Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis

Overview

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

For subjects in longitudinal follow up, this study will: 1. Collect detailed clinical and demographic information about each subject at enrollment and during follow up, 2. Obtain and store imaging data from the subject at entry and during follow up, 3. Obtain and store serum, plasma and urine samples from the subject at entry (after matching) and during follow up, 4. Obtain and store DNA from the subject, 5. Obtain and store DNA from the biological parents, 6. Obtain and store quality of life data from the subject and parents at enrollment and during follow up

Conditions