先天性非溶血性黄疸的临床病理与中医证候特征  

Clinical Pathology and TCM Syndrome Features of Congenital Non-hemolytic Jaundice

作　　者：刘香丽 马素平[2] 王欣欣 张丽慧[2] 王娇[1] 杨晓青[3] LIU Xiangli;MA Suping;WANG Xinxin;ZHANG Lihui;WANG Jiao;YANG Xiaoqing(Department of Pathology,the First Affiliated Hospital of Henan University of Traditional Chinese Medicine,Zhengzhou 450000,China;Department of Spleen,Stomach and Hepatobiliary,the First Affiliated Hospital of Henan University of Traditional Chinese Medicine,Zhengzhou 450000,China;Paediatric Hospital Laboratory,the First Affiliated Hospital of Henan University of Traditional Chinese Medicine,Zhengzhou 450000,China;Beijing You’an Hospital,Beijing 100167,China)

机构地区：[1]河南中医药大学第一附属医院病理科,河南郑州450000 [2]河南中医药大学第一附属医院脾胃肝胆科,河南郑州450000 [3]河南中医药大学第一附属医院儿科医院实验室,河南郑州450000 [4]北京佑安医院病理科,北京100167

出　　处：《河南医学研究》2022年第23期4225-4229,共5页Henan Medical Research

基　　金：河南省科技攻关计划(222102310502);河南省中医药科学研究专项课题(2018JDZX004)。

摘　　要：目的分析先天性非溶血性黄疸的临床表现及病理特征,探讨中医证候、肝穿刺活检病理对临床诊断的指导意义。方法收集2014年4月至2019年10月河南中医药大学第一附属医院及北京佑安医院收治的以血清胆红素异常为主要表现的32例非病毒性、非溶血性黄疸患者,分为间接胆红素(IBIL)升高组和直接胆红素(DBIL)升高组,回顾性分析其病理特征、中医证候、肝功能及基因变化特点。结果IBIL升高组:Crigler-Najjar综合征Ⅱ型(CNS-Ⅱ)的血清IBIL高于Gilbert综合征(P<0.05);7例CNS-Ⅱ中2例出现纤维化;UGT1A1基因检测发现新的碱基突变点c.1471 G>A。DBIL升高组:Dubin-Johnson综合征特异的组织学表现为肝细胞质内棕黑色色素颗粒;进行性家族性肝内胆汁淤积症2型(PFIC2)组织学表现为淤胆明显且出现桥接纤维化。中医证候:IBIL升高组和DBIL升高组阳黄比率分别为44.44%、40.00%,阴黄比率分别为55.56%、60.00%,两组的阴黄占比均多于阳黄,但两组之间差异无统计学意义(P>0.05)。结论超声引导下经皮肝穿刺病理活检是诊断先天性非溶血性黄疸具体类型的重要检查方法,肝组织病理结果对诊断有重要意义;中医证候方面阴黄占比多于阳黄,对中医治疗具有一定的指导意义。Objective To analyze the clinical manifestations and pathological features of congenital non-hemolytic jaundice,and to explore the guiding significance of TCM syndromes and liver biopsy pathology for clinical diagnosis.Methods Thirty-two patients with non-viral and non-hemolytic jaundice with abnormal serum bilirubin as the main manifestation who were admitted to the First Affiliated Hospital of Henan University of Traditional Chinese Medicine and Beijing You’an Hospital from April 2014 to October 2019 were collected.The patients were divided into indirect bilirubin(IBIL)elevated group and direct bilirubin(DBIL)elevated group,and their pathological features,TCM syndromes,liver function and gene changes were retrospectively analyzed.Results IBIL elevated group:Crigler-Najjar syndrome typeⅡ(CNS-Ⅱ)serum IBIL was higher than Gilbert syndrome(P<0.05),2 out of 7 cases of CNS-Ⅱdeveloped fibrosis,and UGT1A1 gene detection revealed new Base mutation point c.1471 G>A.DBIL elevated group:Dubin-Johnson syndrome-specific histological manifestations were brown-black pigment particles in the cytoplasm of liver cells,and progressive familial intrahepatic cholestasis type 2(PFIC2)histological manifestations were obvious cholestasis and bridging fibrosis.In TCM syndromes,the proportion of Yanghuang in the elevated IBIL group and the elevated DBIL group was 44.44%and 40.00%,respectively,and the proportion of Yinhuang in the two groups was 55.56%and 60.00%,respectively.The proportion of Yinhuang in the two groups was more than that of Yanghuang,but there was no statistical difference between the groups(P>0.05).Conclusion Ultrasound-guided percutaneous liver biopsy is an important method for diagnosing specific types of congenital non-hemolytic jaundice.The pathological results of liver tissue are of great significance for diagnosis.In terms of TCM syndromes,the proportion of Yinhuang is more than that of Yanghuang,which has certain guiding significance for TCM treatment.

关 键 词：先天性 黄疸 临床病理 中医证候 

分 类 号：R272[医药卫生—中医儿科学]