Wilson＇s disease （WD）, or hepatolenticular degeneration, is an autosomal recessive inheritance disorder of copper metabolism caused by ATP7B gene mutation. As WD is an inherited disease of the nervous system that i...Wilson＇s disease （WD）, or hepatolenticular degeneration, is an autosomal recessive inheritance disorder of copper metabolism caused by ATP7B gene mutation. As WD is an inherited disease of the nervous system that is not curable; early diagnosis with early and life-long treatment leads to better prognoses. Currently, the recommended treatment for WD is integrated Chinese and Western medicine. A number of studies indicate that treatment of integrative medicine can not only enforce the de-copper effect but also improve liver function, intelligence, and other factors. This article reviewed in detail the advantages of WD treated with Chinese and Western medicine together.展开更多
Gandou decoction(GDD),a well-known traditional Chinese medicine(TCM)formula,has been widely used for decades to treat Wilson's disease(WD)in China due to its remarkable clinical effects.However,the chemical consti...Gandou decoction(GDD),a well-known traditional Chinese medicine(TCM)formula,has been widely used for decades to treat Wilson's disease(WD)in China due to its remarkable clinical effects.However,the chemical constituents of GDD still remain unclear because of their complexity.In this work,a reliable and sensitive strategy based on ultra-performance liquid chromatography coupled with quadrupole time-of-flight tandem mass spectrometry(UPLC-Q-TOF-MSE)and UNIFI informatics platform was applied to investigate the chemical components in GDD.In total,96 compounds including anthraquinones,alkaloids,protostane triterpenoids,flavonoids,triterpenoid saponins,tannins,curcuminoids,etc,were identified or tentatively characterized from GDD by comparing their retention time,accurate mass within 5 ppm error and MSE fragmentation patterns.Among them,eleven compounds were confirmed unambiguously with reference standards.Representative compounds in different chemical structure types were analyzed in fragmentation patterns and characteristic ions.Moreover,to better understand the chemical contribution of individual herbs to the whole decoction,the corresponding each herb in GDD was also detected.This study developed a rapid method for characterizing the chemical constituents in GDD,which could not only be used for chemical standardization and quality control,but also be helpful for further research of GDD in vivo.展开更多
目的探讨5羟色胺(5-HT)、褪黑素(MT)、白介素(IL)-1β、巨噬细胞移动抑制因子(MIF)与肝豆状核变性(Wilson s disease,WD)抑郁障碍的相关性,及肝豆汤(GDD)干预治疗对其的影响。方法采用回顾性研究方法,选取安徽省中医院脑病科2017年1月至...目的探讨5羟色胺(5-HT)、褪黑素(MT)、白介素(IL)-1β、巨噬细胞移动抑制因子(MIF)与肝豆状核变性(Wilson s disease,WD)抑郁障碍的相关性,及肝豆汤(GDD)干预治疗对其的影响。方法采用回顾性研究方法,选取安徽省中医院脑病科2017年1月至2020年12月收治的92例湿热内蕴型WD伴抑郁障碍患者,进行大体评定量表(GAS)及汉密尔顿抑郁量表-24(HDRS-24)评分;选取同期健康体检者40例为健康对照组。ELISA法测定研究对象血清5-HT、MT、IL-1β、MIF水平,并分析WD抑郁障碍患者四项指标水平与GAS评分及HDRS-24评分的相关性。92例患者中,予二巯基丙磺酸钠(DMPS)驱铜治疗的42例为对照组;予GDD+DMPS治疗的50例为GDD组,分析GDD治疗对患者血清5-HT、MT、IL-1β、MIF水平、GAS及HDRS-24评分的影响。结果与健康对照组比较,WD抑郁障碍患者血清5-HT、MT水平下降明显,IL-1β、MIF水平上升显著(P<0.01)。GAS、HDRS-24分别与5-HT、MT呈负相关,与IL-1β、MIF呈正相关(P<0.01)。联合GDD治疗后WD抑郁障碍患者5-HT、MT水平上升显著,IL-1β、MIF下降明显(P<0.01)。治疗4周后,两组HDRS-24评分均降低,GDD组GAS评分降低(P<0.01)。与对照组相比,治疗后GDD组的GAS和HDRS-24评分均更低(P<0.05,P<0.01)。结论5-HT、MT、IL-1β、MIF可考虑作为评估WD抑郁障碍及WD脑损害程度的生物学指标,驱铜治疗基础上联合GDD干预对改善WD抑郁障碍具有重要意义。展开更多
