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  • 1
    Electronic Resource
    Electronic Resource
    Long term renal outcome of childhood haemolytic uraemic syndrome (1992)
    Springer
    Pediatric nephrology 6 (1992), S. 275-275 
    Details
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00878373
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  • 2
    Electronic Resource
    Electronic Resource
    Anti-neutrophil cytoplasmic antibodies and anti-endothelial cell antibodies (1991)
    Springer
    Pediatric nephrology 5 (1991), S. 256-259 
    Details
    ISSN: 1432-198X
    Keywords: Anti-neutrophil cytosolic antibody ; Anti-endothelial cell antibody ; Vasculitis ; Wegener's granulomatosis ; Kawasaki disease ; Systemic lupus crythematosus ; Haemolytic uraemic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Anti-netrophil cytosolic antibodies (ANCA) and anti-endothelial cell antibodies (AECA) have been identified in a wide variety of disorders, but their pathophysiological role remains unclear. ANCA appear to be particularly associated with various forms of vasculitis including Wegener's granulomatosis, Kawasaki disease and microscopic polyartiritis. Cytoplasmic staining (cANCA) on indirect immunofluorescence is associated with extrarenal disease and a perinuclear pattern (pANCA) with renal limited disease. The cANCA antigen appears to be proteinase 3 and that for pANCA myeloperoxidase. AECA have been detected in systemic lupus erythematosus, scleroderma and dermatomyositis but are also found in systemic vasculiitis. Kawasaki disease, haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura and renal allograft recipients at the time of rejection. Their presence appears to be correlated with disease activity and they may be directed against epitopes on as yet unidentified infective agents that precipitate some of the diseases in which they are found that cross-react with antigenic sites exposed on endothelial cells. Measurement of these antibodies has a diagnostic role, facilitates monitoring of disease activity and may prove valuable in understanding the pathogenesis of the diseases in which they are found.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01095967
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  • 3
    Electronic Resource
    Electronic Resource
    Lymphocyte subpopulations, interleukin-2 and interleukin-2 receptor expression in childhood nephrotic syndrome (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 135-139 
    Details
    ISSN: 1432-198X
    Keywords: Nephrotic syndrome ; Lymphocyte ; Interleukin-2
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abnormal T lymphocyte function and reduced interleukin-2 (IL-2) production have been implicated in the pathogenesis of the nephrotic syndrome (NS). We investigated: (1) lymphocyte subpopulations and expression of IL-2 receptor (IL-2R) on T cells using two-colour flow cytometry, (2) serum IL-2 and (3) the soluble component of IL-2R (sIL-2R) in serum, using enzyme-linked immunosorbent assay, in 38 children with NS. All children, except those in remission, had marked proteinuria. They were divided into groups according to renal pathology: (1) steroid-sensitive NS (SSNS) not receiving prednisolone therapy, (2) SSNS on prednisolone, (3) focal segmental glomerulosclerosis (FSGS), (4) SSNS in remission and not receiving prednisolone therapy, (5) congenital NS (CNS). Results were compared with 26 age-matched controls. Total T lymphocytes (CD3) were reduced in groups 1 and 2; CD4 count was reduced in groups 1–4; CD8 count increased in groups 2 and 3; CD8 and CD19 (B lymphocytes) were significantly reduced in group 5. Increased IL-2R expression (CD25) on CD4 lymphocytes was noted in groups 1, 2 and 3 implying activation of these cells. In patients with SSNS, increased serum sIL-2R was recorded during relapse (1,273±497 U/l vs. 913±401 U/l in remission,P〈0.005) but free serum IL-2 was not detectable at any stage. The specific alterations in lymphocyte subpopulations in SSNS and FSGS would imply an involvement of the immune system distinct from that in CNS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00865458
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  • 4
    Electronic Resource
    Electronic Resource
    Autosomal recessive polycystic kidney disease: long-term outcome of neonatal survivors (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 302-306 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Congenital hepatic fibrosis   ;   Hypertension   ;   Portal hypertension   ;   End-stage renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract.  Autosomal recessive polycystic kidney disease causes renal and hepatic dysfunction in childhood. We describe the clinical outcome of 52 children with this diagnosis born between 1950 and 1993. Currently 23 are alive, 24 dead and 5 have been lost to follow-up; 1 has been dialysed and 7 transplanted. Life-table analysis of the patients surviving the 1st month of life revealed an actuarial renal survival of 86% at 1 year and 67% at 15 years. The probability of requiring anti-hypertensive treatment was 39% at 1 year and 60% at 15 years of age. Bleeding from gastro-oesophageal varices occurred in 8 patients at a mean age of 12.5 years, and was preceded by haematological evidence of hypersplenism in 6 of them. The study indicates a relatively good prognosis for patients with this condition who survive the neonatal period and emphasises the importance of early detection and appropriate management of systemic and portal hypertension.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050281
