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[Chemotherapy alone or as adjuvant treatment to surgery for alveolar and cystic echinococcosis].

An overview is presented of the various therapy options in cystic and alveolar echinococcosis based on the recent literature and upon personal experience with chemotherapy over the last 20 years. In regions with an optimal surgical infrastructure and relatively small patient cohorts, long-term chemotherapy is practically limited to patients with inoperable echinococcosis, i. e. < 10 % cystic and around 70 % alveolar echinococcosis.

Aged↗

[Treatment of hydatid echinococcosis].

In Russia the first one-stage operation for hepatic echinococcosis was performed by A.A. Bobrov by his method in 1895. At present there is much experience in surgical treating echinococcosis. With the advent of new ultrasonic diagnostic methods, computed tomography, and new germicide treatment methods have changed. The analysis of 228 patients with echinococcosis treated from 1983 to 1999 was carried out. The traditional surgical interventions were performed in 121 patients; the mortality was 2.5%, the relapse rate 22.3%. Minimum invasive interventions (punctures, hydatid cyst drainage, followed by germicidal treatment) were performed as the only treatment in 68 patients and with subsequent traditional interventions in 22 patients. Experimental studies demonstrated that the retention of gigantic acephalocysts in traditional treatment is the main cause of echinococcal dissemination. 80-100% Glycerin is the most effective germicide. Chemotherapy as the only treatment may be applied only in very minor cysts inaccessible to puncture. A combination of minimum invasive procedures and preoperative (1 course) and postoperative (2-3 courses) chemotherapy is the most effective.

Animals↗

Human echinococcosis: diagnostic value of computed tomography.

The diagnostic value of computed tomography was assessed in 44 patients with alveolar or cystic echinococcosis. The CT-examination was concentrated mainly on the liver, which was involved in all patients. It was extended to other organs, i.e. to lung, intra- or retroperitoneal space, bone and brain if necessary. The CT image of the liver in cystic echinococcosis was often characteristic. It showed sharply outlined masses with attenuation values similar to water. Internal structures were caused by daughter cysts. Furthermore, typical marginal calcium deposits indicated regressive changes in the host membrane. In all cases with alveolar echinococcosis in indistinct solid mass was found, often with central necrotic colliquation and plaque-like calcifications within the tumor.

Adult↗

[Various complications of primary echinococcosis].

Secondary echinococcosis occurs as a complication of a primary cyst. The importance of operative technique during the removal of the primary cyst is emphasized, because the intraoperative disemination occurs most frequently after a surgical performance. Secondary echinococcosis generates by asexual regressive metamorphosis of larval element intro larval forms. Two cases of secondary abdominal echinococcoosis will be described ensuing after removal of the primary liver cyst. One patient, who has been operated because of the pulmonary echinococcosis cyst before, was cured in our hospital having the secondary echinonoccosis of the lungs and pleural cavity.

Echinococcosis↗

Comparison of the dot immunobinding assay and two enzyme-linked immunosorbent assay kits for the diagnosis of liver cystic echinococcosis.

The dot immunobinding assay for the detection of hydatid antigen-specific antibodies (HA-DIA) was evaluated in patients with liver cystic and alveolar echinococcosis in comparison to two commercial ELISA kits. In 30 patients, E. granulosus infection (CE) was confirmed by histopathology or by the presence of parasite protoscoleces and/or hooks or specific antigen 5 (Ag5) in cyst fluid samples obtained by the fine needle aspiration biopsy (FNAB). Infection of E. multilocularis (AE) was diagnosed in two patients by the detection of specific anti-Em2(plus) ELISA and -Em18 Western blot antibodies and finally confirmed by histopathology. The HA-DIA using bovine hydatid antigens showed a high sensitivity in serum samples from CE patients; specific antibodies were found in 29 of 30 CE patients (96.7%). One negative result has been observed in a patient 2.6 years after radical surgery with a subsequent albendazole chemotherapy. The Echinococcosis ELISA(R) (Dialab Diagnostic) was positive in 23 CE cases (76.7%). The correlation between the HA-DIA and the Echinococcosis ELISA(R) was statistically significant. By contrast, Echinococcus granulosus IgG ELISA(R) (Bordier Affinity Products) gave positive results in only 12 of 30 CE patients (40.0%). Sera from two AE patients were high positive in all three methods analysed in our study. In non-endemic areas, due to the between-strains variations and differences in cyst immunogenic activity, related to the natural history of the parasite, a choice of an optimal method for a diagnosis of liver cystic echinococcosis has been discussed.The high diagnostic sensitivity and a faster one-step procedure, in comparison to traditional enzyme immunoassays, make the HA-DIA a very useful method for the diagnosis of CE in non-endemic areas, especially in a case of small or degenerating lesions and sterile echinococcal cysts with a low immunogenicity. The positive serology for CE frequently requires additional differentiation with E. multilocularis-specific antibodies.

