Wilson病の治療—特にペニシラミン療法を中心として

TREATMENT OF WILSON'S DISEASE, ESPECIALLY ON PENICILLAMINE THERAPY

I．緒言

　Wilson病は，血清セルロプラスミンの低下，尿中銅排泄量増加，肝，脳，腎，角膜など組織への銅の過剰沈着をもつて特徴づけられる遺伝性代謝疾患である。臨床症状は肝硬変，脳特に基底核の海綿状変化や膠細胞の変性による神経症状，腎性アミノ酸尿，糖尿，腎機能低下，角膜における色素沈着，白内障など，銅の沈着のいちじるしい部分の変性にもとづく多様な症状を呈する。

　本症の予後は一般にきわめて不良であり，早ければ発病1週間で肝不全のために死亡し，多くは2〜5年の経過で肝または神経症状のために死亡する。自然治癒はまつたく認められず，放置すれば遅かれ早かれ100％死亡するおそるべき疾患である．

From the experiences of penicillamine the-rapy it 10 cases of Wilson's disease, the effect and side effects of penicillamine may be con-cluded as follows:

1) The copper eliminating effect of penici-llamine is greater than BAL and EDTA-Ca.

2) Appearance of the clinical effect of pe-nicillamine appears to be later than BAL. Effects in inhibition of progress of the disease and maintenance of the improved condition, however, last longer than the older agents.

3) As it is given orally, it can be used with-out pain even in children and is convenient for the home treatment.

4) Periodical examination is advisable as it should be administered for a long time al-though no severe side effects, which neces-sitated discontinuation of medication, have been noted so far.

In Wilson's disease, death will take place unless it is treated before the development of irreversible changes. Therefore, early diag-, nosis and treatment are important for the complete control of the disease.