Däumling S, Buriot D, Trung P H, Griscelli C, Lalama M C, Belohradsky B H
Infection. 1980;8 Suppl 3:248-54. doi: 10.1007/BF01639590.
Fifteen patients aged between three and 27 years were examined clinically and immunologically. Common to all patients were severe recurring cutaneous and pulmonary staphyloccal infections, chronic eczema, eosinophilia and an extremely elevated serum IgE level. Eight of the patients had in addition facial dysplasia characterised by coarse features, prognathism and poorly formed external ears. Marked osteoporosis, particularly of the vertebral bodies, was observed in eight patients. A constant defect of granulocyte chemotaxis was found in only three patients; fluctuating or constantly normal chemotaxis occurred in six patients. Polycloncal hypergammaglobulinemia was detected in 14 patients, elevated IgD in two patients, a partial T-cell defect in two patients and a history of lack of antibody response in one patient. Therapeutic trails anti-H2 receptor-antihistamines did not produce lasting or satisfactory clinical or immunological results in the pathogenetically unidentified disease.
对15名年龄在3岁至27岁之间的患者进行了临床和免疫学检查。所有患者的共同症状为严重复发性皮肤和肺部葡萄球菌感染、慢性湿疹、嗜酸性粒细胞增多以及血清IgE水平极高。此外，8名患者存在面部发育异常，表现为面容粗糙、下颌前突和外耳发育不良。8名患者观察到明显的骨质疏松，尤其是椎体。仅3名患者发现粒细胞趋化性持续缺陷；6名患者趋化性波动或持续正常。14名患者检测到多克隆高丙种球蛋白血症，2名患者IgD升高，2名患者存在部分T细胞缺陷，1名患者有抗体反应缺乏病史。在病因不明的疾病中，抗H2受体抗组胺药的治疗试验未产生持久或令人满意的临床或免疫学效果。
