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  • 1
    Electronic Resource
    Electronic Resource
    Mapping of the gene for autosomal recessive polycystic kidney disease (ARPKD) to chromosome 6p21–cen (1994)
    [s.l.] : Nature Publishing Group
    Nature genetics 7 (1994), S. 429-432 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Autosomal recessive polycystic kidney disease (ARPKD) is one of the major hereditary nephropathies in children predominantly presenting in early childhood. The clinical picture is variable but there is a fatal outcome in many cases. We have performed linkage analysis in 16 ARPKD families and ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/ng0794-429
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  • 2
    Electronic Resource
    Electronic Resource
    Low-dose cyclosporin A therapy in cadaver renal transplantation in children (1989)
    Springer
    Transplant international 2 (1989), S. 203-208 
    Details
    ISSN: 1432-2277
    Keywords: Pediatric renal transplantation ; Cyclosporin, low-dose, in children ; Growth, cyclosporin, in kidney transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-one pediatric patients undergoing a first cadaveric kidney transplantation were followed for at least 2 years after grafting. They were divided into two groups: those treated with methylprednisolone plus azathioprine (AZA) and those treated with methylprednisolone plus low-dose cyclosporin A (CyA; median dose 109 mg/m2 per day ≙ 3.4 mg/kg per day after 1 year). The steroid dosage given was significantly lower in the second group. The 4-year graft survival rate was 68% for the AZA group and 78% for the CyA group. Renal function did not differ significantly in the two groups; after 1, 2, and 3 years, the median 24-h creatinine clearance was 79, 69, and 51 ml/min/1.73 m2, respectively, for the AZA group and 78, 63, and 68 ml/min/1.73 m2, respectively, for the CyA group. Linear growth was similar in the two groups. We conclude that in pediatric patients the results of low-dose CyA immunosuppression do not differ significantly from those obtained with AZA in terms of graft survival, renal function, or growth.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02414535
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  • 3
    Electronic Resource
    Electronic Resource
    Effect of ramipril on ambulatory blood pressure and albuminuria in renal hypertension (2000)
    Springer
    Pediatric nephrology 15 (2000), S. 113-118 
    Details
    ISSN: 1432-198X
    Keywords: Key words Hypertension ; ACE inhibitors ; Chronic renal insufficiency ; Ambulatory blood pressure ; Proteinuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Inhibition of the angiotensin-converting enzyme (ACE) exerts a renoprotective effect in adult patients with chronic kidney disease. We evaluated prospectively changes in blood pressure (BP), protein excretion and renal function after administration of the long-acting ACE inhibitor ramipril as monotherapy during 6 months in 14 moderately hypertensive children aged 5–18 years with various nephropathies. Four patients initially had a decreased glomerular filtration rate (GFR below 60 ml/min/1.73 m2). BP was evaluated by ambulatory 24-h monitoring. After 2 weeks of treatment by oral ramipril (1.5 mg/m2 once daily), mean values of systolic and diastolic 24-h ambulatory BP fell by more than 5 mmHg in nine patients. In eight patients the dose was doubled. At the end of the study systolic BP was below the 95th percentile in 9 and diastolic BP in 13 patients. The initially reduced nocturnal dip increased significantly. Of 11 patients with an increased albumin excretion (median 1.3 g/g creatinine), 6 responded to ramipril by a median reduction of 78% (range 24–83%), whilst in 5 albuminuria increased (median +19%). GFR was well preserved and no other adverse effects from the drug were noted. The study demonstrates that ramipril is an efficacious antihypertensive agent in children with renal hypertension. It is well tolerated, even in mild renal insufficiency. In addition, the drug has a persistent antiproteinuric action in about half of the patients contributing to conserve renal function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670000422
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  • 4
    Electronic Resource
    Electronic Resource
    Atrial natriuretic peptide and sodium homeostasis in chronic renal failure (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 397-400 
    Details
    ISSN: 1432-198X
    Keywords: Aldosterone ; Atrial natriuretic peptide ; Chronic renal failure ; Dopamine ; Noradrenaline ; Sodium homeostasis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to evaluate the possible role of vasoactive hormones in the mechanism of exaggerated sodium loss due to reduced renal mass we measured plasma concentration of atrial natriuretic peptide (ANP), aldosterone, plasma renin activity (PRA), plasma noradrenaline, and dopamine, in 12 children with advanced chronic renal failure (mean CIn17.8-2.6,x± SEM, CPAH93.5±17 ml/min per 1.73 m2, FENa7.0±0.95%). No patient had clinical signs of volume overload. Plasma concentrations of ANP were not significantly different from those of healthy agematched controls (29.2±7.2 vs 23.2±3.1 fmol/ml) and did not correlate with urinary sodium excretion. Plasma concentrations of aldosterone, PRA and noradrenaline, were also within the physiological range, while plasma dopamine levels were elevated (260±36 vs 98±11 pg/ml, 〈0.001). Our data do not support the notion that ANP or the renin-aldosterone axis play a major role in the adaptation of remaining nephrons to maintain long-term sodium balance in normotensive children with chronic renal failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00850214
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  • 5
    Electronic Resource
    Electronic Resource
    Body growth in urinary tract malformations (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 151-155 
    Details
    ISSN: 1432-198X
