Abstract
After more than 30 years, the medical world has come to accept the virtues of newborn screening for the diagnosis of cystic fibrosis (CF). Evidence from Europe, Australia and the USA has demonstrated benefits in terms of nutrition, lung function and survival into adulthood [1, 2]. Similarly, evidence exists that children who present with meconium ileus do worse from the respiratory viewpoint than those diagnosed on newborn screening, supporting the argument that genotypic–phenotypic differences remain incompletely understood [3]. Such observations have been further reinforced by work from the AREST CF (Australian Respiratory Early Surveillance Team for Cystic Fibrosis) group from Australia, demonstrating the early onset of structural lung disease [4, 5], and the London CF consortium [6] and American researchers [7] who have recently shown the benefits on lung function in young children with CF diagnosed by newborn screening compared with those diagnosed at a later stage when symptomatic. The complementary nature of structural and functional lung assessment in providing a broader assessment of the status of young people with CF [8] highlights additional concerns for clinicians managing the routine care of “well” infants and young children with CF. The accumulated evidence argues that the lungs of young children with CF are vulnerable from birth and, whilst newborn screening is clearly beneficial, it is routine practice in many centres to place an emphasis on airway clearance [9] from the start with a view to improving short-term and long-term outcomes. However, this is not necessarily the case, according to a recent Cochrane Review which …
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