Emphysema in young adult survivors of moderate-to-severe bronchopulmonary dysplasia

Abstract

Improved survival following extreme preterm birth complicated by bronchopulmonary dysplasia (BPD) is resulting in an increasing number of affected infants surviving to adulthood. The aim of the present pilot study was to describe the functional and structural pulmonary sequelae of moderate and severe BPD in a population of adult survivors.All babies were cared for at one institution (King Edward Memorial Hospital, Subiaco, Australia). Subjects born between 1980 and 1987 with birthweight ,1,500 g and requiring supplementary oxygen at 36 weeks post-menstrual age were identified from a complete neonatal database and recruited prospectively. Local physicians were concurrently asked to refer suitable patients. Demographics, respiratory symptoms and examination results, pulmonary function tests and computed tomography images were acquired.In total, 21 subjects were studied. Of these, 12 were female, the median (range) age was 19 (17-33) yrs and 15 (71%) had persistent respiratory symptoms. The median (range) forced expiratory volume in one second (FEV1) z-score was -0.77 (-8.20-1.37), the forced expiratory flow at 25-75% of forced vital capacity was -1.81 (-6.00-0.75) and the diffusing capacity of the lung for carbon monoxide was -5.04 (-13.17--1.24). Computed tomography was carried out on 19 subjects and all had abnormal findings, with emphysema being the most common, present in 84% of subjects. The extent of radiological emphysema was inversely related to the FEV1 z-score.Young adult survivors of moderate and severe bronchopulmonary dysplasia may be left with residual functional and characteristic structural pulmonary abnormalities, most notably emphysema.

Bronchopulmonary dysplasia and emphysema: in search of common therapeutic targets

“…In addition, the fact that in various genetically engineered mouse models, disturbed alveolarization was followed by later occurrence of emphysema allows one to raise the assumption of a predisposition to develop emphysema acquired in infancy. Supporting this view, young adult survivors of BPD may be left with residual structural and functional lung abnormalities, including emphysema [60].…”

The building blocks of mammalian lung development

“…These can result from premature birth, genetic variation, or antenatal conditions such as intrauterine infection (Baraldi and Filippone, 2007;Shi et al, 2007). Longitudinal studies of individuals born prematurely, with or without acute lung disease at birth, show that these people generally have decreased lung function and/or more respiratory problems throughout adolescence (Vrijlandt et al, 2006;Narang et al, 2008;Wong et al, 2008). Furthermore, it is often suggested that these individuals will be at increased risk of developing diseases associated with the normal age-related decline in lung function, such as chronic obstructive pulmonary disease (COPD; Maciewicz et al, 2009).…”

Nature and severity of lung function abnormalities in extremely pre-term children at 11 years of age

“…Computerised tomography has revealed a high incidence of structural alterations in these early survivors of BPD [3]. By contrast, since widespread introduction of antenatal corticosteroids and neonatal surfactant therapy in the early 1990s, BPD is now largely restricted to more immature infants delivered during the early saccular phase and ''new'' BPD has been reported to be characterised by disruption of alveolar development, with reduced alveolar number and enlarged airspaces, but less pulmonary fibrosis and lung injury than previously described [4].…”

“…Nevertheless, the degree of persistent airway obstruction, as reflected by spirometry, has remained remarkably constant [2,5,6]. Given that diminished forced expiratory volume in 1 s (FEV1) is a marker of all-cause premature mortality [7] and that those with low LF at initial assessment tend to remain low at subsequent assessments and vice versa [8], there is concern that survivors of preterm birth may be at risk of early onset chronic obstructive pulmonary disease in adulthood [3].…”