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  • 1
    Electronic Resource
    Electronic Resource
    Null alleles of the fourth component of complement and HLA haplotypes in familial systemic lupus erythematosus (1985)
    Springer
    Immunogenetics 21 (1985), S. 299-311 
    Details
    ISSN: 1432-1211
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Eight families (121 individuals) with two or more members affected with systemic lupus erythematosus (SLE) were analyzed for histocompatibility antigens (HLA-A, B, C, DR, MT, and MB) and complement antigens (C4A, C4B, and BF). These data were correlated with serological markers (antinuclear antibodies, single- and double-stranded anti-DNA, anti-SM, anti-nRNP, anti-Ro [SS-A], anti-La [SS-B], and biological false-positive tests for syphilis and clinical features. Fifteen members had SLE, and 19 had other immune diseases (subacute cutaneous lupus erythematosus, discoid lupus erythematosus, hypothyroidism, insulin-dependent diabetes mellitus, primary, Sjogren's syndrome, immune thrombocytopenic purpura, rheumatoid arthritis, and multiple sclerosis). Twenty-three healthy relatives (seroreactors) had significant titers of circulating antibodies, as did 2 of 17 spouses. There was an increased frequency of null C4 alleles in those individuals with SLE (60%) and healthy relatives (50%) as compared with spouses (24%). Multivariate analysis showed a significant association between SLE and female sex (P=.006), whereas there was no significant association revealed between female sex and other immune diseases. Patients with SLE also had a higher frequency of either C4A or C4B null alleles (P=.01) than those with immune diseases. The C4A homozygous null phenotype was more common in SLE patients than in seroreactors (P=.02). There was a higher frequency of HLA-DR2 and DR3 in individuals with SLE than in those with immune disease (P=.08), seroreactors (P=.02) and normal relatives (P =.002). One totally C4-deficient patient with SLE was identified. These families demonstrate an important association between SLE and the C4 null allele and the HLA-DR2 and DR3. These risk factors, however, cannot account for the development of disease in all individuals.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00430796
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  • 2
    Electronic Resource
    Electronic Resource
    Neonatal obstructive uropathy (1988)
    Springer
    Pediatric nephrology 2 (1988), S. 48-55 
    Details
    ISSN: 1432-198X
    Keywords: Obstructive uropathy ; Neonate ; Renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neonatal obstructive uropathy is the leading cause of renal failure during infancy. A variety of upper and lower genito-urinary (GU) tract disorders may cause obstructive uropathy. The prompt diagnosis and evaluation of obstructive uropathy is imperative since appropriate intervention and follow-up can lead to excellent early recovery of function. The role of in utero diagnosis is primarily to alert physicians to the presence of obstructive uropathy and permit rapid initiation of care at birth or earlier in certain instances. The GU tract must be evaluated for evidence of dilatation, for reflux and for renal function using radionuclide studies, voiding cystourethrograms, cystoscopy and pyelography. The early course of neonatal obstructive uropathy has been improved by institution of GU diversion and primary repair, yet the effect of early surgical intervention and medical therapy in preventing renal failure remains unclear. Further studies are needed to identify prognostic features heralding progression to renal failure and how such progression can be prevented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00870380
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  • 3
    Electronic Resource
    Electronic Resource
    Hypoproteinemia in the hemolytic-uremic syndrome of childhood (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 72-73 
    Details
    ISSN: 1432-198X
    Keywords: Hemolytic-uremic syndrome ; Hypoproteinemia ; Hypoalbuminemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective review of admission serum protein concentration in 18 children with hemolytic-uremic syndrome (HUS) showed significantly decreased serum total protein, albumin and globulin concentrations upon adminission compared with 22 matched controls (P〈0.003). One child with atypical disease without diarrhea had normal serum protein concentrations. A strongly positive correlation (P=0.006) was found between the age of HUS patients with diarrhea and their lowest total protein concentrations. In 10 children who eventually required hemodialysis, there was a significantly negative correlation (r=−0.8316,P=0.01) between the admission serum albumin and the patients' highest creatinine levels, suggesting that hypoproteinemia may be a risk factor in the development of renal failure. The pathophysiological and clinical significance of hypoproteinemia in HUS needs further investigation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00861576
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  • 4
    Electronic Resource
    Electronic Resource
    Hereditary partial deficiency of the third component of complement associated with minimal change nephrotic syndrome (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 608-610 
    Details
    ISSN: 1432-198X
