1/19. Hyperimmunoglobulin E syndrome associated with nephrotic syndrome.A 21-year-old man was admitted to Kure National Hospital with nephrotic syndrome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months. Renal biopsy gave a diagnosis of membranoproliferative glomerulonephritis. Steroid therapy decreased urinary protein loss and hypoproteinemia, and his pruritic skin rash was improved. patients with hyperimmunoglobulin E syndrome have a defective immune response, especially to Staphylococcus aureus infection. Continuous antigen stimulation may have caused this patient's renal histological damage as in immune complex glomerulonephritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/19. Membranoproliferative glomerulonephritis type I, mixed cryoglobulinemia and lymphoma in the absence of hepatitis c infection.Chronic hepatitis c virus infection has been linked to cryoglobulinemia, membranoproliferative glomerulonephritis, and malignant B-cell lymphoproliferation, suggesting a possible pathogenetic link between these disorders. We report a patient with the latter clinical triad in the absence of hepatitis c infection. We postulate that the persistent and dysregulated immunologic activity associated with chronic antigen stimulation, inflammation and/or B-cell malignancy induces nephritogenic autoantibodies, including cryoglobulins, that produce a similar clinical syndrome in genetically susceptible individuals. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/19. Membranoproliferative glomerulonephritis in two siblings: report and literature review.There is evidence of a genetic basis in some cases of idiopathic membranoproliferative glomerulonephritis (MPGN) types I and III, particularly those occurring in families. The clinical and morphological features and disease course in two siblings with MPGN are described. In the male sibling, both clinical and morphological features as well as serum complement profile suggested type I MPGN; electron microscopy appearance in the female sibling was consistent with type III MPGN. Both patients had treatment-resistant nephrotic syndrome which evolved into renal insufficiency in the girl. No hereditary complement deficiencies were found in siblings or their parents. Both children exhibited HLA-A24; -B27, w4; -DR11, 52; -DQ3 antigens. Between 1981 and 1996, 18 patients from eight families with unequivocal diagnosis of MPGN I or III had been described. The mode of inheritance appeared to be autosomal dominant or X-linked in four of these families. In 11 patients, including our 2, in whom HLA typing was performed, eight had the hla-a2 antigen. Similarities and discrepancies regarding clinical and morphological features and outcomes were evident in these intrafamilial cases, suggesting either a similar genetic background or a multigenic origin of MPGN. The familial occurrence of the MPGN, highlighted by our report, supports the concept that genetically determined factors may be involved in the pathogenesis of the disease.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/19. Membranoproliferative glomerulonephritis in sibs.Idiopathic membranoproliferative glomerulonephritis (MPGN) is a chronic renal disease with variable clinical expression and several distinct morphological subtypes. Two sibs, aged 10 and 13, presented with clinical and laboratory findings of MPGN at the time of admission. After an interval of one year, the diagnosis of MPGN was established by renal biopsies. The complement pathway was unremarkable. HLA typing in the unrelated parents and the two male sibs revealed common HLA A2,A11,Bw60, DR2,DQw1 antigens in the brothers. Of these antigens, A2 has been reported previously in cases of MPGN. The other antigens regarding this disease need to be evaluated from the standpoint of genetic importance.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
5/19. Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases.Factor H (FH) is the major regulatory protein of the complement alternative pathway, with a structure consisting of a tandem array of 20 homologous units, called short consensus repeats (SCR). Reported are 16 FH-deficient patients. Among six patients with homozygous deficiency, four presented with membranoproliferative glomerulonephritis, and two with atypical hemolytic uremic syndrome (HUS). The ten other patients had heterozygous FH deficiency and developed atypical HUS. HUS onset occurred from birth to midadulthood, and disease progression was variable. Four children with homozygous or heterozygous FH deficiency and HUS underwent renal transplantation, which was successful in three but failed as a result of recurrence of HUS in one patient. All but one patient exhibited alternative pathway-mediated complement consumption, with no detectable FH antigenic levels or with 50% immunochemical or functional FH levels in the case of complete or partial deficiency, respectively. The molecular mechanisms of the deficiency were documented in all cases by exon-specific sequencing analysis. These mechanisms included nucleotide substitutions, insertion, or deletion located in SCR 2, 7, 11, 13, 15, and 20, leading to an amino acid substitution or to a stop codon. This report emphasizes the variability in the clinical progression of kidney diseases associated with FH deficiencies. Genetic analysis reveals the molecular abnormalities associated with FH deficiencies to be polymorphous.