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Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis
Insights
Focal biliary cirrhosis is uncommon in infants with cystic fibrosis (CF) but affects over 20% of older children. Mucus accumulation in bile ducts and cholestasis are key findings in affected infants.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Pulmonology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Biliary cirrhosis is a known complication, but its prevalence in infants with CF requires further investigation.
Purpose of the Study:
- To determine the incidence and characteristics of focal biliary cirrhosis in infants and children with cystic fibrosis.
- To investigate the association between biliary pathology and age in CF patients.
Main Methods:
- Postmortem examination of liver tissues from infants and children diagnosed with cystic fibrosis.
- Histopathological analysis to identify focal biliary cirrhosis, mucus accumulation, cholestasis, and periportal changes.
Main Results:
- Focal biliary cirrhosis was found in 5/47 infants (<3 months), 5/32 infants (3-12 months), and 18/67 children (>1 year).
- Excessive mucus in intrahepatic bile ducts was observed in infants under 3 months.
- Cholestasis was present in 18/26 infants, and nonspecific periportal changes were noted in younger infants but not in those over 1 year.
Conclusions:
- Focal biliary cirrhosis is an age-dependent finding in cystic fibrosis patients.
- Mucus accumulation and cholestasis are significant pathological features in the livers of infants with CF.
Abstract:
Focal biliary cirrhosis is an uncommon finding in infants with cystic fibrosis, but it is present in more than a fifth of surviving children and adolescents. It was found at postmortem examination in only five of 47 infants with CF younger than 3 months, in five of 32 infants from 3 to 12 months, and in 18 of 67 children older than 1 year. In infants under 3 months, excessive mucus in intrahepatic bile ducts was seen in 11 necropsies; in 15 others there were only nonspecific periportal changes. Cholestasis was found in the livers of 18 of the 26 infants. Excessive mucus in the biliary tree was occasionally associated with periportal changes and cholestasis in older infants. The periportal changes, which are regarded as nonspecific, were never found in infants more than 1 year of age.