1/67. IgA antibasement membrane nephritis with pulmonary hemorrhage.Goodpasture's syndrome has characteristically been described as being mediated by IgG antibodies. We have recently seen a 55-year-old man who developed renal failure and hemoptysis; a renal biopsy showed linear deposits of IgA and C3 involving glomerular and tubular basement membrane. serologic tests for detecting (IgG) antiglomerular basement membrane antibodies were negative. Elution studies of kidney and lung showed the presence of an IgA antibasement membrane antibody only. The patient's serum contained IgA, but not IgG, antibodies reactive with glomerular and tubular basement membrane of normal human kidney and alveolar basement membrane of normal human lung. Attempts to transfer disease with the patient's IgA antibody to a monkey and to Lewis and Brown-norway rats were unsuccessful. immunoglobulin a antibasement membrane antibody must be considered in the design of immunoserologic procedures for the diagnosis of Goodpasture's syndrome.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/67. Coexistence of Fabry's disease and necrotizing and crescentic glomerulonephritis.The coexistence of Fabry's disease, an X-linked hereditary disease, and other renal diseases, has rarely been described in the same patient. Combined Fabry's disease and pauci-immune necrotizing and crescentic glomerulonephritis (NCGN) is hitherto unreported. We present the clinical and pathologic data of two patients with combined Fabry's disease and NCGN. Both patients presented with fevers of unknown origin and progressive renal insufficiency, however, lacked any other pathognomic signs of Fabry's disease such as acroparesthesias, dyshidrosis, and cutaneous angiokeratomas. The possible pathogenic mechanisms and causal relationship between the two disease processes are discussed.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
3/67. Fibrillary glomerulonephritis associated with crescents as a therapeutic challenge.Most cases of fibrillary glomerulonephritis (FG) terminate in end-stage renal disease within a few years. We report on two female patients (41 and 50 years old) with the diagnosis of FG associated with crescentic glomerulonephritis, a combination found in 20% to 25% of cases of FG. A broad spectrum of infectious disease and systemic immunologic disorders could be ruled out by specific assays. Both patients had severely impaired renal function, nephrotic syndrome, and hypertension. Based on the biopsy finding with necrotizing FG, treatment was started with corticosteroids and cyclophosphamide. In both patients, renal function recovered markedly within 6 months of treatment, in one case remaining stable for 3.5 years. Whether or not cyclophosphamide treatment changed the course of the FG itself or counteracted the acute crescentic process cannot be determined from these two patients. Based on these promising preliminary findings and the poor prognosis of FG, however, we recommend cyclophosphamide treatment of patients with FG and additional crescentic glomerulonephritis. For a systematic evaluation of the therapeutic options in FG, a multicenter clinical trial should be conducted.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
4/67. Successful simultaneous renal transplantation and abdominal aortic aneurysmectomy.At the time of related donor renal transplantation, a 49-year-old man with chronic glomerulinephritis was found to have a large fusiform aneurysm involving the internal and external iliac arteries, the abdominal aorta, and both common iliac arteries. transplantation and abdominal aneurysmectomy using a standard Dacron bifurcation graft were successfully carried out. This patient has had no associated complications and is currently five years after transplantation and aneurysmectomy, with excellent renal function. It is believed that transplantation may now be offered to an older age group of patients with end-stage renal disease in whom atherosclerosis wll have developed as a natural process of aging.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
5/67. Simultaneous occurrence of perimembranous glomerulonephritis and glomerular amyloidosis.The results of electron microscopic examination of renal biopsies from 3 patients with rheumatoid arthritis treated with penicillamine are presented. All 3 patients developed a nephrotic syndrome upon discontinuation of penicillamine therapy. When viewed with the electron microscope, segmental forms of perimembranous glomerulonephritis (Stages I-II of Ehrenreich and Churg) and glomerular renal amyloidosis Grade I-III were observed. In all three cases the nephrotic syndrome was considered to be due to the simultaneous occurrence of the two disease processes. In 2 cases perimembranous glomerulonephritis with immuno-complex-deposits was assumed to be the dominant factor in the causation of the disease, in the other case amyloidosis was the principle abnormality.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
