Objective To investigate the clinical features of patients with Caroli disease. Methods The clinical data were collected from41 patients who were diagnosed with Caroli disease in The Fifth Medical Center of Chinese PLA General Hospital from April 2015 to January2020,and the patients were divided into type I group with 16 patients and type Ⅱ group with 25 patients. A retrospective analysis was performed for general information,laboratory markers,and clinical features. The independent samples t-test was used for comparison of normally distributed continuous data between groups,and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between groups; the chi-square test was used for comparison of categorical data; a Spearman correlation analysis was also performed. Results The type Ⅰ group had a significantly higher level of albumin( Alb) than the type Ⅱ group( t = 0. 976,P = 0. 048),and the type Ⅱ group had a significantly higher prothrombin time( PT) than the type I group( Z = 3. 115,P = 0. 001). Compared with the type I group,the type Ⅱ group had significantly higher incidence rates of esophageal and gastric varices,upper gastrointestinal bleeding and/or tarry stool,and portal hypertension( χ2= 6. 077,5. 468,and 2. 403,P = 0. 002,0. 019,and 0. 028). In the patients with type Ⅱ Caroli disease,the level of cholinesterase was negatively correlated with the incidence rates of esophageal and gastric varices and portal hypertension( r =-0. 468 and-0. 436,P = 0. 018 and 0. 029); Alb level was negatively correlated with the incidence rate of esophageal and gastric varices( r =-0. 561,P = 0. 004); red blood cell count was negatively correlated with the incidence rates of esophageal and gastric varices,upper gastrointestinal bleeding and/or tarry stool,and portal hypertension( r =-0. 662,-0. 566,and-0. 436,P < 0. 001,P = 0. 003,and P = 0. 029); hemoglobin count was negatively correlated with the incidence rates of esophageal and gastric varices,upper gastrointestinal bleeding and/or tarry stool,and portal hypertension( r =-0. 605,-0. 590,and-0. 510,P = 0. 001,0. 002,and 0. 009); PT was positively correlated with the incidence rates of esophageal varices and portal hypertension( r = 0. 488 and 0. 520,P = 0. 013 and 0. 008).Conclusion Compared with the patients with type I Caroli disease,the patients with type Ⅱ Caroli disease have a higher incidence rates of esophageal and gastric varices,upper gastrointestinal bleeding and/or tarry stool,and portal hypertension,with the changes in clinical indicators such as the decrease of Alb level and the increase of PT level,and they tend to have poor prognosis.
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