Molecular, cellular, and clinical studies of human inborn errors of immunity have revolutionized our understanding of their pathogenesis, considerably broadened their spectrum of immunological and clinical phenotypes, and enabled successful targeted therapeutic interventions. These studies have also been of great scientific merit, challenging a number of immunological notions initially established in inbred mice while revealing previously unrecognized mechanisms of host defense by leukocytes and other cells and of both innate and adaptive tolerance to self.

中文翻译：

---

人类与生俱来的免疫缺陷：不断膨胀的宇宙。

对人类先天性免疫缺陷的分子、细胞和临床研究彻底改变了我们对其发病机制的理解，大大拓宽了它们的免疫学和临床表型谱，并实现了成功的靶向治疗干预。这些研