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The evolution of liver disease in cystic fibrosis
S C Ling1, J D Wilkinson, A S Hollman
1Department of Child Health, University of Glasgow, Yorkhill NHS Trust, Glasgow G3 8SJ, UK. lingsimon@hotmail.com
Insights
Liver abnormalities are common in children with cystic fibrosis (CF) and can be predicted by biochemical changes. However, these hepatic issues do not impact nutritional status in CF patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Cystic Fibrosis Research
Background:
- Liver disease is a known complication in cystic fibrosis (CF).
- Understanding the progression and impact of liver abnormalities in pediatric CF is crucial for patient management.
Purpose of the Study:
- To prospectively track the development of liver abnormalities in children with CF.
- To evaluate the relationship between liver disease and nutritional status in this population.
Main Methods:
- Longitudinal follow-up of 124 children with CF over a median of four years.
- Annual assessments included clinical examination, biochemistry, ultrasound, anthropometry, and bacterial colonization.
- Data analysis focused on the evolution of liver abnormalities and their correlation with nutritional markers.
Main Results:
- At baseline, significant proportions of children exhibited biochemical (42%) and ultrasound (35%) liver abnormalities.
- During follow-up, 68% of children developed clinical or ultrasound evidence of liver abnormality.
- Abnormal biochemistry often preceded or coincided with new liver abnormalities, but no association was found with nutritional status.
Conclusions:
- Hepatic abnormalities are prevalent in children with CF and frequently preceded by biochemical changes.
- The development of liver disease in pediatric CF does not appear to negatively affect nutritional status.
Objectives:
To describe prospectively the evolution of liver abnormalities in cystic fibrosis (CF), and to assess their impact on nutritional status.
Study Design:
124 children (61 boys) with CF (median age, 5.4 years; range, 0.1-13.9) were followed longitudinally for a median of four years. Annual clinical examination, biochemistry, and ultrasound assessment were performed. Chrispin-Norman score, anthropometry, and bacterial colonisation of airway secretions were measured at each assessment.
Results:
At initial assessment, 45% of the patients had no liver abnormalities, 42% had biochemical abnormality, 35% ultrasound abnormality, and 6% had clinical abnormality of the liver. In this cross sectional analysis, abnormal biochemistry was present in 40% of children with ultrasound or clinical abnormalities, but when longitudinal follow up data were analysed, abnormal biochemistry preceded or coincided with abnormal ultrasound or clinical hepatosplenomegaly in three quarters of 53 children developing new abnormalities. Eighty four of 124 children (68%) showed ultrasound or clinical evidence of liver abnormality at some point during the four years of follow up. No association was found between liver disease and nutritional status.
Conclusions:
Hepatic abnormality was common in this group of children with CF, was often predicted by intermittent biochemical abnormalities, and was not associated with deterioration in nutritional status.