Long Term Outcomes Following Early Infection And Inflammation In Cystic Fibrosis Lung Disease
Funder
National Health and Medical Research Council
Funding Amount
$75,006.00
Summary
This study involves the long-term follow up of birth cohort of 98 infants with cystic fibrosis, with the objectives of assessing the impact of lower airway infection and inflammation in infancy and early childhood on the development of CF lung disease over 20-25 years, and of describing the development of the lower airway microbiota in early CF lung disease and its relationship to clinical outcomes.
We have shown that premature birth leads to abnormalities in kidney structure and function. This project will determine in human infants, whether premature birth when combined with poor growth in the womb leads to an increase in these kidney abnormalities. Using animal studies we will examine specific factors which may adversely impact on kidney growth before and after premature birth. The findings are very relevant to the long-term kidney health of indigenous Australians.
Role Of Viruses In The Development Of Lung Disease In Cystic Fibrosis
Funder
National Health and Medical Research Council
Funding Amount
$1,223,186.00
Summary
This study will investigate how lung disease starts in babies with cystic fibrosis and the role of viral infections in this process. The new knowledge gained will help us move towards treatments that prevent or delay the start of lung disease, something not currently possible. We believe this new treatment paradigm will lead to improved quality and extent of life of those with cystic fibrosis.
The Centre For Research In Childhood Early Respiratory Disease
Funder
National Health and Medical Research Council
Funding Amount
$2,621,023.00
Summary
The Centre for Research in Childhood Early Respiratory Disease is dedicated to the prevention of lung disease in young children with chronic respiratory disease. We aim to better understand the process of lung disease to identify predictors of disease and to treat it better. We will investigate the psychosocial effects of early interventions to better manage families. We will facilitate collaborations to provide best practice guidance and will train the next generation of doctors and researchers
Professor Stick is a career clinician researcher in Paediatric Respiratory Medicine. He has established worl-leading research teams focused on respiratory diseases in young children such as cystic fibrosis and asthma. He has has developed innovative methods to study early childhood respiratory diseases and has been responsible for training scores of clinicians and scientists in the field. He has received national and international awards in recognition of his achievements.
Hepatic Fibrogenesis In Paediatric Cholestatic Liver Disease.
Funder
National Health and Medical Research Council
Funding Amount
$254,250.00
Summary
Liver disease in children causes a significant impact on lifespan and quality of life. The commonest causes of liver disease in children are cholestatic, or diseases related to obstruction of bile flow out of the liver. In ways we are only beginning to understand, obstruction of bile flow stimulates liver scar formation which, if untreated, leads to replacement of normal liver tissue and ultimately to failure of the liver. In infants, the most common and serious cholestatic liver disease is bili ....Liver disease in children causes a significant impact on lifespan and quality of life. The commonest causes of liver disease in children are cholestatic, or diseases related to obstruction of bile flow out of the liver. In ways we are only beginning to understand, obstruction of bile flow stimulates liver scar formation which, if untreated, leads to replacement of normal liver tissue and ultimately to failure of the liver. In infants, the most common and serious cholestatic liver disease is biliary atresia. It develops at, or shortly after birth with progressive destruction of the bile ducts, responsible for transporting bile out of the liver. Without early diagnosis and surgery these infants develop progressive liver scarring leading to liver failure and death or liver transplantation within 1-2 years. It is the commonest reason for liver transplantation in children (55-60%) in the Western world. Even with successful surgery, most, if not all patients will come to liver transplantation over the subsequent 25 years because of ongoing, but slower, scar formation. In older children, diseases like cystic fibrosis cause bile duct blockages leading to progressive liver scarring that is slower and unpredictable, contributing to ill health in up to 20% of patients and death from end stage liver disease or liver transplantation in 5%. Using liver tissue from children with these two disorders we have been able to identify the key cells that control the liver scar process, the Hepatic Stellate Cell. We now need to investigate the role of bile constituents on the scar-forming process in these two diseases. We will utilise a well characterised animal model to investigate the influence of bile constituents on cells isolated from this model and apply these findings back to patient samples to determine their role in paediatric cholestatic liver disease. This will help us to better understand the disease process and importantly, develop more effective and earlier treatment.Read moreRead less
Early Origins Study Of Cystic Fibrosis-related Diabetes
Funder
National Health and Medical Research Council
Funding Amount
$128,188.00
Summary
Cystic Fibrosis is a genetic condition that causes recurrent lung infections and early death. Some patients also develop diabetes which causes a more rapid decline. Many young children with CF have “pre-diabetes” but guidelines do not recommend screening high glucose in this group. In order to intervene early, this study aims to determine if “pre-diabetes” in very young children with Cystic Fibrosis causes poorer growth, lung function or an increase in bacterial infections to.
A Randomized Controlled Trial Of Effects Of Early Life Exposure To General Anaesthesia On Neurobehavioural Outcomes In Children With Cystic Fibrosis (CF)
Funder
National Health and Medical Research Council
Funding Amount
$587,240.00
Summary
Anaesthesia permits surgeries and other interventional procedures that benefit the health of children to be performed painlessly and non-traumatically. This study will provide critical information about whether the drugs used commonly for general anaesthesia represents a risk to very young children in terms of their neurobehavioural development.