[Bullous form of malignant histiocytosis].
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Biomedical subjects
Publications and source records attributed to G Leverger.
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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.
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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.
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"Xantholeukemia" is a rare disease which combines a juvenile chronic granulocytic leukemia with skin xanthomas and normal serum cholesterol level. The prognosis is variable, usually lethal, sometimes chronic with possible spontaneous cure. We collected 11 patients aged 3 months to 3 years and half at diagnosis. Nine died, 7 with a terminal "blastic" marrow. Blastic crisis was sometimes present at the onset, sometimes started later, between 9 months and 9 years after the onset. One child is still alive but in blastic crisis. One young girl is hematologically normal 13 years after the onset. At the onset, she had no bad prognostic signs. And she received no treatment. Five children had "café au lait" spots, 4 of them had familial history of neurofibromatosis. This frequent association confirms a genetic predisposition. Eight patients had one or several examinations of bone marrow and blood cytogenetics. Only one had an evident clonal abnormality: monosomy 7 (45 XY, -7) in all bone marrow cells. This monosomy 7 was discovered 21 months after the onset and 3 months before the blastic crisis. No chemotherapy had been used before this karyotype. Chemotherapeutic agents were essentially corticosteroids, 6 mercaptopurine and cytarabine. They gave an imperfect result and did not influence the outcome; therefore an aggressive treatment or a bone marrow transplantation should be discussed.
We report our experience in the treatment of meningeal relapses in acute lymphoblastic leukemia (ALL) with intraventricular chemotherapy via an Ommaya reservoir. We treated 5 children in this way with some complications secondary to the use of the reservoir: methotrexate leukoencephalopathy, bacterial meningitis, reservoir malfunction. But patient comfort homogeneous drug distribution when injected via the Ommaya reservoir and the possibility of long term meningeal remission justify the discussion of the use of an Ommaya reservoir use in meningeal relapses in ALL.
Fanconi's anaemia (FA) is a hereditary disease transmitted in a recessive manner, characterized by congenital malformations and bone marrow aplasia. A high rate of chromosome breakage is observed in mitoses of cultured blood cells, but the caryotypes are normal. Forty-four patients (27 boys and 17 girls) were followed in the same department between 1962 and 1976. Most were treated with androgens, sometimes combined with corticosteroids. Nine patients died of acute granuloblastic leukaemia, with more than 25% bone marrow blasts; in three of these, cytogenetic examination showed clonal anomalies. Five patients were in preleukaemic state with non-blastic bone marrow; 4 showed clonal anomalies and 2 of these died of aplasia; the 5th patient had gross liver and spleen enlargement and died of haemorrhage. Among the 30 remaining patients 12 are still alive and 18 died of cerebral haemorrhage (7), hepatic failure (3), cardiac failure (1), pancreatitis (1), septicaemia (2) or graft-versus-host reaction after bone marrow transplantation. One patient transplanted 4 years ago has complete chimerism and is still alive without treatment. The incidence of leukaemic or preleukaemic state in this series was 30%, while no case of leukaemia was observed in 200 patients with acquired aplastic anaemia. Neither parents norsibship had leukaemia. Androgen treatment apparently did not increase the risk of leukaemia which developed within 1 to 13 years (mean = 5 years) of the diagnosis, was preceded by a 2 1/2 year long preleukaemic state with clonal chromosomal anamolies and invariably was of the granulocytic type. None of the patients developed cancer. The median survival in this series was 4 1/2 years.
Eleven infants were treated at the Institut Gustave-Roussy between 1967 and 1980 for a yolk sac tumor localized to vagina. All the children had high serum alpha-foetoprotein levels. The average age was 10 months and the first symptoms were vaginal hemorrhages. The volume of the tumor and tumoral extension, as well as the biopsy, were appreciated by vaginal examination under general anesthesia. Treatment fluctuated during these years; 7/11 children were cured and since 1977, 6/8 (average follow-up 3 1/2 years) with the following therapeutic schedule: primary chemotherapy reducing the tumoral volume, and either partial colpectomy or curietherapy or both. So, these 6 girls were cured with a conservative treatment, preserving uterus and vagina. Two of them had moderate sequelae due to curietherapy.
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In order to study the value of the tetrazolium nitroblue test (TNB) in the localisation of urinary tract infections, the authors performed this test in 33 patients with a urinary tract infection and in 20 control children suffering from no bacterial infection. The 33 infected children were divided into three groups on the basis of clinical and bacteriological criteria (dysuria, frequency, fever, abdominal pain, inflammatory syndrome, immunofluorescence of bacteriuria, serum antibody levels): -- group A (8 children): upper urinary infection -- group B (14 children): lower urinary infection -- group C (11 children): urinary infection of undetermined site. The number and percentage of TNB positive polynuclears was significantly higher in the children of group A than in the children of group B (p 0.001). By contrast there was no significant difference between the results obtained: in the non-infected control group and in group B -- in group A and group C. These results, confirming a previous study by Bjorksten and de Chateau, show the value of the TNB test in the localisation of urinary tract infection in the child.
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Intravascular embolization of catheter fragments is a severe and rare complication of indwelling intravenous catheters. The estimated rate of incidence is 0,1% of central venous catheter insertions. This report of three cases shows how this complication may occur and suggests preventive measures. Mortality from arrhythmia-related cardiac arrest, septic and thrombo-embolic complications, and the risk of perforation of the heart argue for an immediate extraction of the broken catheter. Surgical removal used to be the only method; a non-surgical technique is now available, in which the embolized fragment is removed by means of a loop snare. No death has been associated with this technique which seems to be safe and reliable.