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  • 1
    Electronic Resource
    Electronic Resource
    Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: A long-term follow-up (2001)
    Copenhagen, Denmark : Munksgaard International Publishers
    Pediatric allergy and immunology 12 (2001), S. 0 
    Details
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2–14 years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) and signs of complement activation in serum. In addition, C3 allotyping was performed in 32 patients. Observation time ranged from 2 to 20 (median 11) years. C3 NeF activity was detected at least once in 60% of the patients (in 11 of 26 with type I, in 15 of 17 with type II, and in four of seven with type III). C3 NeF-positive patients had significantly reduced levels of CH50 and C3 and elevated levels of C3dg/C3d. During follow-up, C3 levels were persistently normal in 62% of the patients with MPGN type I and in 43% with type III but in only 18% with type II. C3 allotype frequencies differed from those found in healthy controls with a significant shift to the C3F/C3FS variants in C3 NeF-positive patients. C3b(Bb)P as a marker for alternative pathway activation was not increased in C3 NeF-positive patients. Despite the presence of C3 NeF activity, C3 levels remained normal in six patients throughout the observation period. C3 NeF became undetectable in six patients, whereas seven developed C3 NeF activity during follow-up. There was no significant difference in renal survival probability in patients with or without C3 NeF activity. Neither C3 variants nor continous low C3 or low CH50 levels had any prognostic value for the clinical outcome. No factor H deficiency was detected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1034/j.1399-3038.2001.012003166.x
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  • 2
    Electronic Resource
    Electronic Resource
    Mutations in a novel gene, NPHP3 , cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosis (2003)
    [s.l.] : Nature Publishing Group
    Nature genetics 34 (2003), S. 455-459 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Nephronophthisis (NPHP), a group of autosomal recessive cystic kidney disorders, is the most common genetic cause of progressive renal failure in children and young adults. NPHP may be associated with Leber congenital amaurosis, tapeto-retinal degeneration, cerebellar ataxia, cone-shaped epiphyses, ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/ng1216
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  • 3
    Electronic Resource
    Electronic Resource
    Renal function in predialysis children with chronic renal failure treated with erythropoietin (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 69-73 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Anemia ; Chronic renal failure ; Erythropoietin ; Renal function
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. To assess the effect of long-term administration of human recombinant erythropoietin (EPO) on renal function, 11 anemic children aged 1.4 – 17.2 years were followed for 10 – 61 (mean 31) months on treatment. During EPO therapy the mean hemoglobin rose from 8.1 to 11.1 g/dl at the last observation. The final maintenance dose ranged between 70 and 300 U/kg per week. The rate of deterioration of renal function was calculated by comparing the slope of the regression lines of reciprocal serum creatinine values (SCr) derived from a mean of 20 values per patient obtained over 8 – 50 (mean 29) months before and a mean of 24 SCR values during EPO therapy. The individual slopes improved after initiation of EPO therapy in all but 3 patients, but the mean change of slope (from –0.0521 to –0.0299) was not significant. The study suggests that in most children with predialysis chronic renal failure long-term administration of EPO is not associated with accelerated deterioration but rather with delayed deterioration of renal function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050234
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  • 4
    Electronic Resource
    Electronic Resource
    How to assess the rate of progression of chronic renal failure in children? (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 499-504 
    Details
    ISSN: 1432-198X
    Keywords: Rate of progression ; Renal failure ; Trials ; Statistics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Methodology for assessing progression of chronic renal failure and performing and analysing trials has never been a favourite topic in nephrology. The aim of this paper is to discuss from a statistician's point of view the advantages and disadvantages of different direct (glomerular filtration rate, creatinine clearance, reciprocal and serum creatinine_ and indirect (need of renal replacement therapy, halving of glomerular filtration rate, doubling of serum creatinine) markers of renal function, and their statistical evaluation. The use of cross-tabulation, regression analyses/slope techniques and survival analyses for this purpose is discussed. Furthermore, a new approach for the evaluation of progression, the so-called progression charts, is described in detail. This paper reveals that analysing progression of renal failure is not an easy task. However, there are a number of pragmatic approaches which seem to be suitable.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856550
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  • 5
    Electronic Resource
    Electronic Resource
    Alport's syndrome as a cause of renal failure in Europe (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 411-415 
    Details
    ISSN: 1432-198X
    Keywords: Hereditary nephritis ; Alport's syndrome ; End-stage renal failure ; Sex ratio ; Geographical distribution
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We studied the geographical distribution, male to female ratio, and age at the start of renal replacement therapy (RRT) for end-stage renal failure (ESRF) in 600 patients with hereditary nephritis with nerve deafness (Alport's syndrome) reported to the European Dialysis and Transplant Association Registry since 1975. Annual age- and sex-specific acceptance rates for RRT showed a variable peak incidence according to country, ranging between, 0 and 2.4 patients per million population in males aged 15–24 years, but with only about half this incidence in females. In Scandinavian countries there were very few females who started RRT, and males were older than in the rest of Europe. The overall male to female ratio was 4:1. The median age at the start of RRT was: males (n=479) 24.3 years (1st quartile 19.5 years; 3rd quartile 31.5 years); females (n=121) 31.5 years (1st quartile 23.0 years; 3rd quartile 43.2 years). Our study provided confirmation that males reach ESRF earlier than females. In addition, we detected previously unrecognized geographical differences.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849245
