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

Pediatr Pulmonol. 2009 Mar;44(3):297-9. doi: 10.1002/ppul.20992.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Aspergillosis, Allergic Bronchopulmonary / complications*
  • Aspergillosis, Allergic Bronchopulmonary / diagnosis
  • Aspergillosis, Allergic Bronchopulmonary / drug therapy
  • Asthma / drug therapy
  • Asthma / etiology
  • Child, Preschool
  • Eczema / drug therapy
  • Eczema / etiology
  • Endocrine System Diseases / complications*
  • Endocrine System Diseases / diagnosis
  • Endocrine System Diseases / genetics
  • Forkhead Transcription Factors / genetics
  • Genes, X-Linked / genetics
  • Glucocorticoids / therapeutic use
  • Humans
  • Infant
  • Intestinal Diseases / complications*
  • Intestinal Diseases / diagnosis
  • Intestinal Diseases / genetics
  • Lymphoproliferative Disorders / complications*
  • Lymphoproliferative Disorders / diagnosis
  • Lymphoproliferative Disorders / genetics
  • Male
  • Mutation
  • Prednisolone / therapeutic use

Substances

  • FOXP3 protein, human
  • Forkhead Transcription Factors
  • Glucocorticoids
  • Prednisolone