Gastrooesophageal reflux is present in some 45-89% asthmatic patients. Symptoms of GER are not only gastrooesophageal, and recently increased attention is focused on extraoesophageal symptoms where in particular the relationship of GER and asthma or chronic cough is investigated. At our clinic we implemented a pilot study with the objective to monitor the presence of pathological GER in patients with asthma and to assess whether antireflux therapy will influence the respiratory complaints of the patients. The group was formed by 14 patients selected at random with different severity of asthma and different symptoms of GER. The patients had a baseline examination evaluating the presence of GER (24-hour pH metry) and pulmonary function (FEV1). In case of a pathological GER the patients were treated by antireflux therapy and then check-up examinations were made. It was found that after treatment of GER in patients with asthma in particular subjective symptoms improved such as cough and pyrosis which leads to a substantial improvement of the quality of life. On the other hand reflux treatment did not exert a basic effect on pulmonary functions and it was not possible to reduce the medication of asthma.

The authors submit a retrospective investigation of 50 patients hospitalized at the intensive care unit of the Medical gastroenterological department, Faculty Hospital Brno treated in 1999 with the diagnosis of acute haemorrhage into the upper digestive tract. In the investigated group the most frequent cause of haemorrhage was portal hypertension (21 patients, 32.8%) and a peptic gastroduodenal lesion (15 patients, 23.4%). During the investigation period 12 patients died (18.8%), 6 developed haemorrhage as a complication of a serious condition (decompensated cirrhosis of the liver). In haemorrhage from oesophageal and gastric varicosities pharmacotherapy is equally important as endoscopic intervention.

The aim of this study was to determine the incidence of autoantibodies to asialoglycoprotein receptor (ASGPR, anti-ASGPR) in chronic hepatitis C patients and to characterize the anti-ASGPR-positive and anti-ASGPR-negative patients in more detail. A total of 79 chronic hepatitis C patients were screened for the presence anti-ASGPR by ELISA. Anti-ASGPR were detected in 11 (13.9%) patients. No significant differences were found between the anti-ASGPR-positive and anti-ASGPR-negative patients in age, alanine transaminase (ALT) activity, histological findings and response and tolerance to alpha-interferon (alpha-IFN) therapy. The male predominance in the anti-ASGPR positive group was statistically significant. It was surprising that other tested autoantibodies (antinuclear autoantibodies [ANA], smooth muscle autoantibodies [SMA], type 1 liver-kidney microsome autoantibodies [LKM-1], anti-thyroglobulin and thyroid microsome autoantibodies) and increased levels of immunoglobulins A, G and/or M were observed significantly more frequently in the anti-ASGPR-negative group.

The authors submit the case-history of a 29-year-old man, followed up on account of liver steatosis with a toxic-nutritional etiology who developed, after previous increased physical exertion and alcohol abuse, fever associated with major muscular weakness. Gradually he developed an amental delirious state which was evaluated as suspect delirium tremens. Fever of 40-41 degrees C continued, the patient developed muscular rigidity, tremor and hypotension. After intubation during which succinylcholine was administered, the patient's condition deteriorated further with a rise of temperature and muscular rigidity. The patient developed acute renal failure with anuria and the necessity of repeated haemodialyses and severe acidosis of the mixed type on account of which he was intubated and switched to artificial ventilation. According to the case-history clinical and laboratory picture of the disease (extremely high creatine kinase activity, hyperkalaemia, acidosis, hepatorenal failure) malignant hyperthermia was suspected. After a single intravenous injection of sodium dantrolene, 2.5 mg/kg, the temperature dropped and within 24 hours the patient was afebrile. Gradually the acidosis improved, the blood pressure became stabilized and artificial ventilation was no longer used. The patient was discharged after 34 days in hospital in a state of cardiopulmonary compensation with mild polyuria but without signs of retention of nitrogenous substances with sideropenic anaemia and marginal creatine kinase and lactate dehydrogenase values. Within one month after discharge the laboratory values reached normal levels and only slight muscular weakness and greater fatiguability persisted.