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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 69 (1991), S. 536-551 
    ISSN: 1432-1440
    Keywords: Vasculitis ; Polyarteritis ; Wegener's granuloma ; Anti-neutrophil Cytoplasmic antibody (ANCA) ; Schonlein-Henoch purpura ; Glomerulonephritis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary After a long period during which few new data on the vasculitides emerged, the past decade has seen a real explosion of data on the subject. This began with basic clinical descriptions and the deliniation of long term outcome as survival improved to a major extent under the influence of improved treatment regimes. In parallel, better understanding of the immunopathology has emerged. Of particular interest have been descriptions of autoantibody systems in vasculitis which seem to be specific to vasculitis and therefore diagnostically useful: the antineutrophil cytoplasmic antibodies (ANCA). Whether or not these are pathogenetically significant as well as useful remains a matter for debate. In parallel, anti endothelial cell antibodies have been described, but their role (if any), in pathogenesis remains equally obscure. There are some suggestions that vasculitis is becoming more common, but increased awareness and the availability of ANCA have undoubtedly increased awareness of the subject.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 4 (1990), S. 193-198 
    ISSN: 1432-198X
    Keywords: Membranous nephropathy ; Proteinuria ; Nephrotic syndrome ; Prednisone ; Prednisolone ; Hepatitis B ; Chlorambucil
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Membranous nephropathy is predominantly a disease of middle-aged and elderly individuals, and is thus rather an uncommon finding in proteinuric and nephrotic children. In children, it differs in several important respects from the disease as seen in adults: an apparent associated cause is more common, macroscopic haematuria is seen quite frequently, a relapsing course is more often noted, renal venous thrombosis is not found and evolution into renal failure is the exception. Nevertheless, a proportion of children with membranous nephropathydo evolve into renal failure, and their management is discussed with particular reference to recent papers on the treatment of membranous nephropathy in adults. An aggressive search for associated disease is worthwhile in children, and one should wait to see what the evolution or proteinuria and renal function may be. If a progressive course becomes evident, then a trial of treatment with corticosteroids is worthwhile, but if this is ineffective then a more aggressive approach involving the use of alkylating agents may be justified. It remains undetermined what the best regime in children and adolescents may be.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 2 (1988), S. 490-497 
    ISSN: 1432-198X
    Keywords: Vasculitis ; Wegener's granuloma ; Polyarteritis ; Antileucocyte cytoplasm antibodies (ANCA) ; Immune complexes ; Cellmediated immunity ; Immunosuppressive treatment ; Plasma exchange
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Vasculitis is rare in childhood, apart from Henoch-Schönlein purpura, and paediatric nephrologists see few cases of Wegener's granuloma or microscopic polyarteritis. In contrast, this pattern is reversed in adults. Recently much information about the presentation and longer-term course of both these conditions has been described in adults, and more aggressive treatment of severe cases with renal failure has improved the outlook from a 5-year survival of about 5%–80% or more. In parallel, it has become evident that the abundant immune complexes in the circulation of patients with vasculitis may play little or no role in the pathogenesis of the vasculitic lesions. Current interest centres both on the possible role of cellmediated immunity and the recent finding of antibodies which react with antigens in the cytoplasm of leucocytes. These antibodies seem to be of clinical use in defining patients with vasculitis, especially when only the kidney is involved at a clinical level. The nature of this leucocyte antigen (or antigens) and the possible role of antibodies directed against them in the pathogenesis of the disease are subjects of current work in many loboratories.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-198X
    Keywords: Renal transplantation ; Recurrent glomerulonephritis ; Focal segmental glomerulosclerosis ; Cyclosporin A ; Nephrotic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-nine allografts were placed in 43 patients with renal failure from focal segmental glomerulosclerosis (FSGS): 27 allografts were put into 16 children aged less than 15 years, and 32 allografts into 27 adolescents and adults. Recurrence of FSGS was noted histologically in 13 allografts, 10 in 8 children and 3 in adults. None of the 9 children and 24 adults who never developed an allograft nephrotic syndrome showed FSGS in their allograft biopsies. The age of onset was a strong risk factor for recurrence: recurrent FSGS developed in 8 of 16 children (50%) but only in 11% of adolescents and adults (3 of 27 patients). Although the time from apparent onset to renal replacement treatment was shorter in those with recurrence than those without in the children, there was no difference in the time spent on dialysis prior to transplantation. Mesangial prominence was observed in the original biopsy in 12 of 13 patients with recurrence, and recurrence rate was similar in living and cadaver donor allografts; class I MHC matching was similar in those with and without recurrence. Three allografts treated with cyclosporin A as well as 9 with azathioprine showed recurrence. Of 9 second or subsequent allografts placed in those with recurrence in the first allograft, only 3 showed further recurence. rence. In 3 re-grafted after 13, 11 and 5 years, normal function was seen.
    Type of Medium: Electronic Resource
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