Penicillamine is a breakdown product of penicillin, which is an amino acid containing sulfhydryl groups. The clinical application is dextro-penicillamine. Penicillamine is a white or nearly white fine crystalline powder, very soluble in water. Oral 1h about 57% is absorbed, and quickly reach the peak of blood concentration, distribution throughout the body, but still mainly in the plasma. Penicillamine is combined with monovalent and divalent copper ions deposited in plasma and tissues in patients with hepatomegaly to form stable penicillamine-monovalent copper complexes and Cu-penicillamine mixed-valence complexes excreted from the kidney; on the other hand, penicillamine can induce MT synthesis, which forms non-toxic complexes with copper to reduce the symptoms caused by copper accumulation. In 1956, Walshe was the first to treat 2 cases of hepatomegaly with penicillamine and confirmed the increase of urinary copper excretion, and in the same year, 6 additional cases were reported, and in 1960, the efficacy of penicillamine in treating patients with hepatomegaly was observed and summarized in detail. Since then more than 40 years penicillamine has been the drug of choice for the treatment of hepatomegaly at home and abroad. However, nearly half a century of clinical practice confirmed that penicillamine has more adverse reactions, early gastrointestinal symptoms and allergic reactions can occur, a few can cause leukopenia or (and) thrombocytopenia, hemolytic anemia, etc.; especially in the long-term use of penicillamine, often occur in the skin purpura, a few can lead to granulocyte deficiency and aplastic anemia; some patients can also lead to systemic lupus erythematosus, severe Some patients may also suffer from systemic lupus erythematosus, myasthenia gravis and immune diseases such as nephritis-pulmonary hemorrhage syndrome. According to our statistics, more than 40% of the patients with hepatomegaly treated with penicillamine in external hospitals had one or more adverse reactions, and the duration of adverse reactions ranged from a few hours to about 2 months, with more than half of them occurring within 2 weeks. The clinical manifestations of adverse reactions are: leukopenia; anemia; drug rash with high fever; skin purpura platelet reduction, epistaxis, gum bleeding, upper gastrointestinal bleeding; loss of appetite and nausea and vomiting, bloating diarrhea and constipation, systemic lupus erythematosus, etc. Many patients have worsened symptoms (twisted and deformed limbs, extreme difficulty in swallowing, etc.) and lost their ability to live and are bedridden for years. The presence of adverse effects of penicillamine and the many effects of liver damage and transient or persistent worsening of neurological function at the beginning of treatment (such effects can occur in 20% to 50% of patients) have limited its use to some extent. Some studies have concluded that 50% of patients with neurological symptoms experience exacerbation of symptoms and irreversible neurological damage with penicillamine, so routine use of penicillamine should be avoided in early patients with neurological or psychiatric symptoms. Therefore, other drug options should be considered for treatment at the beginning. As Walshe later pointed out in The Lancet, “treatment of hepatomegaly must look forward to new copper repellent drugs to complement penicillamine”. Many scholars in China have conducted in-depth comparative studies on the clinical therapeutic effects of several copper repellent drugs and their toxicity and adverse effects. Some studies have shown that DMSA has better long-term efficacy than penicillamine, especially for those who are allergic to penicillamine or ineffective in treatment. Studies have shown that DMSA, DMPS and penicillamine all have certain damaging effects on human lymphocyte DNA; penicillamine has significantly higher DNA damage than DMPS and DMSA. Therefore, as copper repellent treatment, penicillamine is no longer emphasized as the drug of choice for the treatment of hepatomegaly, but should be based on the therapeutic effects, toxicity and adverse effects of copper repellent drugs, clinical symptoms of patients, clinical examination The treatment should be individualized according to the therapeutic effects, toxicity and adverse effects of copper repellent drugs, clinical symptoms and clinical examination results. Therefore, the prognosis of patients with the same condition is significantly affected by different choices of copper repellent drugs. Our clinical treatment for hepatomegaly is mainly a strict low-copper diet and regular individualized copper repellent treatment (there are more than 10 types of clinical conditions, each with different treatment plans, and each patient with the same condition has different treatment plans depending on complications and comorbidities, and the same patient has different treatment plans depending on the treatment period), with a clinical efficiency of more than 95%.