Yancheva Slaveya G, Velani Asha, Rice Alexandra, Montero Angeles, Hansell David M, Koo Sergio, Thia Lina, Bush Andrew, Nicholson Andrew G
Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
National Heart and Lung Institute, Imperial College, London, UK.
Histopathology. 2015 Oct;67(4):501-8. doi: 10.1111/his.12672. Epub 2015 Apr 13.
We have analysed levels of bombesin-positive neuroendocrine cells (NECs) in neuroendocrine cell hyperplasia of infancy (NEHI) and other childhood interstitial lung diseases (chILDs) in order to validate proposed histological criteria for NEHI and investigate its aetiology.
The extent of bombesin-positive cells within airway epithelium was analysed in lung biopsies from seven patients diagnosed with NEHI, including two classified previously as non-diagnostic, and other chILDs (n = 64) with age ranges of 1 month-18 years. NECs were counted and calculated as a percentage of airways containing NECs, average percentage of NECs per airway, percentage of airways with >10% NECs and number of neuroendocrine bodies (NEBs). Correlation with age and gender was also undertaken. Patients with NEHI had the highest average percentage of bombesin-positive NECs per airway compared to other chILDs. However, NEH was also seen in many other chILDs, and appears to be most prominent in disorders associated with lung immaturity such as histological patterns associated with surfactant protein-related disorders and pulmonary interstitial glycogenosis.
NEH may, to a degree, be a marker of airway immaturity rather than the direct cause of NEHI. This possibility is supported by the fact that the number of bombesin-positive NECs decreased with age in this cohort, independent of disease type. The average percentage of bombesin-positive NECs per airway appears to be the best histological criterion for assessing the extent of NECs in the context of NEHI.
我们分析了婴儿期神经内分泌细胞增生症（NEHI）及其他儿童间质性肺疾病（chILDs）中胃泌素释放肽阳性神经内分泌细胞（NECs）的水平，以验证针对NEHI提出的组织学标准并探究其病因。
分析了7例诊断为NEHI患者（包括2例先前分类为无法确诊的病例）以及其他chILDs患者（n = 64，年龄范围为1个月至18岁）肺活检组织中气道上皮内胃泌素释放肽阳性细胞的范围。对NECs进行计数，并计算为含有NECs的气道百分比、每个气道中NECs的平均百分比、NECs>10%的气道百分比以及神经内分泌体（NEBs）的数量。还进行了与年龄和性别的相关性分析。与其他chILDs相比，NEHI患者每个气道中胃泌素释放肽阳性NECs的平均百分比最高。然而，在许多其他chILDs中也可见到神经内分泌细胞增生（NEH），并且在与肺不成熟相关的疾病中最为突出，例如与表面活性蛋白相关疾病和肺间质糖原沉积症相关的组织学模式。
在一定程度上，NEH可能是气道不成熟的标志物，而非NEHI的直接病因。这一可能性得到以下事实的支持：在该队列中，胃泌素释放肽阳性NECs的数量随年龄增长而减少，与疾病类型无关。每个气道中胃泌素释放肽阳性NECs的平均百分比似乎是评估NEHI背景下NECs范围的最佳组织学标准。