    Keywords: Body growth ; Urinary tract malformations ; Hydronephrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Body height and height velocity were analysed in 54 children with obstructive urinary tract malformations over a mean period of 8.7 years, using new auxological methods. At the time of diagnosis, 9% of patients had a height of more than 2 standard deviations below the normal mean. Mean relative height changed significantly from the first to the last observation, the standard deviation score (SDS) increasing from −0.16 to +0.36 in patients with hydronephrosis compared with normal children (P〈0.05) and from −0.63 to +0.02 SDS in those without hydronephrosis (P〈0.005). The pathogenesis of the described growth disturbance is not clear. Stepwise multiple regression analysis pointed to a possible link between the duration of antibiotic treatment and the recovery of growth capacity, but improved growth could not clearly be attributed to any medical or surgical treatment. The synchronized average growth velocity curve was similar to that of healthy children and showed a normal pubertal spurt. Final height and target height calculated from parents' height differed only slightly from that of the normal population.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00864383
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  • 6
    Electronic Resource
    Electronic Resource
    Haemorheological changes in uraemic children in response to erythropoietin treatment (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 777-777 
    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/BF01213358
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  • 7
    Electronic Resource
    Electronic Resource
    In vivo prostanoid formation during acute renal allograft rejection (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 6-6 
    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/BF00868246
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  • 8
    Electronic Resource
    Electronic Resource
    The paediatric registry of the European Dialysis and Transplant Association: 20 years' experience (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 758-768 
    Details
    ISSN: 1432-198X
    Keywords: European Dialysis and Transplant Association ; Dialysis and transplantation of children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The first publication of the paediatric registry of the European Dialysis and Transplant Association appeared in 1971. Since then nearly 50 further articles have appeared and this paper provides a full bibliography as well as tracing the changes in the provision of care, methods and results of treatment of end-stage renal disease in childhood over the last 20 years.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01213347
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  • 9
    Electronic Resource
    Electronic Resource
    Urinary excretion of glomerular basement membrane-related peptides in children with renal disorders (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 428-435 
    Details
    ISSN: 1432-198X
    Keywords: Glomerular basement membrane ; Type IV collagen ; Alport's syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Urinary excretion of glomerular basement membrane (GBM)-related peptides was analysed in 72 patients with a variety of renal diseases by immunoblotting using polyclonal antibodies against either collagenase or pepsin digests of human GBM. The specificity of the antibodies was verified by elution of antibodies bound to urinary GBM-related peptides on nitrocellulose blots and demonstration of reactivity of the eluted antibodies with the respective GBM digests. Furthermore, six mice immunized with urinary GBM-related peptides all developed focal linear deposits of mouse IgG along their GBM, linear and mesangial deposits of C3 in the glomeruli and serum antibodies reactive with human GBM. Monoclonal antibodies against urinary GBM-related peptides of one of the mice reacted with different peptides of the non-collagenous and collagenous domains of type IV collagen, the major structural protein of GBM. In the majority of the 75 patients' urines tested, excretion of GBM-related peptides with molecular weights of 33, 50, 80 and 150 kilodaltons (kD) was detectable. Patients with a diminished glomerular filtration rate (GFR) demonstrated excretion of the 33 kD peptide more frequently (91%) and never of the 80 kD peptide as compared with patients with normal GFR (33 kD [42%] 80 kD [87%]). The pattern of urinary GBM-related peptides was not specific for the underlying renal disease as in Alport's syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849249
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  • 10
    Electronic Resource
    Electronic Resource
    Experience with deflazacort in children and adolescents after renal transplantation (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 457-463 
    Details
    ISSN: 1432-198X
    Keywords: Key words Deflazacort ; Renal transplantation ; Renal function ; Body growth ; Obesity ; Bone mineralization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Deflazacort (DFZ) has been proposed as an alternative drug for immunosuppression after renal transplantation (TX), with fewer side effects than conventional glucocorticoids. We investigated renal function, body growth, body fat, and bone mineral density (BMD) after switching from oral methylprednisolone (MPR) to equivalent doses of DFZ 1–9 years after TX in 20 patients aged 5–20 years, selected because of severe adverse effects from previous steroid therapy. At conversion the patients received a mean dose of 7.4±2.4 mg DFZ/m2 per day. The drug was continued for a mean of 3.7 (1.2–5.5) years. Under DFZ, the glomerular filtration rate dropped slightly (NS). A single rejection episode occurred. Growth velocity significantly improved in the 1st year on DFZ treatment and height standard deviation score (SDS) increased steadily after introduction of DFZ (from –2.64 to –1.96 after 4 years, P=0.06). However, in 10 prepubertal children the height gain (+0.20 SDS in 2 years on DFZ) was not significant and the overall mean annual growth rate after TX was similar to that in 10 matched prepubertal TX children on continued MPR treatment. Relative obesity, estimated from mean body mass index corrected for height, was reduced from +1.11 SDS at the start of DFZ to +0.71 SDS after 2 years (P=0.03) and to +0.39 SDS after 4 years (NS). BMD-SDS of the lumbar spine (L2–4) increased after 1 year on DFZ (P=0.005). In conclusion, DFZ is well tolerated and safe in pediatric patients after TX. It improves relative obesity and bone mineralization. However, body growth is not significantly influenced pre puberty.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050792
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