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  • 1
    Electronic Resource
    Electronic Resource
    IgA nephropathy, the most common cause of glomerulonephritis, is linked to 6q22–23 (2000)
    [s.l.] : Nature America Inc.
    Nature genetics 26 (2000), S. 354-357 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] End-stage renal disease (ESRD) is a major public health problem, affecting 1 in 1,000 individuals and with an annual death rate of 20% despite dialysis treatment. IgA nephropathy (IgAN) is the most common form of glomerulonephritis, a principal cause of ESRD worldwide; it affects up to 1.3% ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/81677
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  • 2
    Electronic Resource
    Electronic Resource
    Complement profiles in acute post-streptococcal glomerulonephritis (1988)
    Springer
    Pediatric nephrology 2 (1988), S. 219-223 
    Details
    ISSN: 1432-198X
    Keywords: Acute post-streptococcal glomerulonephritis ; Complement ; C3 ; C4
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract It is well known that the hypocomplementemia of acute post-streptococcal glomerulonephritis (APSGN) is characterized by markedly reduced serum concentrations of C3 and moderately reduced levels of C5 and properdin (P). However, the extent of the activation of the classical pathway is not well defined and only limited data are available concerning serum concentrations of terminal components other than C5. In serial serum specimens from 14 children with APSGN, the presence and extent of C4 activation was directly assessed by measurement by rocket immunoelectrophoresis for C4 and C4 (C4d/C4 ratio). Elevated values for this ratio, indicating C4 activation, were found in 8 of 14 of the initial serum specimens, and in some patients the ratio remained elevated for several weeks. In contrast, the serum C4 level was low in only 1 specimen (the specimen with the highest C4d/C4 ratio). However, in 10 patients C4 concentrations within the normal range rose in serial serum specimens. Serum C2 concentrations were depressed in the initial specimens from 5 patients. The concentrations of 13 other complement component and control proteins were also measured in these specimens. Levels of terminal components, other than C5, in the initial serum specimens were normal except for depressed C8 in 3 of 13 patients and depressed C6 in 1 of 14. Of these 4 individuals, 3 had the lowest C3 levels in the study. It is concluded that the classical complement pathway is frequently activated in patients with APSGN early in the condition and that subtle abnormalities in C6 and C8 levels occasionally occur.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00862594
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  • 3
    Electronic Resource
    Electronic Resource
    Nephrosis, peritonitis and complement deficiency (1990)
    Springer
    Pediatric nephrology 4 (1990), S. 575-575 
    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/BF00869845
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  • 4
    Electronic Resource
    Electronic Resource
    Treatment of IgA nephropathy in children: efficacy of alternate-day oral prednisone (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 529-532 
    Details
    ISSN: 1432-198X
    Keywords: IgA nephropathy ; Treatment ; Prednisone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have previously reported our experience with the use of alternate-day prednisone in the treatment of 6 patients with IgA nephropathy who have clinical or pathological risk factors for disease progression. We have now treated a total of 13 patients and followed them from 4 to 10 years. Patients received an alternate-morning dose of prednisone for 2–4 years. Dosage began at 60 mg/m2 for 3 month, was reduced to 30 mg/m2 by 1 year and 15 mg/m2 by 2 years. At last observation, urinary protein excretion was normal in 12 patients and no patient had hematuria. Twelve patients had normal estimated glomerular filtration rate (GFR) and one had renal insufficiency (GFR=38 ml/min per 1.73 m2). A renal biopsy was performed in 11 patients after 2 years of treatment. Activity score decreased from 5.2 to 4.3 (P=0.03) and chronicity score increased from 2.2 to 2.8 (P=0.12). There were no complications of treatment. When compared with a historical group, the treated patients had a significant improvement in urinalysis (P〈0.00001) and preservation of normal GFR (P=0.03). We conclude that alternate-day prednisone therapy may benefit patients with IgA nephropathy. A large prospective controlled trial is needed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00852535
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  • 5
    Electronic Resource
    Electronic Resource
    Terminal complement complexes in acute poststreptococcal glomerulonephritis (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 671-676 
    Details
    ISSN: 1432-198X
    Keywords: Acute poststreptococcal glomerulonephritis ; Complement ; Terminal complement complexes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Activation of the complement cascade occurs in most cases of acute poststreptococcal glomerulonephritis (APSGN) and results in the formation of the terminal complement complexes (TCC). To examine the possible role of TCC in the pathogenesis of glomerular injury in APSGN, we studied 30 patients with the clinical diagnosis of APSGN. All patients had an elevated plasma SC5b-9 concentration at the onset of clinical nephritis. Serial plasma concentrations showed an inverse linear relationship with time after onset of clinical disease (r=−0.59,P=0.0008), while plasma C3 concentrations showed a positive linear relationship (r=0.78,P=0.0001). Renal biopsies of 5 patients demonstrated co-localization of C5b-9, S-protein, and C3 deposition in a glomerular capillary loop and mesangial distribution. Urinary excretion of TCC in the acute phase of APSGN was not elevated and was not a useful marker of disease activity. These data suggest that in APSGN with terminal complement pathway activation the local generation of TCC may contribute to the pathogenesis of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00869086
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  • 6
    Electronic Resource
    Electronic Resource
    Inherited factor H deficiency and collagen type III glomerulopathy (1995)
    Springer
    Pediatric nephrology 9 (1995), S. 11-15 
    Details
    ISSN: 1432-198X
