Cantani A, Ferrara M
Department of Pediatrics, University of Rome La Sapienza, Italy.
Allergol Immunopathol (Madr). 1988 Nov-Dec;16(6):429-37.
Primary immunodeficiences (ID), once considered to be very rare, are now increasingly recognized thanks to more knowledge in the immunological field and to the availability of more sophisticated diagnostic techniques. The respiratory tract and especially the lung are the most common targets of primary ID, and respiratory infections and/or failure are the most frequent causes of early death when the immune defect is not corrected with HLA-identical bone-marrow trans lant or, in few selected cases, with thymic extracts. Many patients with Ig deficiency are severely affected by chronic and recurrent respiratory infections, unfortunately leading to bronchiectasis in the majority of such patients. Today early i.v. gammaglobulin administration can prevent bronchiectasis, and this increases the chances of a better prognosis and of an improved quality of life as well.
原发性免疫缺陷病（ID），曾被认为非常罕见，如今由于免疫学领域知识的增多以及更先进诊断技术的应用，越来越受到认可。呼吸道尤其是肺部是原发性免疫缺陷病最常见的靶器官，当免疫缺陷未通过 HLA 配型相同的骨髓移植或在少数特定情况下通过胸腺提取物纠正时，呼吸道感染和/或呼吸衰竭是早期死亡的最常见原因。许多 Ig 缺乏患者受到慢性复发性呼吸道感染的严重影响，不幸的是，大多数此类患者会发展为支气管扩张。如今，早期静脉注射丙种球蛋白可预防支气管扩张，这也增加了获得更好预后和改善生活质量的机会。