Journal Article↗

Salvage treatment with amphotericin B in progressive human alveolar echinococcosis.

Most patients with alveolar echinococcosis are diagnosed at a late stage when the disease has advanced to unresectable hepatic lesions. These patients require lifelong therapy with benzimidazoles, the only medical treatment currently available. To date, no treatment option remains for patients with benzimidazole intolerance or treatment failure. Amphotericin B was recently shown to exert antiparasitic activity in vitro. Here, we report the efficacy of amphotericin B in human alveolar echinococcosis. In three patients with extensive disease and without further treatment options, disease progression had been documented over several months. They were treated with amphotericin B intravenously at a dose of 0.5 mg/kg of body weight three times per week. Follow-up parameters were physical examination, laboratory parameters, and imaging techniques. Amphotericin B treatment effectively halted parasite growth in all three patients. The antiparasitic effect was most evident by spontaneous closure of cutaneous fistulae in two patients and by constant size of parasitic lesions during treatment, as assessed radiologically. Metabolic activity in parasitic areas was visualized by positron emission tomography and significantly decreased during treatment. However, progressive affection of the heart in one patient could not be stopped. All patients currently continue on amphotericin B and have been treated for 25, 17, and 14 months, respectively. We introduce amphotericin B as salvage treatment for alveolar echinococcosis patients with intolerance or resistance to benzimidazoles, as it effectively suppresses parasite growth. Amphotericin B is not parasitocidal; therefore long-term treatment has to be anticipated.

Adolescent↗

[Surgical tactics in associated echinococcosis of the liver and lung].

Over 10 years operations were conducted in 28 patients with hydatid disease with concurrent involvement of the liver and lungs, which accounted for 5.76% of all patients with hydatid disease of the thoracic and abdominal organs. The following variants were encountered: uncomplicated echinococcosis of the right lung and right hepatic lobe (13 patients); uncomplicated echinococcosis of the left lung and liver (3); suppurative hydatid cyst of the liver with rupture into the pleural cavity and the development of pyothorax (9); rupture of a hepatic hydatid cyst into the bronchial tree of the right lung with the development of a cysto-bronchial fistula (3 patients). In the group of 28 patients with concurrent involvement of the liver and lung 18 were operated on through a thoraco-phreno-laparotomic approach, 9 through a transpleural approach, and one patient underwent a two-stage operation. The success of the treatment is determined by early recognition of the concurrent echinococcosis and the choice of the optimal surgical tactics. One-stage echinococcotomy from the liver and lung is the operation of choice in concurrent affection of the liver and right lung.

Adolescent↗

[Current problems of echinococcosis (Echinococcus multilocularis)].

Alveolar echinococcosis is by far the most dangerous human parasitosis in Central Europe. For the one part this is due to the fact that the parasite is localised in the liver, and for the second part it is due to the infiltrative growth of the larval cestode tissue. The life cycle of echinococcus multilocularis takes place primarily between the fox as final host and mice as intermediate hosts. In this cycle, man is an accidental host and is infected orally via the intake of cestode eggs. The individual channels of infection and their frequency have so far only been subject to speculation. The infection rate of foxes is highest in the range of the Swabian mountain pastures (known as Schwäbische Alb) (up to 55 per cent), but recently values of more than 30% have been registered in the adjacent areas as well. No accurate data are available on the infection rate in humans; seroepidemiological data indicate a prevalence between 0.1 and 0.5 per thousand. IHA, IIFT and ELISA are the most frequently used methods in serodiagnostics of echinococcosis. The specific E. multilocularis antigen should be used instead of the commercially available E. granulosus antigen when examining patients for alveolar echinococcosis. In clinical diagnostics, sonography and especially CT are well tried. If the parasitic infestation is discovered early, radical surgery is the method of choice. Chemotherapy with mebendazole (e.g. Vermox) is still unsatisfactory despite improvements. Possibly an echinococcus therapy of foxes via baits containing praziquantel may be a possibility to minimize the transfer to the human organism.