    Keywords: Nephrotic syndrome ; Third component of complement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a 10 year old patient admitted to the Children's Hospital of Buffalo with hypocomplementemia associated with steroid responsive minimal change nephrotic syndrome. The sibling also had a low serum C3 concentration and all family members studied hadC 3 slow phenotypes. Factor I levels were at the lower limit of normal in the patient and his brother. Functional assays for CH50, total hemolyticC 3 and serum concentration of C2, C4−C9 and factors B and H were all within normal limits. This case confirms that a depressed serum complement level can occur in minimal change nephrotic syndrome and indicates that this depression could represent a preexisting inherited rather than an acquired deficiency. The findings are consistent with the pressence of a null or hypomorphicC 3 slow allele in hypocomplementemic family members. Additional studies are needed to resolve the association between the inherited partialC 3 deficiency and minimal change nephrotic syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00853597
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  • 5
    Electronic Resource
    Electronic Resource
    Intravenous immunoglobulin in minimal change nephrotic syndrome: a crossover trial (1990)
    Springer
    Pediatric nephrology 4 (1990), S. 32-35 
    Details
    ISSN: 1432-198X
    Keywords: Minimal change nephrotic syndrome ; Immunoglobulin ; Albumin ; Prednisone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine whether intravenous immunoglobulin (IVGG) would be an efficacious adjunct in the treatment of childhood minimal change nephrotic syndrome (MCNS), we enrolled ten patients with frequently relapsing or steroid-dependent MCNS in a double-blind crossover clinical trial. At the time of relapse of the nephrotic syndrome, patients were assigned to treatment with a single outpatient infusion of IVGG (800 mg/kg) or intravenous albumin as a control. The relapse was treated concurrently with standard doses of oral prednisone. At the time of the next relapse, patients who had first received IVGG were treated with albumin, and vice versa. There were no significant differences in the length of remission between the IVGG and albumin treatments. The study had a power of 0.72 to detect a true difference of 45 days between the two therapies. We conclude that in the dose of drug used in this trial, administered at the time of relapse in conjunction with prednisone therapy to children with frequently relapsing or steroid-dependent MCNS, IVGG does not lead to a clinically important extension of the period of remission.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00858434
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  • 6
    Electronic Resource
    Electronic Resource
    Complement and glomerulonephritis — an update (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 226-232 
    Details
    ISSN: 1432-198X
    Keywords: Complement ; Glomerulonephritis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The complement (C) system in man and its relationship to disease has been the subject of intensive research. In this review, we update the information concerning the nature of the various C components, and present some of the similarities between structure and function of the C components and their respective genes. The clinical problems which are encountered in individuals with acquired C abnormalities and with a genetically determined deficiency of a single component provide helpful clues to understanding the affected patients and the possible functional importance of the particular deficient component. The steady progress in identifying both normal variants of C components and the gene defects which produce C deficiencies offers the prospect of correlating structure of the C components with possible pathogenic roles in disease. Genetically determined C abnormalities are more commonly recognized during childhood. An appreciation of the basic aspects of the C system is a helpful tool for the pediatric nephrologist.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00864413
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  • 7
    Electronic Resource
    Electronic Resource
    Partial H (β1H) deficiency and glomerulonephritis in two families (1982)
    Springer
    Journal of clinical immunology 2 (1982), S. 110-117 
    Details
    ISSN: 1573-2592
    Keywords: Complement ; H ; C3 ; IgA nephropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract H (β1H) controls the C3b amplification loop by its ability to displace Bb from the alternative pathway convertase, C3b,Bb, and acts as a cofactor with I (C3b inactivator) to produce inactive C3b. Serum C3 levels are dependent to a large extent on the levels of H and I. Partial H deficiency was found in two families. The index case in Family 1 had vasculitis, thrombocytopenia, proteinuria, and depressed serum H and C3 levels. The index case in Family 2 had depressed serum H and B (Factor B) levels and IgA nephropathy which progressed to renal failure. His sister also had IgA nephropathy and depressed serum H and C3 levels. The depressed serum C3 level, B level, and H level could be responsible for the development of the immune diseases found in some members of these families.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00916894
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