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 investigate the clinical and laboratory features of neonatal paroxysmal nocturnal hemoglobinuria (PNH). Methods The data of 3 neonates with PNH admitted in our hospital were summarized. Their clinical features, hemogram and haemolysis were analyzed. The characteristics of erythrocyte and granulocyte CD55 / CD59 expression were analyzed. Results neonatal PNH clinical manifestations of jaundice in neonatal persistence, pale, progressive anemia, the typical soy sauce color urine is not easy to find. 3 cases of children with erythrocyte CD55 expression defects, 2 cases of erythrocyte CD59 expression defect, 1 cases of granulocyte CD55 / CD59 detection and found that there are defects in the expression, diagnosis of PNH. Conclusions Neonatal PNH is extremely rare. Because of its clinical manifestations different from adults, it is not easy to find out. The detection of CD55 / CD59 expression in blood cells can definitely diagnose the disease. Therefore, clinical suspicion of hemolytic disease, the need to consider the possibility of PNH, the use of the current advanced diagnosis and treatment technology, starting from the pathogenesis of PNH, the positive detection of CD55 / CD59 blood cells to early diagnosis of neonatal PNH, so as not to Misdiagnosis, misdiagnosis.