Animals↗

[Clinical experiment studies in the treatment of echinococcosis with mebendazole (Vermox)].

3 patients with echinococcosis were treated with Mebendazol conservatively. One patient got a drug allergy. The other two were operated after a conservative therapy during 3 years in one case and in the course of 110 days in the other case. Dead as well as vital echinococcus cysts and protobrood-cysts were found intraoperatively. The intraabdominal transmission of protobrood-cysts to white infantile mice led to a secondary echinococcosis. The surgical therapy appeared more efficacious in contrast to the conservative treatment. A combination of surgical therapy and conservative treatment is to be recommended in severe disseminated forms of the echinococcosis.

Adult↗

[Echinococcosis--case report and review of the literature].

Echinococcosis is a rare disease in central Europe. But with the increasing numbers of foreign citizens, this disease becomes more important in cases of cystic processes of unknown origin. It is still very difficult to achieve a diagnosis and a subsequent therapy, particularly in cases of multiple cysts, means a long lasting process containing certain risks. We report on a 20 years old female patient, suspected to have a severe inflammatory pelvic disease. We found multiple abdominal cysts, not originating from or involving ovaries, tubes, bowel, liver or kidneys. The serologic investigations proved our clinical suspicion on an echinococcosis. But neither serology nor clinical investigations could provide any indication on which kind of echinococcosis we found. We started a therapy with high doses of mebendazole to achieve a shrinkage of the multiple cysts to enable a surgery.

Adult↗

Alveolar echinococcosis of the liver: sequelae of chronic inferior vena cava obstructions in the hepatic segment.

BACKGROUND: The clinical pattern and long-term course of chronic inferior vena cava (IVC) obstructions are variable and depend on the underlying cause, the segment involved, and the extension of secondary thrombosis. Pertinent data on IVC obstructions in well-defined series of patients are lacking. We report the sequelae of chronic IVC obstructions in the hepatic segment in 11 consecutive patients derived from a cohort of 104 patients with alveolar echinococcosis of the liver. METHODS: Based on the results of computed tomography scans, 11 patients (7 men, 4 women; mean age, 53.4 years) with IVC obstructions were selected from an ongoing prospective long-term chemotherapy trial comprising 104 patients with alveolar echinococcosis studied at yearly intervals according to a protocol. Obstruction of the IVC in the 11 patients existed for a mean duration of 8.6 years. In these patients, magnetic resonance imaging was performed to assess the morphologic features and extension of the IVC obstruction, as well as the collateral venous pathways. Patency and valvular function of the femoropopliteal veins were analyzed by color-coded duplex ultrasonography. RESULTS: Total occlusions of the IVC were evident in 8 patients (73%) and subtotal stenoses in 3 patients (27%). Only 4 patients (36%) exhibited signs and symptoms of chronic venous insufficiency of the lower extremities; 2 (18%) of the 4 had a history of swelling in the lower extremity. Seven patients (64%) had no lower extremity symptoms. One patient had a history of pulmonary embolism. Abdominal collateral veins were documented in 5 patients (45%) by using magnetic resonance imaging; however, they were clinically evident in only 3 patients (27%). In the 8 patients with IVC occlusion, thrombosis ended at the confluence of the hepatic veins. Obstruction of the IVC was limited to the hepatic segment in 2 patients (18%) and extended to the distal IVC or the iliofemoral veins in 6 patients (54%). Chronic venous insufficiency was present only if the femoropopliteal veins had been involved in the thrombotic process, showing residual venous obstruction, valvular incompetence, or both. Bilateral renal vein thrombosis with moderate proteinuria was observed in 2 patients (18%). The main collateral drainage was achieved through the ascending lumbar, azygos, and hemiazygos veins. CONCLUSIONS: In patients with alveolar echinococcosis, obstruction of the IVC in the hepatic segment often develops asymptomatically and rarely leads to the impairment of renal function. The collateral circulation fully compensates for obstruction of the IVC. Thrombotic involvement and valvular incompetence of the femoropopliteal veins seems to determine the development of chronic venous insufficiency of the lower extremities.

