[Wilsons disease]. / Wilsonova choroba.

Wilsons disease is an autosomal recessive genetic disorder in which copper accumulates in tissues, especially in the liver and the brain. The genetic defect affects the P type ATPase gene (ATP7B). More than 500 mutations causing Wilsons disease have been described. The most common mutation in Central Europe concerns H1069Q. The symptoms of Wilsons disease include hepatic or neurological conditions. The hepatic condition is manifested as steatosis, acute or chronic hepatitis or cirrhosis. The neurological conditions are most often manifested after the age of 20 as motor disorders (tremor, speech and writing disorders), which may result in severe extrapyramidal syndrome with rigidity, dysarthria and muscle contractions. The dia-gnosis is based on clinical and laboratory assessments (neurological signs, liver lesions, low ceruloplasmin, increased free serum copper, high Cu volumes in urine, KayserFleischer ring). The dia-gnosis is confirmed by a high Cu level in liver tissue or genetic proof. Untreated Wilsons disease causes death of the patient. If treated properly the survival rate approximates to the survival rate of the common population. The treatment concerns either removal of copper from the body using chelating agents excreted into the urine (Penicillamine, Trientine) or limitation of copper absorption from the intestine and reducing the toxicity of copper (zinc, ammonium tetrathiomolybdate). In the Czech Republic, Penicillamine or zinc is used. A liver transplant is indicated in patients with fulminant hepatic failure or decompensated liver cirrhosis. In the family all siblings of the affected individual need to be screened in order to treat any asymptomatic subjects.

[Hepatic cein catheterisation -  selected assessment aspects]. / Katetrizace jaterních zil -  vybrané aspekty hodnocení

INTRODUCTION: Hepatic vein catheterisation and portal hypertension assessment using the value of portal hepatic gradient (HVPG) is currently a method of choice. METHODOLOGY: In our paper we shall compare HVPG with the so called direct gradient -  using the difference in pressure in the portal vein and free hepatic vein in 5 groups of patients with liver cirrhosis. RESULTS: Hepatic vein catheterisation is reliable for assessing the portal hypertension in the group of patients with liver cirrhosis of ethylic etiology. In patients with liver cirrhosis resulting from hepatitis B, Wilsons disease or primary biliary cirrhosis, a statistically significant difference between HVPG and the direct gradient has been found. In patients with liver cirrhosis resulting from hepatitis C the obtained values differed but without statistical significance. CONCLUSION: In catheterisation of hepatic veins the HVPG value in liver cirrhosis with a presinusoidal component may be reduced, which has to be primarily taken into account when assessing the relationship to some critical values of the portal hepatic gradient.

Anemia as a predictor of response to antiviral therapy in chronic hepatitis C.

BACKGROUND: The standard therapy for chronic HCV infection is the administration of pegylated interferons in combination with ribavirin. Anemia is a dose-dependent side-effect of ribavirin administration. The degree of anemia could be indicative of the individual exposure to ribavirin. AIMS: 1) To evaluate the correlation of HGB level decreases at specified time-points with a sustained virological response during the antiviral treatment. 2) To compare these parameters with the virological predictors for responses. METHODS: A retrospective analysis of cohort, which comprised 164 patients treated with standard therapy at a tertiary center in Prague, Czech Republic. RESULTS: We identified several predictive factors for a sustained virological response in females: baseline HGB level ≤140 g/l (p=0.025), maximum drop from baseline >40 g (p=0.039), and a HGB drop in week 4 >30 g (p=0.044). There was only one predictor identified for males: reaching the lowest HGB level after week 19 (p=0.021). The strongest positive predictor of response was a rapid virological response. A low viral load (<600 000 IU/ml) at baseline was not associated with a sustained response in either gender. CONCLUSIONS: The parameters of HGB decrease during antiviral treatment are better correlated with a sustained response in females. None of these response predicting parameters was as significant as that of rapid virological response as that of rapid virological response (Tab. 4, Fig. 1, Ref. 15).

