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.
Cox比例风险模型结果显示:在校正年龄、性别、HDL-C、TG、ALT、Hs-CRP、家庭收入、受教育程度后,CHS每增加1分,NAFLD的发病风险比为0.73(0.72~0.74)。CHS与NAFLD发病风险的剂量-反应关系,RCS结果表明(取25%、50%和75% 3个截点):CHS连续型变量与NAFLD呈非线性关系(P<0.05)。将CHS的不同分组代入模型,校正相同的混杂因素后,以Q1组为对照组,Q2组、Q3组、Q4组发生NAFLD的风险逐渐降低(P for trend<0.05),Q2组发病风险比为0.78(0.74~0.81),Q 3组发病风险比为0.57(0.54~0.59),Q4组发病风险比为0.38(0.36~0.41)。在男女性及年龄的分层分析中,也得到了类似的结果(P for trent<0.05)(表 2)。
表
2
心血管健康评分影响NAFLD发病的Cox风险比例模型
项目
例数
发病例数
发病率(/千人年)
HR(95%CI)
P值
总体
Q1组
10 057
4104
77.88
Q2组
17 732
5956
61.33
0.78(0.74~0.81)
<0.001
Q3组
10 321
2737
46.37
0.57(0.54~0.59)
<0.001
Q4组
12 401
2468
33.69
0.38(0.36~0.41)
<0.001
性别
<0.0011)
男
Q1组
9336
3655
73.94
Q2组
14 723
4492
54.84
0.77(0.73~0.80)
<0.001
Q3组
7492
1725
39.79
0.57(0.53~0.60)
<0.001
Q4组
6402
1097
28.92
0.52(0.39~0.45)
<0.001
女
Q1组
721
449
137.57
Q2组
3009
1464
96.32
0.79(0.71~0.88)
<0.001
Q3组
2829
1012
64.65
0.56(0.50~0.63)
<0.001
Q4组
5999
1371
38.81
0.37(0.33~0.42)
<0.001
年龄
<0.0011)
≤45岁
Q1组
3106
1164
67.79
Q2组
5584
1773
55.02
0.83(0.77~0.90)
<0.001
Q3组
3751
890
39.91
0.58(0.53~0.64)
<0.001
Q4组
6192
1016
27.14
0.37(0.34~0.41)
<0.001
>45岁
Q1组
6951
2940
82.75
Q2组
12 148
4183
64.46
0.76(0.72~0.79)
<0.001
Q3组
6570
1847
50.29
0.56(0.53~0.60)
<0.001
Q4组
6209
1452
40.54
0.42(0.39~0.45)
<0.001
注:模型校正了年龄、性别、HDL-C、TG、ALT、Hs-CRP、家庭收入、受教育程度。1)P for interaction<0.001。
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