Indications and results of liver transplantation for Echinococcus alveolar infection: an overview

Background: Alveolar echinococcosis (AE) of the liver, caused by the larval stage of the fox tapeworm Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. It is one of the rare parasitic diseases for which a parasitolytic drug is not yet available, and AE is lethal in the absence of appropriate therapeutic management. Complete surgical resection of the parasite at an early stage of infection provides favourable prospects for cure, but, due to a long clinical latency, many cases are diagnosed at an advanced stage, so that partial liver resection can be performed in only 35% of patients. Benzimidazole (BZM) treatment is given in inoperable cases but these compounds are only parasitostatic, and lifelong therapy is required. During the past 20 years some centres have considered liver transplantation (LT) for the treatment of incurable AE. Methods: Our review summarizes the results of this experience based on a series of 47 European patients who received transplants between 1985 and 2002, tries to specify the real place of LT for AE, and underlines the measures that could be undertaken in the future to improve the results. Results: Five-year survival was 71%. Five-year survival without recurrence was 58%. Major technical difficulties related either to previous laparotomies or to the loco-regional involvement were observed. The nine early deaths concerned AE patients with a long past-history of symptomatic AE (iterative cholangitis, secondary biliary cirrhosis). Five late deaths were directly related to ongoing AE, located in the brain in three cases, a very rare AE location that was not investigated before LT in these patients. Conclusions: In general, the pre-LT screening for distant AE metastases appeared insufficient in this series. Heavy immunosuppressive schemes, absence or delayed re-introduction of BZM after LT have clearly played a role in this unfavourable course. This unique experience indicates that, despite major technical difficulties, LT for incurable AE is feasible and could be discussed in very symptomatic cases. Before LT, interventional radiology should be preferred to repeated laparotomies. Pre-LT and post-LT BZM treatment is mandatory. A careful evaluation of possible distant metastases should be done before the decision for LT is made. After LT, the possibility of an ongoing AE must be permanently kept in mind. This could be reduced by lightening the immunosuppressants, carefully following the specific circulating antibodies, and applying a systematic radiological evaluation, not only to the graft but also to the lungs and the brai

Indications and results of liver transplantation for Echinococcus alveolar infection: an overview

International audienceAlveolar echinococcosis (AE) of the liver, caused by the larval stage of the fox tapeworm Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. It is one of the rare parasitic diseases for which a parasitolytic drug is not yet available, and AE is lethal in the absence of appropriate therapeutic management. Complete surgical resection of the parasite at an early stage of infection provides favourable prospects for cure, but, due to a long clinical latency, many cases are diagnosed at an advanced stage, so that partial liver resection can be performed in only 35% of patients. Benzimidazole (BZM) treatment is given in inoperable cases but these compounds are only parasitostatic, and lifelong therapy is required. During the past 20 years some centres have considered liver transplantation (LT) for the treatment of incurable AE

Échinococcose alvéolaire en région parisienne…

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Survival strategy of Echinococcus multilocularis in the human host

International audienceAs exemplified by "aborted" calcified liver lesions commonly found in patients from endemic areas, Echinococcus multilocularis metacestodes develop only in a minority of individuals exposed to infection with the papasite. Clinical research has disclosed some aspects of the survival strategy of E. multilocularis in human hosts. Clinical observations in liver transplantation and AIDS suggest that suppression of cellular/Th1-related immunity increases disease severity. Most of the studies have stressed a role for CD8+ T cells and for Interleukin-10 in the development of tolerance. A spontaneous secretion of IL-10 by the PBMC seems to be the immunological hallmark of patients with progressive forms of alveolar echinococcosis (AE). IL-10-induced inhibition of effector macrophages, but also of antigen-presenting dendritic cells, may be operating and allowing parasite growth and survival. The genetic correlates of susceptibility to infection with E. multilocularis are clearer in humans than in the mouse model. A significant link between MHC polymorphism and clinical presentation of AE has been shown, and the spontaneous secretion of IL-10 in patients with a progressive AE is higher in patients with the HLA DR3+, DQ2+ haplotype. Clustering of cases in certain families, in communities otherwise exposed to similar risk factors, also points to immuno-genetic predisposition factors that may allow the larva to escape host immunity more easily. The first stage of larval development may be crucial in producing "danger signals" stimulating the initial production of cytokines. Therapeutic use of Interferon alpha is an attempt to foil the survival strategy of E. multilocularis

Imaging aspects and non-surgical interventional treatment in human alveolar echinococcosis.

International audienceAlveolar echinococcosis (AE) of the liver caused by the metacestode of the fox tapeworm Echinococcus multilocularis is characterized by a multivesicular structure surrounded by an extensive fibro-inflammatory host reaction. The lesions behave like a slow-growing liver cancer, without sharp limits between the parasitic tissue and the liver parenchyma. Invasion of biliary and vascular walls is another hallmark of this severe disease. Moreover, the poor vascularization of the parasitic mass often leads to necrosis in the central part of the lesion. This explains why liver abscess due to superimposed bacterial infection of the necrotic area may occur in this disease. Currently, a range of imaging techniques can be used at the different stages of management of AE. For diagnosis, ultrasonography remains the first line examination. For a more accurate disease evaluation, aiming to guide the surgical strategy, computerized tomography, Magnetic Resonance (MR) imaging, including cholangio-MR imaging are of importance, providing useful complementary information. More recently, Positive-Emission Tomography using [18F] fluoro-deoxyglucose has been developed for the follow-up of inoperable AE patients under long-term benzimidazoles therapy. This approach seems very promising to assess inflammatory activity and thereby to indirectly depict parasitic activity. Non-surgical interventional procedures, mainly percutaneous biliary and/or centro-parasitic abscesses drainages, are currently a major aspect in the care of incurable AE patients and have largely contributed to the improvement of survival in this situation during the past 20 years. They may also be used as a bridge before a curative surgical procedure in symptomatic patients presenting a life-threatening bacterial and/or fungal infection. It is also very useful in inoperable patients to overcome similar infectious episodes

Em+ Elisa: a serological test able to predict long term outcome in inoperable alveolar echinococcosis patients under benzimidazole therapy

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L’échinococcose alvéolaire humaine en France en 2010 / Box – Human alveolarechinococcosis in France, update 2010

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Echinococcoe alvéolaire

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Diagnosis and treatment of alveolar echinococcosis of the liver in immunocompromised patients

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