Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilso...Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilson s disease presenting as pigmented gallstone pancreatitis have not been reported in the literature.In the present report,we describe a case of a 37-year-old man who was admitted with jaundice and abdomina pain.The patient was diagnosed with acute pancreatitis,cholangitis,and obstructive jaundice caused by pigmented gallstones that were detected during retrograde cholangiopancreatography.However,because of his long-term jaundice and the presence of pigmented gallstones,the patient underwent further evaluation for Wilson’s disease,which was subsequently confirmed.This patient’s unique presentation exemplifies the overlap in the clinical and laboratory parameters of Wilson’s disease and cholestasis,and the difficulties associated with their differentiation.It suggests thatWilson’s disease should be considered in patients with pancreatitis,cholangitis,and severe protracted jaundice caused by pigmented gallstones.展开更多
目的:研究辅助诊断肝型Wilson病(WD)患者肝纤维化无创诊断技术指标的水平及其检测意义。方法:回顾分析2021年8月~2022年9月在安徽中医药大学神经病学研究所附属医院住院的126例肝型与脑型WD患者,检测并比较两型患者的肝纤维化无创诊断...目的:研究辅助诊断肝型Wilson病(WD)患者肝纤维化无创诊断技术指标的水平及其检测意义。方法:回顾分析2021年8月~2022年9月在安徽中医药大学神经病学研究所附属医院住院的126例肝型与脑型WD患者,检测并比较两型患者的肝纤维化无创诊断技术指标的水平,包括肝脏B超单点剪切波弹性成像(pSWE)值、血清肝纤维化指标[透明质酸(HA)、层黏蛋白(LN)、Ⅳ型胶原(CⅣ)、Ⅲ型前胶原N端肽(PⅢNP)]及肝纤维化无创模型指数[天冬氨酸氨基转移酶和血小板比率指数(APRI)、肝纤维化4因子指数(FIB-4)、γ-谷氨酰转肽酶和血小板比值(GPR)、De-Ritis指数、哥德堡大学肝硬化指数(GUCI)评分及King评分]。结果:肝型WD与脑型比较pSWE[2.2(1.5,2.5)m/s vs 1.3(1.2,1.7)m/s]、HA[148.4(50.3,426.1)ng/ml vs 50.0(50.0,90.1)ng/ml]、LN[72.5(43.9,128.4)ng/ml vs 40.4(30.9,47.7)ng/ml]、CⅣ[168.7(92.2,327.0)ng/ml vs 66.9(55.5,88.4)ng/ml]、PⅢNP[13.0(9.4,23.6)ng/ml vs 8.8(6.3,10.4)ng/ml]、APRI[1.1(0.6,2.1)vs 0.3(0.2,0.5)]、FIB-4[1.4(0.8,3.5)vs 0.9(0.5,1.4)]、GPR[0.8(0.4,2.2)vs 0.2(0.1,0.4)]、GUCI评分[1.4(0.8,3.1)vs 0.4(0.3,0.7)]及King评分[13.9(7.0,33.7)vs 4.5(3.0,8.5)]均显著增加;De-Ritis指数在两组间差异无统计学意义(P>0.05)。结论:肝纤维化无创诊断技术指标(De-Ritis指数除外)均可用于辅助判断肝型WD的肝纤维化状况;三者联合运用,对及早发现肝型WD肝脏纤维化,防止发生严重肝病,具有重要临床价值。展开更多
文摘Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilson s disease presenting as pigmented gallstone pancreatitis have not been reported in the literature.In the present report,we describe a case of a 37-year-old man who was admitted with jaundice and abdomina pain.The patient was diagnosed with acute pancreatitis,cholangitis,and obstructive jaundice caused by pigmented gallstones that were detected during retrograde cholangiopancreatography.However,because of his long-term jaundice and the presence of pigmented gallstones,the patient underwent further evaluation for Wilson’s disease,which was subsequently confirmed.This patient’s unique presentation exemplifies the overlap in the clinical and laboratory parameters of Wilson’s disease and cholestasis,and the difficulties associated with their differentiation.It suggests thatWilson’s disease should be considered in patients with pancreatitis,cholangitis,and severe protracted jaundice caused by pigmented gallstones.
文摘目的:研究辅助诊断肝型Wilson病(WD)患者肝纤维化无创诊断技术指标的水平及其检测意义。方法:回顾分析2021年8月~2022年9月在安徽中医药大学神经病学研究所附属医院住院的126例肝型与脑型WD患者,检测并比较两型患者的肝纤维化无创诊断技术指标的水平,包括肝脏B超单点剪切波弹性成像(pSWE)值、血清肝纤维化指标[透明质酸(HA)、层黏蛋白(LN)、Ⅳ型胶原(CⅣ)、Ⅲ型前胶原N端肽(PⅢNP)]及肝纤维化无创模型指数[天冬氨酸氨基转移酶和血小板比率指数(APRI)、肝纤维化4因子指数(FIB-4)、γ-谷氨酰转肽酶和血小板比值(GPR)、De-Ritis指数、哥德堡大学肝硬化指数(GUCI)评分及King评分]。结果:肝型WD与脑型比较pSWE[2.2(1.5,2.5)m/s vs 1.3(1.2,1.7)m/s]、HA[148.4(50.3,426.1)ng/ml vs 50.0(50.0,90.1)ng/ml]、LN[72.5(43.9,128.4)ng/ml vs 40.4(30.9,47.7)ng/ml]、CⅣ[168.7(92.2,327.0)ng/ml vs 66.9(55.5,88.4)ng/ml]、PⅢNP[13.0(9.4,23.6)ng/ml vs 8.8(6.3,10.4)ng/ml]、APRI[1.1(0.6,2.1)vs 0.3(0.2,0.5)]、FIB-4[1.4(0.8,3.5)vs 0.9(0.5,1.4)]、GPR[0.8(0.4,2.2)vs 0.2(0.1,0.4)]、GUCI评分[1.4(0.8,3.1)vs 0.4(0.3,0.7)]及King评分[13.9(7.0,33.7)vs 4.5(3.0,8.5)]均显著增加;De-Ritis指数在两组间差异无统计学意义(P>0.05)。结论:肝纤维化无创诊断技术指标(De-Ritis指数除外)均可用于辅助判断肝型WD的肝纤维化状况;三者联合运用,对及早发现肝型WD肝脏纤维化,防止发生严重肝病,具有重要临床价值。
