RECRUITING

Impact of Elastin Mediated Vascular Stiffness on End Organs

Description

Background: People with Williams Syndrome (WS) and supravalvular aortic stenosis (SVAS) have less elasticity in their blood vessels. This is called blood vessel stiffness. Blood vessels may have focal narrowings called stenoses or may just be globally more narrow. Objectives: Researchers want to see how blood vessel differences in people with Williams Syndrome and supravalvular aortic stenosis affect organs in the body including the heart, gut, kidneys, and brain. Eligibility: People ages 3-85 who have WS or SVAS Healthy volunteers ages 3-85 Design: * Participants will have yearly visits for up to 10 years. All participants will be offered the same tests. * Participants will give consent for the study team to review their medical records. If the participant is a child or an adult with WS, a parent or guardian will give the consent. * Participants will visit the NIH where they will have a physical exam and medical history. Based on their health history, participants will undergo a series of imaging tests and measures of blood vessel function over the course of 2-4 days. Tests of cognitive abilites will also be performed. Blood will be drawn and an IV may be placed for specific tests.

Study Overview

Study Details

Study overview

Background: People with Williams Syndrome (WS) and supravalvular aortic stenosis (SVAS) have less elasticity in their blood vessels. This is called blood vessel stiffness. Blood vessels may have focal narrowings called stenoses or may just be globally more narrow. Objectives: Researchers want to see how blood vessel differences in people with Williams Syndrome and supravalvular aortic stenosis affect organs in the body including the heart, gut, kidneys, and brain. Eligibility: People ages 3-85 who have WS or SVAS Healthy volunteers ages 3-85 Design: * Participants will have yearly visits for up to 10 years. All participants will be offered the same tests. * Participants will give consent for the study team to review their medical records. If the participant is a child or an adult with WS, a parent or guardian will give the consent. * Participants will visit the NIH where they will have a physical exam and medical history. Based on their health history, participants will undergo a series of imaging tests and measures of blood vessel function over the course of 2-4 days. Tests of cognitive abilites will also be performed. Blood will be drawn and an IV may be placed for specific tests.

Impact of Elastin Mediated Vascular Stiffness on End Organs

Impact of Elastin Mediated Vascular Stiffness on End Organs

Condition
Williams Syndrome
Intervention / Treatment

-

Contacts and Locations

Bethesda

National Institutes of Health Clinical Center, Bethesda, Maryland, United States, 20892

Saint Louis

Washington University School of medicine, Saint Louis, Missouri, United States, 63110-1093

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.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

  • * be between the ages of 3 and 85
  • * have a presumed or confirmed diagnosis of WS (genetic testing is not performed in this research study).
  • * have a parent/guardian available to provide consent and assist in answering medical questions
  • * not be pregnant
  • * be between the ages of 3 and 85
  • * have clinical features suggestive of SVAS or an SVAS-like condition OR have no clinical features of SVAS or an SVAS-like condition but have genetic testing results that imply affected status (SVAS has decreased penetrance). No genetic testing will be done as part of this protocol.
  • * have a parent/guardian available to provide consent and assist in answering medical questions if they are a minor (not applicable to adults)
  • * be between the ages of 3 and 85
  • * have clinical or research genetic testing that reports gene variation in a non-ELN gene in the WS region.
  • * have a parent/guardian available to provide consent and assist in answering medical questions if they are a minor or if they have cognitive impairment that would impede their ability to consent on their own behalf.
  • * be between the ages of 3 and 85
  • * not have clinical features or genetic profile suggestive of WS, SVAS or an SVAS-like condition. No genetic testing will be performed in this research study.
  • * have a parent/guardian available to provide consent and assist in answering medical questions if they are a minor (not applicable to adults)

Ages Eligible for Study

3 Years to 85 Years

Sexes Eligible for Study

ALL

Accepts Healthy Volunteers

Yes

Collaborators and Investigators

National Heart, Lung, and Blood Institute (NHLBI),

Manfred Boehm, M.D., PRINCIPAL_INVESTIGATOR, National Heart, Lung, and Blood Institute (NHLBI)

Study Record Dates

2032-02-25