Taking people in United StatesNo phase

Following how noncirrhotic portal hypertension develops over time

Follows people with liver blood vessel pressure problems to learn how the disease develops.

Key facts

Who runs it: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Where: Bethesda, United StatesSee the site
Trial code: NCT02417740
Registry title: Natural History of Noncirrhotic Portal Hypertension
Status: Recruiting
People taking part: 400 people, planned
Started: 27 Jul 2015
Expected to end: 4 Sept 2029
Type of study: Observational, 4 groups
Record updated: 9 November 2026

Who can take part

AI summary

From the trial's eligibility criteria. Machine-read from the registry text on this page, so it can be wrong. The registry text below and the trial team decide who can take part.

The trial is looking for people aged 12 and older who have noncirrhotic portal hypertension (NCPH) or are at risk of getting it because of another disease.

Age: 12 to 100 years (adults 18 and older, and young people 12 to 17).
Sex: All sexes can join.
Disease stage: A known diagnosis of NCPH, or being at risk for NCPH because of an underlying disease such as CGD, SCD, Mastocytosis, CVID, CF or CHF.

Main reasons for not taking part

Pregnancy
Other liver diseases that usually lead to cirrhosis, or cirrhosis confirmed by liver biopsy
Active hepatitis B or hepatitis C infection
Active substance abuse (alcohol, inhaled or injection drugs) within the past year
Signs of liver cancer or bile duct cancer

The registry criteria, word for word

Show all 18 criteriaShow less

Who cannot take part

Bile duct obstruction as suggested by imaging studies done within the previous six months
Active substance abuse, such as alcohol, inhaled or injection drugs within the previous one year (assessed during subject interviews by subject self-report)
Evidence of other forms of liver disease that typically result in cirrhosis
Evidence of hepatocellular carcinoma; either alpha-fetoprotein (AFP) levels greater than 50 ng/ml (normal <6.6ng/ml) and/or ultrasound (or other imaging study) demonstrating a mass suggestive of liver cancer
Any other severe condition, which in the opinion of the investigators would impede the patient s participation or compliance in the study
Evidence of active Chronic Hepatitis B infection as defined by the presence of hepatitis B surface antigen (HBsAg) in serum and elevated HBV DNA (>10,000 IU/mL)
Hepatitis C as defined by the presence of hepatitis C RNA in serum
Evidence of other liver disease such as primary sclerosing cholangitis, primary biliary cirrhosis, Wilson s disease, autoimmune hepatitis as defined by either liver histology or laboratory abnormalities
Hemochromatosis as defined by presence of 3+ or 4+ stainable iron on liver biopsy or homozygosity for C282Y. Patients with iron saturation indices of >45% and serum ferritin levels of >300 ng/ml for men and >250 ng/ml for women will undergo genetic testing for hemochromatosis
An individual who meets any of the following criteria will be excluded from participation in this study:
Pregnancy
The presence of cirrhosis confirmed by liver biopsy
Evidence of cholangiocarcinoma as suggested by liver histology
Inability to comply or give written informed consent

Other criteria

INCLUSION CRITERIA:; In order to be eligible to participate in this study, an individual must meet all of the following criteria:
Male or female, aged >= 18 years of age, and minors 12-17 years of age
Women of childbearing potential must agree to use birth control unless they are menopausal or had hysterectomy
Known diagnosis of NCPH, or to be at the risk for NCPH by virtue of underlying disease processes such as but not limited to; CGD, SCD, Mastocytosis, CVID, CF, and CHF

Check if you might fit

Answer for yourself. This is not a decision: only the study team can say whether you can take part.

Are you between 12 and 100 years old?

Where in United States

National Institutes of Health Clinical CenterBethesda, United StatesContact: Jaha Norman-WheelerTaking people

About the trial

What the trial wants to find out

Background:

Read full original descriptionShow less

- 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.

The 4 groups in the trial

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.
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).
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.
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).