Abstract
The association of neural, vasculr, and immunologic abnormalities in ataxia telangiectasia is unexplained. We studied the teratogenic effects of B6 as an experimental model because it is associated with (1) neonatal convulsions and (2) impaired immunologic response in rats. Pregnant rats were given a B6-deficient diet and 4-desoxypyridoxine, an inhibitor of B6. The control group had the same plus excess pyridoxine. Tje fetuses were examined at 20 days. Treated rats developed clinical signs of B6 deficiency and gained less weight than the control. Mean weight of 108 B4-deficient fetuses was 1.63 g; mean crown rump length was 28.3 mm. The 61 control fetuses had a mean weight of 3.24 g and a mean length of 37.0 mm; no anomalies were found in the control group. Of the treated group, 48% had digital defects, 20% had cleft palates, 12% had omphaloceles, 8% had micrognathia, and 6% had exencephaly.
Though the thymus and spleen are both significantly smaller (p < 0.001) in the treated group, only the spleen is strikingly hypoplastic. We anticipate that intrauterine B6 deficiency will cause a lifelong immunologic defect.
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Nelson, T., Davis, S. & Shepard, T. Severe Pyridoxine Deficiency: A Model for Ataxia Telangiectasia. Pediatr Res 4, 441 (1970). https://doi.org/10.1203/00006450-197009000-00031
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DOI: https://doi.org/10.1203/00006450-197009000-00031