Milovanović D D, Tomasević R, Bogdanović G
Department of Neurology and Psychiatry of Children and Young People, Belgrade, Yugoslavia.
Srp Arh Celok Lek. 1998 Sep-Oct;126(9-10):327-34.
The introduction of penicillamine in the treatment of Wilson's disease (hepatolenticular degeneration) was a historical event [1]. D-pericillamine (d-PAM) showed some potential side effects such as myasthenia, kidney toxicity, etc. In previous decade the treatment of Wilson's disease (WD) with zinc sulphate started successfully [4]. Danks [7] described the use of exchanged transfusions, peritoneal dialysis and plasmapheresis in the treatment of young patients with WD in acute liver failure. These patients had acute copper poisoning. The results of this study were beneficial.
Therapeutic plasmapheresis (PF) has been used in many diseases in which immunological mechanisms are proved [8, 9]. We started with using PF in the treatment of two young chronic patients with Wilson's disease. The clinical picture of patients became worse, probably due to the decrease in cupriuretic effect of d-PAM. One patient (1) did not take d-PAM regularly. In this study PF was performed with the use of haemonetics V-50 and filter 704. During the treatment with PF, 2000 ml of plasma was always exchanged, i.e. removed. During the treatment with PF the patients were hospitalized at the Department of Neurology and Psychiatry of Children and Young People.
Patient No. 1. A 24-year-old man, born in 1965, came with a coarse tremor. He has been diagnosed as WD at the age of 18. Kayser-Fleischer rings were found in the cornea by slit-lamp examination. The disease began when he was 15 years old with polymorphous difficulties. In time tremor became more severe. It was apparent when the patient made any movement. The first treatment with PF (1) lasted from February 13 to March 16, 1989. The patient had 10 PF without any side effect. During the treatment with PF the patient felt better. After PF-tremor was reduced significantly, and subjective and objective condition ameliorated. The patient (1) was readmitted to the hospital on June 24, 1991 for continuation of the treatment with PF. Anamnestic data and neurological examination revealed progression of the disease. His condition became worse, especially tremor. He could not write a single letter (Figures 1-5) and walk without help of the others. His tremor became the severest when he made any kind of voluntary movement. The second treatment with PF started on June 25, 1991 and finished on July 16, 1991. He received 9 PF in this series. The treatment was without side effects. Tremor was reduced approximately by 15%. Plasma copper examined before and after 9 PF showed different values (Table 2). This difference (2.79 mumol/l; 23.70%) in plasma copper level was removed from circulation. Patient No. 2. A 23-year-old man, born in 1966, came to the hospital with acute exacerbation of WD. At the age of 16 latent psychosis was diagnosed. One year later diagnosis of WD was established in the hospital when he was 17 years old. Kayser-Fleischer rings were found in the cornea by slit-lamp examination. The treatment with d-PAM and other drugs (BAL, symptomatic therapies, sedatives, antidepressants, etc.) has been accompanied with good and long-term remissions and short exacerbation. A few years later exacerbation became longer and longer and worse and worse. He was admitted to the hospital on November 28, 1989 with acute relapse of WD. His condition was very difficult, completely bedridden. The treatment with PF started on December 26, 1989 and lasted to January 25, 1990. The treatment with PF was without d-PAM. He was only given symptomatic therapies. After a few PF he demonstrated side effects with nausea, sometimes vomiting, face sweating, pulse rate of about 120/min while blood pressure was normal. Therefore he was given human albumin in the next day, and no side effects were observed. The removal of little plasma copper from blood circulation correlated well with a small improvement in clinical symtpomatology. The rigidity was reduced and voluntary movements bec
青霉胺用于治疗威尔逊病（肝豆状核变性）是一个具有历史意义的事件[1]。D-青霉胺（d-PAM）显示出一些潜在的副作用，如肌无力、肾毒性等。在过去十年中，硫酸锌成功地开始用于治疗威尔逊病（WD）[4]。丹克斯[7]描述了在治疗患有急性肝衰竭的WD年轻患者时使用换血、腹膜透析和血浆置换的情况。这些患者有急性铜中毒。这项研究的结果是有益的。
治疗性血浆置换（PF）已用于许多已证实存在免疫机制的疾病[8,9]。我们开始将PF用于治疗两名患有威尔逊病的年轻慢性病患者。患者的临床症状恶化，可能是由于d-PAM的促尿铜排泄作用降低。一名患者（1）未规律服用d-PAM。在本研究中，使用血液学V-50和704过滤器进行PF。在PF治疗期间，总是置换出2000毫升血浆，即去除。在PF治疗期间，患者在儿童和青少年神经精神病科住院。
患者1。一名24岁男性，出生于1965年，前来就诊时伴有粗大震颤。他在18岁时被诊断为WD。通过裂隙灯检查在角膜中发现了凯-弗环。该病始于他15岁时，伴有多种不适。随着时间的推移，震颤变得更加严重。当患者进行任何动作时都很明显。第一次PF治疗（1）从1989年2月13日持续到3月16日。该患者进行了10次PF，没有任何副作用。在PF治疗期间，患者感觉更好。PF治疗后震颤明显减轻，主观和客观状况均有改善。患者（1）于1991年6月24日再次入院继续进行PF治疗。既往病史数据和神经系统检查显示疾病进展。他的病情恶化，尤其是震颤。他无法写一个字母（图1-5），在没有他人帮助的情况下无法行走。当他进行任何自主运动时，震颤最为严重。第二次PF治疗于1991年6月25日开始，于1991年7月16日结束。他在这一疗程中接受了9次PF。治疗没有副作用。震颤大约减轻了15%。9次PF前后检测的血浆铜显示出不同的值（表2）。血浆铜水平的这种差异（2.79微摩尔/升；23.70%）从循环中被清除。患者2。一名23岁男性，出生于1966年，因WD急性加重前来医院就诊。16岁时被诊断为潜在精神病。一年后，他17岁时在医院确诊为WD。通过裂隙灯检查在角膜中发现了凯-弗环。使用d-PAM和其他药物（二巯丙醇、对症治疗、镇静剂、抗抑郁药等）进行治疗，伴有良好且长期的缓解以及短暂的病情加重。几年后，病情加重变得越来越频繁且越来越严重。他于1989年11月28日因WD急性复发入院。他的病情非常严重，完全卧床不起。PF治疗于1989年12月26日开始，持续到1990年1月25日。PF治疗期间未使用d-PAM。仅给予对症治疗。经过几次PF治疗后，他出现了副作用，包括恶心、有时呕吐、面部出汗、脉搏约120次/分钟，而血压正常。因此，第二天给他输注了人白蛋白，未观察到副作用。从血液循环中去除少量血浆铜与临床症状的轻微改善密切相关。僵硬程度减轻，自主运动变得……
