Analysis on clinical characteristics and pathological characteristics of 6 cases of congenital liver fibrosis
10.3969/j.issn.1671-8348.2024.20.003
- VernacularTitle:6例先天性肝纤维化患者的临床特征及病理学特点分析
- Author:
Yubao XIE
1
;
Junmin JIANG
;
Huanming XIAO
;
Meijie SHI
;
Pengtao ZHAO
;
Yingxian LI
;
Xiaoling CHI
Author Information
1. 广州中医药大学第二附属医院/广东省中医院肝病科,广州 510120
- Keywords:
congenital hepatic fibrosis;
clinical features;
imaging;
pathology
- From:
Chongqing Medicine
2024;53(20):3055-3059,3064
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical features,imaging and pathological characteristics of the patients with congenital liver fibrosis(CHF).Methods The medical case data of 6 patients with pathological-ly diagnosed CHF in this hospital from January 2011 to June 2021 were retrospectively analyzed,and the clini-cal menifastations,laboratory indicators,imaging characteristics,pathological manifestations,treatment and outcomes were summarized.Results The clinical classification in 6 cases of CHF was mainly portal hyperten-sion(66.67％),and the most common clinical manifestations and signs were hepatosplenomegaly(83.33％),melena(50.00％)and abdominal distension(33.33％).Total bil irubin was normal,and only 2 cases(33.33％)were mildly elevated.The prothrombin time was in the normal range in 4 cases(66.67％),and mild abnormality in 2 cases(33.33％).Imaging showed abnormal liver morphology in 6 cases,spleen enlarge-ment in 5 cases(83.33％),portal vein widening in 3 cases(50.00％),diffuse dilatation of intrahepatic bile ducts in 2 cases(33.33％),and complicating Caroli disease in 1 case(16.67％).The pathological manifesta-tions were normal liver parenchymal cells,periportal fibrosis,and manifold-manifold bridging-like fibrosis.Six cases received the conventional liver protection therapy,3 cases underwent splenectomy+pericardia vascular dissection,the median follow-up time was 68.2 months,1 case died due to liver failure,and 5 cases were rela-tively stable.Conclusion The patients with hepatic disease whose portal hypertension is inconsistent to the degree of hepatic function damage,especially those complicating polycystic kidney disease should perfect the liver puncture pathological examination and genetic testing to clarify the diagnosis,and conduct the genetic counseling and intervention treatment as soon as possible.
