Bowen T J, Ochs H D, Altman L C, Price T H, Van Epps D E, Brautigan D L, Rosin R E, Perkins W D, Babior B M, Klebanoff S J, Wedgwood R J
J Pediatr. 1982 Dec;101(6):932-40. doi: 10.1016/s0022-3476(82)80013-9.
We studied two patients with delayed umbilical cord detachment, recurrent bacterial infections, inability to form pus, rapidly progressive periodontitis, and persistent leukocytosis. The phagocytes of both patients were strikingly abnormal in their ability to adhere to surfaces. The adherence of polymorphonuclear leukocytes to endotoxin-coated glass coverslips, glass beads, or nylon wool was markedly reduced. Scanning electron microscopy of the few adherent polymorphonuclear leukocytes from both patients showed a failure to flatten and form fine pseudopods. In vivo polymorphonuclear leukocyte and monocyte chemotaxis assessed by skin window and skin chamber methods was dramatically impaired, and in vitro chemotaxis was severely depressed. Chemiluminescence of zymosan- but not phorbol-stimulated polymorphonuclear leukocytes was markedly reduced. Allogeneic polymorphonuclear leukocytes transfused into these patients functional normally, indicating that the defect is intrinsic to the cells and not a secondary phenomenon. A 180-kilodalton glycoprotein normally present in the particulate fraction of polymorphonuclear leukocytes was found to be completely absent in Patient 1 and present in low concentration in Patient 2. We postulate that the glycoprotein deficiency interferes with the migration of polymorphonuclear leukocytes from the bloodstream into the interstitial space and to the site of infection.
我们研究了两名患有脐带延迟脱落、反复细菌感染、无法形成脓液、快速进展性牙周炎和持续性白细胞增多症的患者。两名患者的吞噬细胞在黏附于表面的能力方面均表现出显著异常。多形核白细胞对内毒素包被的玻璃盖玻片、玻璃珠或尼龙棉的黏附明显减少。对两名患者中少数黏附的多形核白细胞进行扫描电子显微镜检查发现，它们无法扁平并形成细小的伪足。通过皮肤窗口和皮肤腔室方法评估的体内多形核白细胞和单核细胞趋化性显著受损，体外趋化性也严重降低。酵母聚糖刺激而非佛波醇刺激的多形核白细胞的化学发光明显减少。输入这些患者体内的同种异体多形核白细胞功能正常，表明缺陷是细胞固有的，而非继发现象。在多形核白细胞的颗粒部分中正常存在的一种180千道尔顿的糖蛋白，在患者1中完全缺失，在患者2中浓度较低。我们推测，糖蛋白缺乏会干扰多形核白细胞从血液进入间质空间并到达感染部位的迁移。
