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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
    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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  • 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
    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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  • 5
    Electronic Resource
    Electronic Resource
    Beneficial effect of second courses of cytotoxic therapy in children with minimal change nephrotic syndrome (1988)
    Springer
    Pediatric nephrology 2 (1988), S. 291-295 
    Details
    ISSN: 1432-198X
    Keywords: Children ; Nephrotic syndrome ; Steroid treatment ; Cytotoxic drugs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Therapeutic guidelines are not available for children with minimal change nephrotic syndrome (MCNS) who experience frequent relapses or develop steroid resistance after a course of cytotoxic therapy. The records of nine children with biopsy-proven MCNS who received two courses of cytotoxic therapy with either chlorambucil or cyclophosphamide were reviewed to evaluate the length of remission, associated side-effects and long-term outcome. Initial cytotoxic therapy was given to five frequent-relapsing patients and four steroid-resistant patients 2–48 months (mean 16 months) following diagnosis of nephrotic syndrome. The second drug was given 4–85 months (mean 27 months) after the first. Steroid-resistant patients attained remissions of 0–81 months (mean 23 months) following the first agent and 13–67 months (mean 32 months) following the second. Frequent-relapsing patients experienced remissions of 0.5–24 months (mean 7.4 months) following the first cytotoxic drug and 3–72 months (mean 22 months) after the second. Remissions following the second agent were equal to or longer than those following the first in the seven patients who received both chlorambucil and cyclophosphamide. In the 19- to 128-month follow-up (mean 66 months), all four steroid-resistant patients experienced infrequent relapses which responded to prednisone. One frequent-relapsing patient remains in remission, three have chronic proteinuria and one still has a frequent-relapsing course. For the select group of patients who become frequent relapsing or steroid resistant after one course of cytotoxic therapy, a second course of cytotoxic therapy may allow time for catch-up growth, as well as improve steroid responsiveness once relapses occur.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858680
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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
    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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  • 9
    Electronic Resource
    Electronic Resource
    Clinical quiz (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 501-502 
    Details
    ISSN: 1432-198X
    Keywords: Polyarteritis nodosa ; Prednisone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00857584
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  • 10
    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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