Función pulmonar: función pulmonar en fibrosis quística / Lung function in cystic fibrosis

La fibrosis quística (FQ) es una enfermedad hereditaria autosómica recesiva, causada por la mutación del gen que codifica la proteína CFTR (cystic fibrosis transmembrane conductance regulator), afecta varios órganos, pero la enfermedad pulmonar es la primera causa de morbimortalidad. El diagnóstico a través del screening neonatal (SNN) y los nuevos tratamientos moduladores del CFTR han aumentado el interés por pesquisar y monitorizar la función pulmonar antes del inicio de los síntomas para lograr un tratamiento adecuado y oportuno con una mejor calidad de vida. Existen numerosas formas de medir la función pulmonar según la edad, colaboración y recursos disponibles. En este artículo se resumen las pruebas clásicas y las más novedosas, como técnicas de imágenes, en la búsqueda de marcadores precoces de daño pulmonar, herramientas con los que cada centro de fibrosis quística debiera contar en la era de tratamientos moduladores del CFTR, que están cambiando el pronóstico de los pacientes con esta enfermedad.

Cystic fibrosis (CF) is an autosomal recessive inherited disease, caused by mutation of the gene encoding the CFTR protein (cystic fibrosis transmembrane conductance regulator), affects several organs, but lung disease is the first cause of morbidity and mortality. Diagnosis through neonatal screening (NNS) and new CFTR modulating treatments have increased interest in screening and monitoring lung function before the onset of symptoms to achieve adequate and timely treatment with a better quality of life. There are numerous ways to measure lung function based on age, collaboration, and available resources. This article summarizes the classic and the most innovative tests, which have emerged from imaging techniques in the search for early markers of lung damage, tools that each cystic fibrosis center should have in the era of CFTR modulating treatments, which are changing the prognosis of patients with this disease.

Eventos cardiorrespiratórios adversos após vacinação primária de recém-nascidos de muito baixo peso / Adverse cardiorespiratory events following primary vaccination of very low birth weight infants

OBJETIVO: Examinar a relação entre vacinação primária de recém-nascidos pré-termo e razões de prevalência de fatores associados a eventos cardiorrespiratórios indesejados, seguindo recomendações do Comitê Consultivo de Práticas de Imunização do Centers for Disease Control and Prevention para imunização de recém-nascidos pré-termo aos 2 meses de idade cronológica. MÉTODOS: Estudo retrospectivo de 2 anos de recém-nascidos de muito baixo peso que receberam vacinação primária. Foram registrados eventos cardiorrespiratórios maiores, como apneia, bradicardia, dessaturação de SpO2, e eventos menores, como instabilidade de temperatura, comportamento inapropriado e reações locais. Foi calculada a razão de prevalência com intervalo de confiança de 95% para fatores associados entre recém-nascidos com e sem eventos cardiorrespiratórios. RESULTADOS: Foram estudados 80 recém-nascidos (mediana de peso ao nascer [:intervalo]: de 970 g [:428-1.490]:), idade gestacional de 27,4 semanas (23,3-33). Ocorreram reações adversas em 35 (44%): eventos menores em 19 (24%) pacientes, eventos maiores em 28 (35%). Recém-nascidos com eventos maiores tiveram idade gestacional significativamente menor (p = 0,008) e incidência mais alta de displasia broncopulmonar (71% versus 48%; p < 0,05). Em recém-nascidos de muito baixo peso com eventos maiores, o número de casos de dessaturação de O2 antes da vacinação foi 3,40 (1,41-8,23) vezes maior, e o tratamento com metilxantina para síndrome de apneia e bradicardia foi 8,05 (2,50-25,89) vezes maior em comparação com recém-nascidos sem eventos maiores. CONCLUSÃO: Eventos cardiorrespiratórios maiores ocorreram em mais de 1/3 de todos os recém-nascidos de muito baixo peso após a vacinação. Os fatores associados foram baixa idade gestacional, displasia broncopulmonar, tratamento com metilxantina e dessaturação de O2 persistente antes da vacinação. A vacinação primária de recém-nascidos de muito baixo peso deve ser realizada sob monitoramento contínuo de parâmetros vitais.

OBJECTIVE: To examine the relationship between primary vaccination of preterm infants and prevalence ratios of associated factors for unwanted cardiorespiratory events, following the recommendation of the Centers for Disease Control and Prevention Advisory Committee on Immunization Practices for immunization of preterm infants at 2 months of chronological age. METHODS: Two-year retrospective study of very low birth weight infants receiving their primary vaccination. Major cardiorespiratory events, such as apnea, bradycardia, SpO2 desaturation, and minor adverse events, such as temperature instability, poor handling and local reactions, were recorded. Prevalence ratio with 95% confidence interval for associated factors between infants with and without cardiorespiratory events was calculated. RESULTS: Eighty neonates were studied (median [:range]: birth weight 970 g [:428-1,490]:), gestational age of 27.4 weeks (23.3-33). Adverse reactions occurred in 35 (44%): minor events in 19 (24%) patients, major events in 28 (35%). Infants with major events had significantly lower gestational age (p = 0.008) and a higher incidence of bronchopulmonary dysplasia (71% vs. 48%; p < 0.05). In very low birth weight infants with major events, O2 desaturations before vaccination were 3.40 (1.41-8.23) times higher and treatment with methylxanthines for apnea and bradycardia syndrome was 8.05 (2.50-25.89) times higher compared to infants without major events. CONCLUSION: Major cardiorespiratory events occurred in over 1/3 of all very low birth weight infants after vaccination. Associated factors were low gestational age, bronchopulmonary dysplasia, methylxanthine treatment, and persisting O2 desaturations before vaccination. Primary vaccination of very low birth weight infants should be performed under continuous monitoring of vital parameters.

Ventilatory strategies and adjunctive therapy in ARDS.

Acute respiratory distress syndrome, a diagnosis based on physiologic and radiological criteria, occurs commonly in critical care setting. A major challenge in evaluating therapies that may improve survival in ARDS is that it is not a single disease entity but, rather, numerous different diseases that result in endothelial injury, where the most obvious manifestation is within the lung resulting in pulmonary oedema. It has been shown that poor ventilatory technique that is injurious to the lungs can propagate systemic inflammatory response and adversely affect the mortality. The current data suggest that high tidal volumes with high plateau pressures are deleterious and a strategy of ventilation with lower tidal volumes and lower plateau pressure is associated with lower mortality. There may be a role for recruitment manoeuvres as well. Other forms of respiratory support still require further research. The present understanding of optimal ventilatory management and other adjunctive therapies are reviewed.