Sleep disorders in Wilson's disease.

BACKGROUND: Wilson's disease (WD) is an autosomal recessive inherited disease with copper accumulation; neurodegeneration is associated with dopaminergic deficit. The aim of the study is to verify sleep co-morbidity by questionnaire and objective sleep examinations (polysomnography, multiple sleep latency test). METHODS: fifty-five patients with WD (22 hepatic, 28 neurological, five asymptomatic form) and 55 age- and sex-matched control subjects completed a questionnaire concerning their sleep habits, sleep co-morbidity, Epworth sleepiness scale (ESS), and answered screening questions for rapid eye movement (REM) behaviour disorder (RBD-SQ). Twenty-four patients with WD and control subjects underwent polysomnographic examination. RESULTS: unlike the controls, patients with WD were more prone to daytime napping accompanied by tiredness and excessive daytime sleepiness, cataplexy-like episodes and poor nocturnal sleep. Their mean ESS as well as RBD-SQ was higher than that of the controls. Total sleep time was lower, accompanied by decreased sleep efficiency and increased wakefulness. Patients with WD had lower latency of stage 1 and stage 2 of non-rapid eye movement (NREM) sleep and less amount of NREM sleep stage 2. One-third of the patients with WD were found to have short or borderline multiple sleep latency test (MSLT) values independent of nocturnal pathology (sleep apnoea, periodic leg movements and/or restless leg syndrome). CONCLUSIONS: patients with WD often suffer from sleep disturbances (regardless of the clinical form). The spectrum of sleep/wake symptoms raises the suspicion that altered REM sleep function may also be involved.

[Efficacy of combination treatment with pegylated interferon plus ribavirin in patients chronically infected with HCV]. / Ucinnost terapie pegylovaným interferonem a ribavirinem u pacientu s chronickou HCV infekcí.

THE AIM OF THE STUDY: To evaluate the efficacy of combined antiviral treatment with pegylated interferon alpha plus ribavirin in patients with chronic HCV infection who have not yet been treated with antivirals (treatment-naive patients). To compare the treatment effect in patients with low (< 600,000 IU/ml) and high (> or = 600,000 IU/ml) initial viremia. METHODS AND TREATMENT REGIME: Treatment-naive patients with chronic HCV infection treated with the combination therapy of pegylated interferon-alpha2a plus ribavirin. Treatment response was evaluated at weeks 12, 24 and 48 when treatment was ongoing and at weeks 12, 24 and 48 after the treatment was finished. Commercially available sets from various manufacturers were used for serum and molecular genetic diagnostics of HCV infection. PATIENT SAMPLE: Antiviral treatment was initiated in 175 patients between 2001 and 2007. The complete data sets suitable for statistical analysis were available for 143 patients. End of treatment response and sustained viral response analyses were conducted separately for HCV genotype 1 (n = 124) and genotype 2 + 3 (n = 7). RESULTS: In the genotype 1 group, 76% of patients achieved end of treatment response and 59% of patients achieved sustained viral response. Both types of response were observed in 100% of the genotype 2 and 3 infected patients. When a correlation between initial viremia and sustained viral response was analysed, no statistically significant difference was observed between patients with low (< 600,000 IU/ml) and high (> or = 600,000 IU/ml) initial viremia. CONCLUSION: The results observed in the present study are generally slightly better than comparable results from large registration studies. In contrary to the published literature, the threshold of 600,000 IU/ml for initial viremia did not correlate statistically significantly with SVR.

[Wilson's disease]. / Wilsonova choroba.

