RECRUITING

Natural History of Noncirrhotic Portal Hypertension

Conditions

Cystic Fibrosis Immunologic Deficiency Syndrome Turner Syndrome Congenital Hepatic Fibrosis Idiopathic Non-Cirrhotic Portal Hypertension Portal Hypertension Portal Fibrosis Liver Disease Varices Splenomegaly Natural History

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Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

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.

Official Title

Natural History of Noncirrhotic Portal Hypertension

Quick Facts

Study Start:2015-07-27

Study Completion:2029-09-04

Study Type:Not specified

Phase:Not Applicable

Enrollment:Not specified

Status:RECRUITING

Study ID

NCT02417740

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Ages Eligible for Study:12 Years to 100 Years

Sexes Eligible for Study:ALL

Accepts Healthy Volunteers:No

Standard Ages:CHILD, ADULT, OLDER_ADULT

Inclusion Criteria	Exclusion 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.	* Pregnancy. * Evidence of other forms of liver disease that typically result in cirrhosis. * 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. * Bile duct obstruction as suggested by imaging studies done within the previous six months. * The presence of cirrhosis confirmed by liver biopsy. * 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 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. * Evidence of cholangiocarcinoma as suggested by liver histology. * Any other severe condition, which in the opinion of the investigators would impede the patient s participation or compliance in the study. * Inability to comply or give written informed consent.

Inclusion Criteria

Exclusion 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.

* Pregnancy.
* Evidence of other forms of liver disease that typically result in cirrhosis.
* 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.
* Bile duct obstruction as suggested by imaging studies done within the previous six months.
* The presence of cirrhosis confirmed by liver biopsy.
* 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 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.
* Evidence of cholangiocarcinoma as suggested by liver histology.
* Any other severe condition, which in the opinion of the investigators would impede the patient s participation or compliance in the study.
* Inability to comply or give written informed consent.

Contacts and Locations

Study Contact

Shani C Scott, R.N.

CONTACT

(301) 451-6983

shani.scott@nih.gov

Theo Heller, M.D.

CONTACT

(301) 402-7147

theoh@intra.niddk.nih.gov

Principal Investigator

Theo Heller, M.D.

PRINCIPAL_INVESTIGATOR

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

Study Locations (Sites)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892

United States

Collaborators and Investigators

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

Theo Heller, M.D., PRINCIPAL_INVESTIGATOR, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Study Record Dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Registration Dates

Study Start Date2015-07-27

Study Completion Date2029-09-04

Study Record Updates

Study Start Date2015-07-27

Study Completion Date2029-09-04

Terms related to this study

Keywords Provided by Researchers

Portal Hypertension
Portal Fibrosis
Liver Disease
Varices
Splenomegaly
Natural History

Additional Relevant MeSH Terms

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