Natural History of Noncirrhotic Portal Hypertension

Launched by NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES (NIDDK) · Apr 15, 2015

Keywords

ClinConnect Summary

The clinical trial titled "Natural History of Noncirrhotic Portal Hypertension" aims to better understand a condition called Noncirrhotic Portal Hypertension (NCPH), which occurs when there is increased pressure in the blood vessels of the liver due to liver diseases. This condition can develop slowly and may not show clear symptoms, leading many people to be unaware that they have a liver issue. The study is open to individuals aged 12 and older who either have NCPH or are at risk of developing it, but they must not have other liver diseases that could lead to more serious complications like cirrhosis or liver cancer.

Participants in this trial can expect to go through several medical tests during the screening phase to determine their eligibility. These tests include physical examinations, blood tests, and imaging studies like ultrasounds and MRIs. If eligible, participants will visit the clinic every six months for ongoing assessments, including repeat tests and evaluations. The study is designed to last indefinitely, allowing researchers to gather long-term data about how NCPH progresses. It's important for potential participants to know that the trial excludes individuals with certain liver diseases or severe medical conditions that could interfere with their participation.

Gender

ALL

Eligibility 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.
• EXCLUSION CRITERIA:
• An individual who meets any of the following criteria will be excluded from participation in this study:
• • 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.