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与肝脾紫癜病相关的范科尼氏病(作者译)

[Fanconi's disease associated with hepato-splenic peliosis (author's transl)].

作者信息

Despert F, Lamagnere J P, Chantepie A, Grangeponte M C, Lejars O, Combe P

出版信息

Arch Fr Pediatr. 1981 Jan;38(1):29-33.

PMID:7224798
Abstract

The authors report a case of Fanconi's disease equilibrated for a period of 8 years by androgen therapy. The patient died after interruption of treatment; the clinical picture comprised hepatomegaly, jaundice, pancytopenia. The autopsy showed hepato-splenic peliosis and acute tuberculosis. The subject of peliosis is brought up to date by a thorough review of current literature and the relationships between steroid treatment and hepatic complications are discussed.

摘要

作者报告了一例通过雄激素治疗病情稳定达8年的范科尼氏病患者。该患者在治疗中断后死亡;临床表现包括肝肿大、黄疸、全血细胞减少。尸检显示肝脾紫癜和急性肺结核。通过全面回顾当前文献,对紫癜这一主题进行了更新,并讨论了类固醇治疗与肝脏并发症之间的关系。

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