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ataxia telangiectasia/feber

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The effects of hyperthermia and ionizing radiation in normal and ataxia telangiectasia human fibroblast lines.

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The effects of 45 degrees C hyperthermia and gamma radiation have been studied in three normal human fibroblast lines (GM38, GM730, WI38) and compared to the effects in two lines derived from patients with the hereditary disease ataxia telangiectasia (AT3BI, AT5BI). All lines, both normal and
Ataxia-telangiectasia (AT) is the most frequent progressive cerebellar ataxia in infancy and childhood. Immunodeficiency which includes both cellular and humoral arms has variable severity. Since the clinical presentation is extremely variable, a high clinical suspicion will allow an early
All cells have intricately coupled sensing and signaling mechanisms that regulate the cellular outcome following exposure to genotoxic agents such as ionizing radiation (IR). In the IR-induced signaling pathway, specific protein events, such as ataxia-telangiectasia mutated protein (ATM) activation

Hyperthermia and thermal tolerance in normal and ataxia telangiectasia human cell strains.

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Three normal human fibroblast strains, two human ataxia telangiectasia heterozygote cell strains, and two human ataxia telangiectasia homozygote cell strains were studied for their thermal responses between 41.0 and 46.0 degrees. The heat sensitivities of all cell strains were comparable, and all
Two homozygous and heterozygous ataxia telangiectasia (AT) cell strains were compared to normal human cells for heat and radiation responses. The thermal sensitivities to one hour heating at 42.0 degrees C were comparable in the five cell strains. Thermal enhancement of radiosensitivity occurred in

Ataxia telangiectasia: Family management.

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Ataxia telangiectasia (AT) is a rare autosomal recessive disease resulting in progressive degeneration of multiple systems in the body. Both A-T homozygote and heterozygote are at increased risk of developing malignancy. We report a family in which three generations were affected by this disorder.

Sensitivity of hyperthermia-treated human cells to killing by ultraviolet or gamma radiation.

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Human xeroderma pigmentosum (XP) or Fanconi anemia (FA) fibroblasts displayed shouldered 45 degrees C heat survival curves not significantly different from normal fibroblasts, a result similar to that previously found for ataxia telangiectasia (AT) cells, indicating heat resistance is not linked to

Thermal enhancement of radiosensitivity in normal and ataxia telangiectasia human cells.

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Normal human and ataxia telangiectasia (AT) fibroblasts were tested for enhancement of radiosensitivity by hyperthermia. In normal fibroblasts, thermal enhancement of radiosensitivity occurred at 42.0 degrees C and 45.0 degrees C and was greatest for simultaneous treatments of heat and radiation.
Ataxia-telangiectasia (AT) is a rare multisystem, neurodegenerative genetic disorder. Patients should be closely monitored due to risk of malignancy development. Due to its wide clinical heterogeneity, it often leads physicians to an inaccurate or missed diagnosis, and insight into this rare disease

Unusual and severe disease course in a child with ataxia-telangiectasia.

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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

Renal lymphoma in ataxia-telangiectasia: CT contribution.

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Ataxia-telangiectasia (AT) or the Louis-Bar syndrome is a familial, multisystem disorder. Patients with the disease are at high risk for malignancy, in particular lymphoreticular neoplasms, and immunodeficiency, rendering them susceptible to infection. We present a 17-year-old girl with AT and a
Post-operative microwave (MW) hyperthermia has been applied as an important adjuvant therapy to enhance the efficacy of traditional cancer treatment. A better understanding of the molecular mechanisms of MW hyperthermia may provide guided and further information on clinical hyperthermia treatment.
Rift Valley fever virus (RVFV) is a highly pathogenic arthropod-borne virus infecting a wide range of vertebrate hosts. Of particular interest is the nonstructural NSs protein, which forms large filamentous fibril bundles in the nucleus. Past studies have shown NSs to be a multifaceted protein

Hodgkin disease in ataxia-telangiectasia patients with poor outcomes.

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BACKGROUND To determine the presenting clinicopathologic features and treatment outcomes of 11 ataxia-telangiectasia (A-T) patients with Hodgkin disease. METHODS We reviewed the charts of 412 A-T patients to ascertain cases of Hodgkin disease. The data analyzed included date of diagnosis, duration

Immune competence and respiratory symptoms in patients with ataxia telangiectasia: A prospective follow-up study

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Ataxia telangiectasia is a multi-system disorder characterized by progressive cerebellar ataxia, malignancies, chronic pulmonary disease and immunodeficiency. The aim of our study was to determine the immune competence and prevalence of respiratory infections and/or chronic cough in classical A-T
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