Bilora F, Petrobelli F, Boccioletti V, Pomerri F
Department of Surgery and Medicine, Padua University.
Minerva Med. 2000 May-Jun;91(5-6):113-6.
Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic "cold" abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after IVIG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.
乔布综合征(或高免疫球蛋白E综合征)是一种罕见的遗传性疾病,其特征为皮肤湿疹、化脓性“冷”脓肿、鼻窦肺部反复感染以及血浆中IgE浓度升高。乔布综合征的治疗效果并不理想,且相关研究案例仍然有限。为描述静脉注射免疫球蛋白(IVIG)疗法对一名患有高IgE综合征和肺炎的37岁女性的疗效。我们在IVIG治疗前后,通过免疫荧光法检测血清IgE,并通过在博伊登小室中的迁移来检测中性粒细胞趋化性。中等剂量的IVIG消除了金黄色葡萄球菌支气管肺炎的临床放射学体征,并改善了细胞学和生物体液参数。静脉注射免疫球蛋白是治疗乔布综合征急性肺炎的一种有效方法。