Jyothi S, Lissauer S, Welch S, Hackett S
Department of Emergency Medicine, Birmingham Childrens Hospital NHS Foundation Trust, Birmingham, UK.
Arch Dis Child Educ Pract Ed. 2013 Oct;98(5):186-96. doi: 10.1136/archdischild-2012-302278. Epub 2013 Aug 5.
Primary immune deficiencies (PIDs) are disorders of the immune system that result in increased susceptibility to infectious disease, autoimmunity and malignancy. They are challenging to paediatricians as they can present anytime from birth to adolescence with a wide variety of signs and symptoms. It is important to diagnose PIDs promptly, especially more severe forms to prevent significant morbidity and mortality. However, significant challenges exist in deciding which children to investigate and when. We aim to give a basic understanding of the human immune system, the different presentations in a child that should alert a paediatrician about the possibility of PID and the possible underlying diagnosis. Additionally, we have developed a framework for a stepwise approach to investigating these children.
原发性免疫缺陷病（PIDs）是免疫系统的疾病，会导致对传染病、自身免疫和恶性肿瘤的易感性增加。它们对儿科医生来说具有挑战性，因为这些疾病在从出生到青春期的任何时候都可能出现各种各样的体征和症状。及时诊断原发性免疫缺陷病很重要，尤其是更严重的形式，以预防严重的发病和死亡。然而，在决定对哪些儿童进行检查以及何时检查方面存在重大挑战。我们旨在让大家对人体免疫系统、儿童中应提醒儿科医生注意原发性免疫缺陷病可能性的不同表现以及可能的潜在诊断有一个基本的了解。此外，我们已经制定了一个逐步调查这些儿童的框架。
