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Ataxia-telangiectasia: Immunodeficiency and survival.

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Authors
van Os, Nienke J H
Jansen, Anne F M
van Deuren, Marcel
Haraldsson, Asgeir
van Driel, Nieke T M
Etzioni, Amos
van der Flier, Michiel
Haaxma, Charlotte A
Morio, Tomohiro
Rawat, Amit
Schoenaker, Michiel H D
Soresina, Annarosa
Taylor, Alexander M R
van de Warrenburg, Bart P C
Weemaes, Corry M R
Roeleveld, Nel
Willemsen, Michèl A A P
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Issue Date
2017-05

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Citation
Ataxia-telangiectasia: Immunodeficiency and survival. 2017, 178:45-55 Clin. Immunol.
Abstract
Ataxia-telangiectasia (AT) is a neurodegenerative disorder characterized by ataxia, telangiectasia, and immunodeficiency. An increased risk of malignancies and respiratory diseases dramatically reduce life expectancy. To better counsel families, develop individual follow-up programs, and select patients for therapeutic trials, more knowledge is needed on factors influencing survival. This retrospective cohort study of 61 AT patients shows that classical AT patients had a shorter survival than variant patients (HR 5.9, 95%CI 2.0-17.7), especially once a malignancy was diagnosed (HR 2.5, 95%CI 1.1-5.5, compared to classical AT patients without malignancy). Patients with the hyper IgM phenotype with hypogammaglobulinemia (AT-HIGM) and patients with an IgG2 deficiency showed decreased survival compared to patients with normal IgG (HR 9.2, 95%CI 3.2-26.5) and patients with normal IgG2 levels (HR 7.8, 95%CI 1.7-36.2), respectively. If high risk treatment trials will become available for AT, those patients with factors indicating the poorest prognosis might be considered for inclusion first.
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http://ac.els-cdn.com/S1521661616303904/1-s2.0-S1521661616303904-main.pdf?_tid=edb0f92c-81c1-11e7-aba6-00000aacb361&acdnat=1502805741_ca23c98fd796efef04e081d5d98401e8
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Archived with thanks to Clinical immunology (Orlando, Fla.)
ae974a485f413a2113503eed53cd6c53
10.1016/j.clim.2017.01.009
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