Wilson's disease is an inherited disorder leading to accumulation of copper in tissues, mainly in the liver and brain. Genetic defect is in the gene coding ATPase type P (ATP7B). The inheritance is autosomal recessive. Up to now, more then 500 mutations causing Wilson's disease were described. The most frequent mutation in Central Europe is mutation H1069Q. The manifestation of Wilson's disease is usually hepatic or neurologic. Hepatic form is manifested by acute or chronic hepatitis, steatosis or cirrhosis. Neurologic involvement is manifested usually after 20 year of age by motor disturbances (tremor, disturbed speech, problems with writing), which could progress into severe extrapyramidal syndrome with tremor, rigidity, dysartria, dysfagia and muscle contracture. Diagnosis is based on clinical and laboratory examinations (neurologic symptoms, liver disease, low serum ceruloplasmin levels, elevated free copper concentration in serum, high urine copper excretion, and presence of Kayser-Fleischer rings). Confirmation of diagnosis is done by hepatic copper concentration in liver biopsy or by genetic examination. Untreated disease leads to the death of a patient. Treatment is based on chelating agents decreasing the copper content by excretion into urine (D-penicillamine, trientine) or on agents preventing absorption of copper from food (zinc, ammonium-tetrahiomolybdene). Patients with asymptomatic Wilson's disease have to be treated as well. In Czech Republic either penicillamine or zinc are used. Liver transplantation is indicated in patients with fulminant liver failure or decompensated cirrhosis. Screening in families of affected patients (all siblings) is obvious.

[Hepatitis C viral infection at the beginning of twenty-first century]. / Infekce virem hepatitidy C na pocátku 21. století.

The article reviews basic information on the epidemiology, origin, diagnostics and therapy of hepatitis C viral infection. Virus of the hepatitis C was identified in 1989. The most frequent transmission pathway till 1992 was the reception of blood derivatives, after that year, when transfusion centres started to use detection sets to prove anti-HCV antibodies, the incidence of post-transfusion hepatitis C dropped almost to zero. The most common route of transmission at present is the intravenous toxicomany, and significant participation represents the medical care. The basic serological marker of HCV infection is the presence of anti-HCV antibodies. Those antibodies signify markers of the human contact with the virus; they need not automatically mean the encounter of infection. More often it is contrariwise--because the C viral hepatitis develops the chronic stadium up in 85%, the anti-HVC positivity signifies usually the active form of infection. To prove the active form of infection it is necessary to identify viral nucleic acids in the serum of the examined patient. The standard therapy of the chronic form of the C viral hepatitis is at present a combination of pegylated interpherons alpha and ribavirin. Such form of therapy can result the permanent elimination of the virus in about 60% of cases. In the C viral hepatitis neither the specific pre-exposition nor post-exposition prophylaxis is available. The only prevention of the transmission of infection is the avoidance of any risk factor of transmission, namely in the medical care.

[Long-term pharmacological treatment of portal hypertension]. / Dlouhodobá farmakologická lécba portální hypertenze.

Portal hypertension is an unavoidable complication of liver cirrhosis, which usually limits the survival (bleeding from esophageal varices, ascites). Increase in portal pressure is not only due to mechanical obstruction of portal circulation, but there is also a dynamic component (endothelial dysfunction of hepatic microcirculation) and increased blood flow through the splanchnic circulation. For the long-term treatment of portal hypertension two groups of medicaments are available at present: non-selective betablockers (vasoconstriction in splanchnic bed) and nitrates (lowering of intrahepatic resistance). Long-term treatment is necessary in these situations: Primary prophylaxis of bleeding from esophageal varices (in patients, who never bled, but with "risk" varices)--non-selective betablockers; secondary prophylaxis (in patients after variceal bleeding)--non-selective betablockers (possibly with nitrates) or endoscopic treatment. It is clearly documented, that this treatment lowers the risk of the first or repeated bleeding from varices and hence lowers the mortality and morbidity due to this complication in patients with liver cirrhosis. Another serious complication of liver cirrhosis is the spontaneous bacterial peritonitis. All patients after that infection have to receive prophylactic treatment with antibiotics. This treatment should be long life, till the disappearance of ascites or till the liver transplantation.

