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在俄罗斯,母亲与胎儿人类血小板同种抗原-1a、-1b和-15不相容是新生儿同种免疫性血小板减少症的主要原因。

Maternal incompatibilities with fetal human platelet alloantigens -1a, -1b and -15 are the main causes of neonatal alloimmune thrombocytopenia in Russia.

作者信息

Khaspekova S G, Golovkina L L, Donush E K, Golubeva N V, Shustova O N, Mazurov A V

机构信息

National Medical Research Center for Cardiology, Russian Ministry of Health, Moscow, Russia.

National Medical Research Center for Hematology, Russian Ministry of Health, Moscow, Russia.

出版信息

Ter Arkh. 2018 Aug 17;90(7):65-69. doi: 10.26442/terarkh201890765-69.

Abstract

AIM

Mechanisms underlying the development of neonatal alloimmune thrombocytopenia (NAIT) in in Russia have been studied.

MATERIALS AND METHODS

Genetic polymorphisms of human platelet alloantigens (HPA) -1, -2, -3, -4, -5, and -15 were evaluated in 27 families having the newborns with NAIT. NAIT was diagnosed according to the following criteria: (1) newborn with thrombocytopenia; (2) mother with no thrombocytopenia and no increase of platelet associated IgG, (3) presence of antibodies reacting with paternal platelets in maternal plasma / serum. HPA genotyping revealed incompatibilities in 23 out of 27 tested families. In these 23 families HPA-1 conflicts were detected in 16 ones (70%). In 8 cases mothers were homozygous carriers of rare HPA-1b allele and in another 8 cases - of HPA-1a allele which cased incompatibilities with fetal HPA-1a and HPA-1b respectively. In 5 out of 23 families (22%) there were incompatibilities with fetal HPA-15 (HPA-15a, n=2 and HPA-15b, n=3), in 1 family - with HPA-5b (4%), and in 1 family - with HPA-3b (4%) alloantigens.

CONCLUSION

In conclusion the main causes of NAIT in Russia were HPA-1a and -1b conflicts and HPA-15 conflicts were the second frequent ones.

摘要

目的

对俄罗斯新生儿同种免疫性血小板减少症(NAIT)发病机制进行了研究。

材料与方法

对27个有患NAIT新生儿的家庭进行了人类血小板同种抗原(HPA)-1、-2、-3、-4、-5和-15的基因多态性评估。NAIT根据以下标准诊断:(1)新生儿血小板减少;(2)母亲无血小板减少且血小板相关IgG未升高;(3)母体血浆/血清中存在与父亲血小板反应的抗体。HPA基因分型显示,在27个检测家庭中有23个存在不相容性。在这23个家庭中,16个(70%)检测到HPA-1冲突。8例母亲是罕见HPA-1b等位基因的纯合携带者,另外8例是HPA-1a等位基因的纯合携带者,分别与胎儿的HPA-1a和HPA-1b不相容。在23个家庭中的5个(22%),存在与胎儿HPA-15(HPA-15a,n = 2;HPA-15b,n = 3)的不相容性,1个家庭(4%)与HPA-5b不相容,1个家庭(4%)与HPA-3b同种抗原不相容。

结论

总之,俄罗斯NAIT的主要原因是HPA-1a和-1b冲突,HPA-15冲突是第二常见原因。

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