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Biomedical subjects

G Schaison

Publications and source records attributed to G Schaison.

At least 181 records · Page 10Linked to original sources
PubMed1985

Complete correction of Glanzmann's thrombasthenia by allogeneic bone-marrow transplantation.

Allogeneic bone marrow transplantation (BMT) successfully corrected type I thrombasthenia in a 4-year-old boy. The donor was his HLA-A, B and D identical 14-year-old brother who was heterozygous for thrombasthenia. A first transplant after conditioning with cyclophosphamide and thoracoabdominal irradiation was rejected, but a second transplant using CCNU, cyclophosphamide, procarbazine and horse antihuman thymocyte globulin in the preparative regimen was successful. Engraftment was proven by studies of platelet membrane antigens, PLA1 and glycoprotein IIb/IIIa complex and by platelet function studies. Haemorrhagic manifestations completely disappeared; platelet membrane markers and clot retraction returned promptly to normal values, and platelet aggregation tests more slowly. Twenty-four months after bone-marrow transplant, the patient was well with mild chronic hepatic graft versus host disease. BMT therefore appears to be a possible treatment for severe inherited platelet disorders.

Blood Platelet Disorders↗
PubMed1985

Endometrial and pituitary responses to the steroidal antiprogestin RU 486 in postmenopausal women.

The effects of the antiprogestin RU 486 on the human endometrium were investigated. Seventeen postmenopausal women were injected with estradiol (E2) benzoate (0.625 mg/day) for 15 days. Progesterone (P) (25 mg/day) and/or RU 486 (100 or 200 mg/day) were given to groups of 2-3 women during the last 6 days of E2 benzoate treatment. Serial blood samples were drawn for the measurement of plasma E2, P, and LH and FSH. An endometrial biopsy was performed on the last day of treatment, and processed for histology or for assays of DNA polymerase alpha, E2-dehydrogenase (E2DH), and P receptor (PR). Treatment with E2 benzoate alone resulted in a marked decrease of plasma gonadotropins; in those patients who received either P, RU 486, or both, in addition to E2 benzoate, the concentrations of plasma LH and FSH were further decreased to premenopausal levels. In absence of glycerol, the affinity of RU 486 for the endometrial PR (Kd = 0.8 nM) was higher than that of P (Kd = 1.2 nM). Glycerol decreased markedly the affinity of RU 486, whereas the affinity of P for the PR was unchanged. RU 486 had negligible affinity for plasma transcortin. Either P or RU 486, but not both together, induced secretory changes in the endometrium as determined from histologic sections of tissue biopsies. Either P or RU 486 decreased DNA polymerase alpha and increased E2-DH activities in the endometrium. Unexpectedly, when P and RU 486 were given together. E2-DH activity remained at the level found in E2-treated women. In vitro cultures of proliferative endometrium treated with the synthetic progestagen R 5020 or with RU 486 also had increased E2-DH activity; RU 486 counteracted R 5020 effects. We conclude that, contrary to previous results with experimental animals, the anti-P RU 486 has some progestomimetic activity in humans under specific conditions. Paradoxically, when given together with P, RU 486 lost most of its progestomimetic activity in the endometrium and behaved as a pure antagonist.

Adult↗
PubMed1985

Gonadotropin-releasing hormone pulsatile administration restores luteinizing hormone pulsatility and normal testosterone levels in males with hyperprolactinemia.

Hyperprolactinemia in men is frequently associated with hypogonadism. Normalization of serum PRL levels is generally associated with an increase in serum testosterone (T) to normal. To determine the mechanism of the inhibitory effect of hyperprolactinemia on the hypothalamic-pituitary-gonadal axis, we studied the effect of intermittent pulsatile GnRH administration on LH pulsatility and T levels in four men with prolactinomas. All patients had high PRL values (100-3000 ng/ml), low LH (mean +/- SEM, 2.2 +/- 0.1 mIU/ml), and low T values (2.3 +/- 0.3 ng/ml), with no other apparent abnormality of pituitary function. GnRH was administered iv using a pump delivering a bolus dose of 10 micrograms every 90 min for 12 days. No LH pulses were detected before treatment. Pulsatile GnRH administration resulted in a significant increase in basal LH levels (6.7 +/- 0.6 mIU/ml; P less than 0.001) and restored LH pulsatility. In addition, T levels increased significantly to normal values in all patients (7.8 +/- 0.4 ng/ml; P less than 0.001) and were normal or supranormal as long as the pump was in use, although PRL levels remained elevated. These data, therefore, suggest that hyperprolactinemia produces hypogonadism primarily by interfering with pulsatile GnRH release.

