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

Cartilage-hair Hypoplasia Clinical Trial

Official title:

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

Clinical Trial Details — Status: Active, not recruiting

Administrative data

• NCT number: NCT02383797
• Other study ID #: TYH2013336
• Status: Active, not recruiting
• Phase: Phase 4
• Start date: February 2015
• Est. completion date: February 2020

Study information

• Verified date: October 2019
• Source: Helsinki University Central Hospital
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• 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

Related Conditions & MeSH terms

• Cartilage-hair Hypoplasia
• Hirschsprung Disease
• Immunologic Deficiency Syndromes

Intervention

Biological:
• Varilrix
Vaccination against varicella in selected cartilage-hair hypoplasia patients

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
Helsinki University Central Hospital

Outcome

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