Endobronchial lesion in a premature neonate.

Endobronchial and endotracheal tumours are rare in the paediatric population, especially in neonates. The common presentation is respiratory distress with persistent lung collapse or hyperinflation. Treatment usually involves endoscopic or surgical lobar resection. This case presents a preterm neonate who developed acute respiratory distress with persistent right upper lobe atelectasis. A flexible bronchoscopy showed a pediculate violaceous endobronchial lesion in the right main bronchus. Endoscopic resection was not possible due to the patient's low weight and the decision was made to support the patient with continuous positive airway pressure (CPAP) at home, while monitoring her closely. Remarkably, the lesion spontaneously resolved, and CPAP support was discontinued successfully. The case emphasises the importance of early bronchoscopy, continuous monitoring and the possibility of using CPAP support until conditions are reunited for an eventual resection. This unique case also demonstrates the potential for spontaneous resolution in some cases.

[Long-term home oxygen therapy in children: evidences and open issues]. / Oxigenoterapia Domiciliária de Longa Duração na Criança: Evidências e Questões em Aberto.

INTRODUCTION: Long-term home oxygen therapy is indicated for patients with chronic hypoxemia. We intend to describe pediatric population on long-term home oxygen therapy followed-up at Pediatric Respiratory Unit of a tertiary care hospital between 2003-2012 and to compare with previous 1991-2000 review; to verify conformity with international and national recommendations and need for specific pediatric national guidelines, non-existent in Portugal. MATERIAL AND METHODS: Retrospective, descriptive and comparative study based on clinical files review. Review the guidelines for oxygen therapy in pediatric population. RESULTS: We studied 86 patients (59.3% males). The median age at the beginning of oxygen therapy was 0.0 (0.0-216.0) months, with a median duration of 15.0 (3.0-223.0) months. The most frequent diagnosis was bronchopulmonary dysplasia (53.5%), followed by bronchiolitis obliterans (14.0%), neurologic disorders (10.5%), cystic fibrosis (8.1%), miscellaneous syndromes (5.8%), sickle-cell disease (3.5%), other neonatal lung diseases (2.3%) and interstitial lung diseases (2.3%). Are maintained on follow-up 53 (61.6%) patients, 38 on oxygen therapy; 12 (13.9%) died. The median time of follow-up was 39.5 (1.0-246.0) months, minim on other neonatal lung diseases and maximum on cystic fibrosis. Comparing with previous review, this shows a relative increase in bronchiolitis obliterans and bronchopulmonary dysplasia patients, with increased duration in the latter, and inclusion of neurologic and hematologic patients. DISCUSSION: Prescription of long-term oxygen therapy in pediatric age mainly occurs in specific diseases of infants and pre-school aged. Neurologic and hematologic patients represent new indications, similarly to international publications. CONCLUSION: The knowledge of national reality and pediatric orientations are needed for care plans and rational prescription.

IntroduçÉo: Oxigenoterapia domiciliária de longa duraçÉo está indicada em doentes com hipoxémia crónica. Pretendemos descrever a populaçÉo em programa de oxigenoterapia domiciliária de longa duraçÉo acompanhada numa Unidade de Pneumologia Pediátrica de Hospital Terciário entre 2003-2012 e comparar com revisÉo de 1991-2000; verificar conformidade com orientações nacionais e internacionais, refletindo sobre necessidade de orientações nacionais especificamente pediátricas, inexistentes em Portugal.Material e Métodos: Estudo retrospetivo, descritivo e comparativo por consulta de processo clínico. Pesquisa de orientações sobre oxigenoterapia em idade pediátrica.Resultados: Incluímos 86 doentes (59,3% rapazes). A idade mediana de início da oxigenoterapia foi 0,0 (0,0-216,0) meses e a duraçÉo mediana de 15,0 (3,0-223,0) meses. O diagnóstico mais frequente foi displasia broncopulmonar (53,5%), seguindo-se bronquiolite obliterante (14,0%), doença neurológica (10,5%), fibrose quística (8,1%), síndromes polimalformativas (5,8%), doença de células falciformes (3,5%), outras doenças pulmonares neonatais (2,3%) e doenças pulmonares intersticiais (2,3%). Mantêm acompanhamento 53 (61,6%) doentes, 38 mantendo oxigenoterapia; 12 (13,9%) faleceram. O tempo mediano de seguimento foi 39,5 (1,0-246,0) meses, mínimo nas outras doenças pulmonares neonatais e máximo na fibrose quística. Comparativamente ao estudo anterior revela aumento relativo dos lactentes com bronquiolite obliterante e displasia broncopulmonar, aumento da duraçÉo nestes últimos e inclusÉode doentes neurológicos e hematológicos.DiscussÉo: A prescriçÉo de oxigenoterapia domiciliária de longa duraçÉo em pediatria ocorre sobretudo em doenças específicas dos lactentes e idade pré-escolar. Doentes neurológicos e hematológicos sÉo novos grupos de prescriçÉo, à semelhança da literatura internacional.ConclusÉo: O conhecimento da realidade nacional e orientações pediátricas sÉo relevantes para organizaçÉo de cuidados eprescriçÉo racional.

