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Familial Hyperbilirubinemia in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

E. Hamel
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôtel-Dieu Hospital Montreal and Université de Montréal
D. Bedard
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôtel-Dieu Hospital Montreal and Université de Montréal
F. Laviolette
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôtel-Dieu Hospital Montreal and Université de Montréal
R. F. Butterworth
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôtel-Dieu Hospital Montreal and Université de Montréal
A. Barbeau*
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal, Hôtel-Dieu Hospital Montreal and Université de Montréal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada H2W 1R7
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Summary:

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The combined metabolic stresses of fasting and the intravenous injection of 50 mg nicotinic acid in Friedreich's ataxia resulted in the delineation of two sub-groups of responses. High bilirubin ataxics maintained abnormally elevated levels of bilirubin, while normal bilirubin ataxics behaved like the normal control group. It is postulated that this finding infers the possible linkage of the gene for Friedreich's ataxia and that for Gilbert's disease.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1978

References

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