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P Rebuffel

Publications and source records attributed to P Rebuffel.

7 recordsLinked to original sources
PubMed1983

Immunoreactive Cu-SOD and Mn-SOD in lymphocytes sub-populations from normal and trisomy 21 subjects according to age.

Copper and manganese superoxide dismutases (Cu-SOD and Mn-SOD) were measured by radioimmunoassay in B and T lymphocytes and macrophages, in patients with trisomy 21 and in matched controls. In the controls, Cu-SOD was present in greater amounts than Mn-SOD and there were quantitative differences in the distribution in the three cellular sub-populations. In trisomy 21, levels of Cu-SOD were raised, with no change in levels of Mn-SOD, supporting the theory of a gene dosage effect. There were significant positive and negative correlations between age and Cu-SOD levels in controls, and a correlation approaching significance for Mn-SOD. In trisomy 21, there was no correlation between age and Cu-SOD levels, and the only significant correlation for Mn-SOD was for B lymphocytes.

Adolescent↗
PubMed1979

Erythrocyte copper levels in children with trisomy 21.

Erythrocyte superoxide dismutase is a cuproprotein displaying increased activity in cases of trisomy 21. In this study, the three erythrocyte copper fractions were compared at constant serum copper levels in children with and without trisomy 21. The labile erythrocyte copper level was found to be identical in both groups of children. Total erythrocyte copper, especially the stable fraction, was increased in cases of trisomy 21. The approximately fifty per cent ob served increase correlates with the augmented superoxide dismutase activity related to the presence of an extra chromosome 21. Measurement of the stable erythrocyte copper fraction could constitute an indirect method for evaluating superoxide dismutase activity.

Child↗
PubMed1978

[Hemoglobin oxygen affinity in children on chronic hemodialysis].

We have studied the changes induced by hemodialysis in the blood oxygen affinity in a group of 8 children with chronic kidney failure. Before dialysis: the affinity was low - P50 (7.40) = 28,3 Torr. Such a decreased affinity could explain in part why the important anemia (2 million RBC on average) observed in these patients was well tolerated. The observed decrease in hemoglobin oxygen affinity was attributed to an increase in 2-3 diphosphoglycerate (2-3 DPG) concentration, resulting from a high level of inorganic phosphates. After dialysis: the plasmatic pH increased markedly (7.52) with a consequent increase in the blood oxygen affinity in vivo (Bohr effect). No substantial change in 2-3 DPG concentration on P50 (7.40) occurred during hemodialysis.

Adolescent↗
PubMedDate not supplied

Type III glycogenosis with multicore structures.

A case of an infantile type III glycogenosis (Forbes disease), confirmed by morphologic and biochemical studies, had light-microscopic, histochemical, and electron-microscopic evidence of multicore structures and type 1 fiber predominance with hypotrophy. This association is discussed with relation to the unusual clinical findings. The authors conclude that two distinct disease entities--Forbes disease and multicore myopathy--may coexist.

Adenosine Triphosphatases↗