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  • 5
    Electronic Resource
    Electronic Resource
    Vincristine and focal segmental sclerosis: do we need a multicentre trial? (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 284-289 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Focal segmental glomerulosclerosis ; Vincristine therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Over the last 10 years, eight children have received vincristine for the treatment of steroid- and cyclophosphamide-resistant nephrotic syndrome at Great Ormond Street Hospital for Children, London. We present our experience of these eight cases and put forward a case for reassessing the effectiveness of vincristine in this disorder. In our series, two children treated with vincristine achieved complete remission with preserved renal function, including relapses in one. Both had primary steroid- and cyclophosphamide-resistant focal segmental glomerulo sclerosis (FSGS). Of the other cases, four also had primary FSGS, one familial FSGS and one mesangioproliferative glomerulonephritis. We discuss in general the pros and cons of vincristine therapy in nephrotic syndrome versus the cytotoxic agents that are currently used and the differences in clinical features among the responders and non-responders in this small group. In addition, we explore why this may have occurred and summarise the literature over the last 25 years, where vincristine appeared to have been beneficial, especially in secondary forms of nephrotic syndrome associated with malignancy. We conclude that vincristine therapy warrants re-examination as it could be a valuable alternative therapeutic agent in some cases of FSGS with relatively minor side effects.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050454
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  • 6
    Electronic Resource
    Electronic Resource
    Serum intact parathyroid hormone and ionised calcium concentration in children with renal insufficiency (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 561-565 
    Details
    ISSN: 1432-198X
    Keywords: Parathyroid hormones ; Kidney failure, chronic ; Ionised calcium ; Hyperparathyroidism, secondary ; Renal osteodystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report our experience of the use of an immunoradiometric assay for intact parathyroid hormone (i-PTH) and the measurement of plasma ionised calcium concentration (PCa2+) in 73 children with chronic renal insufficiency (CRI); plasma creatinine concentration (PCr) 52–856 μmol/l. There was a poor correlation between i-PTH and PCr (r=0.10,n=552) compared with that for C-terminal PTH and PCr (r=0.60,n=248), suggesting that the i-PTH assay is independent of renal function in this group of treated children. A clear response of i-PTH to a low total plasma Ca (tPCa) and PCa2+ was observed. There was a significant positive correlation between both tPCa and PCa2+ (r=0.50,n=389) and the fraction of Ca2+ (the fraction of tCa which was ionised) and PCa2+ (r=0.50,n=389). The finding of a low or normal PCa2+ with a low calculated fraction of Ca2+ was frequently observed, i. e. the measured tPCa was unexpectedly high, suggesting complexing of Ca2+ by accumulated anions in CRI. There was a poor relationship between the plasma albumin concentration and both bound plus complexed Ca (tPCa minus PCa2+) and the fraction of Ca2+ (r=0.15 and −0.17, respectively). The positive predictive value for a raised i-PTH of a tubular reabsorbtion of phosphate of less than 80% was 0.87, and of an alkaline phosphatase greater than 800 U/l was 0.37. We conclude that the immunoradiometric assay for i-PTH should replace indirect assessments of secondary hyperparathyroidism, and that measurement of the Ca2+ concentration is necessary to evaluate the true Ca status in children with CRI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858126
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  • 7
    Electronic Resource
    Electronic Resource
    Effect of cyclosporin A on glomerular filtration rate in children with minimal change nephrotic syndrome (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 404-407 
    Details
    ISSN: 1432-198X
    Keywords: Cyclosporin A ; Glomerular filtration rate ; Nephrotic syndrome ; Nephrotoxicity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cyclosporin A (CyA) is now commonly used in the management of children with steroid-dependent nephrotic syndrome. In order to assess nephrotoxicity related to CyA therapy, we measured glomerular filtration rate (GFR) on 123 occasions in 24 children with minimal change nephrotic syndrome receiving CyA. GFR was estimated from the plasma clearance of51chromium-EDTA every 3 months during CyA therapy of up to 27 months duration. There was a significant reduction in GFR after 3 months of CyA therapy [118±33 (SD) to 93±24 ml/min per 1.73 m2] but no further fall thereafter, although the reduction in GFR was sustained for the duration of CyA therapy. This reduction in GFR appeared to be reversible upon cessation of CyA, but careful monitoring of renal function is necessary in such patients to prevent the development of longer term nephrotoxic sequelae.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856512
