ataxia-telangiectasia

noun atax·ia-tel·an·gi·ec·ta·sia \ -ˌte-ˌlan-jē-ˌek-ˈtā-zh(ē-)ə , -tē- , -tə- \

Definition of ataxia-telangiectasia

:an inherited systemic disorder marked especially by progressive pathological changes in the nervous system resulting in loss of motor coordination and by increased susceptibility to cancer especially of lymphoid tissue

First Known Use of ataxia-telangiectasia

1957


Medical Dictionary

ataxia-telangiectasia

noun

medical Definition of ataxia-telangiectasia

:an inherited, systemic disorder that is marked especially by progressive pathological changes in the nervous system resulting in loss of motor coordination, telangiectasias especially on the eyes, ears, and cheeks, and immunodeficiency leading to increased susceptibility to cancer especially of lymphoid tissue
  • Many children with ataxia-telangiectasia die from respiratory failure or cancer by their teens or early 20s.
  • —Bill MinutaglioThe Dallas Morning News22 Dec. 1994
  • Defects in such a multifunctional protein might explain many of the clinical manifestations seen in patients with ataxia–telangiectasia.
  • —Michael KastanThe New England Journal of Medicine7 Sept. 1995
Note: Ataxia-telangiectasia is inherited as a recessive autosomal trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

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