Actively Recruiting

All Genders
ID04908319

Hepatic Histopathology in Urea Cycle Disorders

Led by Baylor College of Medicine · Updated on 2025-07-20

70

Participants Needed

2

Research Sites

N/A

Total Duration

On this page

Sponsors

B

Baylor College of Medicine

Lead Sponsor

C

Children's National Research Institute

Collaborating Sponsor

AI-Summary

What this Trial Is About

Urea cycle disorders (UCDs) are common inherited liver metabolism conditions. Improved diagnosis and treatments have increased survival rates, revealing some long-term liver complications like dysfunction and fibrosis in some patients. These liver issues vary depending on the specific metabolic defect causing the disorder. The study aims to better understand these complications by examining liver tissue samples from individuals diagnosed with any UCD. This research involves reviewing past medical records and prospectively collecting liver tissue samples from patients with confirmed UCD diagnoses. It includes individuals who have had or are planning to have liver biopsies or transplants. This observational study is conducted across multiple centers within the Urea Cycle Disorders Consortium, including Baylor College of Medicine and Children’s National Medical Center. Participants' liver samples will be evaluated for fibrosis, fat accumulation (steatosis), and glycogen buildup (hepatic glycogenosis). Researchers will analyze histopathology reports, biopsy slides, and tissue blocks to identify these outcomes. The study collects data retrospectively and prospectively, tracking histological changes to better understand liver complications in UCD. Participation involves no interventions and focuses on reviewing existing and new liver tissue findings until study completion in June 2026.

CONDITIONS

Brief Title

Hepatic Histopathology in Urea Cycle Disorders

Who Can Participate

All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Diagnosis of primary urea cycle disorder 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
Not Eligible

You will not qualify if you...

  • Lack of available histopathology report, liver tissue, or slides from liver biopsy or transplant
  • Expected inability to obtain pathology report, liver disease tissue blocks, or pathology slides after liver biopsy or transplant
  • Known secondary liver disease such as chronic viral hepatitis, autoimmune liver disease, short gut syndrome, small bowel syndrome, alcohol liver disease, or TPN-related cholestatic disease

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

Diagnostic Evaluation

Duration - Day 1

Participants undergo liver biopsy or liver transplantation to collect hepatic tissue for histopathology analysis.

1 visit (in-person)

Long-term Monitoring

Duration - Up to study completion date

Participants are observed for hepatic complications such as fibrosis, steatosis, and glycogenosis following tissue analysis.

Trial Site Locations

Total: 2 locations

1

Children's National Medical Center

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

Not Yet Recruiting

2

Baylor College of Medicine

Houston, Texas, United States, 77030

Actively Recruiting

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Research Team

S

Saima Ali, MSN

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

0

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