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  • 8
    Electronic Resource
    Electronic Resource
    Investigation and management of hypertension in children (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 59-68 
    Details
    ISSN: 1432-198X
    Keywords: Hypertension ; Renin ; Radionuclide imaging ; Angiography ; Antihypertensives ; Revascularization ; Angioplasty
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The prevalence of hypertension in children is of the order of 1%–3%. Of these children, 10% will have severe hypertension and in the majority the increased blood pressure will be secondary to renal disease. Nephrologically orientated investigation is therefore important. The most helpful investigations are: peripheral plasmarenin activity,99mTc dimercaptosuccinic acid (DMSA) scan, main and segmental renal vein renin measurements and renal angiography plus123I metaiodobenzyl guanidine (MIBG) scan in suspected phaeochromocytoma. Drug treatment of accelerated hypertension is most successfully undertaken by intravenous labetalol or sodium nitroprusside; sustained moderate-to-severe hypertension by a beta-blocker plus a vasodilator or an angiotensin-converting enzyme inhibitor with or without a diuretic; mild hypertension by diet, diuretic and if necessary by more powerful hypotensives. Surgical treatment by revascularization or nephrectomy and percutaneous transluminal angioplasty can be successful. Utilizing the current range of investigational and therapeutic tools the aetiology and appropriate management of hypertension can be effectively and safely undertaken in the majority of hypertensive children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00866886
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  • 9
    Electronic Resource
    Electronic Resource
    The polymorphonuclear leucocyte count in childhood haemolytic uraemic syndrome (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 130-134 
    Details
    ISSN: 1432-198X
    Keywords: Haemolytic uraemic syndrome ; Polymorphonuclear leucocyte count
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Review of data from 79 children with the haemolytic uraemic syndrome (HUS) showed that the polymorphonuclear leucocyte (PMN) count at presentation in childhood HUS predicts outcome. Logistic regression analysis of several features at presentation identified only the PMN count and the presence of a diarrhoeal prodrome as having a significant effect on the outcome (P〈0.01 andP〈0.001 respectively). The geometric mean PMN count was significantly raised in 70 children who had typical HUS following a diarrhoeal prodrome (D+cases) compared with that of 9 children who had atypical disease without diarrhoea (D-cases) (t-test on log-transformed data,P〈0.005). Fifty-seven children with D+HUS who recovered completely had a significantly lower geometric mean PMN count than D+cases with a bad outcome (P〈0.001). Four of these patients, who died in the acute stage of the disease, had a significantly higher mean count than the rest of the D+patients (P〈0.001). Multiple regression analysis demonstrated that the PMN count in D+cases was not significantly influenced by haemoglobin concentration, platelet count, length of the prodrome, or the administration of antibiotics in the prodromal period. A high PMN count at presentation in D+HUS indicates a poor prognosis. The data emphasise the heterogeneity of HUS and suggest that PMN participate in the pathogenesis of the disorder in typical D + cases but not in atypical D- cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00852893
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  • 10
    Electronic Resource
    Electronic Resource
    Fanconi syndrome, ichthyosis, dysmorphism, jaundice and diarrhoea — a new syndrome (1990)
    Springer
    Pediatric nephrology 4 (1990), S. 308-313 
    Details
    ISSN: 1432-198X
    Keywords: Fanconi syndrome ; Ichthyosis ; Dysmorphism ; Jaundice ; Diarrhoea ; Grey platelet syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe six infants, from consanguineous marriages, with a new syndrome comprising the Fanconi syndrome, ichthyosis, musculoskeletal abnormalities, jaundice and diarrhoea. In addition two of the infants were found to have abnormal platelet morphology — the grey platelet syndrome. No evidence of a recognised metabolic disorder was found in any of the six infants, nor did they appear to be typical of any previously described syndromes. Their progress was poor: they required high fluid and bicarbonate intakes and all died by the age of 6 months of dehydration, acidosis and sepsis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00862505
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