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/19. Polyglandular autoimmune syndrome type III accompanied by common variable immunodeficiency.We identified polyglandular autoimmune (PGA) syndrome type III in a 24-year-old nurse with common variable immunodeficiency (CVID). An immune-mediated disorder, membranoproliferative glomerulonephritis, was diagnosed when she was 15 years old. Clinical examination and laboratory findings revealed a PGA syndrome due to the presence of hypergonadotropic hypogonadism, insufficient growth hormone response and thyroid autoimmunity. The patient had neither adrenal disease nor hypoparathyroidism. Therefore we concluded that this patient has PGA syndrome type III. This is an interesting case, because we could not find any previous report of such coexistence between PGA type III and CVID in a medline search. Coexistence of these two entities may be a result of autoimmunity and the association of both conditions with human leukocyte antigen.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/19. Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome.We investigated the phenotypic expression of factor H mutations in two patients with atypical hemolytic uremic syndrome (HUS). Factor H in serum was assayed by rocket immunoelectrophoresis, immunoblotting, and double immunodiffusion and in tissue by immunohistochemistry. Functional activity was analyzed by hemolysis of sheep erythrocytes and binding to endothelial cells. A homozygous mutation in complement control protein (CCP) domain 10 of factor H was identified in an adult man who first developed membranoproliferative glomerulonephritis and later HUS. C3 levels were very low. The patient had undetectable factor H levels in serum and a weak factor H 150 kDa band. Double immunodiffusion showed partial antigenic identity with factor H in normal serum owing to the presence of factor H-like protein 1. Strong specific labeling for factor H was detected in glomerular endothelium, mesangium and in glomerular and tubular epithelium as well as in bone marrow cells. A heterozygous mutation in CCP 20 of factor H was found in a girl with HUS. C3 levels were moderately decreased at onset. Factor H levels were normal and a normal 150 kDa band was present. Double immunodiffusion showed antigenic identity with normal factor H. Factor H labeling was minimal in the renal cortex. Factor H dysfunction was demonstrated by increased sheep erythrocyte hemolysis and decreased binding to endothelial cells. In summary, two different factor H mutations associated with HUS were examined: in one, factor H accumulated in cells, and in the other, membrane binding was reduced.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
8/19. Post-MRSA infection glomerulonephritis with marked Staphylococcus aureus cell envelope antigen deposition in glomeruli.A 48-year-old male developed massive proteinuria and renal dysfunction after pneumonia caused by methicillin-resistant staphylococcus aureus (MRSA) infection. Examination of a renal biopsy specimen by light microscopy showed severe mesangiocapillary proliferative glomerulonephritis with fibrocellular crescents. Immunofluorescence microscopy showed weak linear staining for immunoglobulin g (IgG), while both the peripheral and mesangial lesions stained for IgA and C3. Immunostaining for a possible antigen related to post-MRSA infection glomerulonephritis, using monoclonal antibody S1D6, revealed marked deposition of S.aureus cell envelope antigen in the glomeruli. Electron-dense deposits were observed in both the subendothelial and the mesangial areas. Focal subendothelial widening accompanied with monocytes or foam cell infiltration was also seen. The findings reflect a typical post-MRSA infection glomerulonephritis caused by S.aureus cell envelope antigen.- - - - - - - - - - ranking = 7keywords = antigen (Clic here for more details about this article) |
9/19. Essential mixed cryoglobulinemia type II.We report a rare case of essential mixed cryoglobulinemia type II with membrano-proliferative glomerulonephritis (MPGN) type I in which HCV was not found. Long-term history of palindromic rheumatism, skin leukocytoclastic vasculitis attacks and micro-normocytic anemia preceded the appearance of cryoglobulinemia. Cryoprecipitate consisted of monoclonal IgMk-RF and polyclonal IgG (essential mixed type II). The newly appreciated cryoglobulinemia was associated with Coombs positive hemolytic anemia. The MPGN in this case had a benign course and responded to complex simple therapies including prevention of exposure to cold, low antigen content diet, treatment of provoking factors such as UTI, and maximal dose of ACE inhibitor. Responsiveness of skin vasculitis to colchicine therapy was restored after a two-month colchicine withdrawal period and therefore corticosteroid and immunosuppressive therapy was postponed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/19. Improvement of immune-complex nephritis associated with hepatitis b surface antigen excess.A case of hypocomplementemic membranoproliferative glomerulonephritis was studied during remission of nephrosis induced by high doses of corticosteroids. hepatitis b surface antigen (HBsAg) and immune complexes were detected in serum and glomeruli. Anti-hepatitis-B surface antibody, undetectable in serum by conventional radioimmunoassays was identified in circulating immune complexes (CIC). On two occasions, improvement in renal function coincided paradoxically with an extreme increase in serum HBsAg levels as well as with marked elevation of CIC. We suggest that, as previously observed in animal models of glomerulonephritis, extreme antigen excess may inhibit glomerular deposition of immune complexes.- - - - - - - - - - ranking = 6keywords = antigen (Clic here for more details about this article) |
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