Cases reported "Glomerulonephritis, IGA"

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1/36. IgA nephropathy in cystic fibrosis.

    The kidney does not usually present specific lesions in cystic fibrosis (CF), although in recent years renal involvement has been reported, particularly amyloidosis and immune complex glomerulonephritis. IgA nephropathy is rare. We report four cases of IgA nephropathy out of five renal biopsies performed in the last three years in patients with CF and renal involvement and discuss the possibility of a relationship between IgA nephropathy and CF.
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2/36. immunoglobulin a nephropathy complicating pulmonary tuberculosis.

    A 31-year-old man who presented with smear- and culture-negative pulmonary tuberculosis had associated macroscopic hematuria, elevation of serum creatinine and immunoglobulin a (IgA) levels, overt proteinuria, and peripheral edema. Renal biopsy revealed focal mesangial proliferation with IgA deposits, and a diagnosis of IgA nephropathy was made. The patient received treatment with isoniazide and rifampin. After 4 months, pulmonary lesions were almost completely healed, and a significant improvement of creatinine clearance with normalization of serum creatinine and IgA levels and disappearance of proteinuria were observed. Treatment with isoniazide and rifampin was discontinued after 6 months, without reappearance of either pulmonary or renal symptoms. Two years after the diagnosis of IgA nephropathy, the patient is in good general condition. serum creatinine and IgA levels are normal, proteinuria is absent, and there is neither macrohematuria nor microhematuria. These findings suggest that IgA nephropathy may be a consequence of tuberculosis, possibly due to an abnormal IgA-mediated immune response against mycobacterium tuberculosis with formation of nephrotoxic immune complexes.
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3/36. IgA nephropathy and Reiter's syndrome. Report of two cases and review of the literature.

    immunoglobulin a (IgA) nephropathy is the commonest type of primary glomerulonephritis worldwide. It has previously been reported in association with the seronegative spondyloarthropathies (ankylosing spondylitis, Behcet's syndrome, psoriatic arthritis, Reiter's syndrome and the postenteritic arthritides). Since this condition was first described in 1968, 5 previous case reports of biopsy-proven IgA nephropathy associated with Reiter's syndrome have been published in the English-language literature. Here we report 2 more such cases, along with a review of the literature describing the association of IgA nephropathy and a number of other immune-complex-mediated glomerulonephritides with the seronegative spondyloarthropathies.
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4/36. A case of renal involvement in persistent immune activation caused by chlamydial salpingitis.

    A 24-year-old woman presented with renal insufficiency, macrohematuria, and mild urinary protein. Polyclonal hypergamma-globulinemia, thrombocytosis, increased concentration of serum, and urinary interleukin (IL)-6 all indicated persistent immune activation caused by a chlamydia trachomatis infection of the fallopian tube. Gynecological treatment with levofloxacin was effective both for the renal symptoms and other immunological parameters. First and second renal biopsy specimens showed an immune-complex glomerulopathy with extensive interstitial infiltration of many types of inflammatory cells, including plasma cells. Thus, we conclude that chlamydial salpingitis must be considered as one causative disease factor for renal involvement by means of its persistent immune activation effects.
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5/36. IgA nephropathy in a patient with chronic granulomatous disease.

    We report the first documented case of X-linked chronic granulomatous disease (CGD) in association with IgA nephropathy in a 22 year old male with multiple soft tissue abscesses. The case highlights the potential role of staphylococcal infections in the pathogenesis of IgA nephropathy and suggests the hypothesis that the neutrophil defect in CGD predisposes to such infections which may trigger IgA immune complex formation.
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6/36. IgA antiglomerular basement membrane nephritis associated with Crohn's disease: a case report and review of glomerulonephritis in inflammatory bowel disease.

