Long-term (over 22 years) follow-up of four familial cases of Caroli's disease.
The authors report a long-term (over 22 years) follow-up of four familial cases, 1 brother and 3 sisters, affected by classic Caroli's disease. The male patient experienced acute cholangitis at the age of 6 and 1 of the 3 sisters at 12 years. The other two sisters were asymptomatic at the time of diagnosis. At the last control, all the patients were alive and have remained symptom-free for more than 22 years. Nevertheless, some data suggest a slowly evolving impairment of the disease even in the absence of a clinical expression. In this paper, the ways in which Caroli's disease is inherited and managed are also discussed.
Divisione di Chirurgia Pediatrica, Ospedale Regionale di Vicenza, Italy.
G
G
Belloli
F
F
Battaglino
C
C
Guglielmini
F
F
Cappellari
D000328:Adult; D016767:Caroli Disease / Q000000981:diagnostic imaging / Q000235:genetics* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging; D008297:Male; D013997:Time Factors; D014463:Ultrasonography