NCT02417740

Natural History of Noncirrhotic Portal Hypertension

Recruiting now Last updated 27 March 2026

What this trial tests

trial in Cystic Fibrosis in 400 participants. Currently enrolling.

Timeline

27 July 2015

Primary endpoint
4 September 2029

4 September 2029

Quick facts

Lead sponsor	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Status	Recruiting now
Study type	OBSERVATIONAL
Enrollment	400
Start date	27 July 2015
Primary completion	4 September 2029
Estimated completion	4 September 2029
Sites	1 location across United States

Conditions studied

• Cystic Fibrosis — all drugs for Cystic Fibrosis →
• Immunologic Deficiency Syndrome — all drugs for Immunologic Deficiency Syndrome →
• Turner Syndrome — all drugs for Turner Syndrome →
• Congenital Hepatic Fibrosis — all drugs for Congenital Hepatic Fibrosis →

Sponsor

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Who can join

Adults 12 to 100, any sex, with Cystic Fibrosis or Immunologic Deficiency Syndrome. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

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.

Publications & conference data

2 peer-reviewed publications reference this trial (live from Europe PMC):

1. Noninvasive Markers Associated With Portal Hypertension and Varices in Patients With Noncirrhotic Portal Hypertension.
   Gopalakrishna H, Mironova M, Majeed NA, Scott S, et al · · 2026 · PMID 41410017 · DOI 10.14309/ctg.0000000000000956
2. Cardiopulmonary changes and its association with clinical features in noncirrhotic portal hypertension.
   Gopalakrishna H, Nguyen ML, Mironova M, Viana Rodriguez GM, et al · · 2025 · PMID 40904883 · DOI 10.3748/wjg.v31.i30.109256