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Biomedical subjects

G Schaison

Publications and source records attributed to G Schaison.

At least 271 records · Page 15Linked to original sources

[Survey of anthracyclines derivatives in haematology (author's transl)].

Anthracyclines, such as daunorubicin (DNR), rubidazone (RBD) and adriamycin (ADR) are intercalating drugs used in cancer chemotherapy. They inhibit synthesis of DNA and RNA, break DNA and inhibit mitochondrial oxidative chain. Their antitumoral experimental activities depend upon type of drug, tumor and route of administration. After i.v. administration, the drug is present in all tissues except central nervous system. Its disappearance from the plasma is biphasic with a long terminal half life, justifying intermittent chemotherapy. Anthracyclines metabolism occurs mainly in liver micrososomes, and 90% metabolites are excreted in the bile. The main toxicity is cardiac, as a congestive heart failure which appears when a cumulated drug dose is overcome. In man only, a few derivatives have been studied, compounds with activity and no cardiotoxicity are still in research. Action of malignancies depends on type of derivative. We use DNR since 1967, it is a remarkable active drug in induction treatment of AML, it is the only active drug on acute promyelocytic leukemia, and it increases number of remissions in all of adult patients and severe forms of children ALL. Adriamycin (ADR) is active on solid tumors (osteosarcoma, breast and thyroid cancers) and lymphomas. With rubidazone (RBD) we obtain 2/3 of remissions in acute monoblastic leukemia, and it is easier to use than DNR and equally active on AML. RBD is also active on severe cases of lymphomas (lymphosarcomas and Hodgkin's disease). A new compound DEA 14 DNR seems interesting: experimental antitumor activity is high (compared to DNR, RBD and ADR) and it appears to possess activity on solid tumors in man.

Adult↗

[Glutathion-synthetase deficiency with 5-oxoprolinuria. Two new cases and a review of the literature (author's transl)].

Hereditary deficiency in glutathion- synthetase is a rare disease presenting up to now either with a congenital non-spherocytic anaemia or with a metabolic acidosis, most often neonatal and accompanied by a pyroglutamic amino-aciduria (5-oxoprolinuria). These two syndrome may be present together or exist independently. Pyroglutamic amino-aciduria is the result of extension of the deficiency to non-haematopoietic cells, in particular renal. Two new cases of glutathion-synthetase deficiency are reported: both with haemolytic anaemia and moderate pyroglutamic amino-aciduria, in the absence of clinical signs of metabolic acidosis. The clinical, haematological and biochemical heterogeneity of the deficiency is illustrated by these two cases and datas from the literature.

Anemia, Hemolytic, Congenital↗

Prognosis and treatment of acute lymphoblastic leukemia. Study of 650 patients.

The complete hematological remission (CHR) rate, duration of remission and survival were studied in relation to age, peripheral blast cell (PBC) count, presence or absence of tumor masses, cytological type, and treatment in 650 patients with acute lymphoblastic leukemia. Prognostic factors were considered separately and divided into prognostic classes. Age and PCB count correlated with both the rate and the duration of CHR. This correlation was still observed for more recent treatment schedules though it appears to be becoming progressively less significant. Meningeal relapses were more common in patients less than 1 year old and in those with a high PCB count. It is suggested that stratification of patients according to such factors as age, PCB count, presence or absence of tumor, and cytological type might be necessary for the design of new treatment protocols and for the evaluation of their results.

Adolescent↗

[The pathological secretion of prolactin (author's transl)].

The problem of hyperprolactinaemia is dominated by the search for an active pituitary adenoma, which, if it is at all large, requires neuro-surgical operation. In cases where a microadenoma is responsible or where the pathological secretion of prolactin is istrogenic, the most serious consequence is sterility. Hyperprolactinaemia in fact works at two levels: on cyclical gonadotrophic activity and on luteal function. Whatever the cause is, bromocriptine allows hyperprolactinaemia to be corrected together with the disturbances of ovulation resulting from it.

Adenoma↗

Beta thalassemia with reticulocytopenia: clinical, biochemical, and ultrastructural studies.

An unusual form of beta thalassemia is described in two children of unrelated families. Its main features are a severe anaemia with a low reticulocyte count associated with an erythroblastic hyperplasia; these characteristics indicate a completely ineffective erythropoiesis. The results of the study of haemoglobin synthesis performed on the bone marrow in vitro showed a greater imbalance in chain synthesis than that typically found in Cooley's anaemia. Ultrastructural studies revealed, in erythroblasts, all the features observed in Cooley's anaemia, although these features were more widely encountered. In addition, two peculiar findings were noted: (1) many inclusion bodies, which were partially or totally surrounded by smooth membranes, and which may indicate an autophagic phenomenon; and (2) a spongy appearance of the chromatin in rare erythroblasts of one of the two patients which resembled that found in congenital dyserythropoietic anaemia type I. These findings suggest that the great imbalance in the synthesis of the haemoglobin chain is responsible for the presence of an increased number of inclusion bodies and results in the death of nearly all the late erythroblasts. Furthermore, the present results point out the need for further study of the synthesis of the globin chains in atypical congenital dyserythropoietic anaemias.