Adult↗

Hydroxypyridinium collagen cross-links in human liver fibrosis: study of alveolar echinococcosis.

Liver samples from patients with three different types of liver diseases, alveolar echinococcosis (a dense and irreversible fibrosis), hepatocellular carcinoma and alcoholic cirrhosis, were analyzed for their content in hydroxypyridinium cross-links found in mature collagen. We demonstrated the presence of small amounts of pyridinoline in control livers (0.27 +/- 0.06 pmol/pmol of collagen). Pyridinoline content was increased in fibrotic livers, with the highest values found in patients with alveolar echinococcosis (up to 1.33 pmol/pmol of collagen). The deoxy analogue of pyridinoline was not detected in either normal or fibrotic livers. Pyridinoline levels, expressed as picomoles per picomole of collagen, were similar in all patients with carcinoma (0.7 +/- 0.05 pmol/pmol of collagen). They were heterogeneous in patients with alveolar echinococcosis and were particularly high in patients with alcoholic cirrhosis (1.04 +/- 0.11 pmol/pmol of collagen). These results demonstrate for the first time the presence of an hydroxypyridinium cross-link in liver fibrosis and suggest that pyridinoline measurement might be an important criterion in assessing the irreversibility of human liver fibrosis.

Amino Acids↗

Improvement of liver resectional therapy by adjuvant chemotherapy in alveolar hydatid disease. Swiss Echinococcosis Study Group (SESG).

In human alveolar echinococcosis radical surgery is the only curative therapy. Local recurrences after "radical" surgery have been observed rather frequently in former years. Postoperative "prophylactic" chemotherapy with benzimidazole derivatives over two years appears to prevent local recurrences according to our experience. In addition five patients of our series who suffered from primarily non-resectable alveolar echinococcosis became radically operable after an average of 2 years of chemotherapy. Our experience suggests that chemotherapy improves resectability and curability of surgery in alveolar echinococcosis.

Benzimidazoles↗

Operative treatment of alveolar echinococcosis of the liver.

This study is based on the experience with 51 cases of hepatic alveolar echinococcosis underwent operative treatment up to the end of 1976. Hepatic resection was carried out in 28 cases with overall operative mortality of 25 per cent, but no death occurred in the last 10 years period during which 12 cases were subjected to the procedure. This apparent improvement of the result is ascribed to the establishment of the strict criteria for operative intervention, i.e., when less than three segments are involved, the hepatic hilum is not highly involved, and the inferior vena cava is not invaded. Marsupialization is employed when hepatic resection is not indicated and the lesion shows liquefaction. Ten cases underwent the procedure with one operative death. Biliary tract reconstruction was carried out in two cases with hilar involvement, but the prognosis was poor. Eleven cases were only with celiotomy. The follow-up studies indicated that the hepatic resection offers the best hope for cure followed by marsupialization. Unlike unilocular echinococcosis in which a cyst grows expansively, the alveolar echinococcosis should be considered clinically malignant, in that it grows invasively and often shows metastatic lesions. Surgical intervention at its early developmental stage is the only definitive way of the treatment. Only recent advances in diagnostic procedures and development of type specific serological studies made it possible to bring the disease under control.

Adolescent↗

[Chemotherapy of alveolar echinococcosis with benzimidazoles. A prospective long-term study].

