Treatment of Hepatolenticular Degeneration with Chaihuang Gandou Powder
Hepatolenticular degeneration is an autosomal recessive inheritance disorder of copper metabolism. Its pathogenesis is copper metabolism disorder in the body results in large quantities of copper deposit in liver, brain and other tissues. The deposition site produces functional and structural damages. The main clinical manifestations are liver symptoms such as jaundice, ascites, liver cirrhosis etc. and extrapyramidal symptoms such as tremor, muscle rigidity, torsion spasm, asophia, dysphagia etc., which may seriously impact on the ability of life. If it cannot be diagnosed with prompt treatment early, patients may be dead within 2 to 6 years after the onset.
The treatment of this disease is as follows. Penicillamine is the drug with good curative effect but often leads to allergic. It has good curative effect in early stage but when the course is more than 2 years the effect is poor. It has side effects such as the bone marrow suppression, nephrotic syndrome, induced autoimmune disease etc. Although liver transplantation can thoroughly improve copper metabolization obstacle, sources of liver donor is difficult and surgery is difficult. Postoperative rejection reaction and expensive treatment costs have greatly limited the clinical application.
This disease belongs to the categories of "tremor", "madness" and "jaundice", "accumulation", "tympanites" etc. in traditional Chinese medicine. Modern Chinese medicine experts believe that it is caused by lack of innate endowment and disorders of the internal organs. All kinds of reports show it has a certain effect, but most are individual case reported, which cannot objectively reflect the effectiveness of the curative effects. Based on this, under the guidance of Professor Chen Jinliang, chief physician Wang Dianhua, our specialty is first to create the academic point of view on the theory of "toxin" theory for the treatment of the disease and put forward the pathological mechanism of “stagnation of liver, resistances of gallbladder, copper poison cohesion, collateral and stasis blocking". They have created the treatment principle of "dispersing stagnated liver qi for promoting bile flow, resolving phlegm and expelling of toxin, dispersing blood stasis and dredging collaterals". They select drugs with effects of dispersing stagnated liver qi, dredging the six hollow organs, expelling of toxin and dredging phlegm as composition and develop "Chaihuang Gandou Powder", which can treat the pathogenesis of "syndrome of stagnation of liver qi and resistances of gallbladder and copper toxicity cohesion" but also can treat the conditions of "collateral and stasis". Through hundreds of clinical observations, it has significant curative effects and the effective rate is 85.6%. At the same time combination with acupuncture therapy, rehabilitation training and low copper diet can improve the efficacy.
If patients can get early diagnosis and regular treatment in time, they can have same life, study and work as the healthy people.
