Oury C, Daumling S, Buriot D, Belohradsky B H, Griscelli C
Arch Fr Pediatr. 1982 Jun-Jul;39(6):353-8.
Nineteen patients exhibiting a susceptibility to infections due to staphylococcus and fungi, allergy and hyper IgE are reported (syndrome described by R. Buckley). Prognathism, coarse features and marked osteoporosis were observed in more than half the patients. Immune disorders were characterized by a defective chemotaxis of granulocytes and depressed in vivo and in vitro immune responses to antigens. These cellular abnormalities could be secondary to a dysregulation of IgE synthesis resulting in an excess of secretion of histamine, heparin and other substances from mast cells. According to this possible mechanism, we suggest a therapy associating anti-histaminic substances which block H1 and H2 receptors and an agent which inhibits mast cell degranulation.
报告了19例对葡萄球菌和真菌感染易感、有过敏反应及高IgE血症的患者（R.巴克利描述的综合征）。超过半数患者存在颌前突、面容粗糙及明显骨质疏松。免疫紊乱的特征为粒细胞趋化性缺陷以及体内和体外对抗原的免疫反应低下。这些细胞异常可能继发于IgE合成失调，导致肥大细胞分泌过多组胺、肝素及其他物质。根据这一可能机制，我们建议采用一种联合疗法，即使用能阻断H1和H2受体的抗组胺物质以及一种抑制肥大细胞脱颗粒的药物。
