[Epidemiology and systematics of cystic and alveolar hydatid disease]

Echinococcus multilocularis, a small tapeworm in foxes, has gained considerable public attention owing to its wide distribution in central Europe. Conversely, diagnosis and treatment of the disease have been significantly improved in recent years. Consequently, the incidence among human populations has remained stable and relatively low for many decades. In southern Germany, France (Franche Comté and Doubs), Austria and Switzerland, the annual incidence ranges between 0.02 and 1.4 new cases per 100,000 inhabitants. The relevance of human AE refers to the high lethality of the untreated disease. Therapy consists predominantly of radical surgery followed by continuous, long-term chemotherapy using albendazole (or mebendazole). The action of chemotherapy alone is parasitostatic rather than parasitocidal; nevertheless, clinical improvement is observed in > 80 % of such cases, including a significantly improved prognosis. Diagnosis relies predominantly on imaging procedures and serology. Immunodiagnosis must be performed early, especially when a preclinical diagnosis is desired upon exposure to infection. Thus, in the framework of seroepidemiological studies, first-time evidence was obtained about the phenomenon of natural resistance in non-diseased persons. This raised the question of a potential immunogenetic predisposition in certain infected persons. E. granulosus (cystic hydatid disease) is practically non-existent as autochthonous infection in central Europe now. Most clinical cases of E. granulosus infections are thus observed among persons who have immigrated from - predominantly - the mediterranean basin.