Allergic bronchopulmonary aspergillosis in a 2-year-old asthmatic boy with immune dysregulation, polyendocrinopathy, enteropathy, X-linked.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is caused by impaired function of CD4+CD25+ regulatory T cells that play an important role in controlling exaggerated Th2 responses. The pathogenesis of allergic bronchopulmonary aspergillosis (ABPA) appears to be closely associated with the Th2 response. We report the first case of ABPA in a 2-year-old asthmatic boy with IPEX, this being an unusually young age for the development of ABPA. Pediatr Pulmonol. 2009; 44:297–299. © 2009 Wiley-Liss, Inc.