RecruitingNCT02417740

Natural History of Noncirrhotic Portal Hypertension

Sponsor

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

Enrollment

400 participants

Start Date

Jul 27, 2015

Study Type

OBSERVATIONAL

Conditions

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

Summary

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.

Eligibility

Min Age: 12 YearsMax Age: 100 Years

Plain Language Summary

Simplified for easier understanding

This study is examining the long-term health outcomes of patients with a rare liver condition called non-cirrhotic portal hypertension (NCPH) — where pressure inside the liver's blood vessels is abnormally high, but without the typical liver scarring — to better understand what causes it and how it progresses. **You may be eligible if...** - You are 12 years of age or older - You have been diagnosed with NCPH, or you have an underlying condition that puts you at risk for it (such as sickle cell disease, cystic fibrosis, chronic granulomatous disease, common variable immunodeficiency, mastocytosis, or chronic heart failure) - Women who could become pregnant must agree to use birth control **You may NOT be eligible if...** - You are pregnant - You have liver disease that typically causes scarring (cirrhosis) - You have active hepatitis B or C infection - You have other liver diseases such as Wilson's disease, autoimmune hepatitis, or iron overload - You have a bile duct blockage - You have liver cancer - You currently use alcohol or drugs heavily Talk to your doctor to see if this trial is right for you.

This summary was AI-generated to explain the trial in plain language. It is not medical advice. Always discuss eligibility with your doctor before enrolling in a clinical trial.

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