Cases reported "Nephritis"

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1/4. Preoperative diagnosis of xanthogranulomatous pyelonephritis.

    Preoperative diagnosis of xanthogranulomatous pyelonephritis may be correctly made in a significant proportion of affected patients thus preventing unnecessary radical surgery especially in the poor-risk patient. The diagnosis should be suggested in the patient with a history of chronic urinary tract infection and certain radiologic features. These include unilateral renal enlargement (either localized or diffuse), nonfunction on excretory urography, presence of renal and/or ureteral calculi, angiographic demonstration of avascular mass or masses with stretched, attenuated intrarenal vessels, prominent capsular and periureteric vessels, and an irregular impaired nephrogram with prominent avascular areas.
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2/4. Acute glaucoma in hemorrhagic fever with renal syndrome (nephropathia epidemica).

    Bilateral eyelid edema, chemosis, conjunctival injection and hemorrhages, shallowing of the anterior chamber with transitory myopia, and acute glaucoma were observed in three patients with hemorrhagic fever with renal syndrome (nephropathia epidemica). One patient had anterior uveitis with posterior synechiae. Two patients had congestion of retinal venules and retinal edema, and one patient had small retinal hemorrhages. fluorescein angiography revealed early leakage of the conjunctival capillaries and delayed filling of the lower retinal arterioles with retinal edema. The acute glaucoma subsided rapidly. The myopia disappeared slowly with a gradual deepening of the anterior chamber after onset of the diuretic phase of the illness. In nephropathia epidemica, the transitory shallowing of the anterior chamber, myopia, and acute glaucoma are due to lesions in the small blood vessels of the ciliary body, resulting in its swelling, with relaxation of the zonules and forward movement of the lens.
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3/4. Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.

    A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.
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4/4. Successful treatment of progressive Henoch-Schonlein purpura nephritis with tonsillectomy and steroid pulse therapy.

    Henoch-Schonlein purpura (HSP) is a systemic disorder characterized by a leukocytoclastic vasculitis involving small vessels with the deposition of IgA immune complexes. The renal involvement is the major cause of morbidity and mortality in patients with HSP. We report here an adult patient with HSP nephritis (HSPN) accompanied by persistent proteinuria and progressive renal dysfunction despite conventional therapy. The patient was successfully treated with tonsillectomy followed by intravenous pulse methylprednisolone and oral prednisone. The combination therapy resulted in a significant decrease in proteinuria, improvement of renal function and the disappearance of microhematuria. The patient finally reached a stage of clinical remission.
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