Treatment of early diagnosed HCV infection in hemodialyzed patients with interferon-alpha. Treatment of hepatitis C.

BACKGROUND: Acute and early diagnosed hepatitis C virus (HCV) infections are rare diagnoses. Patients on regular dialysis treatment (RDT) are at risk of acquiring HCV infection. AIMS OF THE STUDY: (1) To determine the efficacy and safety of two-phase induction treatment of acute and early diagnosed HCV infections in patients on RDT, and (2) to establish the importance of serum HCV RNA testing at defined time points of treatment for the prediction of the therapeutic effect. THERAPEUTIC PROTOCOL: Antiviral treatment consisted of two different phases: phase A therapy was interferon (IFN)-alpha 2b 10 million units (MU) s.c. administered daily for 21 days followed by phase B with IFN-alpha 2b 3 MU s.c. administered 3 times weekly for 12 weeks. RESULTS: (1) Efficacy of the treatment: A sustained virological response (SVR) was achieved in a total of 13/18 patients (72%). SAFETY: We did not observe any serious side effects of the treatment. The most pronounced side effect was the myelosuppression caused by IFN-alpha. (2) SVR prediction: Patients with negative serum HCV RNA at day 6 achieve SVR more frequently than those with positive HCV RNA at day 6 (p = 0.074). CONCLUSIONS: Treatment of acute and early diagnosed HCV infections in hemodialyzed patients is much more effective than treatment of chronic infection. Even relatively high doses of IFN at the beginning of therapy (10 MU daily) are tolerated well by the patients.

Cardiac involvement in Wilson disease.

BACKGROUND: Wilson disease is an inherited autosomal recessive disorder of copper metabolism resulting in pathological accumulation of copper in the liver, brain and other tissues. One of the reported manifestations is cardiac involvement. METHODS: We studied 42 patients with Wilson disease (19 men and 23 women, mean age 34 +/- 10 y) and 42 age- and sex-matched healthy volunteers. All subjects underwent complete echocardiographic examination; 24 h ECG Holter monitoring was performed in 23 Wilson disease patients. RESULTS: In comparison to healthy subjects, patients with Wilson disease had increased thickness of the interventricular septum (9.5 +/- 1.4 vs 8.6+/-1.1 mm, p < 0.01) and left ventriclular (LV) posterior wall (9.1 +/- 1.3 vs 8.2 +/- 1.0 mm, p < 0.01). While the two groups did not differ in LV mass index, relative LV wall thickness was significantly increased in Wilson disease patients compared to control subjects (0.39 +/- 0.06 vs 0.34 +/- 0.04 p < 0.001). Concentric LV remodelling was present in 9 patients (21%) and LV hypertrophy in one patient. Systolic LV function showed a nonsignificant trend towards lower values in Wilson disease patients (EF 62 +/- 5% vs 64 +/- 50%, p = 0.06). Diastolic filling and the frequency of valvular abnormalities were comparable in both groups. The established echocardiographic abnormalities did not correlate with the type of Wilson disease manifestation, the presence of the His1069Gln mutation, laboratory parameters or the duration and type of therapy. Twenty-four-hour ECG Holter monitoring detected ECG abnormalities in 10 patients (42%), the most frequent findings being runs of supraventricular tachycardias and frequent supraventricular ectopic beats. CONCLUSIONS: Cardiac involvement in Wilson disease patients was mild, characterized by LV parietal thickening with an increased prevalence of concentric LV remodelling and a relatively high frequency of benign supraventricular tachycardias and extrasystolic beats.

Double-blind randomized, comparative multicenter study of the effect of terlipressin in the treatment of acute esophageal variceal and/or hypertensive gastropathy bleeding.

BACKGROUND/AIMS: 1) To compare the effect of 2-day application of 0.2 mg terlipressin i.v. every 4 hours (group I) with that of 5-day application of 1 mg i.v. 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.

