摘要
肝豆状核变性是以铜代谢障碍为核心,累及肝脏、肾脏及脑部的常染色体隐性遗传病。中医学认为,该病与“肝-肾-脑”轴功能失调密切相关。肝、肾、脑通过精血互化输布、气化枢机协同、经隧络属贯通,形成生理整体。本文基于“肝-肾-脑”轴阐释肝豆状核变性的病机,肝肾精亏致脑髓失养为发病根本,铜毒内蕴化生湿热、痰瘀互结为致病关键。针对本虚标实的疾病特点,确立标本动态兼治原则:治标以驱铜解毒为先导,配合清热化湿、祛痰化瘀;治本遵循乙癸同源理念,强调肝肾同治以滋髓养脑。施治全程需兼顾驱铜(治标),并按病变重心分层施策,或治肝为主,辅以益肾填髓;或治肾为主,佐以调肝荣脑;或肝、肾、脑三脏同调,实现祛邪复正、通补兼施的治疗目标。
Hepatolenticular degeneration is an autosomal recessive hereditary disease characterized by copper metabolism disorder,which affects the liver,kidneys,and brain.In traditional Chinese medicine,this disease is closely associated with dysfunction of the"liver-kidney-brain"axis.The liver,kidney,and brain form a physiological whole through the mutual transformation and distribution of essence and blood,the coordination of qi transformation,and the connection of meridians and collaterals.This article explores the pathogenesis of hepatolenticular degeneration based on the"liver-kidney-brain"axis.The deficiency of liver and kidney essence leads to the malnutrition of brain marrow,which is the fundamental cause of the disease.The internal accumulation of copper toxins generates dampness-heat and phlegm-stasis,which are the key factors causing the disease.In view of the characteristics of this disease with deficiency in nature and excess in superficiality,the principle of dynamic treating both manifestation and root cause of disease is established:treating the manifestation starts with eliminating copper and detoxifying,combined with clearing heat and dampness,and removing phlegm and stasis;treating the root cause follows the concept of the same origin of yi and gui,emphasizing the simultaneous treatment of liver and kidney to nourish the brain marrow.Throughout the treatment process,copper elimination(treating the symptoms)should be taken into account,and stratified measures should be applied based on the primary focus of the lesion:either treating the liver as the main focus and supplemented by tonifying the kidney and filling the marrow;or treating the kidney as the main focus and assisted by regulating the liver and nourishing the brain;or co-regulating the three zang-organs of liver,kidney,and brain,to achieve the therapeutic goal of eliminating pathogenic factors and restoring normal functions,and combining tonification and purgation.
作者
饶志红
杨文明
杨玉龙
郝文杰
杨悦
刁珂
方淑珍
李雨辰
RAO Zhihong;YANG Wenming;YANG Yulong;HAO Wenjie;YANG Yue;DIAO Ke;FANG Shuzhen;LI Yuchen(The First Affiliated Hospital of Anhui University of Chinese Medicine,Hefei 230031,China;Center for Xin'an Medicine and Modernization of Traditional Chinese Medicine,Institute of Health and Medicine,Hefei Comprehensive National Science Center,Hefei 230012,China;Key Laboratory of Xin'an Medicine,Ministry of Education,Hefei 230012,China;Resource Center for Chinese Medicinal Materials,China Academy of Chinese Medical Sciences,Beijing 100700,China)
出处
《北京中医药大学学报》
北大核心
2025年第9期1270-1277,共8页
Journal of Beijing University of Traditional Chinese Medicine
基金
国家自然科学基金(区域创新发展联合基金)(No.U22A20366)
安徽省中医药科技攻关专项(No.202303a07020004)
安徽省临床医学研究转化专项(No.202204295107020066)。
关键词
肝豆状核变性
“肝-肾-脑”轴
标本兼治
hepatolenticular degeneration
"liver-kidney-brain"axis
treating both manifestation and root cause of disease
