Aspectos clínicos e tomográficos de crianças com bronquiectasias não fibrocísticas em um serviço de pneumologia pediátrica

Bronchiectasis is bronchial dilatation, usually diagnosed by chest tomography. Among the main causes are cystic fibrosis, infectious diseases and immunodeficiencies. In the pediatric age group there is a shortage of studies on non-cystic fibrosis bronchiectasis. We seek to describe epidemiological, clinical and etiological characteristics of children and adolescents with non-cystic fibrosis bronchiectasis accompanied in a pediatric Pneumology service. This is a cross-sectional, descriptive study of a sample of 72 patients under 18 years of age, followed up in the pediatric Pneumology service of a tertiary hospital linked to the Unified Health Sys-tem, reference in pediatrics for patients in the capital and in the interior of the state of Ceará The diagnosis was based on tomographic findings. There was a predominance of males (57%). Although the vast majority (90%) started symptoms before the age of 5, less than half (45%) were diagnosed with bronchiectasis before this age group. Almost all patients (97%) reported cough, 90% had expectoration and 80% reported dyspnea. All patients presented crepitations in the auscultation. The most frequently affected lobes were the lowest (23%) and the predominant classification was cylindrical (65%). Among the etiologies, 39% were of unidentified cause followed by post-infectious (28%) immunodeficiency (21%), probable primary ciliary dyskinesia (8%) and aspiration syndrome (4%). We concluded that despite the early onset of symptoms, there was a delay in the diagnosis of bronchiectasis. The proportion of unidentified and post-infectious causes was high. Greater investment is needed to expand diagnostic resources in reference centres

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

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