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Conjugate Pneumococcal Vaccine in Ataxia Telangiectasia (AT)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00656409
Recruitment Status : Completed
First Posted : April 11, 2008
Last Update Posted : April 11, 2008
Great Ormond Street Hospital for Children NHS Foundation Trust
Information provided by:
Institute of Child Health

Brief Summary:

Ataxia Telangiectasia (AT) is an autosomal recessive inherited condition caused by mutations in the ATM gene1. Patients suffer from neuro-degenerative problems, usually commencing in the second year of life, and affecting predominantly the cerebellum. They also develop the characteristic superficial telangiectases. Between 60 and 80% of affected children are immunodeficient. This is associated with deficiency of immunoglobulin A (IgA ) 2, of IgG23 and of antibody responses to pneumococcal polysaccharides4. Patients suffer recurrent sino-pulmonary infections but a recent study suggests poor correlation between immune status and immunological parameters5. If uncontrolled, recurrent pulmonary infections can contribute to the development of chronic lung disease and bronchiectasis. Preventative management includes continuous prophylactic antibiotic treatment in some with the need for replacement immunoglobulin therapy in only a small proportion of cases. Antibiotics have been reasonably effective in this situation but the emergence of resistance amongst community acquired pneumococcal isolates is a cause for concern. Appropriate immunisation strategies may also have a role.

This study is designed to look at antibody responses in a one versus two dose regimen in a cohort of AT patients recruited through the AT Society a national charitable organisation involved in providing support to families with this condition and in fostering education and research in the field.

Condition or disease Intervention/treatment Phase
Ataxia Telangiectasia (AT) Drug: Conjugated pneumococcal vaccine (Prevenar) Phase 3

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Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 30 participants
Primary Purpose: Basic Science
Official Title: Conjugate Pneumococcal Vaccine in Ataxia Telangiectasia (AT)
Study Start Date : June 2006
Actual Study Completion Date : March 2008

Intervention Details:
  • Drug: Conjugated pneumococcal vaccine (Prevenar)
    Other Name: Prevenar

Primary Outcome Measures :
  1. Immunogenicity of vaccine

Secondary Outcome Measures :
  1. Incidence of adverse reactions to vaccine

Information from the National Library of Medicine

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Ages Eligible for Study:   2 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No

Inclusion Criteria:

  • Genetically confirmed diagnosis of AT

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00656409

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United Kingdom
Great Ormond Street Hospital
London, United Kingdom, WC1N 3JH
Sponsors and Collaborators
Institute of Child Health
Great Ormond Street Hospital for Children NHS Foundation Trust

Layout table for additonal information Identifier: NCT00656409    
Other Study ID Numbers: 04MI07
First Posted: April 11, 2008    Key Record Dates
Last Update Posted: April 11, 2008
Last Verified: April 2008
Keywords provided by Institute of Child Health:
Ataxia telangiectasia,
Conjugated pneumococcal,
Immunogenicity ,
Additional relevant MeSH terms:
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Cerebellar Ataxia
Ataxia Telangiectasia
Spinocerebellar Ataxias
Neurologic Manifestations
Nervous System Diseases
Signs and Symptoms
Cerebellar Diseases
Brain Diseases
Central Nervous System Diseases
Vascular Diseases
Cardiovascular Diseases
Neurocutaneous Syndromes
Genetic Diseases, Inborn
DNA Repair-Deficiency Disorders
Metabolic Diseases
Immunologic Deficiency Syndromes
Immune System Diseases
Heptavalent Pneumococcal Conjugate Vaccine
Immunologic Factors
Physiological Effects of Drugs