    Keywords: Glomerulonephritis ; Factor H ; Complement ; β-1 H globulin ; Collagen type III glomerulopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A non-immune complex-mediated glomerulonephritis associated with persistent hypocomplementemia occurred in a young boy. Measurement of complement components revealed complete factor H deficiency, inherited as an autosomal recessive trait. Evaluation of the renal lesion revealed extensive deposition of type III collagen suggestive of collagen type III glomerulopathy, a recently identified cause of chronic renal insufficiency in children and adults. This report represents the first association of inherited factor H deficiency with collagen type III glomerulopathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858956
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  • 7
    Electronic Resource
    Electronic Resource
    Increased recognition of IgA nephropathy in African-American children (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 435-437 
    Details
    ISSN: 1432-198X
    Keywords: Key words: IgA nephropathy ; Incidence ; African-American children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Based upon the percentage of cases of IgA nephropathy (IgAN) in biopsy series, a lower prevalence has been assumed for African-Americans compared with Americans of European descent. This may be due to a racial difference in the basic underlying pathology of IgAN or to racial differences in patterns of referral and biopsy selection practices. Over the past decade (1985 – 1994), we have found similar incidences of IgAN in Caucasian and African-American children from Shelby County, Tennessee. The incidence was 3.0 cases per million per year for Caucasian and 5.7 cases per million per year for African-American children. IgAN may be more common in African-American children than previously appreciated. Population-based incidence studies will be necessary to determine whether or not our experience has become a more widespread phenomenon.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050311
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  • 8
    Electronic Resource
    Electronic Resource
    Comparable renal graft survival in African-American and Caucasian recipients (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 534-539 
    Details
    ISSN: 1432-198X
    Keywords: Key words: African-American ; Renal transplantation ; Anti-lymphocyte induction therapy ; Predicted graft survival ; Immunosuppression
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. In past years, many pediatric transplant centers found African-American renal transplant recipients to have poor graft survival. Since 1991 anti-lymphocyte induction therapy has been routinely used for pediatric cadaveric (CAD) and living-related donor (LRD) renal allograft recipients at the University of Tennessee, Memphis. Sixteen African-American first renal allograft recipients received induction therapy: 11 CAD allografts (10 OKT3, 1 ATGAM) and five LRD (all ATGAM). Sixteen Caucasian recipients received induction therapy; 3 CAD (all OKT3), 1 living-unrelated donor (OKT3), and 12 LRD (9 ATGAM, 3 OKT3). Mean age at renal transplantation was 11.8 and 10.5 years for African-American and Caucasian recipients, respectively. Predicted graft survival (PGS) estimated by the Kaplan-Meier method for the African-American patients was 94% at both 1 and 3 years, and for Caucasian patients was 94% and 85% at 1 and 3 years, respectively. Eleven African-American CAD recipients had a PGS of 91% at 1 and 3 years. Renal allograft survival for African-American and Caucasian pediatric recipients at our center appears to be comparable. This could be due, in part, to the use of anti-lymphocyte induction therapy. However, other factors, such as improved compliance or better immunological and pharmacological monitoring, may also have contributed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050500
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  • 9
    Electronic Resource
    Electronic Resource
    Prognostic indicators in children with IgA nephropathy — Report of the Southwest Pediatric Nephrology Study Group (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 15-20 
    Details
    ISSN: 1432-198X
    Keywords: IgA nephropathy ; End-stage renal disease ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Investigators in 13 pediatric nephrology centers reviewed clinical and pathological features in 218 children and adolescents with IgA nephropathy (IgAN), with particular emphasis on 80 patients who had follow-up periods of at least 4 years. Potential prognostic markers in the 80 children were compared between 12 (15%) who developed end-stage renal disease (ESRD) versus 68 who did not. The relationship between clinical and pathological features and the subsequent development of ESRD was examined using stepwise linear discriminant analysis in addition to standard univariate analysis. Seven variables were found to be predictive of ESRD: the presence of glomerular sclerotic changes, especially when this was associated with proliferation or sclerosis in 20% or more of the glomeruli, black race; hypertension at biopsy; proteinuria at biopsy; age at presentation; crescents; male sex. Using the resulting discriminant function, development of ESRD could be correctly predicted in 95% of the subjects. We conclude that ESRD is more common in American children with IgAN than was realized previously. Risk factors previously documented in adult studies have been confirmed, especially the presence of glomerular sclerosis, proteinuria, and hypertension.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00868251
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  • 10
    Electronic Resource
    Electronic Resource
    Serious infections due to penicillin-resistant Streptococcus pneumoniae in two children with nephrotic syndrome (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 639-641 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Nephrotic syndrome ; Penicillin-resistant Streptococcus pneumoniae ; Peritonitis ; Bacteremia ; Cephalosporins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Children with nephrotic syndrome are susceptible to the development of invasive bacterial infections, particularly those caused by Streptococcus pneumoniae. Recently, penicillin-resistant pneumococcal infections in children have occurred in increased frequency in some regions. We have seen two children with nephrotic syndrome who developed penicillin-resistant pneumococcal bacteremia and/or peritonitis. A change in the initial therapy from penicillin in the usual dose in these patients must be considered.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050179
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