新生儿阵发性血红蛋白尿临床分析  

Oinical analysis of neonate paroxysmal nocturnal hemoglobinuria

作　　者：周晓苓[1] 何振娟[1] 朱建幸[1] 储淞雯[1] 

机构地区：[1]上海第二医科大学附属新华医院儿童医学中心儿内科,200092

出　　处：《新生儿科杂志》2005年第5期206-209,共4页The Journal of Neonatology

摘　　要：目的探讨新生儿阵发性睡眠性血红蛋白尿(PNH)的临床及实验室检查特点。方法总结我院收治的3例新生儿PNH的病例资料,从临床,血象,溶血检查方面及红细胞,粒细胞CD55/CD59的表达检测等方面分析其特点。结果新生儿PNH临床表现为新生儿期黄疸持续不退、面色苍白、进行性贫血,典型的酱油色样尿不易发现。3例患儿红细胞CD55表达均有缺陷,2例红细胞CD59表达缺陷,1例进行粒细胞CD55/CD59检测,发现亦有表达缺陷,诊断为PNH。结论新生儿PNH极为少见,由于其临床表现不同于成人,不易发现,而通过血细胞CD55/CD59表达的检测,可明确诊断该病。因此,临床上在怀疑溶血病时,同时需考虑PNH的可能,利用目前先进的诊疗技术,从PNH的发病机制出发,积极进行血细胞CD55/CD59表达的检测,以早期明确诊断新生儿PNH,以免漏诊、误诊。Objective To study the characteristic of clinical and laboratory examination of neonate paroxysmal nocturnal hemoglobinuria（PNH）. Methods Analysis the characteristics of clinical hemolysis examination; expression of CD55 and CD59 in erythrocytes, granulocytes. Results The clinical situation of neonate PNH included jaundice in neonatal period, pallor anemia. But the typical soy urine was hard to detect, Hemolysis examination was coincidence with hemolytic manifestation. In all cases, CD55 expression was deficient on erythrocytes membrance. In two cases, CD59 expression was deficient on erythrocytes membrance. Conclusions Clinical situation of neonate PNH is different from adult. Expression of CD55 and CD59 on membrance of erythrocytes and granulocytes are important to diagnose neonatal PNH.

关 键 词：新生儿 阵发性睡眠性血红蛋白尿 CD55 CD59 阵发性血红蛋白尿 新生儿期 临床分析 红细胞CD59 CD59检测 表达检测 

分 类 号：R722.1[医药卫生—儿科]