Bronchoalveolar lavage fluid cytokine profiles in neuroendocrine cell hyperplasia of infancy and follicular bronchiolitis

Orphanet J Rare Dis. 2013 Nov 11;8:175. doi: 10.1186/1750-1172-8-175.


Background: Neuroendocrine Cell Hyperplasia of Infancy (NEHI) and Follicular Bronchiolitis (FB) are rare pediatric diffuse lung diseases with poorly understood pathogenesis and similar clinical presentations. We sought to determine if cellular and cytokine profiles in bronchoalveolar lavage fluid (BALF) from subjects with NEHI and FB would differ from pediatric disease controls.

Methods: BALF was obtained from forty-one subjects classified into four disease groups: NEHI, Cystic Fibrosis (CF), other airway disease controls (DC), and FB during clinically indicated procedures. BALF cellular profiles and ten cytokines were measured and values compared across groups using descriptive and nonparametric statistics.

Results: Significant BALF cellular and cytokine differences were seen across all groups. NEHI subjects exhibited the lowest total absolute white blood cell (WBC) levels with a higher percentage of BALF alveolar macrophages compared to controls. NEHI also had lower levels of IL-1β, MIP-1β and IL-8 and FB had higher levels of IL-1ra, G-CSF and VEGF compared to all groups. IL-6 was elevated in CF and FB.

Conclusions: BALF cytokine and cellular profiles differed between NEHI, FB, CF and DC subjects. This pilot data suggests different and distinguishing inflammatory responses in the airway, with the least inflammatory being NEHI. These data could have diagnostic implications.

MeSH terms

  • Bronchiolitis / metabolism*
  • Bronchoalveolar Lavage Fluid / chemistry*
  • Child
  • Child, Preschool
  • Cytokines / metabolism*
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Leukocytes / metabolism
  • Male
  • Neuroendocrine Cells / metabolism*
  • Neutrophils / metabolism


  • Cytokines