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Biomedical subjects

M Bourel

Publications and source records attributed to M Bourel.

At least 91 records · Page 5Linked to original sources
PubMed1978

Ascorbic acid status in idiopathic hemochromatosis.

Vitamin C status was studied, by means of leucocyte ascorbic acid concentrations, in 67 cases of idiopathic hemochromatosis subdivided into 44 untreated and 25 treated cases (2 patients belonging to both subgroups) and compared to 31 normal subjects and 37 alcoholic cirrhosis patients. The control groups exhibited the following mean levels (+/- SEM): 34.4 +/- 1.9 microgram/10(8) WBC in normals and 22.0 +/- 1.8 microgram/10(8) WBC in alcoholic cirrhosis. In idiopathic hemochromatosis the mean levels were: for the untreated group 19.5 +/- 1.7 microgram/10(8) WBC and for the treated group 34.3 +/- 2.3 microgram/10(8) WBC. These results (1) affirm an important vitamin C deficiency in the untreated disease; (2) suggest that iron overload is the main causal factor in view of the striking difference--to date unreported--between untreated and treated cases of idiopathic hemochromatosis. Besides its possible theoretical interests, this vitamin C deficiency is responsible in idiopathic hemochromatosis for a significant underestimation of the desferrioxamine-induced urinary iron excretion.

Adult↗
PubMed1978

Sigma SR, a new method of measuring erythrocyte sedimentation rate. Its value in studying the action and interactions of non-steroidal anti-inflammatory agents.

Sigma SR is a new method of measuring erythrocyte sedimentation rate (ESR) and is characterized by an unvarying haematocrit, routinely corrected to 35%, and the sum of 4 sedimentation levels at 20, 30, 40, and 50 minutes. Two studies were carried out in patients with inflammatory arthritic disorders; the first in 25 patients treated with 1800 mg ibuprofen daily for 7 days, and the second in 31 patients treated either with indomethacin alone (150 mg/day) or combined with aspirin (1500 mg/day) for 5 days. In addition to the assessment of clinical parameters, the ESR was measured using the classical Westergren and the sigma SR methods. The results showed that there were little or no changes from baseline values in the ESR using the Westergren method. With the sigma SR method, however, statistically significant changes were recorded after treatment and these correlated with the clinical findings which demonstrated the anti-inflammatory action of ibuprofen and the reduction in activity of indomethacin by aspirin.

Adult↗
PubMed1978

[The cardiomyopathy of idiopathic hemochromatosis].

A retrospective study of the case histories of 216 patients with idiopathic haemochromatosis has highlighted the frequency of cardiac involvement in this condition (53%). Two forms can be distinguished: a latent one (65%), in which the changes are predominantly electrocardiographic, and a clinical form (35%) with the features of congestive cardiomyopathy, notable for the rapidity of onset after right heart failure, the degree of cardiomegaly, the constant finding of abnormalities of ventricular repolarisation, the relative frequency of latent disorders of supra-His atrio-ventricular conduction, and the finding of elongation of the isovolumic contraction time on the phonomechanocardiogram. A haemodynamic profile is the same as for non-obstructive hypotonic cardiomyopathies, and is usually associated with a slow rise in left ventricular pressure. The cardiomyopathy, which is the most frequent cause of death, determines the prognosis in this condition. It may be found in association with diabetes and gonad failure. The finding of cardiomyopathy indicates basic treatment by veresection, which may be the only means of establishing a favourable outcome.

Adult↗
PubMed1977

Idiopathic hemochromatosis. Demonstration of recessive transmission and early detection by family HLA typing.

We studied iron overloading and HLA types in 24 sibships of patients with idiopathic hemochromatosis, of which 15 had at least two subjects with overt forms. HLA types of 84 unrelated patients were also investigated. Among siblings there was a significant association (P less than 0.0001) between the presence of hemochromatosis and the possession of the same two HLA haplotypes. The fact that overt forms of hemochromatosis depend on the presence of two specific homologous chromosomes strongly supports a recessive mode of transmission for the overt disease. The haplotypic equilibrium demonstrated in the unrelated patients group is another supporting argument. The lod-score value (2.239 for theta = 0.005) in six families available for study further supports the conclusion that a hemochromatosis gene is closely linked to the HLA-A locus. HLA typing in families with hemochromatosis could provide a means of early detection of subjects at risk before appearance of any sign of iron overload.

Adult↗
PubMed1977

[Reduction of the effects of indomethacin by aspirin. Value of measurement of the articular index and sigma ESR].

Two groups, A and B, were selected at random amongst a total of 31 patients suffering from chronic inflammatory rheumatic disorders. The patients in group A (n = 16) received succesively: Placebo (2d), Indomethacin (5d). Indomethacin + aspirin (5d). The order of the 5 day treatment periods was reversed for the patients in group b (n = 15). The daily dose of indomethacin was 150 mg. That of aspirin was 1500 mg. Four parameters were measured at the end of each period of treatment: total serum indomethacin, articular index (Ritchie), ESR (Westergren) and the sigma ESR - a new technique for the measurement of sedimentation rate. No conclusions could be drawn from the analysis of variations in ESR. Concordant and statistically significnat variations in articular index and the sigma ESR showed a reduction in the activity of indomethacin under the influence of aspirin. The inhibitory effect of aspirin. The inhibitory effect of aspirin continues after the drug stopped. This reduction in indomethacin activity is not related to a decrease in serum concentrations of the medication which are not significantly altered when aspirin is taken.

