title: Clinical features and evolution of alveolar echinococcosis in France from 1982 to 2007: results of a survey in 387 patients.
abstract: OBJECTIVE
  Alveolar echinococcosis (AE) is a rare disease in humans, caused by the larval stage of the fox tapeworm Echinococcus multilocularis.
  
  
  METHODS
  We present here 387 detailed AE cases diagnosed in France from 1982 to 2007 actively identified by a retrospective survey performed in 1997-1998 and prospectively thereafter.
  
  
  RESULTS
  Male:female ratio was 1.03 and mean age 57.8 years at time of diagnosis. Among the 362 complete files (including 347 non dead-out and 15 dead-out lesions), 73% of the patients were symptomatic at first admittance. Among them, 83% presented with clinical patterns evocative either of a digestive or a hepatic disorder. Other symptomatic patients presented with erratic clinical pictures, generally due to metastasis or extra-hepatic location of the parasite. Except for a few patients with particularly severe AE who died shortly after the diagnosis, most patients were treated using benzimidazoles. Their mortality tends to merge with that of the general French population, matched by sex, age, and calendar year. This study also highlights an unexpectedly high frequency of blood-tied family cases (13% of patients submitted to a specific questionnaire).
  
  
  CONCLUSIONS
  Even though the broad set of clinical features provoked by E. multilocularis makes AE a potential diagnostic trap for many physicians, our study revealed an improvement of its prognosis. However, as shown by our findings about the frequency of family cases, there is still a need for studies aimed at better describing this uncommon parasitic disease.
authors.affiliation: TheMA, UMR CNRS 6049, Université de Franche-Comté, 25030 Besançon, France.
authors.forename: Martine
authors.initials: M
authors.lastname: Piarroux
authors.forename: Renaud
authors.initials: R
authors.lastname: Piarroux
authors.forename: Roch
authors.initials: R
authors.lastname: Giorgi
authors.forename: Jenny
authors.initials: J
authors.lastname: Knapp
authors.forename: Karine
authors.initials: K
authors.lastname: Bardonnet
authors.forename: Bertrand
authors.initials: B
authors.lastname: Sudre
authors.forename: Jérôme
authors.initials: J
authors.lastname: Watelet
authors.forename: Jérôme
authors.initials: J
authors.lastname: Dumortier
authors.forename: Alain
authors.initials: A
authors.lastname: Gérard
authors.forename: Jean
authors.initials: J
authors.lastname: Beytout
authors.forename: Armand
authors.initials: A
authors.lastname: Abergel
authors.forename: Georges
authors.initials: G
authors.lastname: Mantion
authors.forename: Dominique A
authors.initials: DA
authors.lastname: Vuitton
authors.forename: Solange
authors.initials: S
authors.lastname: Bresson-Hadni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D000871:Anthelmintics / Q000627:therapeutic use; D001562:Benzimidazoles / Q000627:therapeutic use; D004444:Echinococcosis, Hepatic / Q000175:diagnosis* / Q000453:epidemiology* / Q000469:parasitology / Q000628:therapy; D048210:Echinococcus multilocularis; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D000871:Anthelmintics; D001562:Benzimidazoles