Ranganathan, Sarath; (2002) Lung function in infants with cystic fibrosis: Findings at diagnosis and changes with time. Doctoral thesis (Ph.D), UCL (University College London).

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Abstract

Cystic fibrosis is the most common lethal genetic disorder in the white population. Chronic inflammation and infection result in destruction of lung architecture and respiratory morbidity is the leading cause of death among those with cystic fibrosis. Although neutrophil-dominated inflammation similar to that seen in older subjects has been identified in the lungs of affected infants, the evolution of airway pathology in early infancy remains poorly understood. Interpretation of previous lung function studies is difficult and it remains uncertain whether airway function is impaired at or shortly after diagnosis. In older subjects with cystic fibrosis, spirometric measurements are used to assess airway function. A method to assess forced expiration over an extended volume range in infants has been described recently. This new method was used to measure airway function in a prospective longitudinal study, recruiting infants from five cystic fibrosis centres in London. Lung function was assessed in study and control infants on two occasions. The main aim of the study was to assess the distribution of pulmonary function abnormalities at diagnosis and six months later in infants with cystic fibrosis and to examine the cross-sectional and longitudinal associations of airway function with anthropometric and clinical status. The findings presented in this thesis demonstrate that airway function is diminished soon after diagnosis in infants with cystic fibrosis and does not appear to catch-up during infancy.

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