Adult↗
PubMed1985

Effects of the antiprogesterone steroid RU 486 during midluteal phase in normal women.

UNLABELLED: The antiprogesterone steroid RU 486 (17 beta-hydroxy-11 beta-4-dimethyl-aminophenyl)17 alpha(1-propynyl)estra-4,9-dien-3-one) was given orally to 32 normally cycling women for 4 days, starting on the fourth day of the luteal phase. Uterine bleeding occurred on the third day of RU 486 administration in all 14 women treated with 100 mg/day, in 7 of the 8 women treated with 50 mg, and in 8 of 10 women receiving 25 mg/day. Premature luteal regression induced by RU 486 occurred in 8 women treated with 100 mg/day, in 3 treated with 50 mg, and in 2 receiving 25 mg/day. Plasma LH was measured every 15 min from 0800-1200 h for 5 days in 17 women. Mean LH levels decreased and pulsatile release disappeared in 7 of the 8 women treated with 100 mg, in 2 of 4 receiving 50 mg, and in 1 of 5 treated with 25 mg. RU 486 had no effect when given to 5 women with anovulatory cycles for 4 days starting on day 18 of the cycle. IN CONCLUSION: 1) RU 486, given to normally cycling women at midluteal phase, provokes uterine bleeding. 2) This effect occurs whether or not luteal regression is induced by the compound, indicating that RU 486 acts directly upon the endometrial tissue, very likely at the progesterone receptor level. 3) The drug may impair simultaneously or separately luteal function and gonadotropin secretion in a dose-dependent manner. 4) The lack of antiglucocorticosteroid activity, at the dosage of 100 mg/day, suggests that RU 486 may be useful for fertility control.

Adrenocorticotropic Hormone↗
PubMed1985

Elevated metabolic clearance rate of 1 alpha,25-dihydroxyvitamin D3 in hyperthyroidism.

Metabolic clearance rate (MCR) and daily production rate (DPR) of 1 alpha,25-dihydroxyvitamin D3 (1,25(OH)2D3) were measured in 7 hyperthyroid patients and 8 control subjects after a single injection of tritiated 1,25(OH)2D3. Using a bicompartmental analysis, MCR of 1,25(OH)2D3 was found to be significantly higher in hyperthyroid than in control subjects (11.5 +/- 4.4 vs 5.9 +/- 2.4 ml/min (SD), P less than 0.01). No significant difference in plasma 1,25(OH)2D and DPR of 1,25(OH)2D3 was demonstrated. MCR was again measured 1 month after initiation of treatment in 4 hyperthyroid patients and remained elevated. In 2 of these patients, MCR was once more determined 6 months later and had declined towards normal. No correlation was found between MCR and DPR and either plasma calcium, phosphate, immunoreactive PTH, 1,25(OH)D and T3 values. In conclusion, MCR of 1,25(OH)2D3 is elevated in hyperthyroidism, but this finding is probably unrelated to the effect of thyroid hormones on bone metabolism.

Adult↗
PubMed1985

[Acute hyperleukocytic lymphoblastic leukemia (greater than or equal to 100,000 leukocytes/mm3). Metabolic changes during induction treatment. Study, prevention and treatment].

Acute leukemias with high white blood count have a poor immediate prognosis and the treatment must be started within the first hours following diagnosis. It is necessary to prevent and to treat the severe metabolic disorders observed during induction treatment of acute lymphoblastic leukemia with WBC greater than or equal to 100,000/mm3. We analysed all the metabolic disorders in a retrospective study of 45 patients in order to determine their adequate prevention and treatment. Prevention of hyperuricemia and of secondary renal failure is now possible with urate oxidase, allowing an aggressive and rapid induction. Hyperkalemia can be prevented by urinary alkalinization and hyperphosphoremia with hypocalcemia by high dose intravenous calcium therapy. Renal failure is often transitory and functional. Disseminated intravascular coagulation is treated by heparin and platelets infusion and severe hyperglycemia requires insulin therapy.

