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Biomedical subjects

G Schaison

Publications and source records attributed to G Schaison.

At least 217 records · Page 12Linked to original sources
PubMed1983

[Sickle cell anemia, an example of a constitutional disease of hemoglobin].

Sickle cell anemia is a congenital haemolitic anemia. The replacement of the hydrophilic glutamine residue at the 6 position of the beta chain by the hydrophobic valine leads to severe conformation change in the molecule resulting in sickling. The consequences are haemolysis, vascular stasis and thrombotic crisis. Infection triggers thrombosis and vice-versa. Clinical manifestation is anemia punctuated by intermittent crisis producing infarction. Pneumococcal and salmonella infections are frequent mainly in the lungs and bones. Prognosis is poor, half of the patients dying before 20 years. Prompt treatment of infection is important. Treatment includes good nutrition and administration of folic acid. Transfusions are often usefull to avoid hazards of anesthesia or surgery. Ante natal diagnosis based on smaller sample of foetal blood or amniotic liquid is now possible.

Anemia, Sickle Cell↗
PubMed1983

[Robinow's syndrome].

Clinical and radiologic findings and differential diagnosis of the rare Robinow's syndrome are discussed in relation to one case. Features included nanism associated with multiple malformations such as mesomelic brachymelia, buccofacial anomalies, genital hypoplasia and hemivertebra. Transmission mechanisms are unknown and the karyotype is always normal.

Abnormalities, Multiple↗
PubMed1983

[Refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction. Study of a new case].

The case of an infant with bone marrow dysfunction and exocrine pancreatic deficiency is reported. Bone marrow dysfunction presented at birth, with a refractory sideroblastic anemia later associated with neutropenia and thrombocytopenia. Erythroid and myeloid precursors had a marked cytoplasm vacuolization and very poor in vitro growth. The exocrine pancreatic deficiency was shown by the pancreozymin-secretin stimulation test and by the study of fat digestion. This case report is different from Shwachman's syndrome, but similar to a syndrome of unknown etiology, recently described by Pearson in 4 children. The normality of immune investigations and of the culture of T lymphocyte precursors, in our patient, shows that the bone marrow dysfunction spares the lymphoid lineage. The simultaneous occurrence of bone marrow and pancreatic cells dysfunction suggests either a process acquired during embryonic life, or a gene mutation with pleiotropic effects.

Anemia, Sideroblastic↗
PubMed1983

[Robinow's syndrome. Apropos of a case with thrombopenia].

We report on a 17 month-old boy with Robinow' syndrome characterized by mild dwarfism, brachymelia, frontal bossing, hypertelorism, upturned nose, gingivodental, vertebral and bone age abnormalities, hypoplastic genitalia with normal gestation, birth weight and length and intelligence. There was an unusual association with idiopathic thrombocytopenic purpura and splenomegaly.

Abnormalities, Multiple↗
PubMed1983

[Prognostic value of chromosome anomalies in acute non-lymphoblastic leukemias].

The results of a cytogenetic study on 240 acute nonlymphocytic leukemia patients (187 adults and 53 children) were classified in NN (normal), AN (abnormal and normal) and AA (abnormal). Pronostic value of the classification was presented. A higher proportion of complete remission failures was observed in chromosomally abnormal patients (AN and AA). Survival of patients with complete remission was significantly shorter in AN and AA patients than in NN patients. An excess of constitutional chromosome abnormalities was observed in children.

Acute Disease↗
PubMed1982

[Iron-deficiency anemia. Hematologist's viewpoint].

