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Advances in the management of primary immunodeficiency

机译：原发性免疫缺陷管理的进展

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Primary immunodeficiency disorders (PIDs) are a heterogeneous group of diseases often presenting in childhood. Over 150 disease phenotypes due to 120 gene defects are now recognised. Their hallmark is a propensity to unusual infections but other manifestations include autoimmune phenomena, malignancy and non-immunologic features. Recognition and understanding of PIDs continue to grow rapidly and major advances have been made in both supportive and curative therapies for PID. The cornerstones of management remain antimicrobial treatment (including prophylaxis), immunoglobulin (IG) replacement and stem cell corrective therapies. Effective treatment needs an individualised approach taking into account the natural history of the patient's underlying diagnosis, their personal infection history and current status. These considerations, and the rarity of individual PID diagnoses, have meant that few randomised controlled trials have been conducted in this field. However, it is clear that persons with PID are living much longer and healthier lives, due to better tests for early diagnosis of infection, new anti-infective agents, better Ig replacement regimens and improved outcome after haematopoietic stem cell transplantation.
机译：原发性免疫缺陷疾病（PID）是常见于儿童期的异类疾病。现已认识到由于120种基因缺陷而导致的150多种疾病表型。他们的标志是不寻常感染的倾向，但其他表现包括自身免疫现象，恶性肿瘤和非免疫学特征。对PID的认识和理解继续迅速增长，并且在PID的支持性和治疗性治疗方面都取得了重大进展。管理的基石仍然是抗菌治疗（包括预防），免疫球蛋白（IG）替代和干细胞矫正疗法。有效的治疗需要一种个体化的方法，要考虑到患者基本诊断的自然史，他们的个人感染史和当前状况。这些考虑因素以及个别PID诊断的稀有性，意味着在该领域几乎没有进行过随机对照试验。但是，很明显，由于更好的感染早期诊断测试，新的抗感染药，更好的Ig替代疗法以及造血干细胞移植后的改善结局，PID患者的寿命更长，更健康。

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