1/61. 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) |
2/61. Pleural mesothelioma and membranous nephropathy.Underlying malignancy has been thought to be responsible for 5-10% of the cases of membranous nephropathy in adults, with the risk being highest in patients over the age of 60 years. Solid tumors such as carcinomas of lung or colon, are most often involved. It is presumed that tumor antigens are deposited in the glomeruli; this is followed by antibody deposition and complement activation, leading to epithelial cell and basement membrane injury and proteinuria due to the associated increase in glomerular permeability. We describe a patient with a resistant nephrotic syndrome and massive proteinuria due to membranous nephropathy associated with pleural mesothelioma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/61. nephrotic syndrome due to membranous nephropathy associated with metastatic prostate cancer: rapid remission after initial endocrine therapy.A case of severe nephrotic syndrome (urinary protein excretion 12.9 g/day) due to membranous nephropathy associated with untreated prostate cancer and multiple bone metastases is described. A combination of initial endocrine treatment and steroid therapy resulted in normalization of prostate-specific antigen levels followed by a rapid decrease of urinary protein excretion within 4 months. No proteinuria was subsequently detected. Seven months after the initiation of therapy, the patient remained well with complete clinical remission from the nephrotic syndrome. This rapid achievement of remission may have been due to tumor shrinkage by androgen ablation in addition to steroid therapy of the membranous nephropathy. The nephrotic syndrome is a rare complication of prostate cancer, and, to the best of our knowledge, no previous cases have been reported of membranous nephropathy as one of the first disease manifestations.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/61. Minimal change glomerulonephritis associated with hydatid disease.A 63-year-old man presented to our department with dyspnea and peripheral edema. A cystic mass in the right upper abdomen, consistent with echinococcal disease was discovered. proteinuria was also present, and a nephrotic syndrome was diagnosed. The kidney biopsy revealed minimal change glomerulonephritis. Treatment with the antiechinococcal drug albendazole induced complete remission of the nephrotic syndrome, suggesting an etiopathogenic role for a hydatid antigen in the development of an immune-mediated glomerulonephritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/61. Endocapillary proliferative glomerulonephritis in a patient with parvovirus B19 infection.A 45-year-old woman developed acute nephritic syndrome after erythema infectiosum. Laboratory data on admission showed decreased serum C3, C4, and CH50 levels and the presence of both immunoglobulin (Ig) M and IgG antibodies to human parvovirus B19 (HPV). A renal biopsy showed diffuse endocapillary proliferative glomerulonephritis. Immunofluorescence microscopy indicated 2 granular staining for IgG, IgM, and C3 over the mesangial area and along glomerular capillary walls. HPV antigen was also detected in glomeruli by immunohistochemistry. Electron microscopy showed electron-dense deposits in the subendothelial space and the paramesangial area. These findings suggest that immune complex-type glomerulonephritis is caused by glomerular deposition of HPV antigen-antibody complexes in some patients with HPV infection.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/61. Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant.A Japanese male infant presented with nephrotic syndrome at 41 days. His renal function progressively deteriorated, and he died at 4 months of the age. An open renal biopsy revealed diffuse crescentic glomerulonephritis (CrGN) without immune complex deposition, which is not characteristic of the congenital nephrotic syndrome (CNS). Examination for nephrin antigen using rabbit anti-nephrin extra- and intracellular site antibodies was positive. These clinical observations suggest that the patient had a unique histological variant of CNS. This is the first report of rapidly progressive, pauci-immune diffuse CrGN in infancy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/61. nephrotic syndrome with crescent formation and massive IgA deposition following allogeneic bone marrow transplantation for natural killer cell leukemia/lymphoma.We describe herein a case of nephrotic syndrome (NS) following allogeneic bone marrow transplantation (allo-BMT) for natural killer cell leukemia/lymphoma. Histologic studies defined the diagnosis as crescentic glomerulonephritis with massive immunoglobulin a (IgA) deposition, which has never been reported in NS cases following allo-BMT. Most of the massive infiltrated cells in the interstice were CD3( )CD4(-)CD8( ) T cells derived from the donor. We observed mesangial deposition of haemophilus parainfluenza outer membrane (OMHP) antigen and decreased glycosylation of the IgA1 hinge in the recipient's samples is consistent with the recently reported pathogenesis of IgA nephropathy. Further, the titer of IgA antibody against the donor serum was as high as other IgA nephropathy cases. These findings suggest that NS and crescentic glomerulonephritis in this case occurred as one of the forms of chronic graft-versus-host disease (GVHD), and that IgA deposition was associated with H parainfluenza and decreased glycosylation of the IgA1 hinge.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/61. HBV associated nephrotic syndrome: resolution with oral lamivudine.A 6 year old boy presenting with a five month history of fever, lethargy, and anorexia, was found to have hepatitis b associated membranous glomerulonephropathy and nephrotic syndrome. After two months treatment with oral lamivudine, his proteinuria cleared and serum albumin and aminotransferases normalised, associated with disappearance of hepatitis b e antigen (HBeAg) and appearance of anti-HBeAg antibodies. After 12 months, without side effects, lamivudine was discontinued. He remains well 11 months off treatment.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/61. Cerebral aspergillosis in an infant with corticosteroid-resistant nephrotic syndrome.Cerebral aspergillosis is a devastating disease in patients with a compromised immune system. A unique case of a male infant with corticosteroid-resistant nephrotic syndrome complicated by pulmonary and cerebral aspergillosis is described. The patient rapidly developed coma and neurological symptoms and died soon thereafter. central nervous system aspergillosis was diagnosed radiologically and by detection of aspergillus dna and antigen (galactomannan) in blood and cerebrospinal fluid. Moderate immunosuppressive therapy and antifungal phagocytic dysfunction due to nephrotic syndrome per se may have contributed to the occurrence of invasive aspergillosis in our patient. awareness of this serious complication and early application of diagnostic procedures and antifungal therapy may improve the dismal outcome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/61. nephrotic syndrome associated with fanconi syndrome. Immunopathogenic studies of tubulointerstitial nephritis with autologous immune-complex glomerulonephritis.The nature of renal lesions in a patient with simultaneous onset of the fanconi syndrome and nephrotic syndrome was investigated by immunologic studies of the patient's serum, cryoproteins, and renal tissue. Acute severe tubulointerstitial nephritis and generalized segmental glomerulonephritis were present. Renal tubular epithelial (RTE) antigen, IgG, and Clq were localized in the glomerull and proximal tubules. Cryoprecipitates containing RTE antigen and anti-RTE antigen were isolated from the patient's serum antibody to RTE antigen was detected in the serum of the patient. However, antibody to tubular basement membrane was not found in the cryoproteins or serum. The unusual simultaneous presentation of these two syndromes in our patient possibly represents a common etiology: tubular damage with release of RTE antigen and subsequent development of immune-complex glomerulonephritis mediated by renal tubular epithelial antigen and antibody to this antigen.- - - - - - - - - - ranking = 7keywords = antigen (Clic here for more details about this article) |
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