Suppr超能文献

一名18个月大非血友病儿童体内抗血友病因子(凝血因子VIII)的抑制剂

An inhibitor of antihemophilic factor (factor VIII) in an 18-month-old nonhemophilic child.

作者信息

Stein J, Ratnoff O D

机构信息

Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, Ohio.

出版信息

Am J Pediatr Hematol Oncol. 1993 Aug;15(3):346-50.

PMID:8328652
Abstract

PURPOSE

Inhibitors of factor VIII occur in < or = 20% of severely affected patients with classic hemophilia, but are unusual in nonhemophilic individuals, and have not been reported in very young children. We treated a child with a "high-responding" inhibitor.

PATIENT AND METHODS

Our patient was an 18-month-old boy who had experienced several episodes of life-threatening hemorrhage. The techniques we used to decrease production of factor VIII in our patient were prolonged small doses of alternate day corticosteroids and continued administration of factor VIII.

RESULTS

We controlled the acute bleeding with porcine factor VIII or with recombinant human factor VIIa (rFVIIa). Immune tolerance was successfully achieved using a combination of corticosteroids and daily factor VIII infusions.

CONCLUSIONS

Multimodal therapy aimed at inducing long-term remission, along with stop-gap measures for hemostasis, may be effective for treating children with this acquired coagulopathy.

摘要

目的

因子VIII抑制剂见于≤20%的重度典型血友病患者,但在非血友病个体中不常见,且在幼儿中未见报道。我们治疗了一名有“高反应性”抑制剂的儿童。

患者与方法

我们的患者是一名18个月大的男孩,曾经历数次危及生命的出血事件。我们用于减少患者体内因子VIII生成的技术是隔日小剂量长期使用皮质类固醇并持续输注因子VIII。

结果

我们用猪源性因子VIII或重组人因子VIIa(rFVIIa)控制了急性出血。通过联合使用皮质类固醇和每日输注因子VIII成功实现了免疫耐受。

结论

旨在诱导长期缓解的多模式治疗,以及止血的权宜措施,可能对治疗患有这种获得性凝血病的儿童有效。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验