Dataset from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH)

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

本研究的具体研究目标如下：
1. 通过对比超声检查显示回声异质性模式的囊性纤维化（cystic fibrosis）受试者与超声检查显示回声均质模式的囊性纤维化受试者，明确超声检查结果能否预测肝病进展为肝硬化的风险。
2. 构建涵盖血清、血浆、尿液及脱氧核糖核酸（DNA）的数据库与生物样本库（biorepository），以助力相关科研工作，明确肝硬化发生的机制、结局、遗传风险因素及生物标志物。
3. 对比超声检查显示回声异质性模式的囊性纤维化儿童与超声检查显示回声均质模式的囊性纤维化儿童，明确二者在健康相关生活质量、肺功能状态或营养状况方面是否存在差异。
4. 明确肝脾血管的多普勒流速测量结果能否预测肝硬化发生风险的升高。
5. 明确研究周期内超声检出的肝硬化是否会进展为门静脉高压（portal hypertension）。
6. 明确回声均质的肝脏是否会进展为肝硬化或回声异质性肝脏。
7. 明确研究开展至第6年时已被检出肝硬化的受试者，在后续随访期间门静脉高压并发症的发生频率。