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Alveolar echinococcosis


Hépato-Gastro. Volume 16, Number 2, 111-28, mars-avril 2009, Mini-revue

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Author(s) : Solange Bresson-Hadni, Carine Richou, Eric Delabrousse, Dominique A Vuitton, Laurence Millon, Oleg Blagosklonov, Georges Mantion

Summary : Alveolar echinococcosis (AE) is a rare parasitic disease caused by the intra-hepatic growth of the larval stage of the cestode Echinococcus multilocularis. It is a potentially fatal, chronically progressive disease that is characterized by a long asymptomatic period in which development of an invasive tumour-like multi-vesiculated lesion occurs. Vascular and biliary structures, as well as adjacent organs, may be involved in the parasitic process. This disease is observed only in the Northern hemisphere. Carnivores, typically foxes, but also domestic dogs and cats are definitive hosts, harbouring the small adult tapeworms in their intestine. Many species of rodents act as intermediate hosts, allowing the liver development of the metacestode. Human is an aberrant intermediate host, contaminated either by eating non-cooked vegetables and berries contaminated by faeces of infected carnivores or by touching such animals. New factors, such as increasing fox populations, invasion of cities by foxes, seem to currently modify the epidemiology of AE and to cause extension of the disease in new areas. Efficient drugs able to destroy the larva are currently lacking. A partial surgical resection is the only curative treatment in case of a rather limited disease, a situation which becomes more frequent due to the wide use of abdominal ultrasonography. Liver transplantation may be exceptionally indicated in patients with life-threatening complications. Interventional radiological procedures, mainly per-cutaneous biliary and/or centro-parasitic abscesses drainages, currently play a major role in the management of incurable AE patients or as a bridge to radical resection. In all cases, parasitostatic benzimidazole therapy, using albendazole, is mandatory for at least two years after curative resection and lifelong in inoperable patients. All these acquisitions have substantially improved the prognosis of AE patients. Finally, the immunopathogenesis of this disease is becoming better understood. Genetic characteristics are involved in susceptibility/resistance to E. multilocularis metacestode. Tolerance towards E. multilocularis is influenced by the parasite itself, especially through carbohydrate antigens of its laminated layer. It is especially operating via “anti-inflammatory-tolerogenic” cytokines IL-10 and TGF-β. Interferon-α restores a host-defence by modulating cytokine balance and allows clinicians to consider therapeutic immunomodulation for AE in the future.

Keywords : alveolar echinococcosis, Echinococcus multilocularis, liver parasitosis, benzimidazoles, albendazole, surgery, interventional radiology

 

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