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Alveolar echinococcosis

Orpha number ORPHA284
Prevalence of rare diseases 1-9 / 1 000 000
Inheritance
  • Sporadic
Age of onset Variable
ICD 10 code
  • B67.5
MIM number -
Synonym(s) -

Summary

Alveolar hydatid disease is caused by the larvae of the tenia Echinococcus multilocularis. It is only found in the most northern parts of the hemisphere (Alaska, Canada, Siberia, Northern China, and Japan), and in Central Europe. In France, it has been reported in the East (Lorraine, Franche-Comté) and in the Massif Central. Infection is due to the ingestion of wild berries or the handling of fox cadavers. It occurs in rural areas and it affects men and women alike. Symptoms appear 5 to 15 years after infection, and resemble those of liver cancer. Diagnosis is based on clinical signs and serology. Spontaneous progression is severe. Treatment relies upon benzimidazoles, mainly albendazole, and surgery. *Author: L. Paris, M.D. (February 2005)*.

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