[Rifaximin in the treatment of hepatic encephalopathy]. / Rifaximin v lécbe jaterní encefalopatie.

Hepatic encephalopathy is a frequent and serious complication of liver cirrhosis. Usually it is treated by non-absorbable disaccharides or antibiotics and its treatment is often difficult and associated with undesirable effects. The objective of our investigation was to evaluate the safety and effectiveness of a new antibiotic used in this indication--rifaximine. With rifaximine, 400 mg three times per day, a total of 25 patients were treated for a 10-day period. Significant improvement of the manifestations of encephalopathy occurred (evaluated by the grade of encephalopathy, test of combining numerals, the degree of flapping tremor and the arterial ammonia level). None of the patients developed undesirable effects. Rifaximine seems an effective, safe drug for hepatic encephalopathy.

[Risk factors for transmission of hepatitis C in the the Czech population]. / Rizikové faktory prenosu viru hepatitidy C (HCV) v ceské populaci.

BACKGROUND: Recent reports from all over the world have repeatedly indicated a change in the incidence of individual risk factors for hepatitis C virus (HCV) infection transmission compared with the pattern in the late 1980s and early 1990s. In the Czech Republic, HCV is very often referred to as an addicts' disease, rare in the general population. To establish the incidence of individual risk factors for HCV infection transmission in a group of patients on follow-up at the Department of Internal Medicine I. General University Hospital in Prague 2. METHODS AND RESULTS: The group of patients included 216 individuals (127 men, 89 women) with documented HCV infection. The mean age of the patients was 40.2 years (10-81 years; SD 14.3). The risk factors were identified on the basis of evaluation of the patient's medical history, and/or their medical records if available. The presence of at least one of the following risk factors was regarded as the source of infection (the figure in brackets gives the incidence of the respective factor in the examined group in percent): blood product transmission (15%), intravenous drug injection (16%), inclusion into a regular dialysis program (12%), profession-related risk of transmission (10%), sexual contact with an infected individual (2%), surgery including dental surgery (14%), invasive examination (6%), and tattooing (1%). No risk factor for infection transmission was identified in 24% of cases. CONCLUSIONS: It has been shown a risk factor for infection transmission can be identified, through careful examination of medical history data, in the Czech population in as much as 76% of cases. An important finding is the fact the infection can be regarded as iatrogenic in as much as 57% of cases. Our data clearly show HCV infection is not exclusively a disease of intravenous drug addicts.

[Importance of liver biopsy in chronic hepatitis C virus infection]. / Význam jaterní biopsie pri chronické infekci virem hepatitidy C.

INTRODUCTION: The importance of liver biopsy and knowledge of the histological activity of liver les on in chronic hepatitis C virus (HCV) infections is widely discussed recently. There are attempts to find an alternative evaluation which will make it possible to avoid liver biopsy. The crucial question in patients with chronic HCV infection is to differentiate patients with already developed liver cirrhosis from those with chronic hepatitis. OBJECTIVES: 1. To evaluate the impact of the calculation of the discrimination score of liver cirrhosis (DSC) for prediction of liver cirrhosis in the histological assessment. 2. To assess the correlation of prediction of cirrhosis liver based on clinical signs and actual histological verification. 3. To evaluate the frequency of unexpected histological findings not correlating with the clinical picture. GROUP OF PATIENTS: The group was formed by 139 patients. In all patients during the baseline examination the patient's history data were analyzed as well as possible physical signs of liver cirrhosis. In all patients also, based on laboratory values before liver biopsy, the DSC according to Bonacini was calculated. Furthermore agreement between the histological finding of liver cirrhosis and chronic hepatitis with DSC values was assessed. RESULTS: 1. Based on calculation of DSC it is possible to predict accurately the existence of cirrhosis of the liver or chronic hepatitis only in 31% patients. In 69% patients even comprehensive evaluation of the type of DSC is not a sufficient guide for assessment of the hepatic lesion. 2. Even clinical signs of cirrhosis are not a quite reliable guide for its prediction. In 8% patients of our group the histological finding of liver cirrhosis was a surprise and in 3.5% patients cirrhosis of the liver was not confirmed despite the presence of clinical signs. 3. The frequency of other histological findings participating in the development of the hepatic lesion in chronic HCV infection was minimal. In the authors group as such only steatosis and toxic damage of hepatic tissue by alcohol were identified. These findings were, however, suspected already before biopsy. Steatosis can be however considered also a manifestation of HCV infection. CONCLUSION: The results of the trial support the view that liver biopsy is in the majority of cases irreplaceable for evaluation of the severity of the hepatic affection in chronic HCV infection.