Blood Cell Count↗

[Splenic lesions during benign inoculation lymphoreticulosis].

The authors describe the fibro-granulomatous lesions with scar formation on a splenectomy specimen removed for familial spherocytosis, eight weeks after the onset of cat scratch disease. This original observation, apparently unique in the literature, is in favour of the blood spread of the agent responsible for benign inoculation lymphoreticulosis, and suggests a pathogenic theory for the unusual visceral manifestations.

Cat-Scratch Disease↗

Localization chromatid breaks in Fanconi's anemia, using three consecutive stains.

The location of 339 break points was analyzed in three patients with Fanconi's anemia, using three consecutive stains: ordinary Giemsa, Q-banding, and R-banding. Almost all the breaks seem to take place in the Q bands, using R-banding, and in the R bands, using Q-banding. A very important artifact, varying according to the method used, is thus demonstrated. In fact, the breaks take place in the interbands, between R and Q bands. The breaks were also localized in relation to sister chromatid exchanges (SCEs), seen after BUDR treatment. There is a clear excess of breaks at place of SCE (29%). This may indicate a possible correlation between breaks and SCEs.

Anemia, Aplastic↗

[Recurrence with gonadic localization of acute leukemia. 113 cases].

Amongst 1500 cases of acute lymphoblastic leukaemia, affecting both sexes, the authors noted 111 testicular recurrences and 2 ovarian recurrences, i.e. an incidence of 16 percent. Clinical diagnosis is easy and testicular needle biopsy was reserved for doubtful cases only. Amongst 12 lymphographies, only one showed lumbar node involvement. Testicular involvement may exist before, after or in association with a bone marrow or a meningeal relapse. In 47 cases the first signal recurrence was a testicular tumour. The majority of these recurrences occured during the first two years of the disease, but late recurrences affecting the gonads are possible. Mean survival after an isolated testicular recurrence was 14 months, identical with meningeal recurrences. General chemotherapy and local radiotherapy were in general followed by an in situ or contralateral recurrence. Bilateral irradiation at a dose of 2400 rads is the best local treatment. However, in the light of the frequency of bone marrow recurrences a short time after, intensification of general treatment is necessary.

Biopsy, Needle↗

[Antigonadotropic actions of prolactin. Study of 10 cases of women with hyperprolactinemia].

In order to determine the pituitary or ovarian site of the anti-gonadotrophic action of prolactin (PRL), ten women with hyperprolactinaemia were studied in the following way: 1) Repeated estimations of PRL, gonadotrophins (LH and FSH), plasma estradiol and progesterone during six weeks of treatment with bromocriptine. 2) Verification of the effects of estradiol benzoate on LH and FSH levels before and after normalisation of prolactin. 3) Exploration of the ovarian response to the administration of human menopausal gonadotrophin. Without it being possible to exclude any direct effect of prolactin on the ovary, it may be affirmed that the hormone decreases the sensitivity of the gonadotrophic cells to the positive feedback mechanism exerted by plasma estradiol.

Adult↗

[Treatment of acute leukemia].

Evaluations of 650 patients with acute lymphoblastic leukemias (A.L.L.) and of 596 patients with acute granulocytic leukemias (A.G.L.) ara analyzed. The patients were treated in the department of Professeur Jean Bernard at Saint-Louis Hospital between 1964 and 1976. In A.L.L., prognosis is influenced primarely by age, being worse in infants less than 1 year old and in adults: it is also influenced by the blastic load and by other parameters such as cytology and immunological markers which could not be studied in all patients. The correlation between high blastic load and the T lymphoblasts variety is noteworthy. Random studies are still necessary to find out the best drug combinations and the optimal duration of treatment, but the necessity for meningeal prophylaxis, reinduction treatments and of L-A sparaginase consolidation is no longer discussed. Prognostic stratification is useful for therapeutical evaluation and should load to therapeutical modulation. In acute granulocytic leukemias, age is also a significant parameter and remission rate is lower for patients over 50 years of age. Cytology and cytochemistry allow to recognize acute promyelocytic leukemias and acute monoblastic leukemias which bear specific initial risks (D.I.C. for A.P.L., renal failure for acute monoblastic leukemia) but which are highly sensitive to Daunorubicin and Rubidazone respectively. In A.M.L. remission duration is not influenced by age and seems to be improved by intermittent treatment. The role of immunotherapy is still under study.

Acute Disease↗