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丙酮酸激酶缺乏症患者因细小病毒感染引发的再生障碍危象

Aplastic crisis due to parvovirus infection in pyruvate kinase deficiency.

作者信息

Duncan J R, Potter C B, Cappellini M D, Kurtz J B, Anderson M J, Weatherall D J

出版信息

Lancet. 1983 Jul 2;2(8340):14-6. doi: 10.1016/s0140-6736(83)90005-3.

Abstract

A thirteen-year-old boy with congenital haemolytic anaemia due to pyruvate kinase (PK) deficiency had an aplastic crisis. A serum parvovirus-like virus (SPLV) was demonstrated in the blood by electron microscopy and, subsequently, IgM and IgG antibodies to the prototype SPLV B19 were detected. In an attempt to define the level of erythropoiesis that is involved in parvovirus-induced bone marrow suppression, the levels of circulating early erythroid progenitors (burst forming units erythroid, BFU-E) were monitored during the crisis and recovery period. The virus-containing plasma inhibited the formation of BFU-Es from non-immune subjects and this effect was neutralised by convalescent serum. Colony forming units granulocyte-macrophage (CFU-GM) were also inhibited but this was probably non-specific since neutralisation did not reverse the effect. These experiments, together with the clinical data, suggest a selective effect of SPLV at the stage of erythroid progenitors.

摘要

一名13岁因丙酮酸激酶(PK)缺乏导致先天性溶血性贫血的男孩发生了再生障碍危象。通过电子显微镜在血液中发现了一种血清细小病毒样病毒(SPLV),随后检测到针对原型SPLV B19的IgM和IgG抗体。为了确定细小病毒诱导的骨髓抑制所涉及的红细胞生成水平,在危象期间和恢复期监测了循环早期红细胞祖细胞(红细胞爆式集落形成单位,BFU-E)的水平。含病毒的血浆抑制了非免疫受试者BFU-E的形成,而恢复期血清可中和这种作用。粒细胞-巨噬细胞集落形成单位(CFU-GM)也受到抑制,但这可能是非特异性的,因为中和作用并未逆转这种效应。这些实验以及临床数据表明,SPLV在红细胞祖细胞阶段具有选择性作用。

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