RecruitingPrimary Sclerosing CholangitisLiver DiseasesCholangitis, Sclerosing

Primary Sclerosing Cholangitis in Children

Eligible age

2–25 yrs

Accepts

All genders

Locations

10 states

Healthy volunteers

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About this study

Primary sclerosing cholangitis (PSC) is a rare liver disease that damages the liver's bile ducts. Bile ducts are tiny tubes that carry bile from the liver to the small intestine. Bile is a liquid produced by the liver that helps us absorb and use the nutrients in the food we eat. In people with PSC, the bile backs up into the liver and will damage it, causing scarring of the liver. The purposes of this study are to: * Collect medical and other data to learn more about PSC, how it progresses, and identify factors that may cause the disease to progress more quickly. * Ask questions about how PSC symptoms affect your child's life to learn more about its impact on your child's daily functioning * Children with PSC who are seen at one of the participating clinical sites in the Childhood Liver Disease Research Network (ChiLDReN) will be asked to contribute information, DNA, and other specimens. The information and specimens will be available to investigators to carry out approved research aimed at learning more about the possible causes and long-term effects of PSC.

Sponsor: Arbor Research Collaborative for Health

You may qualify if…

✓ Patients with the clinical diagnosis of large or small duct PSC made at any time prior to enrollment are screened for eligibility to participate in this prospective cohort study. The site PI will determine eligibility following review of MRCP or ERCP images with the site radiologist to confirm presence of an abnormal cholangiogram at the time of diagnosis of large duct PSC. Liver histopathology obtained at the time of diagnosis of small duct PSC will be reviewed with the site pathologist prior to enrollment.
✓ Individuals must meet all of the Inclusion criteria in order to be eligible to participate in the study:
✓ 1. Aged 2 through 25 years at time of screening.
✓ 2. Diagnosis of large duct PSC based on review of cholangiogram by MRC, ERC, or intraoperative cholangiogram (IOC) by the site radiologist and interpreted to be consistent with PSC, based on one or more of the following:
✓ Focal structuring of the bile duct(s)
✓ Dominant stricture of the common bile duct
✓ Saccular dilatation of bile duct(s)
✓ Beaded appearance of bile duct(s)

You may not qualify if…

✕ An individual who meets any of the following criteria at baseline will be excluded from participation in this study.
✕ 1. History of liver transplantation
✕ 2. History bone marrow transplantation
✕ 3. History of primary or acquired immunodeficiency predisposing to secondary sclerosing cholangitis, for instance: hyper-IgM syndrome, severe combined immunodeficiency (SCID) syndrome, common variable immunodeficiency (CVID) syndrome, cartilage hair hypoplasia syndrome, or HIV/AIDS
✕ 4. History of histiocytosis, including Langerhans cell histiocytosis (LCH), or hemophagocytic lymphohistiocytosis (HLH)
✕ 5. History of ischemic cholangitis
✕ 6. History of portal vein thrombosis with biliopathy, veno-occlusive disease, or abdominal radiation vasculopathy
✕ 7. History of recurrent pyogenic cholangitis