6/67. Azurocidin-specific-ANCA-related idiopathic necrotizing crescentic glomerulonephritis.An 80-year-old woman who had rapidly progressive glomerulonephritis unaccompanied by systemic vasculitis is described. On renal biopsy, she showed necrotizing crescentic glomerulonephritis by light microscopy and pauci-immune glomerular lesions by immunofluorescent study. No dense deposits were present on electronmicroscopic study. On serum examination, indirect immunofluorescent study showed perinuclear pattern antineutrophil cytoplasmic antibody (ANCA), but myeloperoxidase-ANCA and proteinase 3-ANCA were both negative. Her serum reacted only to azurocidin excluding other ANCA antigens: bactericidal permeability-increasing protein, cathepsin g, elastase, lactoferrin, or lysozyme. serum creatinine level decreased, and c-reactive protein turned negative after steroid therapy. Azurocidin-ANCA also turned negative. It is suggested that azurocidin-ANCA might have been related to the inflammatory process of pauci-immune necrotizing crescentic glomerulonephritis in this patient.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
7/67. Extra corporeal membrane oxygenation and plasmapheresis for pulmonary hemorrhage in microscopic polyangiitis.Early initiation of extracorporeal membrane oxygenation to treat acute hypoxemic respiratory failure secondary to massive pulmonary hemorrhage in microscopic polyangiitis in children can be life-saving while awaiting control of the autoimmune disease process by plasmapheresis and immunosuppression.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
8/67. A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosis.We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone. He was admitted to our hospital on November 22 following the appearance of bilateral leg edema in October 2000. Laboratory tests at presentation revealed nephrotic syndrome with renal impairment. Renal biopsy specimens revealed necrotizing glomerulonephritis with crescent, but immunofluorescence study showed lack of staining for immunoglobulins or complement components. Chest X-ray and CT showed abnormal shadows in the right upper lung field, and cryptococcus neoformans was isolated in a transbronchial lung biopsy. After the diagnosis of pulmonary cryptococcosis was made, the patient was treated with 200 mg/day fluconazole. The pulmonary abnormal shadows immediately improved and urinary protein excretion dramatically decreased. A second renal biopsy, performed about 2 months after the first biopsy, showed disappearance of crescent. Electron microscopic examination of the second renal biopsy showed partial effacement of foot processes without electron-dense deposits. Our findings suggest that necrotizing glomerulonephritis with nephrotic syndrome in this patient represented pauci-immune T-cell-mediated injury related to pulmonary cryptococcosis.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
9/67. diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener's granulomatosis.We report a case of a 47-year-old woman with Wegener's granulomatosis complicated by central diabetes insipidus. The patient had initially seronegative polyarthritis which mostly responded well to methotrexate and steroid therapy. Eight months later the patient suffered from polyuria and polydipsia. There were no abnormalities of the anterior pituitary hormones. MR images showed only loss of brightness of the posterior pituitary. Extensive evaluation of the patient revealed the presence of ANCA, in c-ANCA pattern and also PR3 positivity. Three months later findings of glomerulonephritis, as suggested by an active urine sediment and gradual proteinuria, and, finally, asymptomatic pulmonary nodules completed the clinical picture of Wegener's disease within 1 year. Renal biopsy showed crescent formation in two glomeruli, consistent with ANCA-related glomerulonephritis which showed pauci-immun depositions by direct immunofluorescence. diabetes insipidus symptoms mostly regressed; renal and pulmonary findings completely disappeared with glucocorticoid and pulse cyclophosphamide treatment. These findings show that diabetes insipidus may rarely develop early in the disease process and ANCA positivity was directly indicative of Wegener's granulomatosis before the classic clinical signs of the disease.- - - - - - - - - - ranking = 0.0069147407082427keywords = process (Clic here for more details about this article) |
10/67. autopsy case of microscopic polyangiitis with crescentic glomerulonephritis and necrotizing pancreatitis.Herein is reported the case of an 84-year-old woman who initially manifested rapidly progressive glomerulonephritis following a urinary tract infection. Laboratory findings showed a high titer of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Treatment with high-dose i.v. steroids resulted in clinical recovery and an undetectable MPO-ANCA titer. Two months later the patient was readmitted in a state of severe shock. Laboratory examination showed the deterioration of renal function, leukocytosis, and coagulation abnormalities consistent with disseminated intravascular coagulation (DIC). The patient died 12 days later. The post-mortem examination revealed necrotizing pancreatitis due to acute-stage vasculitis typified by fibrinoid necrosis of the arterioles and venules, and crescentic glomerulonephritis with healed-stage vasculitis. In the lungs, capillaritis with diffuse alveolar hemorrhage was not evident, but arteriolitis and phlebitis were occasionally seen. This case represents an unusual complication of necrotizing pancreatitis in the setting of microscopic polyangiitis. Thus, it is important to consider reactivation independent of the titer of ANCA in the course of the disease.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
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