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  • 1
    Electronic Resource
    Electronic Resource
    Salivary excretion of endogenous proteins in nephrotic syndrome in children (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 411-414 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Nephrotic syndrome ; Size selectivity ; Charge selectivity ; Saliva
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Size and charge selectivity of capillary permeability in the salivary glands of nephrotic children were investigated by measuring salivary excretion of endogenous plasma proteins of different size and charge. We examined 10 children with steroid-sensitive nephrotic syndrome (SSNS) in relapse and subsequent remission, 11 with steroid-resistant nephrotic syndrome, and 11 healthy children (controls). Albumin [mol. wt. 66 kilodaltons (kDa), isoelectric point (pI) 4.9] was measured by radioimmunoassay, transferrin (mol. wt. 77 kDa, pI 5.9) and immunoglobulins IgG1 (mol. wt. 150 kDa, pI 7–9) and IgG4 (mol. wt. 150 kDa, pI 〈6) by enzyme-linked immunoabsorbent assay. In saliva, no significant differences were found between the four groups of children for any of the four proteins. Also, the saliva/plasma ratios of the four proteins were not different among the four groups. From these data, we conclude that in subjects with SSNS in relapse, neither size nor charge selectivity of salivary gland capillary permeability are affected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050306
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  • 2
    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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  • 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
    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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  • 5
    Electronic Resource
    Electronic Resource
    Variability of urine albumin excretion in normal and diabetic children (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 414-419 
    Details
    ISSN: 1432-198X
    Keywords: Insulin-dependent diabetes mellitus ; Children ; Urine albumin excretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The variability of urine albumin excretion (UAE) was studied in normal and diabetic children and, in addition, the best method of expressing the data was investigated. In 39 timed overnight urine samples from diabetic children, the urine albumin creatinine clearance ratio (CA/CC) was compared with the urine albumin creatinine concentration ratio (UA/UC), the urine albumin excretion rate (UAER) and the urine albumin concentration (UA). UA/UC predicted CA/CC (r=0.95) better than either UAER (r=0.83,P〈0.02) or UA (r=0.90), 0.1〉P〉0.05). The within-individual and the between-individual variability in overnight UA/UC in 171 urine samples from 73 normal children was compared with that of 406 urine samples from 119 diabetic children, using a “random effects type 2 nested analysis of variance” model. Geometric mean (range) UA/UC (mg/mmol) in diabetic children, 0.55 (0.04–6.90), was greater than in normal children, 0.33 (0.05–2.10,P〈0.01), and 18% of diabetics had a value of UA/UC above the normal range. Within-individual variance was the same in normals (0.12) and diabetics (0.12), but between-individual variance in diabetics (0.18) was much greater than in normals (0.03). These data show that within-individual observations for both normals and diabetics are highly but equally variable. Furthermore, from these data, it is possible to infer that a minimum of five estimations are necessary per individual to estimate the true mean value of urine albumin excretion with reasonable confidence.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00850218
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  • 6
    Electronic Resource
    Electronic Resource
    Tubular proteinuria in reflux nephropathy: post ureteric re-implantation (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 559-563 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Reflux nephropathy   ;   Primary vesico-ureteric reflux   ;   Ureteric re-implantation   ;   Tubular proteinuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We studied urine protein excretion in 55 adults with reflux nephropathy (median age 26.9 years) who had had normal blood pressure, renal function and ureteric re-implantation in childhood. Urine retinol binding protein (RBP), N-acetyl-β-D-glucosaminidase (NAG), albumin, bacteriuria, systolic blood pressure, glomerular filtration rate (GFR), peripheral plasma renin activity (PRA) and the degree of renal scarring were measured in each subject; 20 had bilateral and 35 unilateral renal scarring; 5 were hypertensive and none were in renal failure. Urinary NAG and RBP excretions were significantly greater in the study group than in 34 healthy controls (median age 29.7 years). Within the study group, NAG excretion significantly correlated with PRA (P = 0.02). RBP excretion correlated with PRA, systolic blood pressure and the laterality (bilateral vs. unilateral) of scarring (P〈0.01). Urinary albumin excretion correlated with systolic blood pressure (P = 0.03). We conclude that increased urinary protein, especially NAG and RBP excretion, occur late after ureteric re-implantation in reflux nephropathy independent of GFR. Its association with PRA supports the concept of segmental perfusion and filtration as an important mechanism that may explain the above findings.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050160
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  • 7
    Electronic Resource
    Electronic Resource
