Abstract
The specific aims for this study are:
1. To determine if sonographic findings predict the risk of progression of liver disease to
cirrhosis by comparing cystic fibrosis subjects with heterogeneous echogenicity pattern
on ultrasound to those with normal echogenicity pattern on ultrasound
2. To develop a database and biorepository of serum, plasma, urine and DNA to aid the
investigations in ascertaining the mechanisms, consequences, genetic risk factors and
biomarkers for the development of cirrhosis
3. To determine if there are differences in health related quality of life, pulmonary or
nutritional status in children with cystic fibrosis who have a heterogeneous echo
pattern on ultrasound compared to those who have a normal echo pattern on ultrasound
4. To determine if Doppler velocity measurements of hepatic and splenic vessels predict an
increased risk for the development of cirrhosis.
5. To determine if cirrhosis on ultrasound progresses to portal hypertension during the
study period
6. To determine if homogeneous liver progresses to either cirrhosis or heterogeneous liver.
7. To determine the frequency of complications of portal hypertension during follow up in
those identified with cirrhosis by year 6 of the study
