None listed
Conditions
Brief summary
Although the lungs in children with cystic fibrosis (CF) are essentially normal at birth, airway infection and inflammation may begin within the first few weeks of life. As postnatal lung development, including significant alveolarisation continues during the first 2-3 years of life, this is a vulnerable period where undiagnosed infection and chronic inflammation may lead to irreversible tissue damage and lung disease. Identifying and treating infection (especially with Pseudomonas), in early childhood may prevent significant lung disease, reduce morbidity and improve survival. Early infection is often subclinical and conventional screening methods such as sputum microbiology, chest radiology and lung function, are either not possible or insensitive to detect disease in this age group. Upper airway cultures such as throat swabs or cough suction specimens correlate poorly with lower airway pathogens and the currently available systemic markers of inflammation lack sensitivity. Bronchoaclveolar lavage (BAL) is considered the gold standard for identifying airway infection and inflammation in young children with CF. The association between BAL markers of infection and inflammation and sensitive infant lung function testing such as the multiple breath washout test which determines the lung clearance index has not been studied. This study will investigate early CF lung disease by assessing the relationships between BAL markers of infection/inflammation, the lung clearance index, systemic markers of neutrophil inflammation (S100 proteins) and urinary desmosine. The study will also determine the prevalence of gastro-oesophageal reflux and the relationship between BAL pepsin and airway infection and inflammation.
Interventions
Newborn screened infants with cystic fibrosis less than 3 years of age attending the Cystic Fibrosis clinic at Sydney Children's Hospital Randwick will be prospectively recruited for bronchoalveolar lavage, infant lung function testing, a chest xray and venepuncture.
A control group of similarly aged healthy infants undergoing sedated cardiac echo or a DMSA scan will also be recruited for infant lung function testing over the same period thus serving as the normative or reference population for
Newborn screened infants with cystic fibrosis less than 3 years of age attending the Cystic Fibrosis clinic at Sydney Children's Hospital Randwick will be prospectively recruited for bronchoalveolar lavage, infant lung function testing, a chest xray and venepuncture. A control group of similarly aged healthy infants undergoing sedated cardiac echo or a DMSA scan will also be recruited for infant lung function testing over the same period thus serving as the normative or reference population for infant lung function. Healthy in fants and infants with CF will undergo sedated lung function on a single occasion over a duration of approximately one hour.
Sponsors
Dr Yvonne Belessis MBBS(Hons) FRACP MPH PhD
Eligibility
Sex/Gender
All
Age
0 to 5 Years
Healthy volunteers
No
Inclusion criteria
Cystic fibrosis infants under age 3 years and healthy control children under 3 years of age will be recruited for sedated lung function testing. CF children up to 5 years of age will also undergo venepuncture and urine analysis for desmosine determination
Exclusion criteria
Chronic neonatal lung disease Other respiratory disease Cardiac disease Neuurologic disease Respiratory infection in the 3 weeks prior to infant lung function testing
Outcome results
None listed