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

Helsinki University Central Hospital5 enrolled

Overview

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 Type

INTERVENTIONAL

Allocation

Purpose

PREVENTION

Masking

NONE

Enrollment

Conditions

Cartilage-hair Hypoplasia

Interventions

Eligibility

Sex: ALLMin age: 12 Months

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

Humoral response to vaccination

Time frame: 4-6 weeks post-vaccination

Cell-mediated response to vaccination

Time frame: 4-6 wks post-vaccination

Number and severity of adverse events to vaccination

Time frame: 0-60 days