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Defining the Basis of Fibromuscular Dysplasia (FMD)

Recruiting
Conditions
Spontaneous Coronary Artery Dissection
Fibromuscular Dysplasia
Cervical Artery Dissection
Registration Number
NCT01967511
Lead Sponsor
Icahn School of Medicine at Mount Sinai
Brief Summary

The purpose of this study has evolved and expanded since its inception. Originally the intent was to establish the functional, molecular and genetic profile of fibroblasts from Fibromuscular Dysplasia (FMD) patients as compared to carefully matched control subjects. While this remains among the objectives, the study has been expanded to undertake a fully powered cross-tissue systems genetics analysis of FMD, and now also the related arteriopathies spontaneous coronary artery dissection (SCAD) and cervical artery dissection (CvAD). The overall objective is to disclose the core biologic mechanisms of these disorders.

Detailed Description

Specific aims

* Specific aim 1: To establish a library of fibroblasts, DNA, plasma and serum from patients with FMD, SCAD and CvAD and unaffected healthy control subjects.

* Specific aim 2: To perform a fully powered cross-tissue systems analysis of the key regulatory gene networks and disease drivers underlying FMD, SCAD and CvAD.

* Specific aim 3: To cross-compare the molecular and genomic profiles of FMD, SCAD and CvAD to establish the degree of biologic similarity among these disorders.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
600
Inclusion Criteria
  • Patients of any age and freely willing to participate. For patients < 18 years of age consent will be via parents.
  • Fluency in either English or Spanish.
  • Signed, informed consent
  • For FMD, SCAD or CvAD subjects - a clinical diagnosis of FMD, SCAD or CvAD with fulfillment of standard diagnostic criteria.
  • For healthy controls - no clinical features of FMD, SCAD or CvAD and absence of any major ongoing systemic disease including any condition requiring hospitalization, immune suppression, intravenous or injected medications or that result in functional impairment in the performance of activities of daily living. Healthy controls will be matched to enrolled FMD patients on the basis of gender and approximate age (within a 5 year window of another FMD subject).
Exclusion Criteria
  • Patients who have co-morbidities which reduces life expectancy to one year.
  • Patients with any solid organ or hematological transplantation, or those in whom transplantation is considered.
  • Active autoimmune disease.
  • Illicit drug use.
  • HIV positive.
  • Prior malignancy.
  • Any other form of vascular disease, including other arteriopathy coronary artery disease or peripheral vascular disease
  • Family history of arteriopathy other than FMD, SCAD or CvAD (e.g. Ehlers-Danlos syndrome)

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Identification of regulatory gene networkssingle time point at study enrollment

The identification of regulatory gene networks, and their key drivers, underlying FMD, SCAD and CvAD

Secondary Outcome Measures
NameTimeMethod
RNA sequencingsingle time point at study enrollment

To define and compare the genomic (RNA sequencing) profile of fibroblasts from FMD, SCAD and CvAD subjects versus healthy control subjects

Identification of molecular featuressingle time point at study enrollment

To cross-compare the molecular features of FMD, SCAD and CvAD

Identification of genomic featuressingle time point at study enrollment

To cross-compare the genomic features of FMD, SCAD and CvAD

Circulating cytokinesingle time point at study enrollment

To define and compare the circulating cytokine profile of FMD versus healthy control subjects.

Trial Locations

Locations (1)

Icahn School of Medicine at Mount Sinai

馃嚭馃嚫

New York, New York, United States

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