# Continuation of ChiLDReN, the Childhood Liver Disease Research Network: Indiana University

> **NIH NIH U01** · INDIANA UNIVERSITY INDIANAPOLIS · 2022 · $480,001

## Abstract

PROJECT SUMMARY/ABSTRACT
Pediatric liver disease has significant morbidity and mortality. Biliary atresia (BA) accounts for more than
half of pediatric liver transplants. Genetic causes of neonatal cholestasis include Alagille's syndrome
(ALGS), alpha-1 antitrypsin deficiency (A1AT), Progressive Familial Intrahepatic Cholestasis (PFIC), bile
acid synthetic defects, mitochondrial hepatopathies, and cystic fibrosis (CF). All of these diseases can
progress to cirrhosis. The goals of the ChiLDReN network are to follow large numbers of these subjects
longitudinally to allow accumulation of clinical data and biospecimens for studies that can help diagnose,
use biomarkers to prognosticate for, and treat these diseases. Translational research founded on this
remarkable database and biorepository can result in new insights regarding pathogenesis, disease
modifiers, and ultimately therapy. We propose to continue participation in the ChiLDReN Research
Network; our center will recruit patients with ChiLDReN diseases aggressively, retain them for longitudinal
follow-up, and contribute to ongoing studies. We will continue to actively participate in the IMAGINE
clinical trial studying the effect of an intestinal bile-acid transport inhibitor on pruritus. We will continue to
participate in the longitudinal FORCE study of transient elastography to monitor hepatic fibrosis in children
with biliary atresia, A1AT, and ALGS. The CF studies will continue to evaluate the role of ultrasound in
predicting the development of cirrhosis in children with CF (PUSH), the role of transient elastography
(ELASTIC), and magnetic resonance elastography (MRE) to non-invasively monitor fibrosis progression,
and the progression of cirrhosis in this disease. We will participate in the development and implementation
of the Primary Sclerosing Cholangitis (PSC) study, beginning with a database study and progressing to a
clinical trial. The ultimate goal of the network is to learn more about natural history and pathogenesis in
order to inform therapy and clinical management of cholestatic diseases in children.
The Pilot and Feasibility clinical trial proposed by the IU center is a study of Minimal Hepatic
Encephalopathy (MHE) in children with cirrhosis/portal hypertension due to biliary atresia. A panel of
neurocognitive tests of executive function and a parent survey instrument will be used to evaluate children
with biliary atresia-related cirrhosis. Children who are diagnosed with MHE will participate in a clinical trial
of lactulose therapy, acting as their own control, with repeat neurocognitive testing after 3 months.
ChiLDReN data will be used to begin to examine risk factors for the condition. This pilot will generate data
about the frequency of this important sequela of chronic liver disease and guide future multicenter studies
investigating its treatment.

## Key facts

- **NIH application ID:** 10416032
- **Project number:** 5U01DK084536-14
- **Recipient organization:** INDIANA UNIVERSITY INDIANAPOLIS
- **Principal Investigator:** Jean Pappas Molleston
- **Activity code:** U01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $480,001
- **Award type:** 5
- **Project period:** 2009-09-10 → 2024-05-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10416032

## Citation

> US National Institutes of Health, RePORTER application 10416032, Continuation of ChiLDReN, the Childhood Liver Disease Research Network: Indiana University (5U01DK084536-14). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10416032. Licensed CC0.

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