Georgia Pediatric Pulmonology Associates, P,C,, 1100 Lake Hearn Drive, Suite 450, Atlanta, GA 30342, USA.
Orphanet J Rare Dis. 2013 Nov 11;8:175. doi: 10.1186/1750-1172-8-175.
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.
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.
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.
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.
婴儿神经内分泌细胞增生症(NEHI)和滤泡性细支气管炎(FB)是两种罕见的儿科弥漫性肺部疾病,其发病机制尚未完全阐明,临床表现相似。我们试图确定 NEHI 和 FB 患者支气管肺泡灌洗液(BALF)中的细胞和细胞因子谱是否与儿科疾病对照组存在差异。
在临床指征下,从 41 名受试者中获取 BALF,这些受试者分为四个疾病组:NEHI、囊性纤维化(CF)、其他气道疾病对照组(DC)和 FB。使用描述性和非参数统计方法比较各组间 BALF 细胞谱和十种细胞因子的差异。
所有组之间均存在明显的 BALF 细胞和细胞因子差异。NEHI 组的总绝对白细胞(WBC)水平最低,与对照组相比,BALF 肺泡巨噬细胞的比例更高。与所有组相比,NEHI 组的 IL-1β、MIP-1β 和 IL-8 水平较低,而 FB 组的 IL-1ra、G-CSF 和 VEGF 水平较高。CF 和 FB 组的 IL-6 升高。
NEHI、FB、CF 和 DC 受试者的 BALF 细胞因子和细胞谱存在差异。这些初步数据表明,气道中的炎症反应不同且具有区别性,NEHI 的炎症反应最轻。这些数据可能具有诊断意义。