How is liver pox sex nucleus denaturation treated

summary

Most people don't know about hepatopox degeneration, and few people encounter it. However, we still need to understand it. Hepatolenticular degeneration was first described by Wilson in 1912, so it is also called Wilson's disease. Hepatolenticular degeneration is an autosomal recessive disorder of copper metabolism. It is characterized by liver cirrhosis and basal ganglia damage caused by copper metabolism disorder. The understanding of the pathogenesis of hepatolenticular degeneration has reached the molecular level. Let's share some experience.

How is liver pox sex nucleus denaturation treated

First: D-penicillamine (PCA): it is the first choice drug for this disease. It is a powerful metal chelator. It can form a non-toxic complex with copper in the liver, which can promote its clearance in the tissue deposition site and reduce the toxicity of free copper. Penicillamine reacts with the same kind of daughter complex in tissue to form copper penicillamine complex, which is excreted from urine. It should be easy to absorb by oral administration. Side effects include nausea, allergic reaction, myasthenia gravis, arthropathy, no blister, a few can cause leukopenia and aplastic anemia. Optic neuritis, lupus syndrome. Exfoliative dermatitis, nephrotic syndrome and other serious side effects. In addition, penicillamine skin test should be done when patients first use the drug, and negative patients can use it. It is necessary to take medicine for a long time or even for a lifetime, and sufficient vitamin B should be supplemented.

Second: dimercaptopropanesulfonic acid (DMPS): DMPS 5mg / kg dissolved in 5% glucose solution 500ml, intravenous drip slowly, once a day, 6 days as a course of treatment, 1-2 days rest between two courses, continuous injection for 6-10 courses. The main adverse reactions were anorexia, mild nausea and vomiting. It can be used in patients with mild, moderate and severe liver damage and neuropsychiatric symptoms.

Third: triethylene hydroxyl tetramethylamine (TetA): the pharmacological effect is similar to that of D-penicillamine, which is the main drug used in the treatment of intolerance to penicillamine. The side effect is small, but the drug source is difficult and the price is expensive.

matters needing attention

When there is tremor and myotonia, it can be taken orally with benhexol. Clonazepam is the first choice for patients with gross tremor. Dystonia can be treated with benhexol, compound levodopa preparation, dopamine receptor agonist, clonazepam, nitrazepam, baclofen, and drugs for limited dystonia. Local injection of botulinum toxin type a can be used in patients with no response. Clonazepam, nitrazepam, haloperidol and benhexol can be used for choreographic movements and hand foot hyperactivity disorder. Antipsychotic drugs such as perphenazine, risperidone, haloperidol and clozapine can be used for patients with obvious mental symptoms, and antidepressants can be used for patients with depression. Drugs for liver protection should also be used for a long time.