Cyclosporin-induced muscular toxicity.
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Biomedical subjects
Publications and source records attributed to Y Lebranchu.
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The progress in organ transplantation is leading an increase in the demand for organs. Any non-harvested organs from a dead child mean at least a delay or even the loss of hope for life. All brain dead children do not undergo organ harvesting because there are still obstacles. The definition of brain death must be clear and include the destruction of all the brain system. Intensive care units must be motivated by improved contacts with transplantation teams. The number of parents who refuse could decrease with better public information, good contacts with medical teams and the quality of discussion with parents when a child dies. Energy should be concentrated on improving the collective consciousness about this new aspect of medicine.
Ultrasound techniques and radionuclide studies are very often used to assess vascularization of renal transplants. Although, in acute tubular necrosis or acute rejection, it is difficult to choose between these two techniques, in 3 recent cases of renal artery thrombosis, we conclude that color coded Doppler is preferable to nuclear medicine or duplex system to diagnose this form of thrombosis.
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Thirty French children (18 males, 12 females; mean age 20 +/- 18 months) presenting with Kawasaki disease were treated with high-dose intravenous plasmin-modified immunoglobulins (Veinoglobulines Institut Mérieux, France) during the acute phase of the disease. The total dose ranged from 1 to 2.5 g/kg (mean 1.88 +/- 0.50) administered in 1 to 5 infusions. In every case the clinical symptoms disappeared rapidly. Hyperfibrinaemia and hyperleucocytosis with granulocytosis regressed within one week, and high platelet counts within 2 to 3 weeks. the disappearance of immunological abnormalities paralleled that of clinical signs. Three-dimensional echocardiography showed dilatation of the left coronary artery in only one of the 30 children at 1 and 6 months, but coronary arteriography performed during the 6th month gave normal results. Intravenous immunoglobulin therapy seems to be effective in preventing the development of coronary aneurysms in patients with Kawasaki disease. The immunoregulatory function of immunoglobulins accounts for this effectiveness and for their value in immune diseases.
Autoantibodies to the cytoplasmic components of neutrophil polymorphonuclear granulocytes (ACPA) were searched for by indirect immunofluorescence on ethanol-fixed smears from normal white blood cells in 248 sera from patients with Wegener's granulomatosis, Kawasaki disease and various other vasculitides, connective tissue diseases, nephropathies and infiltrative pulmonary diseases. Apart from a weak positivity in a few Kawasaki disease sera, ACPA were found in Wegener's granulomatosis (and micropolyarteritis) only, with a correlation between ACPA titers and disease activity. These results confirm the value of ACPA in the diagnosis and evaluation of activity of Wegener's granulomatosis.
Serum IgG subclass levels were measured using an indirect competitive immunoenzymatic assay with monoclonal antibodies in 221 patients affected with definite immunodeficiency (ID) syndromes and 229 patients presenting with infection patterns suggestive of ID, but with normal immunoglobulin class levels and no clear evidence of ID. In common variable ID and IgG-IgA deficiency with normal or high IgM, subclass imbalance (mostly IgG1-IgG3 or IgG2-IgG4 deficiency) was the rule, with a higher incidence of severe infections in IgG2-IgG4 defects. One-fifth of patients with IgA deficiency, especially those with autoimmune cytopenia, had subclass deficiencies with no significant correlation with the occurrence of infections. Subclass (mostly IgG2-IgG4) deficiencies were also observed in severe combined ID, defective expression of HLA class II antigens, chronic mucocutaneous candidiasis, and IgM deficiency. Subclass levels were normal in all but one (who was IgG3 deficient) patient with the Wiskott-Aldrich syndrome and in the Buckley's syndrome, except for an unusual patient who presented with low IgG and IgA levels. Subclass (mainly IgG2) deficiency occurred in 24% of infected patients without known ID.
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Improved results of organs transplantation have resulted in an increased request for organs. All brain-dead children do not undergo organ harvesting for 3 main reasons. First, the definition of brain death, which should remain strict, is not accepted by everyone, in spite of the laws on this topics. Then, organizing organs harvesting requires specific hospital structures, a co-ordinator and motivated medical staff. Finally, parent's authorization is mandatory before organs harvesting in children. Parents still often refuse. An improvement might be reached by informing the general public, a maintained trust in the medical profession and the quality of the talk with the parents when the child dies. All energies should tend to develop the collective consciousness in order to improve this new aspect of medicine.
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The first patient suffered from a very severe atopic dermatitis with intense pruritus and thickened skin. He had also recurrent infections, particularly related to Staphylococcus coagulase +, and axillary and inguinal lymphodermopathy. The use of tetracosactide given intramuscularly allowed controlling the evolution of his atopic dermatitis. After several months of treatment, the skin became less infiltrated, lymphodermopathy disappeared and no severe infection had happened. The second patient had a less severe atopic dermatitis and recurrent infections without any particular severity. Topical corticosteroids allowed to control the atopic dermatitis. These two patients had high levels of circulating IgE and an important deficiency of polymorphonuclear chemotaxis which was evaluated by migration through boyden room. Study of the family showed atopic manifestations in several members, but with lower levels of IgE. The most characteristic abnormality of this syndrome is the according to considerable increase of IgE. The deficit in polymorphonuclear chemotaxis may vary according to time and even become normal. The prognosis over long periods remains to be determined.
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