Schmalstieg F C
Department of Pediatrics, University of Texas Medical Branch, Galveston.
Semin Hematol. 1993 Oct;30(4 Suppl 4):66-71.
Leukocyte adherence deficiency (LAD) is characterized by bacterial infections at peripheral tissue sites whose defense requires exit of neutrophils from the vasculature. LAD is unique in that one of the basic and common cell properties, adherence, is involved in this defect in host defense. The molecular understanding of LAD catalyzed a continuing revolution in the investigation and understanding of cell adherence phenomena inside and outside of the immune system. The explosive gain of knowledge directly attributable to the recognition of LAD may make this disease the most important inborn error of host defense yet recognized. The recognition of this defect through the interaction of a number of investigators is explored in this report. An attempt is made to reveal the background events that made the discovery of this defect possible. The relationship between the understanding of the physiology of this disease and the rapid understanding of the molecular defect is examined. Finally, the significance of this disease and its understanding in relation to biologic discovery in general are discussed.
白细胞黏附缺陷症（LAD）的特征是外周组织部位发生细菌感染，而这些部位的防御需要中性粒细胞从血管系统中逸出。LAD的独特之处在于，宿主防御缺陷涉及到一种基本且常见的细胞特性——黏附。对LAD的分子学理解推动了免疫系统内外细胞黏附现象研究和理解的持续变革。直接归因于对LAD认识的知识爆炸性增长，可能使这种疾病成为迄今所认识到的最重要的先天性宿主防御缺陷。本报告探讨了通过众多研究者的相互作用对这一缺陷的认识过程。试图揭示使发现这一缺陷成为可能的背景事件。研究了对该疾病生理学的理解与对分子缺陷的快速理解之间的关系。最后，讨论了这种疾病的意义及其在一般生物学发现中的理解。
