OBJECTIVE: To evaluate the efficacy and safety of gandouling plus sodium dimercaptosulphonate(DMPS) on neurological Wilson's disease(WD) in patients.METHODS: We retrospectively evaluated the clinical records of 12...OBJECTIVE: To evaluate the efficacy and safety of gandouling plus sodium dimercaptosulphonate(DMPS) on neurological Wilson's disease(WD) in patients.METHODS: We retrospectively evaluated the clinical records of 125 WD patients with neurological syndromes who were treated with gandouling plus sodium DMPS or DMPS used alone. All patients had a history of neurological deterioration during their diseases courses. The clinical efficacies, adverse reactions, and results of the various hematological and biochemical investigations were recorded for statistical analysis.RESULTS: 92.30%(60 patients) of the WD patients treated with the combined therapy experienced an improved or stable neurological condition paral-leled by a significantly improved GAS score. Meanwhile, the WBC and PLT counts stabilized, liver function and renal function were improved or remained stable. The combined therapy also obviously promoted the 24-h urinary copper excretion. In particular, only 30.76% of the WD patients experienced mild adverse reactions, including neurological deterioration in 5 patients(7.69%), hepatic worsening in 1 subject(1.89%), which was less frequently than those in the control group treated with DMPS only.CONCLUSION: Our findings indicate that the safety and efficacy of gandou-ling plus DMPS is superior to those of DMPS used alone in the WD patients with neurological symptoms.展开更多
OBJECTIVE： To investigate the effect and safety of Gandouling plus low-dose D-penicillamine for treating patients with Wilson＇s disease （WD） who have neurological symptoms. METHODS： WD patients with neurological ...OBJECTIVE： To investigate the effect and safety of Gandouling plus low-dose D-penicillamine for treating patients with Wilson＇s disease （WD） who have neurological symptoms. METHODS： WD patients with neurological symptoms were divided into two groups： a treatment group （n = 53） and a control group （n = 50）. The treatment group received anti-copper therapy with a combination of Gandouling and low-dose D-peni- cillamine （10 mglkg）, whereas the control group was with conventional dose D-penicillamine （20 rag/ kg） monotherapy. The clinical efficacies, adverse re- actions, and results of the various hematological and biochemical investigations were recorded and analyzed statistically. RESULTS： Overall, 98.11% of the WD patients treated with the combined therapy experienced alleviation of their neurological condition （paralleled by a significantly improved Global Assessment Scale score or remained stable）. Their white blood cell and platelet counts stabilized, and their liver function was improved or remained stable. The combined therapy also obviously promoted improved 24-h urinary copper excretion. Only 15.09% of the WD patients with the combined therapy experienced adverse reactions, including neurological deterioration in one case （1.89%） and hepatic worsening in one case （1.89%）, which was less frequent than that in the control group given conventional-dose D-penicillamine monotherapy. CONCLUSION： Treating WD patients with neurological symptoms using Gandouling plus low-close D-penicillamine is effective and safe.展开更多
基金Supported by National Natural Science Foundation of China(No.81673811,81473534,81774299)
文摘OBJECTIVE: To evaluate the efficacy and safety of gandouling plus sodium dimercaptosulphonate(DMPS) on neurological Wilson's disease(WD) in patients.METHODS: We retrospectively evaluated the clinical records of 125 WD patients with neurological syndromes who were treated with gandouling plus sodium DMPS or DMPS used alone. All patients had a history of neurological deterioration during their diseases courses. The clinical efficacies, adverse reactions, and results of the various hematological and biochemical investigations were recorded for statistical analysis.RESULTS: 92.30%(60 patients) of the WD patients treated with the combined therapy experienced an improved or stable neurological condition paral-leled by a significantly improved GAS score. Meanwhile, the WBC and PLT counts stabilized, liver function and renal function were improved or remained stable. The combined therapy also obviously promoted the 24-h urinary copper excretion. In particular, only 30.76% of the WD patients experienced mild adverse reactions, including neurological deterioration in 5 patients(7.69%), hepatic worsening in 1 subject(1.89%), which was less frequently than those in the control group treated with DMPS only.CONCLUSION: Our findings indicate that the safety and efficacy of gandou-ling plus DMPS is superior to those of DMPS used alone in the WD patients with neurological symptoms.
基金Supported by National Natural Science Foundation of China:Study on the Effect of Gandou Tablets in Mitophagy by Willson Disease Model TX Mouse(No.81503443)
文摘OBJECTIVE： To investigate the effect and safety of Gandouling plus low-dose D-penicillamine for treating patients with Wilson＇s disease （WD） who have neurological symptoms. METHODS： WD patients with neurological symptoms were divided into two groups： a treatment group （n = 53） and a control group （n = 50）. The treatment group received anti-copper therapy with a combination of Gandouling and low-dose D-peni- cillamine （10 mglkg）, whereas the control group was with conventional dose D-penicillamine （20 rag/ kg） monotherapy. The clinical efficacies, adverse re- actions, and results of the various hematological and biochemical investigations were recorded and analyzed statistically. RESULTS： Overall, 98.11% of the WD patients treated with the combined therapy experienced alleviation of their neurological condition （paralleled by a significantly improved Global Assessment Scale score or remained stable）. Their white blood cell and platelet counts stabilized, and their liver function was improved or remained stable. The combined therapy also obviously promoted improved 24-h urinary copper excretion. Only 15.09% of the WD patients with the combined therapy experienced adverse reactions, including neurological deterioration in one case （1.89%） and hepatic worsening in one case （1.89%）, which was less frequent than that in the control group given conventional-dose D-penicillamine monotherapy. CONCLUSION： Treating WD patients with neurological symptoms using Gandouling plus low-close D-penicillamine is effective and safe.
