Publications by authors named "Noel L"

1. To determine whether treatment with octreotide, a somatostatin analogue, may diminish or prevent long-term diabetic renal hypertrophy and nephropathy, uninephrectomized streptozotocin-diabetic rats maintained under moderate glycaemic control (approximately 300 mg/dl) were treated with either placebo (n = 10 rat/group) or octreotide for 14 weeks. Uninephrectomized non-diabetic rats given either placebo or octreotide served as controls.

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Autoantibodies directed against polymorphonuclear neutrophils (PMN) have been observed in serum from patients with ulcerative colitis (UC), Crohn's disease (CD) and primary sclerosing cholangitis (PSC) using indirect immunofluorescence and fixed granulocyte ELISA. Our study demonstrates the presence in the serum of these patients of autoantibodies which bind to an azurophilic granule component distinct from proteinase 3, elastase and myeloperoxidase. These autoantibodies thus belong to the ANCA family, but their antigen specificity differs from the already characterized ANCA antigens.

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Sera from 273 French haemophiliacs who had received non viral inactivated concentrates, were tested for antibodies to HCV by first and second generation assays. Antibodies to HCV were detected in 66% of the sera by the first generation assays (anti-C 100-3) reaching 100% by the second generation assays. None of the 53 patients only exposed to solvent-detergent treated Factor VIII or IX concentrates had HCV seroconversion.

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The specific antigens to antineutrophil cytoplasmic antibodies (ANCA) and their relations with renal diseases begin to be well-known. In patients with systemic vasculitis, two major antigens have been recognized: proteinase 3 and myoloperoxydase. These enzymes are located in the azurophilic granules of neutrophils and migrate to the surface of these cells when they are activated.

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In a prospective study 163 patients aged 17 years or less admitted to a children's hospital between April 1985 and December 1990 with traumatic hyphema were treated with tranexamic acid, 25 mg/kg given orally every 8 hours to a maximum of 1500 mg every 8 hours for 5 days. Secondary hemorrhage occurred in 5 patients (3%), none of whom had more than one rebleeding episode. In contrast, 24 (8%) of 316 patients aged 17 years or less admitted to the same hospital between January 1977 and March 1985 with traumatic hyphema who were not treated with antifibrinolytics had a secondary hemorrhage, several more than once, giving a rebleeding rate of 33/316 (10%).

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A National Register of transfusion-transmitted infections was opened by the French Society of Blood Transfusion on 1 October, 1986. Out of 54 initially reported cases of HIV-infection, allegedly transmitted by blood components, further investigation could be completed in 33 cases. The transfusional origin of contamination was considered as established or probable in 28/33 cases, either because a potentially infectious unit was identified among those transfused to the recipient (23/28), or because the recipient was known to be seronegative before transfusion (5/28), or both (10/28).

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ANCA positive sera, detected by the standard immunofluorescence method, derived from 37 patients with vasculitis were studied using formalin-acetone fixed chronic myelocytic leukemia cells (CML). All 37 sera were positive on CML cell smears. Furthermore formalin-actone fixation selectively impaired antinuclear antibody binding without reducing ANCA staining and thus facilitated differentiation of these autoantibodies which is often difficult with the standard immunofluorescence method.

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Two unrelated mentally retarded patients were found to have an interstitial deletion of 18q12. They were a 2-year-old, short, macrocephalic and autistic girl, and a 5-year-old boy. Six other liveborn patients with comparable deletion have been so far identified.

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Antineutrophil cytoplasmic autoantibodies have been recently reported in sera from patients with inflammatory bowel disease. We report our experience based on 90 patients with ulcerative colitis, 148 patients with Crohn's disease, and 60 controls. Determination of antineutrophil cytoplasmic autoantibodies was performed by the indirect immunofluorescence technique on ethanol fixed leucocytes.

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The frequency, isotype, and specificity of antineutrophil cytoplasmic antibodies were investigated in a cross-sectional study of 100 patients with IgA nephropathy and 30 children with Henoch-Schönlein purpura. Two of the patients with IgA nephropathy had high titres of antineutrophil cytoplasmic antibodies which were of IgG isotype and confirmed as antimyeloperoxidase antibodies in solid-phase ELISA and inhibition experiments. Antineutrophil cytoplasmic antibodies were not detected in the children with Henoch-Schönlein purpura and none of the patients in either group had IgA antineutrophil cytoplasmic antibodies.

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The Human Monitoring Laboratory (HML) has used the International Commission on Radiological Protection's Report on Reference Man and Canadian anthropomorphic data as guidance to design and construct a family of phantoms corresponding to Reference Man (PM), Reference Woman (PF), Reference Ten-Year-Old (P10), Reference Four-Year-Old (P4), Ninety-five Percentile Man (PM95), and Five Percentile Man (PM5). The PM series also has an accessory chest section (PMacc) to better simulate lung depositions. The phantoms are constructed from high-density polyethylene and fitted with end-recessed filling caps to minimize leakage problems.

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Necrotizing and crescentic glomerulonephritis without immunoglobulin deposits (also called pauci-immune glomerulonephritis) is the cause of approximately 50 percent of acute renal failures of glomerular origin. Our study, based on 40 case-records selected on histological criteria, and on data from the literature, shows that in most cases, if not all, this type of glomerulonephritis is part of a wider systemic vasculitis which predominantly affects the glomerular capillaries. Anti-neutrophil cytoplasm antibodies are detected in three-quarters of the cases, with a specific distribution that varies according to the clinical features.

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Graft antiglomerular basement membrane nephritis in patients with Alport's syndrome (AS) is a unique complication related to the glomerular basement membrane (GBM) abnormality characteristic of the disease. Its prevalence and clinical significance however remain unknown. We used strict criteria of AS to select 30 patients (26 men, 4 women), aged 17 to 44 years (m: 27) in whom 35 grafts (30 first, 5 second) had been performed at our center between 1968 and 1988.

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Ocular motor apraxia is characterized by a deficiency in the generation of voluntary saccadic eye movements despite the presence of spontaneous saccades. We report two cases of isolated ocular motor apraxia, both in children, that developed following cardiac surgery. Electro-oculography was done in one case and showed slow saccades and decreased pursuit gain.

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The antigenic specificity and clinical distribution of the antineutrophil cytoplasmic antibodies (ANCA) in kidney diseases have recently been extensively studied. In patients with systemic vasculitis, the great predominance of two major ANCA antigens, proteinase 3 (PR3) and myeloperoxidase (MPO), is now established. PR3 and MPO are colocalized in the azurophilic granules of neutrophils and translocated to the cell surface during activation, and thus are able to interact with autoantibodies after neutrophil preactivation.

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