Immunodeficiency in Cartilage-hair Hypoplasia: Sub-project on Safety of Vaccination Against Chickenpox
Phase 4
- Conditions
- Cartilage-hair Hypoplasia
- Interventions
- Biological: Varilrix
- Registration Number
- NCT02383797
- Lead Sponsor
- Helsinki University Central Hospital
- 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.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- UNKNOWN
- Sex
- All
- Target Recruitment
- 5
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 & Design
- Study Type
- INTERVENTIONAL
- Study Design
- SINGLE_GROUP
- Arm && Interventions
Group Intervention Description Cartilage-hair hypoplasia (CHH) Varilrix 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.
- Primary Outcome Measures
Name Time Method Humoral response to vaccination 4-6 weeks post-vaccination Cell-mediated response to vaccination 4-6 wks post-vaccination Number and severity of adverse events to vaccination 0-60 days
- Secondary Outcome Measures
Name Time Method