(NEHI, Persistent Tachypnea of infancy)

Extremely rare: it is estimated to represent 8 % of the interstitial lung pathologies of the child. A mutation of the NKX2-1/TTF-1 (14q13.3) or FOXP1(17q23.2) genes is involved in some cases.

Reminder: During fetal life, neuroendocrine cells are present in the bronchi and bronchioles. They are either isolated within bronchial cells or grouped together to form neuroendocrine bodies. These neuroendocrine cells have both the role of a nerve cell and that of an endocrine cell, and have an important role in the growth and development of the lungs and in the production of surfactant. After birth, neuroendocrine cells should normally almost disappear.

In case of hyperplasia of the neuroendocrine cells of the infant, too many neuroendocrine cells are observed in the respiratory tract. This leads to chronic inflammation of the lung and bronchoconstriction. The clinical picture corresponds to what is called 'persistent tachypnea of the infant'. The first symptoms are tachypnea or dyspnea occurring during exercise, during breast or bottle feeding and sometimes at rest. Other signs may appear: intercostal indrawing, wheezing, cyanosis in case of hypoxemia, feeding difficulties, digital clubbing.

Treatment: corticosteroids (generally not very effective), hydroxychloroquine or azithromycin (antibiotic). Oxygen therapy during the night and sometimes during the day or during exercise. Food supplements (energy drinks) or feeding by nasogastric tube or gastrostomy may be necessary to ensure normal growth.

Anesthetic implications:

SpO2 at room air; gentle ventilation; lung biopsy.

References :

-        Liptzin DR, Pickett K, Brinton JT, et al.
Neuroendocrine cell hyperplasia of infancy. Clinical score and comorbidities.
Ann Am Thorac Soc 2020; 17: 724-8        

Updated: October 2021