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[重型地中海贫血中的脾功能亢进。脾脏部分去动脉化治疗。关于5例的初步结果]

[Hypersplenism in thalassemia major. Treatment by partial dearterialization of the spleen. Preliminary results apropos of 5 cases].

作者信息

Girot R, Revillon Y, Barritault L, Gire R, Beringer A, Boiteux F

出版信息

Presse Med. 1984 Sep 15;13(31):1881-4.

PMID:6237327
Abstract

Partial dearterialization of the spleen is a surgical method aimed at reducing the volume of the spleen whilst preserving its defensive properties against bacterial infections. Four children with thalassaemia major and one with congenital dyserythropoiesis who developed hypersplenism in the course of their disease underwent the operation and were subsequently followed up for periods of 24 to 36 months. The effectiveness of the method was demonstrated by a smaller enlargement of the spleen, a lesser need for transfusions, a longer life of 51 Cr-labelled red cells and more numerous circulating platelets. Surgery was also effective in reducing post-transfusional iron overload, as shown by the decrease and stability of serum ferritin levels in all children subjected to iron chelation. No episode of infection or changes in serum IgM occurred during the follow-up period, and radioisotopic studies of the spleen showed that macrophage function was preserved in the remaining splenic tissue. One child inexplicably developed thrombosis of the splenic vein 1 year after surgery.

摘要

脾脏部分去动脉化是一种外科手术方法,旨在减小脾脏体积,同时保留其抵抗细菌感染的防御特性。4名重型地中海贫血患儿和1名先天性红细胞生成异常患儿在病程中出现脾功能亢进,接受了该手术,随后进行了24至36个月的随访。该方法的有效性体现在脾脏肿大程度减小、输血需求减少、51铬标记红细胞寿命延长以及循环血小板数量增多。手术在减少输血后铁过载方面也很有效,所有接受铁螯合治疗的患儿血清铁蛋白水平均下降且保持稳定。随访期间未发生感染事件或血清IgM变化,脾脏的放射性同位素研究表明,剩余脾组织中的巨噬细胞功能得以保留。1名患儿在术后1年不明原因地出现了脾静脉血栓形成。

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