BACKGROUND: Mebendazole and albendazole are the drugs of choice for the treatment of alveolar echinococcosis. In this prospective study we present and evaluate the outcome of the long-term treatment with both drugs. PATIENTS AND METHODS: Forty-four patients were treated with either mebendazole or albendazole and they were followed up for an average of 42 months. Success of treatment was defined as non-progression for more than 1 year. RESULTS: The overall success-rate was approximately 80% (35/44). An initial regimen was recurrence-free in 64% of cases under mebendazole and in 73% of cases under albendazole. Half of the cases with recurrent disease could be stabilized after changing the therapeutic regimen. Seven patients received a continuous regimen with albendazole. They were observed over an average of 19 months without signs of progression nor significant side effects. CONCLUSION: This open-labelled prospective study demonstrates the high therapeutic efficacy of both mebendazole and albendazole with similar response rates in the treatment of alveolar echinococcosis. In Germany, serum levels for mebendazole can easily be obtained at numerous institutes, while serum levels for albendazole are rarely available. On the other hand, albendazole reduces costs by over 40%. A simplified mode of intake and a reduced number of side effects argue in favor of the preferred use of albendazole. Albendazole in alveolar echinococcosis is only licensed for intermittent application. Nonetheless, continuous treatment may be considered in inoperable cases or progressive disease.

Adult↗

The clinical and surgical features of right-sided intracardiac masses due to echinococcosis.

Right-sided cardiac echinococcosis shows special clinical and surgical features beyond the rareness of echinococcosis in this position, leading to serious and life-threatening complications. We examined our cardiac hydatid cyst patients, retrospectively, and report our experience of the surgical treatment of right-sided cardiac hydatid cysts. Between 1985 and 2000, seven patients were transferred to our department from the cardiology department with a diagnosis of cystic cardiac masses which were highly suspected of being hydatid cysts. Two were males and 5 were females. In 3 patients the hydatid cyst was located in the right ventricle, and one was in the right atrium. The mean age of the patients was 37 years (ranging from 12 to 60 years). One patient had preoperative pulmonary emboli. In all right-sided cardiac echinococcosis patients, cardiopulmonary bypass was used. All cysts were cleaned after quilting the cystic cavities, and daughter cysts were removed carefully. The cavities were closed with purse-string sutures. Postoperatively, one patient had pulmonary emboli. In all patients, mebendazole was administered postoperatively. When a right-sided cardiac hydatid cyst is diagnosed, early surgical treatment should be performed under open-heart surgery conditions. During the operation, a single cannula in the superior vena cava should be used until fibrillation, and after clamping, the cannula for the pulmonary artery inferior vena cava should be inserted.

Adolescent↗

Prevention of alveolar echinococcosis--ecosystem and risk management perspectives in Japan.

We focused on the epidemiology of alveolar echinococcosis especially in Japan and discussed control measures to prevent an epidemic. No effective control measures against alveolar echinococcosis have been identified thus far because it is difficult to fully understand the ecology of the parasite and its hosts, i.e. the precise infection route to humans. In Hokkaido, Japan, infection rates among red foxes have recently risen even in low endemic districts. Infection seems to be spreading not only among wild foxes but also among domestic dogs. Despite only sporadic reports of human cases in Japan, we predict that the incidence of alveolar echinococcosis will increase in the near future if no effective preventive measures are put in place. An Echinococcus multilocularis epidemic would have the potential to affect the economy of Hokkaido, due to its impact on the agricultural and tourist industries. Well-designed epidemiological surveys are, therefore, urgently required prior to large outbreaks, based on understanding of the ecosystem around E. multilocularis.

Animals↗

Fenced pasture: a possible risk factor for human alveolar echinococcosis in Tibetan pastoralist communities of Sichuan, China.

Alveolar echinococcosis, infection caused by the parasitic helminth Echinococcus multilocularis, is a zoonosis strongly linked to climatic and ecological factors. Cross-sectional survey data were used to test a hypothesis that partial fencing of pastures could promote alveolar echinococcosis transmission in semi-nomadic pastoral communities of the Tibetan plateau, PR China. Using multiple stepwise logistic regression with consideration of factors of age and gender, it was shown that partial fencing around the settlements in winter pasture was significantly and independently associated with the risk of human alveolar echinococcosis in the surveyed villages (P = 0.021). The underlying reason may lie in overgrazing, an assumed cause of population outbreaks of small mammal intermediate hosts of the parasite on the Tibetan plateau. Overgrazing may have been exacerbated by the reduction of communal pastures nearby the settlements due to introduction of partial fencing around group tenure pastures acquired by Tibetan pastoralist families.

Adolescent↗