FibroScan™ in Pediatric Cholestatic Liver Disease (FORCE)

Noninvasive monitoring of liver fibrosis is an unmet and critical need within the clinical
management of children with chronic liver disease. While liver biopsy is often used in the
initial diagnostic evaluation of children with liver disease, subsequent surveillance liver
biopsy is rarely performed in children because of its inherent invasiveness and risks.
Therefore, our understanding of the natural history of fibrosis progression in children is
limited. The patchy nature of fibrosis in many important pediatric liver diseases [e.g.
biliary atresia (BA) and cystic fibrosis liver disease (CFLD)] limits the utility of
sequential liver biopsy even if it were to be employed in clinical practice in pediatrics.
Thus, non-invasive means of assessing liver fibrosis throughout the liver would be highly
desirable and clinically useful in pediatric hepatology. ChiLDReN is poised and uniquely
qualified to conduct a comprehensive longitudinal assessment of the utility of
FibroScan™-specific elastography, liver stiffness measurement (LSM) as a measure of hepatic
fibrosis in children with serious chronic cholestatic liver disease.

Inclusion Criteria:

- Age less than 21 years at the time of enrollment

- Participants enrolled in a ChiLDReN based prospective observational cohort study
(PROBE, BASIC, or LOGIC)

- Willingness and ability to participate in the study for up to 24 months

- One of the following three diagnoses

- Biliary atresia per ChiLDReN criteria or,

- Alpha-1 antitrypsin deficiency (PiZZ or SZ) or,

- Alagille Syndrome per ChiLDReN criteria

Exclusion Criteria:

- BA with known situs inversus or polysplenia/asplenia

- Presence of clinically significant ascites detected on physical examination

- Open wound near expected FibroScan probe application site

- Use of implantable active medical device such as a pacemaker or defibrillator

- Known pregnancy

- Prior liver transplant

- Unable or unwilling to give informed consent or assent