Neuroendocrine Cell Hyperplasia of Infancy

Abstract

Neuroendocrine cell hyperplasia of infancy (NEHI) is a disease of the unknown etiology which is one of the rare, insufficiently explored interstitial lung diseases. It might be encountered among children up to two years old and it is characterized by persistent tachypnea, crackles, hypoxemia without being oxygen-dependent and hyperplasia of bombesin-positive neuroendocrin cells of peripheral respiratory tracts in lung biopsy. The lung CT shows that there are mosaic attenuation air trapping involving more than four lung segments and ground glass opacity mostly marked in the middle lobe of the right lung and lingular segments of the left lung. А precise diagnosis establishment based on a combination of clinical signs and HRCT data is possible without implementation of invasive research methods, lung biopsy in particular. At present there is no specific treatment for this disease; infants diagnosed with NEHI require special monitoring since it is not known whether this disease has a connection with idiopathic diffusive hyperplasia of PNE-cells of adults (IDYPNC) and how this pathology might affect lung development and their function in adults. The most part of patients with NEHI have satisfactory clinical results and there have been no fatal cases. Despite such positive data on this understudied disease, infants with NEHI require special monitoring and comprehensive research since long-term effects of this disease in an older age are still undiscovered. In this article case of NEHI in 1,5 years old child with a complaint for tachypnea and cough is submitted.