Hass R J, Niethammer D, Flad H D, Wildfeuer A, Bültmann B, Haferkamp O, Kleihauer E
Acta Paediatr Scand. 1976 Mar;65(2):241-7. doi: 10.1111/j.1651-2227.1976.tb16544.x.
The report describes the clinical syndroms of a 14-year-old boy which suffered from recurrent infections since early infancy. The clinical and general laboratory findings were similar to "the granulomatous disease of childhood" as described by Bridges et al. (8). The following serum factors were determined: Immunoglobulins, complement factors, isoagglutinins. The following assays with normal or patient's granulocytes were done: Chemotactic activity, nitroblue-tetrazolium test, bactericidal assay, fungicidal assay, myeloperoxidase, monocytes fungicidal assay. Immunological studies include kinetics of phytohaemagglutinin response, effect of serum of the patient on lymphocytic reactivity in vitro and skin tests. The following results were obtained: 1) Assays with normal or patient's granulocytes showed an impaired chemotatic activity, when serum of the patient was added. There was strong indication by treatment of the patient with plasma infusions, that the chemotactic defect is a serum dependent factor. 2) It could be demonstrated that the patient's serum also inhibited the response of lymphocytes to tuberculin and phytohaemagglutinin. Therefore the patient report focuses attention upon the possibility of serum related abnormalities that may influence granulocytic as well as lymphocytic functions leading to recurrent bacterial, fungal and viral infections.
该报告描述了一名14岁男孩的临床症状,他自婴儿早期就反复感染。临床和常规实验室检查结果与布里奇斯等人(8)所描述的“儿童肉芽肿病”相似。测定了以下血清因子:免疫球蛋白、补体因子、同种凝集素。对正常或患者的粒细胞进行了以下检测:趋化活性、硝基蓝四氮唑试验、杀菌试验、杀真菌试验、髓过氧化物酶、单核细胞杀真菌试验。免疫学研究包括植物血凝素反应动力学、患者血清对体外淋巴细胞反应性的影响以及皮肤试验。获得了以下结果:1)当加入患者血清时,对正常或患者粒细胞的检测显示趋化活性受损。通过给患者输注血浆进行治疗有强烈迹象表明,趋化缺陷是一种血清依赖性因素。2)可以证明患者血清也抑制淋巴细胞对结核菌素和植物血凝素的反应。因此,该患者报告关注可能影响粒细胞和淋巴细胞功能导致反复细菌、真菌和病毒感染的血清相关异常的可能性。