Natural History of Noncirrhotic Portal Hypertension

Part of paid clinical trials in Bethesda, Maryland.

Sponsor
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Study ID
NCT02417740
Status
Recruiting
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Conditions

  • Congenital Hepatic Fibrosis
  • Cystic Fibrosis
  • Idiopathic Non-Cirrhotic Portal Hypertension
  • Immunologic Deficiency Syndrome
  • Turner Syndrome

Eligibility Criteria

Sex
ALL
Age
12 Years - 100 Years
Healthy Volunteers
Not accepted

Study Details

Background: \- Noncirrhotic Portal Hypertension (NCPH) is caused by liver diseases that increase pressure in the blood vessels of the liver. It seems to start slowly and not have many warning signs. Many people may not even know that they have a liver disease. There are no specific treatments for NCPH. Objectives: \- To learn more about how NCPH develops over time. Eligibility: \- People age 12 and older who have NCPH or are at risk for getting it. In the past year, they cannot have had other types of liver disease that typically result in cirrhosis, liver cancer, or active substance abuse. Design: * Participants will have 2 screening visits. * Visit 1: to see if they have or may develop NCPH. * Medical history * Physical exam * Urine and stool studies * Abdominal ultrasound * Fibroscan. Sound waves measure liver stiffness. \<TAB\>- Visit 2: * Blood tests * Abdominal MRI * Echocardiogram * Questionnaire * Liver blood vessel pressure (hepatic venous portal gradient (HVPG)) measurement. This is done with a small tube inserted in a neck vein. * They may have a liver biopsy. * All participants will visit the clinic every 6 months for a history, physical exam, and blood tests. They will also repeat some of the screening tests yearly. * Participants with NCPH will also have: * Upper endoscopy test. A tube inserted in the mouth goes through the esophagus and stomach. * At least every 2 years: Esophagogastroduodenoscopy. * At least every 4 years: testing including HVPG measurements and liver biopsy. * Participants without NCPH will also have: * Liver biopsy and HVPG measurements to see if they have NCPH. * Every 2 years: abdominal MRI and stool studies. * The study will last indefinitely.

Key Dates

Start date
Jul 27, 2015
Status verified
May 2026
Primary completion
Sep 4, 2029
Completion
Sep 4, 2029

Study Design

Enrollment
400 participants (estimated)

Arms

  • Arm: Adult with absence of Portal Hypertension
    Confirmed absence of Portal Hypertension will have no findings suggestive of non cirrhotic portal hypertension on liver biopsy and on portal pressure measurements on confirmatory examination.
  • Arm: Adult with presence of Portal Hypertension
    Confirmed Presence of Noncirrhotic Portal Hypertension, through confirmatory testing, tissue diagnosis by liver biopsy and/or portal hypertension (HVPG \>5mmHg).
  • Arm: Minors likely to have the absence of Portal Hypertension
    Minors identified as Confirmed Absence of Noncirrhotic Portal Hypertension will have no abnormal findings on confirmatory examination.
  • Arm: Minors likely to have the presence of Portal Hypertension
    Minors identified as Confirmed Presence of Noncirrhotic Portal Hypertension, have shown they have the disease with a tissue diagnosis by liver biopsy and/or portal hypertension (HVPG \>5).

Primary Outcome Measure

To study the natural history of non cirrhotic portal hypertension. It is an ongoing study. [ Time Frame: Ongoing ]

Central Contacts

Locations (1)

FacilityCityStateZIPSite coordinators
National Institutes of Health Clinical CenterBethesdaMaryland20892
For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR)
[email protected]
800-411-1222

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By research site
National Institutes of Health Clinical Center· Bethesda, MD

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