Immunophenotyping and protein profiling of Fontan-associated plastic bronchitis airway casts.

ABSTRACT:

Rationale

Plastic bronchitis (PB) is a rare and deadly condition that is characterized by the formation of airway casts. It most frequently occurs in children with underlying congenital heart disease that has been surgically palliated by the Fontan procedure. The Fontan circulation results in above-normal central venous pressure, and it has been hypothesized that the formation of airway casts is due to lymph leak. Knowledge of plastic bronchitis pathogenesis is poor and stems mostly from published case reports.

Objectives

To garner information about cast pathogenesis by characterizing inflammatory cell phenotypes in existing formalin-preserved, paraffin-embedded samples and generating protein and cytokine-chemokine profiles of airway cast homogenates.

Methods

We used

SUBMITTER: Racz J 

PROVIDER: S-EPMC3960899 | biostudies-literature | 2013 Apr

REPOSITORIES: biostudies-literature