Adult↗
PubMed1977

Heredity of idiopathic haemochromatosis: a study of 106 families.

More than 80% of the first degree relatives of 106 patients with iron overload - 97 with idiopathic haemochromatosis (I.H.) and nine with haemosiderosis secondary to alcohol induced liver disease (A.H. - were examined. Physical examination and measurement of plasma iron level and UIBC were done in all subjects; relatives who presented with some anomaly were submitted to a desferrioxamine test and, if the latter showed a high urinary iron output, to a liver biopsy. While absent in relatives of A.H. patients, iron overload was present in 78 out of 499 relatives of I.H. patients: 29 major and 49 minor forms. The major forms involved the sibships almost exclusively. The genetic analysis showed much evidence in favour of a recessive or rather intermediate form of inheritance, with heterozygous developing minor forms. However, other modes of transmission, especially polygenic (probably oligogenic), cannot be totally excluded. Data from recent studies showing a strong correlation between I.H. and certain HLA antigens do not conflict with the above conclusions.

Adolescent↗
PubMed1977

alpha-Fetoprotein screening in patients with idiopathic hemochromatosis and liver cirrhosis.

The serum alpha-fetoprotein level was measured by radioimmunoassay in 200 patients when admitted to hospital, 63 with idiopathic hemochromatosis and 137 with liver cirrhosis. In addition, repeated controls were performed in 19 subjects of each group for a mean period of 11 months (range 3--18 months). Elevated alpha-fetoprotein levels were observed initially or during the study period in 15 patients, a malignant liver tumor being demonstrated in 12 of them. In 4 of these patients, the abnormal alpha-fetoprotein concentration was the clue to the diagnosis of an unsuspected malignant hepatoma, but in none of these cases could the tumor be resected. The present results indicate that screening the serum alpha-fetoprotein level may contribute to the detection of malignant hepatoma in high-risk clinical groups, but the practical interest of such screenings may keep limited until more efficient therapeutic methods are developed.

Adult↗
PubMed1977

Accuracy of portein synthesis and in vitro aging. Search for altered enzymes in senescent cultured cells from human livers.

The authors have looked for altered proteins in senescent cultured cells from adult liver. Four enzymes (phosphoglycerate kinase, M2 type pyruvate kinase, glucose phosphate isomerase and glucose-6-phosphate dehydrogenase) have been studied by immunological and enzymatic titration and electrofocusing. In addition, heat stability of glucose-6-phosphate dehydrogenase (G6PD) was appraised in cell crude extracts and in partially purified preparations. Enzymatic aactivity as well as immunological reactivity of the four enzymes studied were identical 16 lines in phase II and in 13 lines in phase III. Electrofocusing pattern of the enzymes from 'young cells' was identical to the ones from 'old cells'. Finally, G6PD from old cells seemed to be more unstable than G6PD from young cells when studied in crude extracts. These differences, however, disappeared as G6PD was partially purified from old or young cultured cells. Consequently, no evidence of altered protein, either missynthesized or posttranslationally modified, was found in the senescent cultured cells studied. Moreover, this work indicated that the modification of heat stability of G6PD from old cells was not due to the enzyme molecule itself but rather to the cell medium.

Aging↗
PubMed1977

Cutaneous manifestations of idiopathic hemochromatosis. Study of 100 cases.

Skin manifestations of idiopathic hemochromatosis (IH) are presented in 100 cases with emphasis on the previously unrecognized high frequency of ichthyosis-like states and koilonychia. In 50 cases with treated and nontreated groups, histological siderosis and clinical skin pigmentation were found to decrease postphlebotomy whereas melanosis, histologically, did not. By skin biopsy in 50 cases versus controls, the location of siderosis in eccrine sweat glands seems specific for IH providing a strong basis for a probable diagnosis of the disease. There are correlations between skin manifestations and other signs of the disease.

Adult↗
PubMed1977

Idiopathic hemochromatosis and iron overload in alcoholic liver disease: differentiation by HLA phenotype.

HLA A and B antigens were determined in two groups of patients: 38 patients with idiopathic hemochromatosis (IH) and 22 patients with iron overload accompanying alcoholic liver disease (AH). As previously described, HLA A3 and HLA B14 antigens appeared more frequently in the IH group (76.3 and 28.9%, respectively) than in a control group of 204 subjects (27 and 3.4%). In the AH group the frequency of A3 (22.7%) and B14 (13.6%) was not significantly different from that observed in controls. The frequency of A3 was significantly lower in the AH group than in the IH group (P less than 0.001). On this basis a clear difference appears between the two conditions with iron overload, and genetic analysis of the results rules out the hypothesis that AH would be a heterozygous form of IH exposed by alcohol.

Adult↗