化痰祛瘀法治疗肝豆状核变性研究进展

肝豆状核变性(WD)在西医学上是铜代谢障碍导致全身多器官损害等为主要临床特征的常染色体隐性单基因遗传疾病,病变影响肝、脑、肾、角膜等重要脏器。中医通过对WD患者临床症状及病理改变的认识,认为WD的基本病理因素为痰浊、瘀血,二者相互作用而发病,以痰瘀互结为主要的病机,贯穿疾病发展始终,故而运用化痰祛瘀法治疗WD在临床中常见。众多研究表明运用中药汤剂、中医外治法化痰祛瘀治疗可以有效缓解WD患者临床症状,发挥排铜优势、改善肝纤维化、改善脑流动力学、抑制炎症和抗氧化应激作用,进而延缓疾病进程,控制疾病发展。现结合新近研究成果,对化痰祛瘀法在WD的临床应用予以综述,以期为临床治疗WD提供中西医结合治疗有效的方案及依据。

Research advance in the treatment of hepatolenticular degeneration by dispelling phlegm and removing stasis

Hepatolenticular degeneration(WD)is an autosomal recessive single-gene genetic disease with major clinical features such as multiple organ damage caused by copper metabolism disorder.The disease affects important organs such as liver,brain,kidney and cornea.Based on the understanding of clinical symptoms and pathological changes of WD patients,traditional Chinese medicine believes that the basic pathological factors of WD are phlegm turbidity and blood stasis,which interact with each other to cause the onset of WD.Phlegm and blood stasis are the main pathogenesis throughout the development of the disease.Therefore,the treatment of WD by removing phlegm and blood stasis is common in clinical practice.Numerous studies have shown that the use of TCM decoction and TCM external treatment to remove phlegm and blood stasis can effectively relieve the clinical symptoms of WD patients,play the advantages of copper drainage,improve liver fibrosis,improve brain flow mechanics,inhibit inflammation and anti-oxidative stress,and then delay the disease process and control the development of the disease.Combined with recent research results,this paper summarizes the clinical application of eliminating phlegm and removing stasis in WD,in order to provide an effective program and basis for the clinical treatment of WD with the combination of traditional Chinese and western medicine.