Cases reported "Nephrotic Syndrome"

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1/42. Recurrent type I membranoproliferative glomerulonephritis in a renal allograft: successful treatment with plasmapheresis.

    Recurrent disease is increasingly recognized as a cause of renal allograft dysfunction and failure. We describe a patient with type I membranoproliferative glomerulonephritis not associated with hepatitis c. The glomerular disease recurred in the renal allograft within 1 month of transplantation, leading to acute allograft dysfunction and nephrotic syndrome. Aggressive treatment with prednisone and plasmapheresis resulted in improvement in kidney function, improvement of the light microscopic picture, and removal of immune complexes from the glomerular subendothelial space.
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ranking = 1
keywords = hepatitis
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2/42. Cryofiltration and oral corticosteroids provide successful treatment for an elderly patient with cryoglobulinemic glomerulonephritis associated with hepatitis c virus infection.

    A 75-year-old man was admitted due to nephrotic syndrome, purpura on the legs, which was associated with hepatitis c virus (HCV), and type II mixed cryoglobulinemia. Renal biopsy revealed features of cryoglobulinemic glomerulonephritis. Since the patient was elderly and the HCV genotype was Ib, interferon-alpha for reducing HCV was not indicated. Four sessions of cryofiltration and the administration of corticosteroids improved the proteinuria and renal function strikingly without adverse effects. This case demonstrates that an elderly patient who has nephrotic syndrome caused by cryoglobulinemic glomerulonephritis associated with HCV can be treated safely by cryofiltration with low doses of oral corticosteroids.
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ranking = 5
keywords = hepatitis
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3/42. interferon-alpha in combination with ribavirin as initial treatment for hepatitis c virus-associated cryoglobulinemic membranoproliferative glomerulonephritis.

    Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis c virus (HCV) infection. In HCV-infected patients with MC, renal involvement worsens the overall prognosis because of a high incidence of infection or cardiovascular disease. The relationship between MC and HCV infection has prompted the use of antiviral therapy. Two patients with chronic HCV infection, type-II MC and membranoproliferative glomerulonephritis (MPGN), presenting as nephrotic syndrome were treated with interferon (IFN)-alpha (3 MU 3 times per week) and ribavirin (15 mg/kg daily) for 6 months. Laboratory tests included measurement of anti-HCV antibodies, HCV rna, and HCV genotyping, and characterization of circulating cryoglobulins. A pretreatment renal biopsy was performed, and the histopathologic lesions were scored according to the index of disease activity. viremia and cryoglobulinemia were suppressed in both patients. However, a complete remission of proteinuria was observed in 1 patient only. The evaluation of the renal biopsy specimens revealed a mild MPGN (activity score: 5/24) in the patient with remission of proteinuria and a severe MPGN (activity score: 15/24) in the patient who maintained a nephrotic-range proteinuria. Although a fully satisfactory treatment is not yet available, we feel that a reasonable therapeutic strategy for HCV-infected patients with MC nephritis could be as follows: (1) antiviral treatment alone for patients with a low-grade kidney involvement, and (2) a short-term course of steroids and cytotoxic drugs followed by antiviral therapy for acute exacerbations and/or rapidly progressive GN.
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ranking = 5
keywords = hepatitis
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4/42. Glomerular involvement in myelodysplastic syndromes.

    Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
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ranking = 1
keywords = hepatitis
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5/42. Nephrotoxicity of interferon alfa-ribavirin therapy for chronic hepatitis c.

    The nephrotoxicity associated with interferon therapy for chronic hepatitis c infection has not been clearly defined. We describe a patient with chronic hepatitis c infection who developed the nephrotic syndrome during treatment with interferon and ribavirin. Renal biopsy revealed focal segmental glomerulosclerosis. She had a virologic and biochemical response to the antiviral therapy, and the nephrotic syndrome improved after termination of antiviral therapy. We place our case report in context with a review of the literature on nephrotoxicity associated with interferon therapy. Because our patient had no other obvious reason for the nephrotic syndrome, we are postulating that it may be secondary to interferon-ribavirin therapy. The temporal relation between the administration of the drug and the detection of toxic affects (nephrotic syndrome) and subsequent improvement upon withdrawal also supports a causative role for interferon-ribavirin. Although nephrotoxicity is rare, it should be emphasized that it can occur anytime after the start of interferon therapy, and physicians treating patients with chronic hepatitis c must be aware of this idiosyncratic, unpredictable, and potentially serious adverse event.
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ranking = 7
keywords = hepatitis
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6/42. role of ribavirin in membranoproliferative glomerulonephritis associated with hepatitis c virus infection refractory to alpha-interferon.

    Chronic hepatitis c virus (HCV) has been associated with several extrahepatic diseases, such as membranoproliferative glomerulonephritis (MPGN). alpha-Interferon is currently the treatment of choice for this association. When this therapy fails clinicians face a difficult challenge due to the lack of useful information in these particularly difficult patients. We report the case of a severe nephrotic syndrome due to MPGN associated HCV infection, in which a triple association--interferon plus ribavirin and cyclophosphamide--was needed to control the disease.
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ranking = 5
keywords = hepatitis
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7/42. Acute onset of nephrotic syndrome during interferon-alpha retreatment for chronic active hepatitis c.

    A 57-year-old woman was scheduled to receive recombinant interferon-alpha retreatment for chronic active hepatitis c. During the course of therapy, the patient showed rapid onset of oliguria, dizziness, edema, and a pre-shock state. She was subsequently admitted to hospital and was diagnosed as having nephrotic syndrome. After admission, albumin-dominant proteinuria persisted despite the discontinuation of interferon therapy. light microscopy of a renal needle biopsy specimen showed interstitial lymphoid cell infiltration, but no marked changes of the glomeruli and no staining for immunoglobulin or complement. Electron microscopy showed diffuse effacement of the glomerular epithelial foot processes, leading to a diagnosis of minimal change nephrotic syndrome with interstitial nephritis. proteinuria resolved after the initiation of oral prednisolone therapy (1 mg/kg per day). The number of patients with chronic hepatitis c requiring interferon retreatment is increasing rapidly. We herein report this rare case of acute onset of nephrotic syndrome during interferon-alpha retreatment.
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ranking = 6
keywords = hepatitis
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8/42. pityriasis rosea-like eruption due to pneumococcal vaccine in a child with nephrotic syndrome.

    A pityriasis rosea-like eruption can occur as a consequence of treatments with gold compounds and captopril. It has rarely been reported to have an association with vaccinations such as smallpox, BCG, hepatitis b, and diphtheria toxoid. It has not previously been documented to develop after pneumococcal vaccination. We report a case of pityriasis rosea-like eruption that developed following pneumococcal vaccination in a child with nephrotic syndrome.
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ranking = 1
keywords = hepatitis
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9/42. 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.
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ranking = 2
keywords = hepatitis
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10/42. Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins.

    The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.
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ranking = 1
keywords = hepatitis
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