Enlarged spleen, fever, increased susceptibility to infections, and thrombocytosis, are manifestations of iron deficiency which are relatively specific of pediatric patients. Iron deficiency anemia is part of everyday pediatrics. Patients are referred to the hematologist in the following situations: 1) Therapy is ineffective for one of the following reasons: the hypochromic anemia is not caused by iron deficiency (hemoglobinopathies); iron is less efficiently used because of transferrin deficiency or infectious, inflammatory or cancerous disease; iron therapy is inadequate either because of insufficient dosage or of suboptimal duration. 2) A relapse occurs in spite of adequate therapy. Before investigating the digestive tract, abnormal hemostasis. Osler-Weber-Rendu syndrome and pulmonary hemosiderosis should be considered. 3) Iron deficiency anemia is less common in adolescents. This condition, known as chlorosis, results mainly from increased needs, unbalanced diet, and onset of menses. In some cases no explanation is found but iron therapy leads to recovery. 4) Difficult problems arise in patients with complex anemias: iron deficiency with folic acid or vitamin B12 deficiency; hyposideremia complicating one of the hemoglobinopathies.

Adolescent↗
PubMed1982

[Clinical activity of m-Amsa and the combination of m-Amsa with cytosine arabinoside].

Of 91 acute leukaemia patients treated with m-Amsa, 19 received intermittent doses, 23 received daily doses and 49 underwent courses with combined m-Amsa and cytosine arabinoside (Ara-C). Intermittent doses had minimal therapeutic activity and toxicity. Among the 23 patients given daily doses, complete remission was observed in 5/12 relapses of ALL and in 2/11 relapses of AML. When Ara-C (200 mg/m2 x 5 days) was administered concomitantly with m-Amsa (200 mg/m2 x 5 days or 120 mg/m2 x 7 days), 17 out of 37 patients with advanced relapses of ALL (13/25 children and 4/12 adults) went into complete remission. While high doses of m-Amsa alone were well tolerated, the combined treatment with high doses of both drugs resulted in severe gastro-intestinal toxicity. Cardiac disorders were observed in patients who had previously received high doses of anthracyclins; there were 5 cases of dysrhythmia and 1 case each of sudden death, ECG alterations and heart failure. In view of its indisputable activity, m-Amsa should be used at an earlier stage in the treatment of acute leukaemias.

Adolescent↗
PubMed1982

[Pathophysiology of Grave's disease (author's transl)].

It has been established that Grave's disease is an autoimmune condition characterized by immunization against TSH receptors. Neither the receptors nor the stimulating immunoglobulins have been identified, but there seems to be two types of antireceptor antibodies: some stimulate the production of hormones or of thyroid stimulating immunoglobulins (TSI) and are responsible for thyrotoxicosis; others stimulate cell proliferation or thyroid growth immunoglobulins (TGI) and account for the diffuse goitre. The mechanism that triggers off autoimmunization is still unknown, but the disease frequently occurs in individuals genetically predisposed, as suggested by the high incidence of some HLA B8 and DR W3 antigens.

Autoantibodies↗
PubMed1982

Poor-prognosis acute lymphoblastic leukemias.

Burkitt's-type leukemias have specific cytologic, immunologic, and cytogenetic characteristics. Initial symptomatology frequently includes abdominal tumors and initial CNS involvement. Despite intensive treatment including high-dose cyclophosphamide, prognosis remains poor in most patients because of failures to achieve complete remission (CR) or because of early relapses, especially CNS relapses. Class III acute lymphoblastic leukemia in children is defined by the presence of two or more unfavorable parameters and recent progress has been achieved by intensive therapy. Cox's multifactorial analysis allows improved discrimination. A phase I protocol for increased-risk leukemias, including testis preventive irradiation and monthly reinductions without continuous maintenance for the first 6 months of CR, seems promising.

Adolescent↗
PubMed1982

[Hodgkin's disease in childhood: long term results].