    Charge and size selectivity of proteinuria in children with idiopathic nephrotic syndrome (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 404-410 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Charge selectivity   ;   Nephrotic syndrome   ;   Proteinuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract.  Experimental studies have pointed to charge selectivity as an important determinant of glomerular permeability to macromolecules. Loss of glomerular basement membrane (GBM) polyanion has been proposed as a cause of the selective proteinuria in minimal change nephrotic syndrome (MCNS). However, the presence of less-anionic albumin in urine than plasma from MCNS and focal and segmental glomerulosclerosis (FSGS) patients has been interpreted both as evidence for partial maintenance of charge selectivity and for involvement of other pathogenic mechanisms. The exact role of charge selectivity in the pathogenesis of nephrotic proteinuria remains controversial. We have examined the clearance of endogenous proteins of differing size and charge in children with idiopathic nephrotic syndrome (NS). Chromatofocusing was used to determine the isoelectric points (pIs) of albumins in paired plasma and urine samples from patients with FSGS (n = 6) and MCNS (n = 6). Charge selectivity was assessed by comparing the pIs of the fractions with the highest albumin concentration (modal pI) in plasma and urine. The difference between the modal pIs was defined as the delta modal pI. Charge selectivity was also assessed from the albumin/transferrin and IgG4/IgG1 clearance ratios; size selectivity from the IgG1/albumin and IgG1/transferrin as well as the IgG4/albumin and IgG4/transferrin clearances. In children with FSGS, the mean (± SD) delta modal pI was  – 0.05 ± 0.16, and in MCNS  – 0.05 ± 0.11. Neither value differed significantly from zero. The albumin/transferrin clearance ratio showed no significant difference between FSGS and MCNS, but the IgG4/IgG1 clearance ratio was significantly higher in MCNS (P〈0.05). Size selectivity was significantly reduced in FSGS compared with MCNS (for IgG1/transferrin P〈0.01 and for IgG1/albumin P〈0.05). For IgG4/transferrin and IgG4/albumin, P was 〈0.05. In conclusion, there was no evidence for residual charge selectivity in idiopathic NS associated with either MCNS or FSGS during nephrotic-range proteinuria. There was a significant loss of GBM size selectivity in children with FSGS with heavy proteinuria compared with children with MCNS with heavy proteinuria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050305
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  • 8
    Electronic Resource
    Electronic Resource
    Diagnostic value of urinary retinol-binding protein in childhood nephrotic syndrome (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 643-647 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Nephrotic syndrome ; Minimal change nephrotic syndrome ; Focal segmental glomerulosclerosis ; N-acetyl-β-d-glucosaminidase ; Retinol-binding protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Urinary excretion of N-acetyl-β-d-glucosaminidase (NAG) and retinol-binding protein (RBP), sensitive markers of renal tubular damage and dysfunction respectively, were evaluated in paired remission and relapse urine samples from 16 patients (median age 12 years), with minimal change nephrotic syndrome (MCNS), in single samples from 5 nephrotic patients (median age 12 years) with focal segmental glomerulosclerosis (FSGS) and in 183 normal controls aged 2–16 years. The NAG and RBP data were expressed as a ratio over urinary creatinine (Cr). The NAG/Cr and RBP/Cr geometric means (ranges) for normal subjects were 11.1 (3.4–35.5) μmol 2-methoxy-4-(2"-nitrovinyl)-phenol (MNP)/h per mmol and 3.1 (0.3–38.8) μg/mmol, respectively. The NAG/Cr data revealed a weak negative correlation with age in normal children, whereas RBP/Cr was independent of age. RBP/Cr and NAG/Cr in MCNS in remission were the same as in controls. In MCNS in relapse, NAG/Cr was significantly elevated (P=〈0.001), while in FSGS both RBP/Cr and NAG/Cr were significantly raised (P=〈0.001 and P〈0.008, respectively). These findings suggest that elevated NAG/Cr may be an indicator of relapse in both MCNS and FSGS and elevated RBP/Cr may allow differentiation between the two.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050519
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  • 9
    Electronic Resource
    Electronic Resource
    Neutrophil activation in the haemolytic uraemic syndrome: free and complexed elastase in plasma (1992)
    Springer
    Pediatric nephrology 6 (1992), S. 50-53 
    Details
    ISSN: 1432-198X
    Keywords: Haemolytic uraemic syndrome ; Neutrophils ; Free and complexed elastase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract There is evidence of neutrophil involvement in the pathogenesis of the haemolytic uraemic syndrome (HUS), and neutrophil release products are thought to cause endothelial cell damage. Elastase is the major lysosmal proteinase liberated by activated neutrophils. In this study we measured both free and complexed elastase. No free elastase activity could be detected in the plasma of patients with diarrhoea-associated (D+) HUS using a specific substrate. However, there was a marked increase in α1-antitrypsin (α1-AT) complexed elastase as measured by a newly developed enzyme-linked immunosorbent assay not only in D+ HUS, but also in non-diarrhoea-associated (D-) HUS. This finding is independent of either a high polymorphonuclear leucocyte count or renal failure. This increase in bound elastase together with our sequential data which demonstrate raised α1-AT complexed elastase levels early in the disease process further support the theory that neutrophil activation is one of the key events in the pathophysiology of this disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856833
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  • 10
    Electronic Resource
    Electronic Resource
    Autosomal recessive polycystic kidney disease (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 43-49 
    Details
    ISSN: 1432-198X
    Keywords: Polycystic kidneys ; Congenital hepatic fibrosis ; Hypertension ; Hyponatraemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical features of 55 cases of autosomal recessive polycystic kidney disease (ARPCKD) have been reviewed. Each had evidence of ARPCKD. The outcomes of 87% were known; 24 had died. Twenty-four of 31 were seen between 1980 and 1986; 7 could not be traced. Forty-five percent presented under 1 month; 38% between 1 month and 1 year; and 9 cases over 1 year. Hyponatraemia occurred in 15 out of 19 aged less than 3 months; hypertension occurred in 65%; splenomegaly in 47% of those surviving more than 3 months. Portocaval shunts were done in 5 aged 2–12 years. Thirteen died of renal failure, 6 under 1 year, and 7 between 1 year and 13 years. Life-table survival rates calculated from birth revealed that 86% were alive at 3 months, 79% at 1 year, 51% at 10 years, and 46% at 15 years. Calculations based on patients who survived to 1 year of age showed that 82% were alive at 10 years and 79% at 15 years. These results reveal an improved prognosis for a condition once assumed to be fatal.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00859625
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