Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients

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Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients
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H Patriquin1, C Lenaerts, L Smith

  • 1Department of Medical Imaging, Sainte-Justine Hospital, Côte Ste-Catherine Montreal, Quebec, Canada.

Radiology
|April 6, 1999
PubMed

Insights

Abnormal liver appearance on ultrasound in children with cystic fibrosis (CF) often indicates poor liver function. Signs of portal hypertension and cirrhosis on ultrasound are particularly linked to abnormal liver function tests in pediatric CF patients.

Area of Science:

  • Pediatric Gastroenterology
  • Medical Imaging
  • Hepatology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the liver.
  • Liver disease is a known complication in children with CF.
  • Abnormal liver architecture on ultrasound may correlate with impaired liver function.

Purpose of the Study:

  • To investigate the relationship between abnormal liver architecture visualized via ultrasonography (US) and abnormal liver function in pediatric patients with CF.
  • To assess the diagnostic value of specific US findings in predicting liver dysfunction.

Main Methods:

  • A cohort of 195 children with CF underwent abdominal US and liver function tests over one year.
  • US findings were categorized (e.g., edema, steatosis, cirrhosis, portal hypertension).
  • Liver function test results (AST, ALT, GGT) were analyzed and compared with US findings.

Main Results:

  • 19% of children had abnormal liver sonograms; 63% of these had abnormal liver function tests.
  • Children with normal liver architecture showed a significantly lower prevalence of biochemical abnormalities.
  • Signs suggestive of portal hypertension and cirrhosis on US were highly associated with abnormal liver function.

Conclusions:

  • A significant correlation exists between abnormal liver architecture on US and abnormal liver function tests in children with CF.
  • Ultrasonographic findings of portal hypertension and cirrhosis are the most specific indicators of abnormal liver function in this population.
Abstract

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