徐春军治疗Wilson病验案一则
增强出版A Case of Wilson's Disease Treated by Professor Xu Chunjun
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1.首都医科大学附属北京中医医院肝病科,北京 100010
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武璇, 闫语, 郑鑫卓, 等. 徐春军治疗Wilson病验案一则[DB/OL].(2023-02-27)[2023-11-05].http://cccl-tcm.cacm.org.cn/thesisDetails?columnId=28614876&Fpath=home&index=0
WU Xuan, YAN Yu, ZHENG Xinzhuo, et al. A Case of Wilson's Disease Treated by Professor Xu Chunjun[DB/OL].(2023-02-27)[2023-11-05].http://cccl-tcm.cacm.org.cn/thesisDetails?columnId=28614876&Fpath=home&index=0
武璇, 闫语, 郑鑫卓, 等. 徐春军治疗Wilson病验案一则[DB/OL].(2023-02-27)[2023-11-05].http://cccl-tcm.cacm.org.cn/thesisDetails?columnId=28614876&Fpath=home&index=0 DOI:
WU Xuan, YAN Yu, ZHENG Xinzhuo, et al. A Case of Wilson's Disease Treated by Professor Xu Chunjun[DB/OL].(2023-02-27)[2023-11-05].http://cccl-tcm.cacm.org.cn/thesisDetails?columnId=28614876&Fpath=home&index=0 DOI:
病史摘要,2,患者王某,女,40岁,角膜K-F环、铜蓝蛋白和24 h尿铜、基因筛查等诊断要素具备,Wilson病西医诊断明确。因病情反复控制欠佳,逐步进展为肝硬化,行脾栓塞术后并发感染,目前出现黄疸、腹水等肝硬化失代偿期严重并发症。本次以肝硬化1年余,伴腹水3月为主诉就诊。四诊信息 牙龈出血,闭经,脱发,形寒肢冷,神倦乏力,脘腹胀满,纳差,大便秘结,小便黄,眠欠安。舌质暗红,边齿痕,舌苔薄白,脉象滑数。中医诊断 臌胀病-痰瘀胶结、气虚水停证。西医诊断 Wilson病(肝豆状核变性)、肝硬化腹水、脾栓塞术后。干预措施 中医治法:益气扶正,活血化痰,利水消癥。生黄芪40 g、茵陈10 g、防己10 g、泽兰15 g、炒酸枣仁30 g、当归10 g、白芍10 g、赤芍10 g、水红花子15 g、佩兰10 g、炒白术10 g、金钱草15 g、赤小豆20 g、楮实子10 g、丹参15 g、百合15 g。全方攻补兼施,以大剂量黄芪大补元气,气血相生,益气、养血之品相配,兼以理气活血,补而不滞、行而不伤。西医治疗:长期口服青霉胺片(0.125 g/片),8片/d。疗效转归 经治疗后,患者牙龈出血,闭经,脱发,形寒肢冷,神倦乏力,脘腹胀满,纳差,眠欠安等临床症状得到明显改善,腹水得到有效控制,铜蓝蛋白、24 h尿铜、直接胆红素等检测指标也逐步好转,生活质量明显提高。
Summary of case history ,Patient Wang, female, 40 years old, with diagnostic elements such as corneal K-F ring, copper blue protein and 24 h urine copper, and genetic screening, had a clear Western diagnosis of Wilson's disease. Due to repeated poor control of his disease, she has progressively progressed to cirrhosis, complicated by infection after splenic embolization, and is currently presenting with severe complications of cirrhosis in the decompensated phase, including jaundice and ascites. The present presentation was made with a complaint of cirrhosis of the liver for more than 1 year with ascites for 3 months.,Symptoms and signs ,Bleeding gums, amenorrhea, hair loss, cold body and limbs, tiredness and fatigue, fullness in the abdomen and stomach, poor appetite, constipation, yellow urine and restless sleep. The tongue is dark red with tooth marks on the sides, the tongue coating is thin and white, and the pulse is slippery.,TCM diagnosis, She is diagnosed as Tympanites, Phlegm and stagnation, Qi deficiency and water retention.,Western medicine diagnosis ,She is diagnosed as Wilson's disease, cirrhotic ascites, and post-splenic embolism.,Therapeutic methods ,Traditional Chinese Medicine Treatment: Benefiting Qi, promoting rectification, invigorating blood, resolving phlegm, inducing diuresis and eliminating symptoms. Radix Astragali 40 g, Yin Chen 10 g, Fang Ji 10 g, Ze Lan 15 g, fried Semen Ziziphi spinosae 30 g, Angelica Sinensis 10 g, Paeoniae Radix Alba 10 g, Paeonia Lactiflora Pall 10 g, Fructus Polygoni Orientalis 15 g, Fortune Eupatorium Herb 10 g, Fried Atractylodes Macrocephalae 10 g, Lysimachiae herba 15 g, Rice Bean 20 g, Fructus Broussonetiae 10 g, Salvia Miltiorrhiza 15 g, Lilium 15 g. The whole formula is based on a combination of attacking and tonifying. Western medicine treatment: Long-term oral administration of penicillamine tablets (0.125 g/tablet) 8 tablets/d.,Clinical outcomes ,After treatment, the patient's clinical symptoms such as bleeding gums, amenorrhea, hair loss, cold body and limbs, fatigue, distention and fullness in the abdomen, poor appetite and poor sleep are significantly improved. Her ascites is effectively controlled, and copper blue protein, 24-h urine copper and direct bilirubin are gradually improved. Her quality of life is significantly improved.
Wilson病经验徐春军
Wilson's diseaseExperienceXu Chunjun
Mulligan C, Bronstein J M. Wilson Disease: An overview and approach to management[J]. Neurol Clin, 2020,38(2):417-432.
中华医学会神经病学分会神经遗传学组. 中国肝豆状核变性诊治指南2021[J]. 中华神经科杂志,2021,54(4):310-319.
梁晨,郑素军. Wilson病治疗研究现状[J]. 实用肝脏病杂志,2021,24(6):930-933.
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