胱氨酸病的生存时间。一项合作研究。

Survival time in cystinosis. A collaborative study.

作者信息

Gretz N, Manz F, Augustin R, Barrat T M, Bender-Götze C, Brandis M, Bremer H J, Brodehl J, Broyer M, Bulla M, Callis L, Chantler C, Diekmann L, Dillon M J, Egli F, Ehrich J H, Endres W, Fanconi A, Feldhoff C, Geisert J, Gekle D, Geschöll-Bauer B, Grote K, Grüttner R, Hagge W, Haycock C B, Hennemann H, Klare B, Leupold D, Löhr H, Michalk D, Oliveira A, Ott F, Pistor K, Rau J, Schärer K, Schindera F, Schmidt H, Schulte-Wissermann H, Verrier-Jones K, Weber H P, Willenbockel U, Wolf H

出版信息

Proc Eur Dial Transplant Assoc. 1983;19:582-9.

PMID:6878257

Abstract

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

摘要

在一项回顾性研究中，确定了六个国家205例胱氨酸病患者的总生存时间。中位生存时间为8.5年。“肾死亡”（因尿毒症死亡的年龄或开始肾脏替代治疗的年龄）的中位时间为9.2年。死于肾死亡的最年轻患者为5.2岁。未发现生存时间存在性别差异。此外，不同国家之间的生存时间也没有差异。总生存曲线分析表明，婴儿型和青少年型胱氨酸病之间没有明显差异。