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

Abstract

BackgroundNeuroendocrine 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.MethodsBALF 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.ResultsSignificant 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.ConclusionsBALF 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.

Novel Application of Aptamer Proteomic Analysis in Cystic Fibrosis Bronchoalveolar Lavage Fluid

“…ELISA assays were performed in the Pediatric CTRC Core Laboratory at Children's Hospital Colorado (see Supporting Information). To cross‐validate SOMAmer measurements in BALF, correlations between SOMAmer values and results of commercially‐available ELISAs were calculated for a subset of 16 proteins and 43 banked BALF samples . The banked BALF samples included seven subjects with CF from this project as well as 36 BALF samples from subjects with a diagnosis of children's interstitial lung disease.…”

Dyspnoea on exertion in a child: A presentation of neuroendocrine hyperplasia of infancy

“…With further research and validation of data in this series, BAL specimens may well be considered diagnostic in time to come. [12] In some cases, where the typical features are not seen on a HRCT chest scan, a lung biopsy may be required to diagnose NEHI. The initial H&E stains may show mild inflammatory changes.…”

“…[5] In the case series which described these exacerbations, oxygen was used in one case, and Zithromax with prednisone in the other; both showed resolution over time. [12] As this syndrome gains more recognition in the international literature, management strategies during an exacerbation will probably be alluded to.…”

Update on Diffuse Lung Disease in Children

“…9,19,[26][27][28][29][30][31][32][33][34][35][36][37] A primary advance in this area is the growing recognition that NEHI can be diagnosed by using a chest CT scan without the need for lung biopsy. Brody et al 9 first fully described the radiologic features of NEHI in 2010.…”