Carcinoembryonic antigen-related cell adhesion molecule 1 is the 85-kilodalton pronase-resistant biliary glycoprotein in the cholesterol crystallization promoting low density protein-lipid complex.

A pronase resistant 85-kd glycoprotein in the Concanavalin A-binding fraction (CABF) of biliary glycoproteins has been reported to act as a promotor of cholesterol crystallization. De Bruijn et al. (Gastroenterology 1996;110:1936-1944) found this protein in a low-density protein-lipid complex (LDP) with potent cholesterol crystallization promoting activity. This study identifies and characterizes this protein. An LDP was prepared from CABF by discontinuous gradient ultracentrifugation. Proteins were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE), blotting and immunochemical staining with anti-carcinoembryonic antigen, CEA-related adhesion molecule 1 (CEACAM1) cross-reacting antibodies. Biliary concentrations of CEA cross-reacting proteins were determined in patients with and without gallstones. Two isoforms of CEACAM1 (85- and 115-kd bands), CEA and 2 CEA cross-reacting protein bands of 40 and 50 kd were found in human bile. All bands were also present in CABF, but only a subfraction of the 85-kd band found in the LDP was resistant to digestion with pronase. CEACAM1-85 exhibited potent cholesterol crystallization promoting activity in vitro and accounted for most of the activity in CABF. Total CEA cross-reacting protein concentrations were the same in gallbladder biles from patients with cholesterol and pigment gallstones but only half of those in biles from nongallstone subjects. In conclusion, we have identified the protein component of the cholesterol crystallization promoting LDP to be CEACAM1-85.

[Importance of hepatitis C virus infection in gynecology and obstetrics]. / Význam infekce virem hepatitidy C v gynekologii a porodnictví.

OBJECTIVE: Diagnostic methods of hepatitis C-virus. SUBJECT: A review article. SETTING: 1st Internal Clinic, General Faculty Hospital in Prague, 1st Medical Faculty, Charles University. SUBJECT AND METHOD: Hepatitis C virus infection importance is increasing during the last few years, in concordance with this fact therapeutical options are being developed very rapidly, too. Majority of cases of HCV infection has a silent clinical course for a long time and patients are often diagnosed in the late stage of liver disease. This is the reason why the diagnosis should be established as early as possible. Gynecologist can be the first person who can observe the symptoms of the infection and therefore he may become the key person in the diagnostic process. CONCLUSION: The basic facts about the diagnostic methods and clinical course of the disease are mentioned in this review. The differences in clinical course of HCV infection in pregnancy and the vertical transmission is discussed in details.

Immunoaffinity isolation of CEACAM1 on hydrazide-derivatized cellulose with immobilized monoclonal anti-CEA antibody.

