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

October 18, 2019 updated by: Paula Klemetti, Helsinki University Central Hospital

Immunodeficiency in Cartilage-hair Hypoplasia: Correlation With Pulmonary Disease, Infections and Malignancy

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.

Study Overview

Status

Unknown

Conditions

Intervention / Treatment

Study Type

Interventional

Enrollment (Actual)

5

Phase

  • Phase 4

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.

Eligibility Criteria

Ages Eligible for Study

1 year and older (Child, Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Primary Purpose: Prevention
  • Allocation: N/A
  • Interventional Model: Single Group Assignment
  • Masking: None (Open Label)

Arms and Interventions

Participant Group / 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

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
Humoral response to vaccination
Time Frame: 4-6 weeks post-vaccination
4-6 weeks post-vaccination
Cell-mediated response to vaccination
Time Frame: 4-6 wks post-vaccination
4-6 wks post-vaccination
Number and severity of adverse events to vaccination
Time Frame: 0-60 days
0-60 days

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Helsinki University Central Hospital

Investigators

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

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

February 1, 2015

Primary Completion (Actual)

October 1, 2019

Study Completion (Anticipated)

February 1, 2020

Study Registration Dates

First Submitted

February 10, 2015

First Submitted That Met QC Criteria

March 3, 2015

First Posted (Estimate)

March 9, 2015

Study Record Updates

Last Update Posted (Actual)

October 22, 2019

Last Update Submitted That Met QC Criteria

October 18, 2019

Last Verified

October 1, 2019

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • TYH2013336

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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