伴有中性粒细胞趋化功能缺陷的塞-乔二氏综合征。

Saethre-Chotzen syndrome associated with defective neutrophil chemotaxis.

作者信息

Etzioni A, Obedeanu N, Benderly A, Gershoni-Baruch R

机构信息

Department of Pediatrics A, Rambam Medical Center, Haifa, Israel.

出版信息

Acta Paediatr Scand. 1990 Mar;79(3):375-9. doi: 10.1111/j.1651-2227.1990.tb11477.x.

DOI:10.1111/j.1651-2227.1990.tb11477.x

PMID:2333756

Abstract

An 11-month-old male infant with Saethre-Chotzen syndrome and recurrent respiratory infections is described. Persistent extremely high leukocytosis warranted evaluation of neutrophil functions. It was found that the opsonophagocytic activity was normal, but neutrophil chemotaxis was markedly decreased. Further studies pointed to an intracellular neutrophil defect causing this motility dysfunction.

摘要

本文描述了一名患有塞特勒-乔岑综合征和反复呼吸道感染的11个月大男婴。持续性极高的白细胞增多症需要对中性粒细胞功能进行评估。结果发现调理吞噬活性正常，但中性粒细胞趋化性明显降低。进一步研究表明存在一种导致这种运动功能障碍的细胞内中性粒细胞缺陷。