中西医结合治疗198例肝豆状核变性患者的近期疗效及随访观察

目的 :观察中西医结合治疗肝豆状核变性 (HLD)患者的近期疗效和 6个月～ 2 4年的随访结果。方法 :198例HLD患者住院期间均静脉滴注二巯基丙磺酸 ,同时口服肝豆片和葡萄糖酸锌进行强力排铜治疗 ;出院后予二巯基丁二酸和青霉胺交替服用 ,并同时服肝豆片和葡萄糖酸锌以维持治疗。结果 :疗程结束时 ,临床痊愈、显效及改善分别为 2 2例 (11.11% )、15例 (7.5 8% )及 131例 (6 6 .16 % ) ,愈显率 18.6 9% ,总有效率 84 .85 %。随访 6个月时 ,愈显率与总有效率均有显著增高 ;随访 6 +个月～ 2 4年 ,临床痊愈、显效率均增高 ,无效和恶化率亦增高 ,而改善率下降。结论 :倘若长期坚持中西医结合维持排铜治疗 ,可使大多数HLD患者生活质量进一步提高 ,并能长期存活 ,而少数不能坚持服药或晚期重症患者 ,往往病情恶化 ,甚至死亡。

英文摘要:

Objective: To observe the short-term effect and follow-up of integrative medicine in treating patients with hepatolenticular degeneration (HLD).Methods: The 198 patients with HLD were treated by strengthened decopper therapy with sodium dimercaptosulphonate by intravenous dripping combined with oral intake of Gandoutablet and zinc gluconate during their hospitalization period.After discharge,the maintaining therapy given to them was dimercaptosuccinic acid and penicillamine alternatively combined with Gandou tablet and zinc gluconate orally.Patients were followed-up for 6 months to 24 years.Results: By the end of therapeutic course in hospital,the clinically cured,markedly improved and improved cases were 22(11.11%),15(7.58%) and 131(66.16%) respectively,the cure + markedly effective rate being 18.69% and the total effective rate 84.85%. The markedly effective rate and the total effective rate were increased significantly at the 6 months follow-up, while in the over 6 months to 24 years follow-up period the cure + markedly effective rate reduced,and ineffective and worsened rate increased as well as improved rate lowered were shown.Conclusion: Most HLD patients would acquire long-term survival and better quality of life if they insist on long-term decopper therapy.Condition would be deteriorated,even death often occur in those who cannot insist the treatment or in severe cases of advanced stage.

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