Status:

RECRUITING

Hepatic Histopathology in Urea Cycle Disorders

Lead Sponsor:

Baylor College of Medicine

Collaborating Sponsors:

Children's National Research Institute

Conditions:

Urea Cycle Disorder

Ornithine Transcarbamylase Deficiency

Eligibility:

All Genders

Brief Summary

This is a multi-site, retrospective chart review as well as a prospective study to evaluate histopathologic findings in liver samples from individuals with any UCD diagnosis. This study will be conduc...

Detailed Description

Urea cycle disorders (UCDs) are among the most common inborn errors of liver metabolism. With early diagnosis and improved treatments, the survival of individuals with UCDs has improved, and this impr...

Eligibility Criteria

Inclusion

  • Diagnosis of primary urea cycle disorder based on clinical suspicion confirmed by enzyme activity, DNA testing or metabolite analysis.
  • History of liver transplantation and/or liver biopsy OR
  • Planned liver transplantation and/or liver biopsy

Exclusion

  • Unavailability of histopathology report from the liver biopsy or explant, or unavailability of liver tissue or slides from the biopsy or explant OR
  • Anticipated inability to obtain pathology report, liver disease, tissue blocks, or pathology slides after liver biopsy or transplantation
  • Known history of a secondary cause for liver disease such as chronic viral hepatitis, autoimmune liver disease, short gut, small bowel syndrome, alcohol liver disease, or TPN-related cholestatic disease

Key Trial Info

Start Date :

February 24 2022

Trial Type :

OBSERVATIONAL

Allocation :

ESTIMATED

End Date :

June 30 2026

Estimated Enrollment :

70 Patients enrolled

Trial Details

Trial ID

NCT04908319

Start Date

February 24 2022

End Date

June 30 2026

Last Update

July 20 2025

Active Locations (2)

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Page 1 of 1 (2 locations)

1

Children's National Medical Center

Washington D.C., District of Columbia, United States, 20010

2

Baylor College of Medicine

Houston, Texas, United States, 77030