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  • 6
    Electronic Resource
    Electronic Resource
    Rate of deterioration of renal function in juvenile nephronophthisis (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 56-60 
    Details
    ISSN: 1432-198X
    Keywords: Age at renal death ; Chronic renal failure ; Nephronophthisis ; Deterioration of renal function ; Serum creatinine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The rate of deterioration of renal function was determined in 29 patients with juvenile nephronophthisis presenting before the age of 18.5 years. The analysis was based on 618 serial determinations of serum creatinine (SCR). By application of a new statistical method the time elapsing between different successive SCR levels was calculated. A more or less homogeneous increase of SCR was noted starting from the time when a SCR of 4 mg/dl was reached. The median time elapsing between an SCR of 2 and 4 mg/dl was 32 months, between 4 and 6 mg/dl 10 months and between 6 and 8 mg/dl 5 months. The median age at the start of renal replacement therapy or death due to uraemia, evaluated by survival analysis, was similar in both sexes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00859627
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  • 7
    Electronic Resource
    Electronic Resource
    Progression of chronic renal failure in a historical group of patients with nephropathic cystinosis (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 466-471 
    Details
    ISSN: 1432-198X
    Keywords: Cystinosis ; Chronic renal failure ; Serum creatinine ; Siblings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a historical group of 205 patients with infantile or adolescent cystinosis treated without cysteamine, the rate of deterioration of renal function was analysed retrospectively. Patient survival curves and renal survival data are presented. Longitudinal data of serum creatinine values (n=3280) in 157 patients were plotted for each patient, smoothed by the method of the running medians and grouped into 12 serum creatinine classes. In every patient the age at the last smoothed serum creatinine value observed in each serum creatinine class was determined. These virtual age values were then summarized per serum creatinine class, expressed as median and centiles and plotted, thus describing the “natural” course of the disease. In 9 pairs of affected siblings the rate of progression showed a median difference of about 12 months. Our data describe the “natural” course of nephropathic cystinosis and can be used as a prognostic aid for recently detected patients. The data can also be applied for the assessment of the influence of new therapeutic strategies on the rate of progression of renal failure in cystinotic patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856532
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  • 8
    Electronic Resource
    Electronic Resource
    The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire (1995)
    Springer
    Pediatric nephrology 9 (1995), S. 685-689 
    Details
    ISSN: 1432-198X
    Keywords: Cystinosis ; Cysteamine ; Phosphocysteamine ; Renal function ; Growth ; Leucocyte cystine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-nine patients with cystinosis were treated with cysteamine or phosphocysteamine in the United Kingdom up to May 1990. Treatment was started at a median age of 3.2 years (range 0.6–24.8 years) and continued for a median duration of 3.0 years (range 0.01–1.2 years). At the end of the study, 46 (78%) patients remained on treatment. One patient developed end-stage renal failure and 6 died. Efficacy was assessed in the 44 pre-transplant patients. The United Kingdom pre-transplant patients had significantly lower plasma creatinine concentrations at 6 and 8 years than a historical group of patients who did not receive cysteamine (P〈0.0001 andP〈0.0003, respectively). There was no significant difference between pretreatment and final post-treatment height standard deviation scores, suggesting maintenance of growth rate. The leucocyte cystine concentration was less than the accepted upper limit of the treatment range (1 nmol 1/2 cystine/mg protein) in only 21% of determinations. There was no significant difference between the mean pre-treatment and final values of leucocyte cystine concentration. The mean final doses of cysteamine (33 mg/kg per day) and phosphocysteamine (37 mg/kg per day base equivalent) were less than the mean dose (51 mg/kg per day) used in a United States multicentre trial. We conclude that cysteamine treatment was beneficial, but further improvements might be achieved by an improvement in monitoring of therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00868711
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  • 9
    Electronic Resource
    Electronic Resource
    An ever-expanding story of cyst formation (2000)
    Springer
    Cell & tissue research 300 (2000), S. 361-371 
    Details
    ISSN: 1432-0878
    Keywords: Polycystic kidney disease PKD1 PKD2 Polycystin-1 Polycystin-2 Two-hit hypothesis Stop-signal hypothesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. Autosomal-dominant polycystic kidney disease represents one of the most common monogenetic human disorders. The cloning of the PKD1 and PKD2 genes, which are mutated in far more than 90% of the patients affected by this disease, has generated high hopes for a quick understanding of the pathogenesis of cyst formation. However, these expectations have not yet been fulfilled, since the function of both polycystin-1 and polycystin-2, the two proteins encoded by PKD1 and PKD2, still remains a puzzle. In this review, we will highlight some of the characteristics of polycystic kidney disease, briefly touch on polycystin-1, and then go on to describe recent results of experiments with polycystin-2, since the latter is the major focus of our work. We will discuss new evidence which suggests that autosomal-dominant polycystic kidney disease actually behaves recessively on a cellular level. Finally, a model will be presented that tries to explain the available data.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004410000215
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