目的:探讨肝豆汤治疗湿热内蕴型Wilson病(WD)伴快动眼期睡眠行为障碍(RBD)的疗效。方法:从2019年4月至2023年8月在安徽中医药大学第一附属医院脑病科入院患者中,选取符合纳入标准的湿热内蕴型WD伴RBD患者62例,由计算机分发器随机分为对...目的:探讨肝豆汤治疗湿热内蕴型Wilson病(WD)伴快动眼期睡眠行为障碍(RBD)的疗效。方法:从2019年4月至2023年8月在安徽中医药大学第一附属医院脑病科入院患者中,选取符合纳入标准的湿热内蕴型WD伴RBD患者62例,由计算机分发器随机分为对照组、观察组各31例。对照组予以常规驱铜治疗,观察组在对照组基础上加用肝豆汤治疗。8 d为1个疗程,共3个疗程。比较两组治疗前、后的中医证候积分、RBD筛查问卷(RBDSQ)评分、RBD筛查问卷香港版(RBDQ-HK)评分、多导睡眠图(PSG)参数、24 h尿铜(24 h U-Cu)水平和游离铜(NCC)水平,观察不良反应。结果:最终完成试验病例60例,对照组和观察组各30例。治疗前,两组各项指标比较差异无统计学意义,资料具有可比性。与本组治疗前比较,两组患者中医证侯积分、RBDSQ和RBDQ-HK评分明显降低,24 h U-Cu水平明显增高,NCC水平明显降低(P<0.05,P<0.01);与对照组比较,中医证侯积分、RBDSQ、RBDQ-HK评分和NCC水平观察组改善更优(P<0.05,P<0.01)。与本组治疗前比较,两组总睡眠(TST)时间、睡眠效率(SE)、入睡/快速眼动(REM)潜伏期、N1/N2/REM期占比、微觉醒指数(ARI)和时相性肌电图活动(P-EMG-A)占比明显改善(P<0.05)。与对照组治疗后比较,观察组TST、SE、REM期占比、ARI和P-EMG-A占比改善更明显(P<0.05)。结论:肝豆汤不仅可以改善湿热内蕴型WD伴RBD患者的中医证候,还可以减轻患者的RBD症状。展开更多
文摘Wilson＇s disease （WD）, or hepatolenticular degeneration, is an autosomal recessive inheritance disorder of copper metabolism caused by ATP7B gene mutation. As WD is an inherited disease of the nervous system that is not curable; early diagnosis with early and life-long treatment leads to better prognoses. Currently, the recommended treatment for WD is integrated Chinese and Western medicine. A number of studies indicate that treatment of integrative medicine can not only enforce the de-copper effect but also improve liver function, intelligence, and other factors. This article reviewed in detail the advantages of WD treated with Chinese and Western medicine together.
基金This research was supported by the National Natural Science Foundation of China(No:81872976,81873038,81303245)Key Project of the National Science Fund of Anhui Province(No:KJ2016A398)+1 种基金Key Project Foundation of Oversea Visiting and Research for the Excellent Young and Middle-aged Faculties in Universities of Anhui Province(No:gxgwfx2018042)Primary Research&Development Plan of Anhui Province(No:201904a07020051).
文摘Gandou decoction(GDD),a well-known traditional Chinese medicine(TCM)formula,has been widely used for decades to treat Wilson's disease(WD)in China due to its remarkable clinical effects.However,the chemical constituents of GDD still remain unclear because of their complexity.In this work,a reliable and sensitive strategy based on ultra-performance liquid chromatography coupled with quadrupole time-of-flight tandem mass spectrometry(UPLC-Q-TOF-MSE)and UNIFI informatics platform was applied to investigate the chemical components in GDD.In total,96 compounds including anthraquinones,alkaloids,protostane triterpenoids,flavonoids,triterpenoid saponins,tannins,curcuminoids,etc,were identified or tentatively characterized from GDD by comparing their retention time,accurate mass within 5 ppm error and MSE fragmentation patterns.Among them,eleven compounds were confirmed unambiguously with reference standards.Representative compounds in different chemical structure types were analyzed in fragmentation patterns and characteristic ions.Moreover,to better understand the chemical contribution of individual herbs to the whole decoction,the corresponding each herb in GDD was also detected.This study developed a rapid method for characterizing the chemical constituents in GDD,which could not only be used for chemical standardization and quality control,but also be helpful for further research of GDD in vivo.
