ABSTRACT
Introducción. Sobre la base de un caso clínico se presenta la descripción de un cuadro intersticial pulmonar por hiperplasia de células neuroendocrinas en un lactante. Método. Seguimiento clínico desde el sexto mes hasta los 17 meses con extensos estudios para descartar otras patologías semejantes. El diagnóstico definitivo fue determinado por taquipnea persistente y rales crepitantes con imágenes en vidrio esmerilado características, en lóbulo medio y língula. La biopsia pulmonar fue normal a la observación con microscopio óptico a pesar de las imágenes evidentes en TAC. La tinción con bombesina demostró acumulaciones anormales de células neuroendocrinas de 10,9% en bronquiolos e hiperinsuflación (Childrens Hospital of Colorado). Resultados. Se confirmó en este lactante con síntomas de cuadro intersticial la patología pulmonar sospechada: hiperplasia de células neuroendocrinas. Ésta debe ser sospechada en un lactante con: taquipnea, rales crepitantes persistentes, posible desnutrición, radiografía hiperinsuflada, tomografía que muestra imágenes en mosaico tipo vidrio esmerilado especialmente en lóbulo medio y língula. Biopsia pulmonar casi normal y aumento porcentual de células neuroendocrinas en bronquios periféricos.
Introduction. A clinical case of a 6 month old infant with symptoms of interstitial lung disease is presented. Methodology. Follow up until 17 months of age is described. Extensive studies were included to rule out other similar pathologies of infancy. Final diagnosis was determined by characteristic clinical symptomatology of persistent tachypnea and crepitant rales, hyperinflated chest x rays, CT scan presenting ground glass opacities in the middle lobe and lingula. Lung biopsy was nearly normal and bombesin staining showed increased percentage of neuroendocrine bronchial cells (10.9%) (Childrens Hospital of Colorado). Results. NEHI was confirmed in this 6 months old infant. This diagnosis should be suspected in a tachypneic infant, with persistent fine rales, possible undernutrition, chest x-rays hyperinflation and CT scan with ground glass opacities more characteristically in middle lobe and lingula. Lung biopsy is near normal but staining with bombesin shows increased number of neuroendocrine cells and neuroendocrine bodies.
Subject(s)
Humans , Infant, Newborn , Infant , Lung Diseases, Interstitial/diagnosis , Tachypnea , Diagnosis, Differential , Tomography, X-Ray Computed , Respiration DisordersABSTRACT
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. .
OBJETIVO: 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 achado mais frequente, em 7 pacientes. As TCAR de controle (realizadas em 10 pacientes) revelaram resultados normais (em 1) e melhorias (em 9). Os achados de biopsia foram inespecíficos, e os resultados para bombesina foram positivos em todas as amostras. A confirmação de HCNEL baseou-se principalmente em achados clínico-tomográficos. Os sintomas melhoraram durante o seguimento (média, 41 meses). Quatro pacientes apresentaram cura clínica. CONCLUSÕES: Os achados clínicos e tomográficos permitiram o diagnóstico de HCNEL nesta série de pacientes, independentemente dos resultados da biopsia pulmonar. A maioria mostrou melhora clínica e ...