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Table 2 Initial clinical presentations of CS patients

From: Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study

  Initial clinical presentation
Patients (%)
Initial clinical presentation leading to first clinical visit
Patients (%a)
Fever 6 (37.5%) 6 (100%)
 Fever + abdominal pain 3 (18.75%) 3 (100%)
Abdominal distention 8 (66.7%) 5 (62.5%)
 Fever + abdominal distention 1 (6.25%) 1 (100%)
Gastrointestinal bleeding 1 (6.25%) 1 (100%)
Fatigue 1 (6.25%) 1 (100%)
Other 1 (6.25%)b  
  1. CS Caroli syndrome
  2. a % of patients in whom the very initial clinical presentation led to the first clinical visit
  3. b The patient went to hospital due to upper respiratory infection and was found to have splenomegaly