Between 1965 and 1976, 83 previously untreated children aged 15 years and under, with biopsy-proven Hodgkin's disease (HD) were evaluated, treated and followed at Hospital Saint Louis, Paris. Clinical stages were IA-IIA for 59 patients, IB-IIB for 19 patients and III-IV for 5 patients. Two main kinds of treatment were used: monochemotherapy-radiotherapy (MCT-RT) for 26 patients who have received mantle field irradiation followed by a monthly Vinblastine injection during 3 years; 57 patients have received a combination of MOPP and radiotherapy (MOPP-RT). The MOPP-RT treated patients have a significantly better survival and relapse free survival than the MCT-RT treated patients (86.9% vrs 76.1% and 83.5% vs 65.4%). Thirteen relapses were observed after a median complete remission of 30 months: 6 patients are now free from disease and one is still under treatment. Ten patients are dead after a 55 months median survival: 7 died from H.D. and 3 from treatment toxicity. No 2nd cancer or leukemia were observed until now. The main long term complications of therapy were sterility in male patients, growth defects and disturbances of thyroid functions.

Adolescent↗
PubMed1982

[Children born of leukemic parents. Apropos of 23 children].

We have studied the children born of leukemic parents who treatment had stopped. In total, 8 women (3 acute myeloblastic leukemias and 5 acute lymphoblastic leukemias) who gave birth to 11 children, and 6 men (all with acute lymphoblastic leukemias) who fathered 12 children were studied. Of these 23 children, two have a severe congenital malformation, one congenital hypopituitarism associated with mid-line defect, and one laparoschisis, and also two benign abnormalities were observed. The children with abnormalities had a leukemic mother, whilst no leukemic father had an abnormal child. It is well known that the toxic effect of chemotherapy is different in the male and the female gonad. These results are compared to those in the literature, and at present it appears difficult to form a clear opinion on the delayed teratogenic effect of chemotherapy. Fecundity and the risk for future generations are unknown. The opening of an international registry would be useful.

Abnormalities, Drug-Induced↗
PubMed1982

[Acute curable preleukemic bone marrow aplasia in children].

The occurrence of a transitory aplasia followed shortly thereafter by an acute leukemia in a known sequence in children is rare. We report 13 observations involving children between 6 months and 10 years of age. Splenomegaly was observed in 5 patients and hepatomegaly in two. There was a tricytopenia in 5 cases, bicytopenia in 4 and an isolated cytopenia in the remaining 4 cases. The phase of aplasia was short, lasting from 6 to 30 days. Complete bone marrow recovery occurred with integral restitution. Remission was spontaneous or followed transfusion or corticosteroid therapy and lasted for 2 to 6 months. The leukemia had no particular character when it appeared: there was one acute myeloblastic leukemia, 11 acute lymphoblastic cases and one sarcoma. Median survival time was 5 to 32 months and relapses were not aplasic. It is noteworthy that among these cases there are two long remissions lasting more than ten years. The interpretation of these observations is difficult because of the following choice: an initially non-leukemic aplasia or a leukemia present at the onset but undetected, camouflaged or confined to several infrequent blast cell islets.

Acute Disease↗
PubMed1981

[Autoimmune thrombocytopenic purpura. Clinical and therapeutic retrospective study of 544 cases (author's transl)].

A retrospective study of 544 patients with autoimmune thrombocytopenic purpura showed that 81 had immunological abnormalities (antinuclear factors and/or positive direct Coombs; test and/or anti-smooth muscle antibodies). After one year, 58-8% of all patients were still in complete remission, irrespective of treatment. The outcome of the disease was studied in relation to therapeutic regimens. Prognosis was worst in patients with immunological abnormalities, since 11% died as compared with 4% of the whole patient population. Most relapses occurred during the first two years following remission.

Adolescent↗
PubMed1981

[Treatment of acute lymphoblastic leukaemia in children over twenty years (author's transl)].

This is a review of the progress achieved in the treatment of acute lymphoblastic leukaemia, based on a series of 1580 children treated in Prof. Jean Bernard's unit, Paris, from 1956 to 1976. The children are retrospectively divided into three prognostic classes and five therapeutic categories. The benefits obtained from successive additions to the therapeutic armentarium during that period are conspicuous in all classes and categories and particularly striking in children with poor initial prognosis. The role of each component of the therapeutic measures applied is discussed.

Adolescent↗