文摘目的探讨5羟色胺(5-HT)、褪黑素(MT)、白介素(IL)-1β、巨噬细胞移动抑制因子(MIF)与肝豆状核变性(Wilson s disease,WD)抑郁障碍的相关性,及肝豆汤(GDD)干预治疗对其的影响。方法采用回顾性研究方法,选取安徽省中医院脑病科2017年1月至2020年12月收治的92例湿热内蕴型WD伴抑郁障碍患者,进行大体评定量表(GAS)及汉密尔顿抑郁量表-24(HDRS-24)评分;选取同期健康体检者40例为健康对照组。ELISA法测定研究对象血清5-HT、MT、IL-1β、MIF水平,并分析WD抑郁障碍患者四项指标水平与GAS评分及HDRS-24评分的相关性。92例患者中,予二巯基丙磺酸钠(DMPS)驱铜治疗的42例为对照组;予GDD+DMPS治疗的50例为GDD组,分析GDD治疗对患者血清5-HT、MT、IL-1β、MIF水平、GAS及HDRS-24评分的影响。结果与健康对照组比较,WD抑郁障碍患者血清5-HT、MT水平下降明显,IL-1β、MIF水平上升显著(P<0.01)。GAS、HDRS-24分别与5-HT、MT呈负相关,与IL-1β、MIF呈正相关(P<0.01)。联合GDD治疗后WD抑郁障碍患者5-HT、MT水平上升显著,IL-1β、MIF下降明显(P<0.01)。治疗4周后,两组HDRS-24评分均降低,GDD组GAS评分降低(P<0.01)。与对照组相比,治疗后GDD组的GAS和HDRS-24评分均更低(P<0.05,P<0.01)。结论5-HT、MT、IL-1β、MIF可考虑作为评估WD抑郁障碍及WD脑损害程度的生物学指标,驱铜治疗基础上联合GDD干预对改善WD抑郁障碍具有重要意义。
文摘目的:探讨肝豆汤治疗湿热内蕴型Wilson病(WD)伴快动眼期睡眠行为障碍(RBD)的疗效。方法:从2019年4月至2023年8月在安徽中医药大学第一附属医院脑病科入院患者中,选取符合纳入标准的湿热内蕴型WD伴RBD患者62例,由计算机分发器随机分为对照组、观察组各31例。对照组予以常规驱铜治疗,观察组在对照组基础上加用肝豆汤治疗。8 d为1个疗程,共3个疗程。比较两组治疗前、后的中医证候积分、RBD筛查问卷(RBDSQ)评分、RBD筛查问卷香港版(RBDQ-HK)评分、多导睡眠图(PSG)参数、24 h尿铜(24 h U-Cu)水平和游离铜(NCC)水平,观察不良反应。结果:最终完成试验病例60例,对照组和观察组各30例。治疗前,两组各项指标比较差异无统计学意义,资料具有可比性。与本组治疗前比较,两组患者中医证侯积分、RBDSQ和RBDQ-HK评分明显降低,24 h U-Cu水平明显增高,NCC水平明显降低(P<0.05,P<0.01);与对照组比较,中医证侯积分、RBDSQ、RBDQ-HK评分和NCC水平观察组改善更优(P<0.05,P<0.01)。与本组治疗前比较,两组总睡眠(TST)时间、睡眠效率(SE)、入睡/快速眼动(REM)潜伏期、N1/N2/REM期占比、微觉醒指数(ARI)和时相性肌电图活动(P-EMG-A)占比明显改善(P<0.05)。与对照组治疗后比较,观察组TST、SE、REM期占比、ARI和P-EMG-A占比改善更明显(P<0.05)。结论:肝豆汤不仅可以改善湿热内蕴型WD伴RBD患者的中医证候,还可以减轻患者的RBD症状。
