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C Chapuis-Cellier

Publications and source records attributed to C Chapuis-Cellier.

25 records · Page 2Linked to original sources
PubMed1977

[Alpha-1-antitrypsin deficiency. Phenotype study of 60 members of the same family].

In two brothers treated for severe pulmonary emphysema, was demonstrated an alpha-1-antitrypsin deficiency associated with a ZZ phenotype (Pi system). The authors carried out a genetic study of the family including 60 members spread over 4 generations. In all, were demonstrated 4 subjects of phenotype ZZ, 29 of phenotype MZ, 3 of phenotype MS ; one subject had a phenotype SZ and 23 members of this family had normal levels of alpha-1-antitrypsin and were of phenotype MM. The disease was transmitted in all cases as an autosomic codominant. The interest of a study of the phenotype in alpha-1-antitrypsin deficiency is emphasized together with the practical steps to be taken on discovery of a subject with the allele responsible for a reduction in serum levels of alpha-1-antitrypsin.

Adult↗
PubMedDate not supplied

[Non specific immunity of children with selective IgA deficiency. Aggravating role of abnormal phenotype of alpha-1-antitrypsin (author's transl)].

Among 998 children with recurrent respiratory diseases 26 children with selective IgA deficiency were found. Three groups were considered according to IgA level in serum: group I with IgA under 0.05 g per litre; group II with IgA between 0.05 and 0.3 g per litre; group III with IgA above 0.3 and under 1 g per litre. Non specific immunity was studied in these patients including immunoglobulin levels, alpha-1-antitrypsin (A.A.T.) phenotypes, phagocytosis of staphylococcus aureus by PMN, lysozyme level, complement system. Cellular immunity was evaluated by IDR tests and rosette forming cells (RE). Only non specific immune systems were disturbed in some patients and appeared as aggravating factors in IgA deficient patients. We found: Abnormal phenotypes of ATT in 11 cases; deficiencies of engulfment in 6 cases, of bactericidal activities of PMN in 7 cases out of 16 studied; decrease of lysozyme level in 4 cases out of 17 studied; increase of IgE level in 9 cases with atopic symptoms in 7 patients. In our experience the chief aggravating factor in IgA deficient patients is abnormal phenotype of AAT.

Adolescent↗
PubMedDate not supplied

[Immunonephelometric analysis of immunoglobulin light chains: agreement between the theoretical and experimental model. Assessment of the reference values of the kappa/lambda ratio and the heavy to light chain ratio].

Since the introduction of fully automated nephelometric systems simultaneous measurements of immunoglobulin light chains kappa (kappa) and lambda (lambda) and IgG, IgA and IgM have become increasingly used for the routine assessment of humoral immunity. From these data two ratios were calculated, the kappa/lambda ratio and the heavy chains to light chains ratio. As changes in these ratios might have some predictive clinical value besides reflecting a monoclonal component, it is necessary to know mean and reference limits of these ratios. On account of differences in the calibration method of the light chains measurements (either free light chains or light chains bound to a complete molecule) and of differences in the calculation method of the heavy chains to light chains ratio we were led to conduct our own investigation. IgG, IgA and IgM and kappa and lambda light chains were immunonephelometrically measured in the sera of 84 blood donors. For each sample theoretical values for kappa + lambda, kappa and lambda, and kappa/lambda were calculated using the existing relation between the concentration of a given immunoglobulin and the concentration of bound light chains. Using the Valtec Protocole and the t test we were able to evidence highly significant differences (p < 10(-4) between theoretical and experimental values of kappa, lambda and kappa + lambda; those differences could be proved to be directly linked to the nephelometric technique itself. However the experimental kappa/lambda ratio did not appear to differ from the theoretical one nor the standardization method to have an effect on the reference values of this ratio, our values (mean and reference limits, 1.81, 1.29-2.53) being very similar to previously published results. Concerning the so called heavy chains to light chains ratio two methods were used to express it, one consisting in the ratio of the theoretical kappa + lambda value to the experimental one with the following results, 1.05 and 0.93-1.18 for the mean and reference limits and the other one using the raw data. The results were as follows: mean 3.50, reference limits 3.11-3.94.

Humans↗