Cases reported "Proteinuria"

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1/14. Botryoid wilms tumor: case report and review of literature.

    A rare case of botryoid wilms tumor is presented. The main clinical manifestations were persistent low-grade fever, malaise, and proteinuria associated with microhematuria. ultrasonography revealed an echogenic mass in the right kidney, and a contrast-enhanced mass was found in the dilated collecting system by contrast-enhanced computed tomography. The surgically resected tumor was a polypoid, light-yellow, glistening mass that occupied a large part of the renal pelvis and originated from the pelvicaliceal wall. Part of the tumor extended to the proximal ureter, resulting in hydronephrosis in the involved kidney. No parenchymal lesion was observed. Microscopic examination revealed epithelial, stromal, and blastemal components, which indicated wilms tumor. infection had occurred in the hydronephrotic kidney, which presumably had caused the major presenting symptoms. The prognosis of our patient and previously reported cases of botryoid wilms tumor was good compared with that of typical wilms tumor, since the botryoid type can be detected at an early stage.
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2/14. Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

    We present two cases of the May-Hegglin anomaly discovered in a patient and one of her two sons. The female patient was known to have proteinuria from the age of 14 and was hospitalized in 1980, at the age of 25 years, because of hypertension and proteinuria (1.5 g/day). thrombocytopenia was found with an abundance of megakaryocytes in the bone marrow. Both steroid treatment and splenectomy failed to ameliorate the thrombocytopenia, thought to be due to idiopathic thrombocytopenic purpura. Progressive renal failure, secondary hyperparathyroidism and uremic osteodystrophy were diagnosed in 1995. In January 1996, when she was hospitalized because of high-grade fever, we saw giant platelets and prominent blue inclusion bodies in almost all granulocytes in the peripheral blood smear. Electron microscopy confirmed the diagnosis of May-Hegglin anomaly in this patient and one of her sons, who at that time showed thrombocytopenia but no renal disease. Three years later, however, at the age of 15, the affected son was found to develop proteinuria. Coexpression of the May-Hegglin anomaly and renal disease, reported previously in a few other patients, may in fact represent a new subentity.
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3/14. Delayed onset of systemic lupus erythematosus in a child with endothelial tubuloreticular inclusion.

    We report here on an 11-year-old Japanese girl who was found to have proteinuria by routine mass screening urinalysis for school children, and who developed systemic lupus erythematosus (SLE) 21 months later. The initial renal biopsy, performed 3 months after the first visit to tokyo Medical University Kasumigaura Hospital (TMUKH), revealed membranous glomerulonephritis. In an immunofluorescent study, IgG was the only positive immunoglobulin found. A "full-house" immunofluorescence glomerulopathy, well known as a predictive finding for lupus nephritis, was not detected. Endothelial tubuloreticular inclusions (ETI) were found by electron microscopy. Because the diagnosis of SLE was not established clinically and serologically, the patient was followed every 3 months without drugs. Her urinary findings returned to normal within 18 months. Three months after the last visit, she was sent to Tsukuba University Hospital (TUH) for fever, arthralgia, dyspnea and butterfly rash. She was diagnosed as having SLE, pleuritis, and pericarditis. Although she was treated with methylpredonisolone and oral prednisolone, she developed cardiac tamponade on the 12th day of admission during the course of pneumococcal septicemia. Finally, she was treated successfully with surgical procedures, antibiotics and oral prednisolone and was discharged. We conclude that ETI is a more significant early sign of SLE than "full-house" immmunofluorescence glomerulopathy, especially in pediatric cases.
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4/14. Collapsing glomerulopathy: a cause of noncardiogenic pulmonary edema.

    Collapsing glomerulopathy is a pathologic diagnosis characterized by obliteration of glomerular capillary lumina, seen most commonly as a primary glomerular disease in young black men. A secondary form with almost identical pathologic features is described in association with human immunodeficiency virus infection. The disease is characterized by heavy proteinuria with variable renal insufficiency at the onset followed by rapid progression to end-stage renal disease with no documented effective therapy. We describe a patient who presented with systemic manifestations, including fever, acute renal failure with massive proteinuria, and noncardiogenic pulmonary edema. Renal biopsy showed classic collapsing glomerulopathy. All known causes of noncardiogenic pulmonary edema were ruled out. The pulmonary syndrome resolved, but the renal disease progressed to end-stage renal disease. We propose consideration of collapsing glomerulopathy in the differential diagnosis of any patient presenting with a multisystem disease including acute renal failure and pulmonary edema.
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5/14. Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks.

    Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increased serum LDH. Moreover, a chest CT evidenced bilateral multiple pulmonary infiltrates and pleural effusion. After the development of proteinuria, a diagnosis of large B-cell intravascular lymphoma was made with a renal biopsy 10 months after the onset of the clinical manifestations. So far, more than 100 cases of IL have been reported and the diagnosis often turned out to be difficult, as clinical signs did not point to a lymphoproliferative disorder. This report confirms that FUO is not only frequently associated with IL but that it even marks the real onset of the disease. We are then tempted to conclude that undiagnosed fever is not so rare in IL and if we call it FUO, it is only because diagnosis is necessarily elusive and hence time-consuming.
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keywords = fever
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6/14. Collapsing glomerulopathy in adult still's disease.

    Idiopathic collapsing glomerulopathy is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis characterized clinically by a male and Afro-Caribbean racial predominance, proteinuria (often nephrotic range), and rapid progression to end-stage renal failure. Pathologically, the typical changes are global glomerular collapse leading to obliteration of glomerular capillary lumina, hypertrophy and hyperplasia of podocytes, and severe tubulointerstitial changes. A secondary form with almost identical pathologic features is described in association with human immunodeficiency virus infection. We describe a female patient who presented with multisystemic manifestations, including high spiking fever, arthralgias, lymphadenopathy, striking hyperferritinemia, and impaired renal function with proteinuria. Renal biopsy showed classic collapsing glomerulopathy. A diagnosis of adult Still's disease was made on the basis of Yamaguchi's criteria. The patient was treated with steroids, resulting in remission of the rheumatological condition closely paralleled by remission of proteinuria and renal function, thereby strongly suggesting a causative link between adult Still's disease and collapsing glomerulopathy in this patient. We propose that collapsing glomerulopathy ought to be considered in adult Still's disease with unexplained renal insufficiency or proteinuria.
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7/14. hantavirus Puumala infection as a cause of fever of unknown origin in a child.

    hantavirus infection due to puumala virus causes nephropathica epidemica, a disorder characterized by fever, haematological abnormalities, mild renal dysfunction and ophthalmological abnormalities. The prevalence in most European countries is low, but the virus can be endemic. In children, hantavirus infection is rare. This paper describes a young girl diagnosed with hantavirus infection. The patient presented with high fever, proteinuria, haematuria and eye lesions, but other typical hallmarks of the disease, such as thrombocytopenia and renal dysfunction, were absent. This case report demonstrates the need to consider the diagnosis of hantavirus infection in children with prolonged fever of unknown origin. The diagnosis is based on serological tests.
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ranking = 7
keywords = fever
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8/14. Aural involvement in loxoscelism: case report and literature review.

    An 11-year-old male presented with fever, rash, and a necrotic lesion on the lobule of the left ear. The lesion became tender and formed an eschar over 4 days. The patient developed leukocytosis, hemolytic anemia, and proteinuria, and was diagnosed with systemic loxoscelism from a brown recluse spider bite. He was managed with supportive therapy and improved in 4 days. Loxoscelism is a clinical diagnosis which should be suspected in an otherwise healthy patient with a necrotic wound, particulary in the endemic Southern and midwestern united states. physicians should be aware of this disease entity and its complications.
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9/14. Acute human immunodeficiency virus infection temporally associated with rhabdomyolysis, acute renal failure, and nephrosis.

    A previously healthy 29-year-old homosexual man presented with a 4-day history of fever, malaise, sore throat, and bleeding gums. rhabdomyolysis, acute renal failure, and nephrotic range proteinuria were also present. The patient was found to have acute human immunodeficiency virus (hiv) infection confirmed by the presence of hiv antigen in his serum and subsequent evolution of an hiv antibody profile typical of acute seroconversion. A kidney biopsy revealed acute tubular necrosis and mesangioproliferative glomerulonephritis, with tubuloreticular inclusions. In the presence of otherwise unexplained acute renal failure, rhabdomyolysis, or new onset nephrotic syndrome, acute hiv infection should be considered in the differential diagnosis.
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ranking = 1
keywords = fever
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10/14. Remission of progressive renal failure in familial mediterranean fever during colchicine treatment.

    colchicine was administered to a 12 year old girl with familial mediterranean fever and progressive renal insufficiency. There was immediate resolution of abdominal attacks together with a dramatic fall in the serum creatinine concentration and the degree of proteinuria. At the same time her severely impaired growth was stimulated.
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keywords = fever
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