Carcinoembryonic cell adhesion molecule 1 (CEACAM1) is a human membrane glycoprotein belonging to the carcinoembryonic antigen (CEA) family and to the immunoglobulin superfamily. It is expressed in apical membranes of many epithelial cells in gastrointestinal and urogenital tract and also in granulocytes and lymphocytes, and its biological effect in human tissues has recently been discussed in literature. The purpose of this study was to isolate CEACAM1 glycoprotein from bile and characterize its purity and recovery which has not been described before. Affinity chromatography of CEACAM1 on hydrazide-activated cellulose with immobilized monoclonal anti-CEA F34-187 antibody is described. The immunoglobulin carbohydrate moiety was oxidized by periodate and then bound to hydrazide-activated matrix. Crude protein fraction from bile was applied on the affinity column and after extensive washing of non-bound proteins CEACAM1 was eluted with 6 M guanidine-HCl. A single immunopositive 85 kDa band was detected on Western blots with anti-CEA antibody after SDS-PAGE. We found out that CEACAM1 was not stainable with any common method of protein staining and the only non-specific method which could detect the 85 kDa band was a lectin staining.

Intraluminal brachytherapy and selfexpandable stents in nonresectable biliary malignancies--the question of long-term palliation.

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 30 Gy) 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 carcinoma 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 as 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.

Extracorporeal shock wave lithotripsy of gallstones with oral dissolution. Results in course of ten years in Czech Republic in correlation to indication criteria.

BACKGROUND/AIMS: We compared the results of treatment of gallbladder stones by extracorporeal shock wave lithotripsy with dissolution during two periods to different criteria of indication. In the first period (1990-1991) we treated patients with stones up to 3 cm in diameter. In the second period (1992-1994) the indication included stones up to 1.5 cm with gallbladder contraction on cholecystography, which was also quantitatively determined by USG exceeding 60% of fasting volume. METHODS: We used Czech made lithotriptors MEDILIT. The shock waves (SW) are produced by an underwater high voltage discharge and the concentration of SW is achieved by reflection. The localization of stones is performed by means of a sonographic probe. After stone localization usually 700 SW are applied to a patient lying in prone position. Oral dissolution was started in both groups of patients two weeks before shock wave application. Ursodeoxycholic acid and chenodeoxycholic were administered in doses of 7.5 mg/kg body weight/day. RESULTS: In the first group of 260 patients (average age 42), which had moderate indication criteria, disappearance of fragments during one year was found only in 148 (57%). The remaining patients were treated symptomatically or recommended to cholecystectomy. In a second group of 160 patients (average age 45), disappearance of fragments during one year was achieved in 125 (78%). We used stricter indication criteria and our results are much better compared to the first group. Only 208 patients from both groups were controlled for more 5 years, in 89 cases of this number was a recidivity of lithiasis and at 20 of them we indicated cholecystectomy, 22 of the patients refused to continue in the therapy, because the stones are asymptomatic. CONCLUSION: In our opinion, this therapy (ESWL combined with oral dissolution) is suitable in 7% patients with gallbladder stones. Its side effects are very small, practically negligible. It can be performed without general anaesthesia and on an out-patient basis. After the advent of laparoscopic cholecystectomy we observed diminished interest of both patients and their physicians for gallbladder ESWL, however, after it has reached a widespread use, a new interest in ESWL and dissolution therapy is emerging, which we attribute to its above-mentioned advantages.

An absence of changes in kidney tissues after an enormous number of shock waves.

Repeated sessions of unilateral extracorporeal shock wave lithotripsy (ESWL) were performed in a patient with multiple bilateral calycolithiasis who suffered from Crohn's disease of long duration and from recurrent gastric ulcers. To achieve complete clearance of stones from the left kidney it was necessary to apply 10,000 shock waves during seven sessions within five weeks. This number is exceedingly high compared to average total doses which were applied to other patients of our group (920 SW per one session). For the treatment was used an electrohydraulic lithotripter (Medipo). A surgical reconstruction of the abdominal wall which had been planned long before was made six weeks after the last ESWL session. The postoperative course was complicated by bleeding gastric ulcer of which the patient died despite an intensive treatment. The autopsy and histology revealed no damage of kidney tissue or adjacent organs caused by ESWL.