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Susceptibility to Infections in Ataxia Telangiectasia

NCT02345135 · Status: COMPLETED · Phase: NA · Type: INTERVENTIONAL · Enrollment: 41

Last updated 2017-11-30

No results posted yet for this study

Summary

Death in Ataxia telangiectasia (A-T) is usually due to cancer or chronic lung failure around 20 years of age. Despite low lymphocyte counts (CD3, CD4, CD8 and CD19), IgA and IgG subclass deficiency opportunistic and acute severe respiratory infections are rare. The prevailing wisdom is that an immunoglobulin replacement therapy is not necessary in most of the patients. However no placebo controlled trials have been performed so far. The aim of this trial was to investigate the prevalence of mild and severe respiratory infections and / or chronic cough in classical A-T patients compared to healthy controls.

Conditions

Interventions

PROCEDURE

Blood collection

In all patients with Ataxia telangiectasia blood was collected at each visit date to determine blood count, lymphocyte subpopulation count and immunoglobulin levels in serum (IgA, IgG, IgM, IgE), as well as alpha feto protein (AFP).

PROCEDURE

Lung function measurement

In all patients - Ataxia telangiectasia and healthy controls - a lung function measurement was done to determine vital capacity (VC), forced expiratory volume in 1 second (FEV1), peak expiratory flow (PEF) and Tiffenau index.

BEHAVIORAL

Symptom diary

All patients - Ataxia telangiectasia and healthy controls - were requested to keep a symptom diary for the months September to March of the years 2012/2013 and 2013/2014 to determine days with symptoms as coughing during day and night, cold and fever, as well missing days at kindergarten/school/work, intake of medication (especially antibiotic treatment) and hospitalisations.

Sponsors & Collaborators

  • CSL Behring

    collaborator INDUSTRY

  • Johann Wolfgang Goethe University Hospital

    lead OTHER

Principal Investigators

  • Stefan Zielen, Prof. Dr. · Johann Wolfgang Goethe University, Childrens Hospital

Study Design

Allocation
NON_RANDOMIZED
Purpose
SUPPORTIVE_CARE
Masking
NONE
Model
PARALLEL

Eligibility

Min Age
2 Years
Max Age
45 Years
Sex
ALL
Healthy Volunteers
Yes

Timeline & Regulatory

Start
2012-09-30
Primary Completion
2016-01-31
Completion
2016-07-31

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Entities

Diseases

Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT02345135 on ClinicalTrials.gov