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Immunodeficiency in Cartilage-hair Hypoplasia: Sub-project on Safety of Vaccination Against Chickenpox

Sponsor
Helsinki University Central Hospital (Other)
Overall Status
Unknown status
CT.gov ID
NCT02383797
Collaborator
(none)
5
Enrollment
1
Arm
60
Duration (Months)

Study Details

Study Description

Brief Summary

The investigatoris will carefully select cartilage-hair hypoplasia patients unexposed to varicella (VZV) and immunize patients in a controlled manner with VZV vaccine. Patients will be selected on the basis of disease severity and the degree of immunodeficiency (including CD4+ T-cell counts). Any potential complication of VZV immunization, such as rash, will be discussed with the patients/caregivers beforehand and acyclovir will be used to treat any VZV related symptoms, consistent with the current practices. The investigators will verify the development of immune response to vaccination by testing for VZV antibodies and cell-mediated immunity.

Condition or Disease Intervention/Treatment Phase
  • Biological: Varilrix
 Phase 4

Study Design

Study Type:
Interventional
Actual Enrollment :
5 participants
Allocation:
N/A
Intervention Model:
Single Group Assignment
Masking:
None (Open Label)
Primary Purpose:
Prevention
Official Title:
Immunodeficiency in Cartilage-hair Hypoplasia: Correlation With Pulmonary Disease, Infections and Malignancy
Study Start Date :
Feb 1, 2015
Actual Primary Completion Date :
Oct 1, 2019
Anticipated Study Completion Date :
Feb 1, 2020

Arms and Interventions

Arm Intervention/Treatment
Experimental: Cartilage-hair hypoplasia (CHH)

Selected CHH patients will be vaccinated against varicella with Varilrix, one dose of 0,5 ml subcutaneously. If no response is documented to the first dose, the second dose of 0,5 ml can be administered.

Biological: Varilrix
Vaccination against varicella in selected cartilage-hair hypoplasia patients
Other Names:
  • ATC J07BK01

    		•

    Outcome Measures

    Primary Outcome Measures

    1. Humoral response to vaccination [4-6 weeks post-vaccination]

    2. Cell-mediated response to vaccination [4-6 wks post-vaccination]

    3. Number and severity of adverse events to vaccination [0-60 days]

    Eligibility Criteria

    Criteria

    Ages Eligible for Study:
    12 Months and Older
    Sexes Eligible for Study:
    All
    Accepts Healthy Volunteers:
    No
    Inclusion Criteria:

    • genetically confirmed cartilage-hair hypoplasia diagnosis

    • age >12 months

    • no history of chickenpox

    • informed concent of the patient/caregiver.

    Exclusion Criteria:

    • positive serum IgG for varicella zoster virus

    • low CD4+ cell counts (<15% or <200 cells/mm3)

    • clinical or laboratory signs of severe immunodeficiency

    • ongoing intravenous or subcutaneous immunoglobulin treatment

    Contacts and Locations

    Locations

    No locations specified.

    Sponsors and Collaborators

    • Helsinki University Central Hospital

    Investigators

    • Study Director: Outi Mäkitie, PhD, Helsinki University Central Hospital and University of Helsinki, Folkhälsan Research Centre, Helsinki Finland

    Study Documents (Full-Text)

    None provided.

    More Information

    Publications

    None provided.
    Responsible Party:
    Paula Klemetti, MD, PhD, Consultant in pediatric gastroenterology and immunology, Helsinki University Central Hospital
    ClinicalTrials.gov Identifier:
    NCT02383797
    Other Study ID Numbers:
    • TYH2013336
    First Posted:
    Mar 9, 2015
    Last Update Posted:
    Oct 22, 2019
    Last Verified:
    Oct 1, 2019
    Additional relevant MeSH terms:
    Osteochondrodysplasias
    Hirschsprung Disease
    Immunologic Deficiency Syndromes

    Study Results

    No Results Posted as of Oct 22, 2019