| Autor: |
Gotoff, Samuel P., Amirmokri, Ebrahim, Liebner, Edwin J. |
| Zdroj: |
American Journal of Diseases of Children; December 1967, Vol. 114 Issue: 6 p617-625, 9p |
| Abstrakt: |
ATAXIA telangiectasia is an inherited disorder characterized by progressive cerebellar ataxia and oculocutaneous telangiectasia.1-3 Generally these patients have recurrent sinopulmonary infections, and defects in cellular immunity and in the immunoglobulin system have been recently documented.4-9 An unusual propensity for the development of neoplasia has become apparent.2-4,10-14 In previous cases the neoplasms have been discovered close to or at the time of death, obviating therapy. Since this is apparently the first instance in which a patient with ataxia telangiectasia has been treated for a lymphosarcoma, we wish to call attention to the severe untoward effect of a partial therapeutic dose of x-irradiation.In addition, we found typical lesions of tuberous sclerosis in the brain and unusual hypertrophic cells in the renal tubules. These findings, associated with the development of a lymphosarcoma, suggest a generalized disturbance in the metabolic control of many cell types. REPORT OF A CASE A |
| Databáze: |
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