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- Title
Unusual and severe disease course in a child with ataxia-telangiectasia.
- Authors
Meyts, Isabelle; Weemaes, Corry; De Wolf-Peeters, Chris; Proesmans, Marijke; Renard, Marleen; Uyttebroeck, An; De Boeck, Kris
- Abstract
Ataxia-telangiectasia (AT) is an autosomal recessive syndrome of combined immunodeficiency. Hallmarks of the disease comprise progressive cerebellar ataxia, oculocutaneous telangiectasia, cancer susceptibility and variable humoral and cellular immunodeficiency. We describe a patient with AT presenting with autoimmune haemolytic anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy and hyper-IgM at the age of 6 months. At the age of 26 months she developed persistent fever, progressive lymphadenopathy and pulmonary nodular infiltrates, which were responsive to steroid therapy.
- Subjects
IMMUNODEFICIENCY; CEREBELLAR ataxia; TELANGIECTASIA; NEUTROPENIA; STEROIDS; THERAPEUTICS
- Publication
Pediatric Allergy & Immunology, 2003, Vol 14, Issue 4, p330
- ISSN
0905-6157
- Publication type
Article
- DOI
10.1034/j.1399-3038.2003.00037.x