中国实用儿科杂志

中国实用儿科杂志 ›› 2023, Vol. 38 ›› Issue (3): 190-193.DOI: 10.19538/j.ek2023030606

• 专题笔谈 • 上一篇    下一篇

婴儿神经内分泌细胞增生症

  

  1. 国家儿童医学中心  首都医科大学附属北京儿童医院呼吸科  国家呼吸系统疾病临床医学研究中心，北京  100045
  • 出版日期:2023-03-06 发布日期:2023-03-28

Neuroendocrine cell hyperplasia of infancy

  1. Respiratory Department，Beijing Children’ Hosp-ital?Affiliated to Capital University of Medical Sciences；China National Clinical Research Center for Respiratory Diseases；National Children’s Medical Center，Beijing  100045，China
  • Online:2023-03-06 Published:2023-03-28

摘要： 婴儿神经内分泌细胞增生症（neuroendocrine cell hyperplasia of infancy， NEHI）是一种成熟不良的婴儿间质性肺疾病。NEHI的病因不明，临床上以呼吸急促、三凹征、爆裂音和低氧血症为特点。病理诊断主要靠蛙皮素的免疫组化，远端气道神经内分泌细胞（neuroendocrine cells， NECs）数目增加， NECs存在于至少75%的气道；在每一气道中NECs占10%；缺乏其他气道和间质疾病的特征，肺高分辨肺CT的特征为中心型的磨玻璃影，主要累及右中叶、左舌叶，以及其他区域的气体滞留。NEHI无特异的治疗方法，主要是氧疗和基本的营养支持治疗。NEHI是一种良性疾病，无死亡报道，病情随年龄增长可缓解。

关键词: 神经内分泌细胞增生, 婴儿, 高分辨CT, 间质性肺疾病

Abstract: Neuroendocrine cell hyperplasia of infancy （NEHI） is an pulmonary dysmaturation disorder of interstitial lung disease in infants. The etiology of NEHI is unknown. The clinical features of NEHI are tachypnea， retraction， crackles and hypoxemia. The diagnosis mainly depends on immunohistochemistry of bombesin. The number of neuroendocrine cells （NECs） increases in the distal airway. NECs exist in at least 75% of the airways; NECs account for 10% in each airway; there is lack of the features diagnosed as the other airway and interstitial diseases. High resolution computed tomography （HRCT） of lungs shows central ground glass opacities， mainly involving right middle lobe and left lingula， and air trapping in other areas. There is no specific treatment for NEHI， mainly  oxygen therapy and basic nutritional support treatment. NEHI is a benign process， no death has been reported， and it is relieved with age.

Key words: neuroendocrine cell hyperplasia, infant, high resolution computed tomography, interstitial lung disease