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Biomedical subjects

G Tobelem

Publications and source records attributed to G Tobelem.

At least 145 records · Page 8Linked to original sources
PubMed1983

[Treatment of chronic autoimmune thrombopenic purpura with intravenous immunoglobulins. Study of 18 cases].

The authors reported the effect of high dose intravenous immunoglobulin therapy in 18 patients with chronic autoimmune thrombocytopenic purpura. A good but transitory response was observed in 60% of the cases. The intensity of the response was variable, the duration was between 11 and 26 days. The mechanism of the effect of this therapy is poorly understood. Since this therapy is expensive and the response inconsistent and transitory, the indications are limited.

Adolescent↗
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

[Moschkowitz's syndrome, or thrombotic thrombocytopenic purpura].

Moschcowitz's syndrome is a rare condition with poor prognosis. It is characterized by a microangiopathic haemolytic anaemia associated with thrombocytopenia, neurological symptoms and renal involvement. The vascular lesions consist of hyaline microthrombi, predominantly made up of platelet aggregates, disseminated in the smaller vessels. The physiopathological mechanisms are still poorly understood and probably multiple. Recent studies have demonstrated abnormalities in the endothelial synthesis of prostacyclin, and in many cases the lack of a plasma factor has been held responsible for intravascular disseminated platelet aggregation. The latest therapeutic attempts suggest that the most effective treatment probably is fresh plasma transfusions associated or not with plasma exchanges.

Adult↗
PubMed1982

[The factor VIII complex: hemophilia A and von Willebrand disease].

The factor VIII complex is a macromolecule with two distinct components. One is the coagulation factor VIII. The other, known as the Willebrand factor, is a polymer which probably acts as a carrier for serum factor VIII. Hereditary disorders can affect either of these two components. Hemophilia A is a coagulation disorders due to decreased factor VIII coagulant activity. Increase of partial activated thromboplastin time parallels disease severity. Hematomas and hemarthrosis in large joints are the main clinical features. In von Willebrand disease, mucocutaneous bleeding is the main symptom. Diagnosis is established by demonstrating disorders of primary hemostasis: prolonged bleeding time and decreased ristocetin-induced platelet aggregation. Two forms of von Willebrand disease have been described. In the quantitative form, decreased synthesis of von Willebrand factor is often responsible for severe clinical manifestations. The qualitative form probably results from defective polymerization of von Willebrand factor subunits. In both these forms, deficient primary hemostasis is a consequence of decreased platelet adhesion to the vascular wall. Clinical and biological features of hemophilia A and von Willebrand disease, as well as their management, are discussed.

Blood Coagulation Tests↗
PubMed1982

[Thrombotic thrombocytopenic purpura or Moschcowitz syndrome].

Moschcowitz syndrome or thrombotic thrombocytopenic purpura is a rare disorder with a poor prognosis. This syndrome is characterized by a microangiopathic hemolytic anemia with thrombocytopenia, neurologic symptoms and renal disease. The vascular lesion consists of disseminated hyaline thrombi in the microvasculature composed mainly of platelet aggregates. The mechanisms are still poorly understood and are probably multiple. Recent data focus on an abnormal endothelial synthesis of prostacyclin and the presence of a factor (or the reduction of its inhibitor) in plasma able to induce intravascular disseminated platelet aggregation. The most efficient therapy seems to be infusions of fresh plasma with or without plasma exchange.

Adolescent↗
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↗