De Martino M, Vierucci A, Rossi M E, Galluzzi F, Salti R, La Cauza C
Boll Ist Sieroter Milan. 1983 Jul;62(3):268-72.
Some neutrophil functions (superoxide anion production, bactericidal activity and chemotaxis) have been examined in four children with Chondrodystrophia Calcificans Congenita. Superoxide anion production and bactericidal activity were in the normal range, but a defect in cellular chemotaxis has been proved. This finding account for predisposition to infection which is frequently present in these patients, and may include this disease among those syndromes in which a deficiency of the immune system is associated with growth failure.
对4例先天性钙化性软骨营养障碍患儿的一些中性粒细胞功能(超氧阴离子生成、杀菌活性和趋化性)进行了检测。超氧阴离子生成和杀菌活性在正常范围内,但已证实细胞趋化性存在缺陷。这一发现解释了这些患者中经常出现的感染易感性,并且可能将这种疾病纳入那些免疫系统缺陷与生长发育迟缓相关的综合征之中。