Cartilage-hair Hypoplasia Clinical Trial
Official title:
Immunodeficiency in Cartilage-hair Hypoplasia: Correlation With Pulmonary Disease, Infections and Malignancy
Verified date | October 2019 |
Source | Helsinki University Central Hospital |
Contact | n/a |
Is FDA regulated | No |
Health authority | |
Study type | Interventional |
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.
Status | Active, not recruiting |
Enrollment | 5 |
Est. completion date | February 2020 |
Est. primary completion date | October 2019 |
Accepts healthy volunteers | No |
Gender | All |
Age group | 12 Months and older |
Eligibility |
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 |
Country | Name | City | State |
---|---|---|---|
n/a |
Lead Sponsor | Collaborator |
---|---|
Helsinki University Central Hospital |
Type | Measure | Description | Time frame | Safety issue |
---|---|---|---|---|
Primary | Humoral response to vaccination | 4-6 weeks post-vaccination | ||
Primary | Cell-mediated response to vaccination | 4-6 wks post-vaccination | ||
Primary | Number and severity of adverse events to vaccination | 0-60 days |