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