Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series

Abstract

Objective: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. Methods: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. Results: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. Conclusions: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment.Keywords: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/treatment; Tomography, X-ray computed. ResumoObjetivo: A hiperplasia de células neuroendócrinas do lactente (HCNEL) é uma forma de doença pulmonar intersticial da infância caracterizada por taquipneia, retrações, estertores e hipóxia. O objetivo deste estudo foi descrever e discutir os achados clínicos, histopatológicos e de imagem em uma série de casos de HCNEL em um hospital pediátrico terciário, enfatizando critérios de diagnóstico e desfechos clínicos. Métodos: Entre 2003 e 2011, 12 lactentes nascidos a termo foram diagnosticados com HCNEL, com base em critérios clínico-tomográficos e acompanhados por 1-91 meses. Quatro lactentes foram submetidos a biopsia pulmonar, e as amostras histopatológicas foram coradas com anticorpo para bombesina. Resultados: Nesta série de casos, os sintomas surgiram ao nascimento em 6 lactentes e em até 3 meses de idade nos outros 6. Em todos os casos, HCNEL estava associada com infecção respiratória aguda. Os achados iniciais em TCAR de tórax foram opacidades em vidro fosco em lobo médio e língula, em 12 pacientes, e em outras regiões medulares, em 10. O aprisionamento aéreo foi o segundo achad...

Persistent Tachypnea of Infancy. Usual and Aberrant

“…Some patients, however, have additional CT abnormalities, such as consolidation, bronchial wall thickening, and interlobular septal thickening. Sometimes ground-glass opacities are absent or have a different distribution (3,6,7,21). With regard to biopsy-proven PIG, the few published CT studies have shown mainly ground-glass opacities in a subsegmental, segmental, or diffuse distribution (11,15), very similar to what was found in NEHI (11): interlobar septal thickening or scarring, hyperinflation, air trapping, multifocal atelectasis (7), and multiple air-filled cystic changes of variable size (12).…”

Update on Diffuse Lung Disease in Children

“…9,19,[26][27][28][29][30][31][32][33][34][35][36][37] A primary advance in this area is the growing recognition that NEHI can be diagnosed by using a chest CT scan without the need for lung biopsy. Brody et al 9 first fully described the radiologic features of NEHI in 2010.…”

Diffuse Lung Disease

“…NEHI is not a life-threatening condition and most patients show clinical and radiographic improvement, especially after two years of age. However, some patients require supplemental oxygen for months to years, and air trapping and exercise intolerance may persist into adolescence (Deterding 2010;Gomes et al 2013). The development of nonatopic asthma has also been reported in follow-up (Lukkarinen et al 2013).…”

“…A frontal CXR (c) obtained at 3 years of age shows continued pulmonary hyperinflation always reliable, especially if airway sampling is limited in the biopsy specimen . A presumptive diagnosis of NEHI can be made without lung biopsy if the clinical presentation and the findings on CT or pulmonary function testing are characteristic (Deterding 2010;Young et al 2010;Brody et al 2010;Kerby et al 2013;Gomes et al 2013). …”