    A case of glomerulonephritis in a 35-year-old man with Crohn's disease is described here. The patient presented with severe diarrhea, nephrotic range proteinuria, hematuria, microangiopathic hemolytic anemia, thrombocytopenia, hypocomplementemia, acute renal failure requiring hemodialysis, cryoglobulinemia, and extensive thrombotic gangrene of the distal upper and lower limbs. The patient did not respond to plasmapheresis and steroid therapy and died of upper gastrointestinal bleeding. Renal tissue obtained at autopsy showed IgA-mediated antiglomerular basement membrane crescentic glomerulonephritis. Linear staining of the glomerular basement membrane by non-IgG antibodies is quite unusual with only 11 cases previously reported in the worldwide literature, 8 caused by IgA. Glomerulonephritis is a rarely reported extraintestinal manifestation of inflammatory bowel disease, and there are only 24 previously described cases that are reviewed and summarized in this report. Glomerulonephritis occurred in the setting of active bowel inflammation in all cases, circulating immune complexes were found in nearly half the cases, and serum complements usually were normal. renal insufficiency and nephrotic range proteinuria were typically present at the time of diagnosis of glomerulonephritis and most often improved in parallel with treatment of the gastrointestinal disorder. The histologic findings were varied and included membranoproliferative glomerulonephritis, mesangioproliferative glomerulonephritis, membranous nephropathy, IgA nephropathy, and IgM nephropathy. Thus, the authors present the first case of glomerulonephritis caused by antiglomerular basement membrane disease in association with inflammatory bowel disease.
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7/36. light and electron microscopic observation in a case of congenital renal proximal tubular dysfunction.

    We report a case of congenital renal proximal tubular dysfunction (CRPTD) accompanied by IgA nephropathy. The mesangial matrix was slightly increased with depositions of IgA and C3. podocytes contained many clear vacuoles. Cuboidal cells, as well as squamous cells, lined the parietal epithelium of Bowman's capsule, although the functional or pathological significance of the cuboidal cells is unknown. The proximal tubular cells appeared to have a less-developed apical endocytic complex, basal infoldings and fewer lysosomes. These morphological changes may reflect proximal tubular dysfunction.
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8/36. IgA nephropathy in patients with congenital C9 deficiency.

    The clinical, histologic, and immunopathological findings of three young Japanese males with congenital C9 deficiency and primary IgA nephropathy are reported. The C9 deficiency was discovered either through mass complement screening, or when low hemolytic activity for CH50 and normal C3 levels were detected in plasma. hematuria and proteinuria were detected at the age of 8 or 9 years as a result of annual urinary screening tests for school children. Renal biopsy showed focal and segmental mesangial proliferation with small epithelial crescents in one patient, and mild, diffuse mesangial proliferation in two. IgA and C3 were deposited predominantly in the mesangial area, and staining for C9 was negative in these patients. Electron microscopy revealed electron dense deposits predominantly in mesangial and paramesangial zones. Immunohistochemical staining in renal biopsy tissues from two patients showed mesangial staining for C5, C8, and S-protein, but staining for C5b-9 neoantigen was completely negative. These results show that the formation of C5b-9 complex is not essential for the induction of human IgA nephropathy, and also for the proliferation of mesangial and even parietal epithelial cells.
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9/36. IgA nephritis in HIV-positive patients: a new HIV-associated nephropathy?

    Four HIV-positive patients were shown to have IgA-associated nephritis on biopsy, including one with anaphylactoid purpura. Three were homosexuals, while the fourth acquired the infection from his mother. All had hematuria, a variable degree of proteinuria and renal disease with a benign course. Serologic studies showed elevated levels of IgA as well as IgA immune complexes and rheumatoid factor. IgA antibodies to multiple hiv antigens were detected by Western blot. Pathologic studies showed tubuloreticular inclusions in endothelial cells and nuclear bodies in interstitial cells in all cases. hiv antigens were not detected in kidney biopsies by monoclonal antibodies nor was HIV viral genome demonstrated by in situ hybridization. The possibility that this represents a unique type of IgA-associated HIV nephropathy is discussed.
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10/36. IgA-dominant glomerulonephritis associated with hepatitis a.

    Unlike hepatitis b and C, renal involvement has been extremely uncommon in patients with hepatitis Avirus (HAV) infection. nephrotic syndrome has been documented as a rare complication in association with HAV infection. In this report, we describe a patient with serologically documented HAV infection, who presented with nephrotic syndrome. The renal biopsy showed an immunoglobulin a- (IgA) dominant glomerulonephritis (GN) with subendothelial immune deposits. This is the second biopsy-proven case report of a patient with acute HAV associated with IgA-dominant immune complex glomerulonephritis and nephrotic syndrome. This is perhaps the first case in which a patient experienced both IgA-dominant glomerulonephritis and cutaneous cryoglobulinemic vasculitis.
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