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Biomedical subjects

G Schaison

Publications and source records attributed to G Schaison.

At least 145 records · Page 8Linked to original sources

Interaction of deletional alpha-thalassaemia with sickle cell beta-thalassaemia and its influence on foetal haemoglobin expression.

A rare association of three haemoglobin defects, viz: traits for deletion form of alpha-thalassaemia, beta-thalassaemia, and sickle cell gene, in a family of French West Indies origin, was studied both at phenotype and genotype levels. In this sickle cell beta-thalassaemia, interacting alpha-thalassaemia is shown to influence the foetal haemoglobin expression. A reverse relationship between the foetal haemoglobin level and the number of alpha genes was observed.

Anemia, Sickle Cell↗

[Hodgkin's disease in children. Current therapeutic strategies].

The observed cure rates in childhood Hodgkin's disease (85 to 95%) and the knowledge of late complications of treatments, radiotherapy as well as chemotherapy, lead to studies aiming at decreasing the treatment burden without compromising these good results. An international workshop helped to make an update of the different presently used strategies. It appeared that the benefit and the risks of the staging laparotomy are still controversial, though most of the teams are now omitting this procedure. Different options have been selected in decreasing the treatments. If a few teams choose to treat by chemotherapy alone, the majority prefer to treat by combined modality therapy allowing to reduce at the same time the intensity of the radiation therapy (doses and fields) and of the chemotherapy (use of chemotherapy devoid of procarbazine and alkylating agents for the favorable cases). The presented french study shows the feasibility of such a strategy.

Adolescent↗

[Endocrine polyadenomatosis of 2a type (MEN 2a). Clinical and genetic study of a family].

In a large kindred with multiple endocrine neoplasia type 2a (MEN 2a) (137 members, 5 generations), bilateral thyroid medullary carcinoma was found in all affected members. Pheochromocytoma was present in 59% of the cases, and was responsible at least for 4 out the 5 deaths related to MEN 2a. Hyperparathyroidism was less frequent (41%). Family screening leads to a reduction in age for diagnosis and to an improvement in the prevalence of complete healing after surgery. Linkage between HLA loci and a dominant gene for MEN 2a was investigated in this kindred. Lod scores for recombination fraction were all negative (-0.47 for a recombination fraction of 0.05). These results comfort the lack of linkage between MEN 2a and the HLA complex.

Adolescent↗

[The value of chromosome anomalies in the prognosis of acute non-lymphoblastic leukemia in children].

Cytogenetic studies performed on 130 consecutive childhood acute non lymphoblastic leukemias (ANLL) and investigated in the same center between 1977 and 1986 have been studied for their prognostic value. Clonal chromosome changes were detected in 68.5% (89/130) of the cases prior to treatment. Complete remission rate and median survival were significantly lower in patients with only abnormal metaphases than in patients with only normal metaphases (NN) or a mixture of normal and abnormal mitoses (AN). The ANLL with translocation t(8;21), which were 58.6% of the M2 ANLL, were not associated with a particularly long survival (16 months) when compared with AN and NN ANLL. The longest survival (26 months) was observed in patients with acute myelomonocytic leukemia with bone marrow eosinophilia. Cytogenetic analysis have a prognostic value in childhood ANLL.

Adolescent↗

[Contragestion].

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Contraceptives, Postcoital↗

[Value of high-dose cytosine-arabinoside in the treatment of resistant acute leukemia].

Sixty-one patients with refractory or relapsed or secondary acute leukemia were treated with high-dose cytosine arabinoside (2-3 g/sq m in intravenous infusion every 12 hr to a 12-36 g/sq m total dose). m-Amsa or another antileukemic drug was given with cytosine arabinoside to 20 patients. Complete remission was achieved in 12 of 27 patients with acute myeloid leukemia, 5 of 8 patients with chemotherapy-induced leukemia, 3 of 7 patients with hematologic disorders in blastic crisis and 5 of 17 acute lymphoblastic leukemia patients. A similar response rate (6/16) was obtained when m-Amsa was given with cytosine-arabinoside. The median duration of remission was short (4 months in acute myeloid leukemia). Bone marrow transplantation was performed in 10 patients during the remission time. This regimen has acceptable toxicity; severe neurologic or hepatic disorders occurred in 18% of patients. These data suggest that high-dose cytosine arabinoside is an effective alternative in the treatment of resistant acute leukemia.

Acute Disease↗

Plasma lipids in juvenile chronic granulocytic leukaemia.

Plasma lipid parameters (triglycerides, total cholesterol and high density lipoprotein cholesterol) were measured in 7 children with juvenile chronic granulocytic leukaemia, of whom 3 were with and 4 without xanthomas. In all cases, whatever the stage of the disease, these parameters were extremely altered. Plasma triglycerides were generally increased, total and HDL cholesterols were very low. No relationship seems to exist between these values and the occurrence of xanthomas.

Child↗

Comparative effects of cyproterone acetate or a long-acting LHRH agonist in polycystic ovarian disease.

