Jabłońska J, Popow J, Rodo M, Członkowska A
Klinika Hepatologii i Nabytych Niedoborów Immunologicznych, Instytut Chorób Zakaźnych, Warszawie.
Pol Arch Med Wewn. 1997 Dec;98(12):542-5.
Wilson's disease is an autosomal recessive disorder characterized by inability to excrete copper, and manifests by hepatic, neurologic or/and psychiatric symptoms. The therapy is available if diagnosis is made in time. The hepatic form of the disease is rarely recognized in Poland. The authors describe two patients with Wilson's disease who developed acute hepatic failure leading to death, in the first case within few months, in the second within few weeks. The diagnosis was established in the terminal stage of the disease and attempts of treatment were uneffective.
威尔逊病是一种常染色体隐性疾病,其特征为无法排出铜,并表现为肝脏、神经或/和精神症状。如果能及时诊断,该病可进行治疗。在波兰,这种疾病的肝脏型很少被识别出来。作者描述了两名威尔逊病患者,他们发展为急性肝衰竭并导致死亡,第一例在几个月内,第二例在几周内。诊断是在疾病的终末期确定的,治疗尝试未取得成效。