Adolescent↗
PubMed1985

[Association of hemoglobin E and thalassemia].

Double heterozygotism Hb E-beta zero thalassemia was discovered in two children born to an Alaouite Syrian family. Clinical, biological and radiological findings were similar to those in Cooley disease. Splenectomy allowed reduction in the frequency of blood transfusions. Hb E disease is frequent in South East Asia and results in a mild hemolytic anemia in homozygous patients. Hb E disease is a thalassemia syndrome with decrease production of beta E RNA messenger, and imbalanced alpha/beta E chains. Association with the thalassemia gene increases the imbalanced of chain synthesis explaining the severity of the disease. These were the first cases of Hb E-thal in Syria.

Child↗
PubMed1984

[Anovulation of hypothalamic origin. Treatment by pulsatile injection of hypothalamic gonadotropin releasing hormone].

In two patients with severe hypogonadotrophic hypogonadism intermittent pulsatile delivery of gonadotrophin hormone (LHRH) 5 to 20 micrograms every 90 minutes by means of the Zyklomat pump promptly resulted in ovulation and pregnancy. The comparative effectiveness of the intravenous and subcutaneous routes was evaluated by radioimmuno assay of LHRH in plasma. The pulsatile patterns of LH was satisfactory with both routes, but plasma levels of LHRH were about 7 times lower with the subcutaneous route. The dosage range (5-20 micrograms) of LHRH must be adjusted to the severity of the hypothalamic deficiency and to the route of administration. The use of the pump, the monitoring of ovulation and the indications for this type of treatment are discussed.

Adult↗
PubMed1984

Subacute and chronic myelomonocytic leukemia in children (juvenile CML). Clinical and hematologic observations, and identification of prognostic factors.

The clinical and hematologic characteristics of 38 children with subacute and chronic myelomonocytic leukemia (S & CMMOL) are described, and the prognostic significance of these characteristics as recorded at diagnosis is reported. The common and distinctive feature of these children was the excessive proliferation of cells of neutrophilic and monocytic series. The disease predominated in younger children, 95% were younger than 4 years, and boys were more affected than girls (22/16). The onset of the disease was heralded most often by acute or subacute symptoms. Splenomegaly was the most common physical finding at diagnosis. Leukocytosis was usually under 100 X 10(9)/l. Monocytosis and granulocytosis were often associated with normoblastosis, and, in some cases, with moderate blastosis (less than or equal to 30%). Severe anemia and marked thrombocytopenia were found in about one third of patients, increased fetal hemoglobin levels in 53%, and increased gamma-globulin levels in 50% of cases. The Philadelphia chromosome was absent in all blood and marrow cell karyotypes. Thirty-three of 38 patients were treated with moderate or intensive chemotherapy, and in all cases treatment never resulted in a complete remission. Terminal acute leukemia occurred in 11 cases. Of the 38 patients, 29 have died (median survival time, 16 months). Initial characteristics predicting a short survival (log-rank test) included: older age (greater than or equal to 2 years) (P less than 0.001), hepatomegaly (P less than 0.05), bleeding (P less than 0.001), thrombocytopenia (P less than 0.01), high counts of blasts and normoblasts in peripheral blood (P less than 0.01, P less than 0.01). Sex, infections, cutaneous manifestations, lymphadenopathy, degree of splenomegaly, hemoglobin levels, fetal hemoglobin, leukocyte counts, percent of blasts in bone marrow, and serum gamma-globulin levels were of no prognostic value. When survival was plotted on a semilogarithmic scale, a change in death rate was evident at the second year of survival suggesting that there may be two subgroups of patients with myelomonocytic picture, one with very rapid, and another with a slower rate of mortality. A stepwise discriminant-function analysis was performed in an attempt to distinguish between those children who lived less than or equal to 2 years and those who lived longer. A linear combination of variables which best discriminated between these two subgroups was found. Nearly all patients could be classified as a short-survivor or long-survivor on the basis of age and platelet, blast, and normoblast counts in peripheral blood.(ABSTRACT TRUNCATED AT 400 WORDS)

Bone Marrow Examination↗