Objective: To study the clinical features of neuroendocrine cell hyperplasia of infancy (NEHI) in order to provide a basis for the management of diagnosis, treatment and prognosis of children with NEHI.
Methods: A retrospective analysis was performed for the clinical data of seven children with NEHI who were diagnosed and treated from January 2014 to March 2016.
Results: Among the seven children with NEHI, there were five boys and two girls. Two children experienced tachypnea since the neonatal period, and five children developed respiratory tract symptoms within 1-6 months after birth. Of the 7 children, 6 had pulmonary crackles, 4 had hypoxemia, and 3 had gastroesophageal reflux. Lung high-resolution CT (HRCT) showed ground-glass opacities in the central region of the lungs in all children, which involved at least two lung lobes. Of the 7 children, 2 had the involvement of more than 4 lobes and 6 had air trapping. All 7 children had an improvement in clinical symptoms after two years of age. One child achieved clinical and CT remission. Four children achieved clinical remission, but still with CT changes.
Conclusions: NEHI often occurs in infancy, with the major clinical manifestations of persistent tachypnea, pulmonary crackles, and hypoxemia. The children with NEHI often present ground-glass opacities in the central region of the lungs and air trapping on HRCT. There is no specific treatment for this disease and most cases have a good prognosis.
目的: 总结婴儿神经内分泌细胞增生症（NEHI）的临床特点，为制订NEHI患儿的诊断、治疗、预后等病程管理提供依据。
方法: 回顾性分析2014年1月至2016年3月诊治的7例NEHI患儿的临床资料。
结果: 7例患儿中，男5例，女2例；2例患儿从新生儿期开始呼吸增快，5例患儿生后1~6个月出现呼吸道症状；6例患儿有肺部爆裂音，4例有低氧血症，3例合并胃食管反流。肺部高分辨率CT（HRCT）显示所有患儿均存在肺中心区磨玻璃样病变，并且至少2个肺叶受累；2例患儿4叶以上肺叶受累；6例患儿有气体潴留。所有患儿在2岁后临床症状好转；1例达到临床和CT缓解；4例临床缓解，但仍有CT改变。
结论: NEHI常婴儿期起病，临床表现主要为持续性气促、肺部爆裂音和低氧血症，HRCT常表现为肺中心区磨玻璃样病变并气体潴留。该病无特异性治疗，大部分病例预后良好。