The multiple meanings of "wheezing": a questionnaire survey in Portuguese for parents and health professionals.

BACKGROUND: Most epidemiological studies on pediatric asthma rely on the report of "wheezing" in questionnaires. Our aim was to investigate the understanding of this term by parents and health professionals. METHODS: A cross-sectional survey was carried out in hospital and community settings within the south of Portugal. Parents or caregivers self-completed a written questionnaire with information on social characteristics and respiratory history. Multiple choice questions assessed their understanding of "wheezing". Health professionals (physicians, nurses and physiotherapists) were given an adapted version. We used bivariate analysis and multivariate models to study associations between definitions of "wheezing" and participants' characteristics. RESULTS: Questionnaires from 425 parents and 299 health professionals were included. The term "wheezing" was not recognized by 34% of parents, more frequently those who were younger (OR 0.4 per 10-year increment, 95% CI 0.3-0.7), had lower education (OR 3.3, 95% CI 1.5-7.4), and whose children had no history of respiratory disease (OR 4.6, 95% CI 2.5-8.7) (all ORs adjusted). 31% of parents familiar with "wheezing" either did not identify it as a sound, or did not locate it to the chest, while tactile (40%) and visual (34%) cues to identify "wheezing" were frequently used. Nurses reported using visual stimuli and overall assessments more often than physicians (p < 0.01). The geographical location was independently associated with how parents recognized and described "wheezing". CONCLUSIONS: Different meanings for "wheezing" are recognized in Portuguese language and may be influenced by education, respiratory history and regional terminology. These findings are likely applicable to other non-English languages, and suggest the need for more accurate questionnaires and additional objective measurement instruments to study the epidemiology of wheezing disorders.

Early nasal continuous positive airway pressure versus INSURE in VLBW neonates.

BACKGROUND: Evidence suggests the INSURE strategy (INtubate-SURfactant administration and Extubate to nasal continuous positive airway pressure [nCPAP]) is superior to mechanical ventilation (MV) with rescue surfactant for the management of respiratory distress syndrome (RDS) in very low birth weight (VLBW) neonates. There is limited data, however, to assess whether INSURE is superior to nCPAP alone. We aimed to compare these two strategies regarding early and late outcomes. METHODS: Retrospective cohort study from Jan/2002 to Aug/2008. We included VLBW neonates with gestational age (GA) ≤ 30 weeks registered in the Vermont Oxford Network that used either nCPAP alone (nCPAP group, N=40), or with INSURE (INSURE group, N=56). We assessed early (RDS, need for rescue surfactant, and CPAP failure with MV) and late outcomes (chronic lung disease of prematurity (CLD), mortality, and composite outcome of mortality and/or CLD). RESULTS: Neonates in the INSURE group had lower mean GA, while other baseline variables were comparable. The proportion of RDS was significantly superior in the nCPAP group (53% vs. 30%; adjusted OR [95% CI] - 0.2 [0.1-0.6]). Therapeutic surfactant was administered to all cases with RDS in the nCPAP group, but to none from the INSURE group. MV was more frequently required in the nCPAP group (11/ 40, 28% vs. 9/ 56, 12%; p=0.04). No statistically significant differences were found in either of the late outcomes. CONCLUSION: The use of nCPAP alone in VLBW neonates with GA ≤ 30 weeks may be associated with slightly poorer early outcomes than the INSURE strategy, but there were no significant differences in CLD or mortality.