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Bilirubin Metabolism — Preliminary Investigation

Published online by Cambridge University Press:  18 September 2015

A. Barbeau ,
G. Breton ,
B. Lemieux  and
R.F. Butterworth
A. Barbeau*
Affiliation:
Clinical Research Institute of Montreal; the Hôpital Hôtel-Dieu de Montreal; and the Centre Hospitalier Universitaire de Sherbrooke
G. Breton
Affiliation:
Clinical Research Institute of Montreal; the Hôpital Hôtel-Dieu de Montreal; and the Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
Clinical Research Institute of Montreal; the Hôpital Hôtel-Dieu de Montreal; and the Centre Hospitalier Universitaire de Sherbrooke
R.F. Butterworth
Affiliation:
Clinical Research Institute of Montreal; the Hôpital Hôtel-Dieu de Montreal; and the Centre Hospitalier Universitaire de Sherbrooke
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, H2W 1R7 Quebec, Canada
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In our studies, high total bilirubin values in the plasma were noted in cases of Friedreich's ataxia. A bimodal distribution of the values indicated the possible presence of two subgroups of patients. In these kindred, we demonstrated an elevation in unconjugated bilirubin with features similar to those reported in Gilbert's syndrome: normal liver function tests, elevation after fasting and day to day variability. We also report preliminary experiments indicating that bilirubin levels may be taurine dependent. We postulate that the defect could be a secondary component of the ataxic disease, possibly indicating a defect in membrane transport.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 3 , Issue 4 , November 1976 , pp. 365 - 372
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

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