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HIDS-MKD: B7 Coreceptor Molecules in Hyper IgD Syndrome Form of Mevalonate Kinase Deficiency

Sponsor
Michigan Technological University (Other)
Overall Status
Withdrawn
CT.gov ID
NCT01568736
Collaborator
(none)
0
Enrollment
4
Locations
48
Duration (Months)
0
Patients Per Site
0
Patients Per Site Per Month

Study Details

Study Description

Brief Summary

The hyper IgD syndrome (HIDS) is an inflammatory disease caused by mevalonate kinase deficiency. There is no cure, and available treatments of HIDS febrile episodes have shown limited clinical efficacy. The development of effective interventions for HIDS is limited by our poor understanding of the disease. The goal of the study is to better characterize the inflammatory response during HIDS episodes and to determine the relationship between this response and blood and urine markers of mevalonate kinase deficiency. This knowledge will help us learn more about the cause of the disease and should lead to the identification of new disease biomarkers that can be used to evaluate clinical efficacy in future therapeutic trials.

The primary hypothesis is that the costimulatory B7 glycoprotein abnormalities identified in the murine MKD model will be recapitulated in sera obtained from human HIDS patients, either before, during or after febrile episodes. The secondary hypothesis is that B7 glycoprotein molecule levels will correlate with clinical symptomatic severity score, other known biomarkers of HIDS, markers of inflammation and or markers of isoprenoid metabolism.

Condition or Disease Intervention/Treatment Phase

    Study Design

    Study Type:
    Observational
    Actual Enrollment :
    0 participants
    Observational Model:
    Case-Only
    Time Perspective:
    Prospective
    Official Title:
    B7 Coreceptor Molecules as Clinically-Relevant Surrogate Biomarkers in the Hyper IgD Syndrome (HIDS) Form of Mevalonate Kinase Deficiency (MKD)
    Study Start Date :
    Mar 1, 2012
    Anticipated Primary Completion Date :
    Mar 1, 2016
    Anticipated Study Completion Date :
    Mar 1, 2016

    Outcome Measures

    Primary Outcome Measures

      Eligibility Criteria

      Criteria

      Ages Eligible for Study:
      18 Years to 89 Years
      Sexes Eligible for Study:
      All
      Accepts Healthy Volunteers:
      No
      Inclusion Criteria:

      • any race or ethnicity

      • diagnosed with HIDS

      Exclusion Criteria:

      • parents' inability to donate blood

      • currently having cancer, renal failure, diabetes, liver disease, thyroid diseases, major infectious diseases or immunodeficiency

      • pregnancy

      • inability to provide consent

      Contacts and Locations

      Locations

      Site City State Country Postal Code
      1 Michigan Techinical University Houghton Michigan United States 49931
      2 University of Nebraska Medical Center Omaha Nebraska United States 68198
      3 Oregon Health & Science University Portland Oregon United States 97239
      4 Radbound University of Nijmegen Medical Centre Nijmegen Netherlands

      Sponsors and Collaborators

      • Michigan Technological University

      Investigators

      None specified.

      Study Documents (Full-Text)

      None provided.

      More Information

      Publications

      None provided.
      Responsible Party:
      Michigan Technological University
      ClinicalTrials.gov Identifier:
      NCT01568736
      Other Study ID Numbers:
      • STAIR 7003
      First Posted:
      Apr 2, 2012
      Last Update Posted:
      Jun 18, 2018
      Last Verified:
      Mar 1, 2012
      Keywords provided by Michigan Technological University
      Hyper IgD Syndrome (HIDS)
      Mevalonate Kinase Deficiency (MKD)
      Additional relevant MeSH terms:
      Mevalonate Kinase Deficiency
      Syndrome

      Study Results

      No Results Posted as of Jun 18, 2018