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Oxygen Transport m Patients with Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

M. A. Bureau ,
Y. Berthiaume ,
R. Begin ,
D. Shapcott ,
B. Lemieux  and
M. Cote
M. A. Bureau
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
Y. Berthiaume
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
R. Begin
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
D. Shapcott
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
B. Lemieux
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
M. Cote
Affiliation:
Le Centre Hospitalier Universitaire de l'Universite de Sherbrooke
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Summary:

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The hypothesis that an abnormal oxygen-hemoglobin dissociation curve is a primary or a secondary defect in patients with Friedreich's ataxia was investigated in 12 subjects with this disease. Hemoglobin and P50 were measured and compared with age and sex matched controls. The mean hemoglobin concentration was 14.2 g% and the P50 was 26.25 ton for the patients and 13.8 g% and 26.27 ton in the controls. These results indicate that the oxygen transport system is normal in this disease and likely exclude an abnormal oxygen dissociation curve as a primary or a secondary factor in the pathophysiology of the cardiomyopathy and the neuromyopathy found in this disease.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 5 , Issue 1 , February 1978 , pp. 97 - 99
Copyright
Copyright © Canadian Neurological Sciences Federation 1978

References

REFERENCES

Bureau, M.A., Ngassam, P., Lemieux, B. and Trias, A. (1976). Pulmonary function in Friedreich’s ataxia. Can. J. Neurol. Sci., 3, 343–348.Google Scholar
Cote, M., Davignon, A., Elias, G., Solignac, A., Geoffroy, G., Lemieux, B. and Barbeau, A. (1976). Hemodynamic findings in Friedreich’s ataxia. Can. J. Neurol. Sci., 3, 333–342.Google Scholar
Edwards, M.J. and Canon, B. (1972). Oxygen transport during erythropoetic re-sponse to moderate blood loss. N. Engl. J. Med., 287, 115–119.Google Scholar
Edwards, M.J., Novy, M.J., Walters, C.L. and Metcalf, E.J. (1968). Improved oxygen release: an adaptation of mature cells to hypoxia. J. Clin. Invest., 47, 1851–1853.CrossRefGoogle ScholarPubMed
Geoffroy, G., Barbeau, A., Breton, G., Lemieux, B., Aube, M., Leger, C. and Bouchard, J.P. (1976). Clinical description and roentgenologic evaluation of patient with Friedreich’s ataxia. Can. J. Neurol. Sci., 3, 279–286.Google Scholar
Sanchez-Cases, G., Cote, M. and Barbeau, A. (1976). Pathology of the heart in Friedreich’s ataxia: review of the literature and report of one case. Can. J. Neurol. Sci., 3, 349–354.Google Scholar
Severinghaus, J.W. (1971). Blood oxygen dissociation line chart: man. In: Altman, P.C. and Dittmer, D.S. (eds) Respiration and circulation, Bethesda, Md., Federation of American Societies for Experimental Biology, pp. 204–206.Google Scholar
Torrance, J., Jacobs, P., Restrepo, A., Eschback, J., Lentant, C. and Finch, C.A. (1970). Intraerythrocytic adaptation to anemia. New Engl. J. Med., 283, 165–169.Google Scholar
Weiss, E.B., Slawsky, P. and Desforges, J.F. (1971). Oxyhemoglobin affinity in chronic pulmonary granulo matosis (Sarcoidosis) and fibrosis. Am. Rev. Resp. Dis., 104, 694–702.Google Scholar