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  • 1
    ISSN: 1432-198X
    Keywords: Key words: Juvenile gout ; Hyperuricaemia ; Familial renal disease ; Allopurinol ; Fractional urate clearance ; Dominant genetic disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We studied 34 apparently healthy children and 2 propositi from kindreds with familial juvenile hyperuricaemic nephropathy (FJHN) – a disorder characterised by early onset, hyperuricaemia, gout, familial renal disease and a similarly low urate clearance relative to glomerular filtration rate (GFR) [fractional excretion of uric acid (FEur) 5.1±1.6%] in young men and women. In addition to the propositi, 17 asymptomatic children were hyperuricaemic – mean plasma urate (368±30 μmol/l), twice that of controls (154±41 μmol/l). Eight of them had a normal GFR (〉80 ml/min per 1.73 m2), and 11 renal dysfunction, which was severe in 5. The FEur in the 14 hyperuricaemic children with a GFR 〉50 ml/min was 5.0±0.5% and in the 5 with a GFR ≤50 ml/min was still low (11.5±0.2%) compared with controls (18.4±5.1%). The 17 normouricaemic children (185±37 μmol/l) had a normal GFR (〉80 ml/min) and FEur (14.0±5.3%). The results highlight the dominant inheritance, absence of the usual child/adult difference in FEur in FJHN and presence of hyperuricaemia without renal disease in 42% of affected children, but not vice versa. Since early allopurinol treatment may retard progression to end-stage renal failure, screening of all relatives in FJHN kindreds is essential.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 9 (1995), S. 647-662 
    ISSN: 1432-198X
    Keywords: Continuous ambulatory peritoneal dialysis ; Peritonitis ; Peritoneal macrophages ; Lymphocytes ; Opsonins ; Complement ; Fibronectin ; Immunoglobulin ; Biofilm
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Continuous ambulatory peritoneal dialysis (CAPD) has come to be extensively used for the treatment of end-stage renal failure in children, and especially infants, such that now more than half of children on dialysis worldwide receive treatment by this means. Peritonitis, however, is commoner in children than in adults receiving treatment, and is a major source of morbidity and treatment failure in children started on CAPD. Only recently has the immunology of the normal peritoneum been studied extensively, with the need to assess the impact of the installation of large volumes of fluid into the peritoneal sac during dialysis. The main phagocytic defences of the peritoneum depend upon a unique set of macrophages which are present free in the peritoneal fluid but also in the submesothelium and in perivascular collections together with B lymphocytes in the submesothelial area. Both the number of macrophages per unit volume and the concentration of opsonic proteins, such as IgG, complement and fibronectin, are reduced to between only 1% and 5% when dialysis fluid is continuously present in the peritoneal sac. In addition, the fluids used for CAPD are toxic to both macrophages and to mesothelial cells. Thus minor degrees of contamination frequently lead to peritonitis and in addition the majority of patients have catheters inserted in their peritoneum which become colonised with organisms capable of producing exopolysaccharide (slime), which promotes adhesion of the organism to the plastic and protects them against phagocytic attack and the penetration of antibiotics. Thus the peritoneum is in a state of continual inflammation, as well as being a markedly more vulnerable site than the normal peritoneum to the entry of organisms. Whether clinical peritonitis appears in this state of chronic contamination probably depends on perturbation in the balance between host defences and the organism. WhilstStaphylococcus epidermidis is the commonest, cause of peritonitis,Staphylococcus aureus and Gram-negative organisms are much more serious and more frequently lead either to temporary catheter removal or discontinuation of dialysis altogether. This review describes, the peritoneal defences in relation to the genesis of peritonitis.
    Type of Medium: Electronic Resource
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