Characterization of Pulmonary Artery Stenoses in Alagille Syndrome - a Medical Record Review

NCT01515631 | Completed

• 1 other identifier: AG9031
• Study Type: observational
• Target: 25
• Locations: 1 country
• Sites: 1

Brief Summary

Patients who have Alagille Syndrome (AGS) also frequently have blockages (or "stenoses") of their pulmonary arteries. Little is known about the degree or variability of these stenoses, or the effect of this disease on the right ventricle (the chamber of the heart which pumps blood to the lungs). This study will first quantify and describe pulmonary artery stenosis in patients with Alagille Syndrome. The study will also assess the effect of these stenoses on the right ventricle. The investigators hope to learn the degree and characteristics of pulmonary artery stenosis in Alagille Syndrome. The investigators also hope to learn the effect of this pulmonary artery stenosis on the right ventricle in patients with Alagille Syndrome. This information is critical in the management of patients with Alagille syndrome, as there is currently no data to guide clinicians on the management of pulmonary artery stenosis. Furthermore, the information from this study may help physicians manage pulmonary artery stenosis in other patients as well.

Conditions

Alagille Syndrome

Outcome Measures

Primary Outcomes (1)

• Assessment of PA in Alagille Patient

  Open

Study Arms (1)

Study

Patients with alagille syndrome

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with Alagille Syndrome. The age range will be from birth through adulthood. There will be no gender or ethnic limitations to study.

You may qualify if:

• Diagnosis of Alagille Syndrome

You may not qualify if:

• None

Sponsors & Collaborators

• Stanford University: lead

Study Sites (1)

Lucile Packard Children's Hospital at Stanford

Palo Alto, California, 94304, United States

Location

MeSH Terms

Conditions

Alagille Syndrome

Condition Hierarchy (Ancestors)

Cholestasis, Intrahepatic; Cholestasis; Bile Duct Diseases; Biliary Tract Diseases; Digestive System Diseases; Liver Diseases; Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn

Study Officials

• Jeffrey Feinstein, MD, MPH

  Stanford University

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: January 18, 2012
• First Posted: January 24, 2012
• Study Start: April 1, 2007
• Primary Completion: February 1, 2018
• Study Completion: February 1, 2018
• Last Updated: September 28, 2018

Record last verified: 2018-09

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)