先天性肾病综合征合并新生儿红细胞增多症1例报告  

Congenital nephrotic syndrome with neonatal polycythemia: a case report

作　　者：韩宗来 杨磊[1] 林沂 李永伟 严超英[1] HAN Zonglai;YANG Lei;LIN Yi;LI Yongwei;YAN Chaoying(Department of Pediatrics,The First Hospital of Jilin University,Changchun 130000, Jilin,China)

机构地区：[1]吉林大学第一医院儿科,吉林长春130000

出　　处：《临床儿科杂志》2018年第3期175-177,共3页Journal of Clinical Pediatrics

摘　　要：目的探讨先天性肾病综合征(CNS)和新生儿红细胞增多症之间的关系及临床诊治。方法回顾分析1例CNS合并新生儿红细胞增多症患儿的临床表现、诊治经过,同时复习相关文献。结果男婴,生后即出现腹胀,随后出现喂养不耐受、反应差;出生第3天血红蛋白249 g/L,红细胞比容0.714 L/L,诊断新生儿红细胞增多症;经部分换血治疗后,症状好转;生后第6天出现水肿,尿蛋白+++,血白蛋白12.7 g/L,血胆固醇8.84 mmol/L,临床诊断CNS,给予口服激素等治疗无效,生后32天死亡。结论 CNS合并新生儿红细胞增多症临床罕见,两者合并存在更易诱发血栓栓塞合并症及器官功能障碍,临床预后差。Objectives To investigate the relationship between congenital nephrotic syndrome(CNS)and neonatal polycythemia,and clinical diagnosis and treatment.Methods The clinical manifestations,diagnosis,and treatment of a case of CNS with neonatal polycythemia were retrospective analyzed,and the related literature were reviewed.Results A male infant had abdominal distention after his birth,followed by feeding intolerance and poor response.On the third day of birth,he was diagnosed of neonatal polycythemia according to the levels of hemoglobin(249g/L)and hematocrit(0.714L/L).And after a partial exchange transfusion,the symptoms were improved.On the sixth day of birth,the infant had edema,urinary protein+++,serum albumin at12.7g/L and blood cholesterol at8.84mmol/L,and was clinical diagnosed of CNS.Oral hormone therapy was ineffective and he died32days after birth.Conclusions CNS combined with neonatal erythrocytosis is rare in clinic.The coexisting of the two is more likely to induce thromboembolism and organ dysfunction,and the clinical prognosis is poor.

关 键 词：新生儿 红细胞增多症 先天性肾病综合征 

分 类 号：R722.1[医药卫生—儿科]