A randomized cross-over study was done to compare the therapeutic efficacy of cyproterone acetate (CPA, 50 mg/day orally) and a depot preparation of the LHRH superagonist (D-Trp6 LHRH 3 mg i.m. once a month) in 10 patients with polycystic ovarian disease (PCO). The two treatment periods were separated by 6 months. Both treatments resulted in marked clinical improvement. In response to CPA treatment, basal plasma gonadotropin, estradiol, estrone, testosterone and androstenedione levels significantly decreased. In response to D-Trp6 LHRH, both basal and stimulated gonadotropin levels were completely suppressed after 3 weeks of treatment. After initial elevation on day 2, plasma ovarian steroid levels fell into the castrate range, without any change in dehydroepiandrosterone sulfate levels. Urinary 3 alpha-androstanediol excretion decreased significantly. In patients with PCO, LHRH-A induced more complete gonadotropin inhibition than did CPA. However, following cessation of either therapy, the disease rapidly recurred.

Adult↗

Increased aromatase activity in pubic skin fibroblasts from patients with isolated gynecomastia.

Aromatase activity (AR) was studied in pubic skin fibroblasts from eight patients with isolated gynecomastia (PSFG) and five normal subjects (PSFC). Cell monolayers were incubated in the presence of [3H]androstenedione (2 nM) for 4 or 24 h. Culture medium was extracted after addition of [14C] carriers to monitor recovery. Metabolites were separated by two successive chromatographic steps. Estrone (E1) and estradiol (E2) were characterized by crystallization, the other metabolites: 16-hydroxyestrone (16 alpha-OHE1) estriol (E3), and epiestriol (epiE3) by their chromatographic migration. AR was expressed either as femtomoles of E2 per microgram DNA (ARE2) or as total aromatized metabolites (ART = E1 + E2 + 16 alpha-OHE1 + E3 + epiE3/microgram DNA). After 4 h of incubation, no ARE2 could be measured in PSFC; it was low but significant in PSFG (0.03 +/- 0.02 (SEM) fmol/microgram DNA, P less than 0.01). The difference in ART was even more striking: 0.28 +/- 0.1 fmol/microgram DNA in PSFC, 3.15 +/- 2.88 in PSFG (P less than 0.05). 16 alpha-OHE1 represented in this latter group 62.5% of total aromatized metabolites vs. 39% in PSFC. After 24 h, ART was 4.17 +/- 3.70 and 1.02 +/- 0.42 fmol/microgram DNA in PSFG and PSFC, respectively (P less than 0.05); E3 + epiE3 represented 50% of the metabolites in both groups. In conclusion, AR is increased in PSFG relative to PSFC and an important oxidative metabolism of estrogens exists in both types of cells. This increased peripheral AR could result in increased formation of estrogens at the target cell site and represent an element of androgen-estrogen imbalance which would favor the development of gynecomastia.

Adolescent↗

[Treatment of Hodgkin's disease in children with chemotherapy and low-dose radiation].

In 1982 a French cooperative study for Hodgkin's disease was designed to minimize adverse side effects of therapy in childhood. Initial staging is clinical, omitting laparotomy. Objectives are: comparing 4 ABVD to 2 MOPP alternating with 2 ABVD in CS IA et IIA, assessing the value of 3 MOPP + 3 ABVD in CS IB, IIB, III et IV in terms of remission induction and duration of remission and assessing the effectiveness of radiotherapy at dose of 20 Gy given to all good responders to chemotherapy in involved fields and lombo-splenic fields for CS IB, IIB, III and IV. We present preliminary results. Of the 120 evaluable patients 94% had a good response to initial chemotherapy and received 20 Gy. The rate of local control of these patients is very high as only 2 relapsed in previously irradiated nodes. Actuarial 3 years survival rate and relapse free survival are respectively 98% and 86%.

Adolescent↗

[HIV infections and transfusions].

The prevalence of AIDS now approaches 1% in patients with hemophilia and laboratory evidence of abnormal immunoregulation is found in 50% of patients with severe hemophilia. Current evidence indicates that a human retrovirus HIV/LAV is the etiologic agent which can be transmitted through the administration of blood products. We reported 7 cases in childhood, 5 acute leukemia, 1 metastatic neuroblastoma, and 1 severe aplastic anemia in whom AIDS occurred after administration of blood products. To date 5 patients are alive and 2 have died of infections. It is suggested that the use of steroids or antineoplastic agents increases the incidence of AIDS in patients infected with HIV/LAV because of altered immune suppression. The maintenance treatment is difficult in these patients. The widespread use of HIV serology to screen donated blood should help to prevent AIDS transmission in leukemic patients.

Acquired Immunodeficiency Syndrome↗

[Importance of anthracyclines in infants in the treatment of acute lymphoblastic and non-lymphoblastic leukemias].

Induction treatment of acute non lymphoblastic leukemia in children and in adults must include anthracyclines, but the best anthracycline is still in discussion. In acute lymphoblastic leukemia the use of anthracycline is discussed according to the initial prognostic factors: needful in high risk patients, useless in good risk. In intermediate risk patient, most cooperative protocols include one anthracycline in induction.

Acute Disease↗