RT Journal Article
SR Electronic
T1 Neuroendocrine cell hyperplasia (NEHI) and NEHI Syndrome: Lung biopsy necessary?
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P3741
VO 44
IS Suppl 58
A1 Marcela Moreno
A1 Silvia Castillo
A1 Bárbara Fernández
A1 Vicente Posadas
A1 Olga Benavent
A1 Amparo Escribano
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P3741.abstract
AB Background: NEHI, initially known as "persistent tachypnea of infancy", is a rare early debut disorder (tachypnea, hypoxemia, crackles and failure to thrive), with a characteristic high resolution computerized tomography (HRCT), and usually favorable evolution without specific treatment, which make doubts about the need for routine biopsy.Objectives: To compare clinical and radiological course of 5 children, 2 with diagnosis of NEHI by lung biopsy and 3, classified as NEHI syndrome (diagnosis by clinical and radiological data without biopsy).Patients: All cases debuted before one year of age (mean 5 months). All had tachypnea (>60 breaths/minute), failure to thrive, oxygen saturation between 85-94% and crackles. HRCT shows a characteristic pattern of ground-glass opacity in the central regions of lungs (more in middle lobe and lingula). No specific changes in bronchoalveolar lavage. Surfactant protein deficiency and mutations were excluded (only one patient with low Pro-SC but normal surfactant protein C). In 2 cases with poor outcome, lung biopsy, which confirmed the diagnosis, was performed (European reference center/Dr. Griese). The outcome was favorable in all cases, without receiving any specific treatment.Conclusion: The clinic and radiological evolution is very similar in our patients. Basing on this, in infants who meet clinical and radiological criteria of NEHI and have favourable evolution, it could be avoid biopsy, as long as they behave as we expect in a NEHI.