부산대학교 도서관

Keywords: XIAP deficiency; Immune dysregulation; IL-18; Tadekinig alfa EN XIAP deficiency Immune dysregulation IL-18 Tadekinig alfa 901 903 3 06/07/22 20220501 NES 220501 To the Editor: X-linked inhibitor of apoptosis (XIAP) deficiency, also known as X-linked lymphoproliferative syndrome type 2 (XLP2), is an inflammasomopathy and adaptive immune deficiency characterized by hyperinflammation and immune dysregulation. We describe this single patient experience to ensure clinicians are aware of potential new therapies for patients with XIAP deficiency since there are limited treated options for patients with this rare disease. Patients with XIAP deficiency have chronically elevated IL-18 and targeting this proinflammatory cytokine may represent a promising precision therapeutic approach to XIAP deficiency. [Extracted from the article]