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1/100. Acute renal failure in an adult patient with Henoch-Schoenlein purpura after episode of macroscopic hematuria.

    A case of severe prolonged acute renal failure with a histological picture of acute tubulointerstitial lesions in an adult patient with Henoch-Schoenlein purpura after an episode of macroscopic hematuria is described. The macroscopic hematuria lasted only for 5 days and the renal biopsy was performed 50 days after the end of the macroscopic hematuria. Restoration of renal function was not complete six months after the beginning of improvement. Fewer than 65 cases of acute renal failure due to tubulointerstitial nephritis in patients with glomerulonephritis and after episode of macroscopic hematuria have been described in the international literature. Only one of these patients was suffering from Henoch-Schoenlein purpura.
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ranking = 1
keywords = nephritis, interstitial
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2/100. Successful treatment of adult-onset Henoch-Schonlein purpura nephritis with high-dose immunoglobulins.

    A 26-year-old woman was admitted for the evaluation of edema and massive proteinuria. She had a history of purpura of the lower extremities, abdominal pain and melena. Laboratory investigations showed hypoalbuminemia, hypercholesterolemia and proteinuria of over 10 g/day. Renal biopsy showed moderate proliferative glomerulonephritis with mesangial immunoglobulin a (IgA) deposition. She was diagnosed as having Henoch-Schonlein purpura nephritis. Oral prednisolone, dipyridamole and intravenous heparin treatment were not effective. Steroid pulse therapy induced a partial improvement of proteinuria to 2-3 g/day. High-dose intravenous immunoglobulin (i.v.-IG) treatment was introduced and a dramatic improvement of proteinuria was noted. I.v.-IG should be fully considered in patients with steroid-resistant Henoch-Schonlein purpura nephritis.
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ranking = 3.4992646956868
keywords = nephritis
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3/100. glomerulonephritis without IgA deposits in a case of Henoch-Schonlein purpura.

    A boy aged 3 years 8 months with Henoch-Schonlein purpura (HSP) developed significant proteinuria with hematuria 2 days after the appearance of purpura rash. Although thought to be purpura nephritis, a percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis (MesPGN) without deposition of immunoglobulin a or complement. Since his urine screening test during a health check at the age of 3.5 years had been unremarkable, HSP might have played a role in the pathogenesis of his non-IgA MesPGN. To our knowledge, non-IgA MesPGN is an uncommon renal manifestation of HSP.
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ranking = 2.9993697391601
keywords = nephritis
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4/100. Low serum C3, leukopenia, and thrombocytopenia: unusual features of henoch-schonlein purpura.

    Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP.
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ranking = 0.49989495652669
keywords = nephritis
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5/100. systemic vasculitis and atypical infections: report of two cases.

    Two cases of systemic vasculitis are described; one presenting with adult Henoch-Schonlein purpura secondary to a concomitant chlamydia infection and the other with leucocytoclastic vasculitis and mesangioproliferative glomerulonephritis secondary to a recent parvovirus B19 infection. association of chlamydial infection has not previously been described with Henoch-Schonlein purpura and this infection should, perhaps, be added to the list of aetiologies of this disease. parvovirus B19 causing significant urinary sediment abnormalities associated with mesangioproliferative glomerulonephritis and leucocytoclastic vasculitis has also not been described previously.
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ranking = 0.99978991305337
keywords = nephritis
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6/100. Relapsing Henoch-Schonlein purpura associated with pseudomonas aeruginosa pyelonephritis.

    Henoch-Schonlein purpura is a systemic vasculitis of unknown cause. It is frequently triggered by a streptococcal upper respiratory tract infection. Other bacteria have been implicated as triggering agents. We report a recurring case of Henoch-Schonlein purpura in a patient with Pseudomonas pyelonephritis. The Henoch-Schonlein purpura remitted only when the infection was eradicated. Pseudomonas infection should be added to the list of bacteria that can trigger Henoch-Schonlein purpura.
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ranking = 2.4994747826334
keywords = nephritis
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7/100. Cardiopulmonary manifestations of Henoch-Schonlein purpura.

    Henoch-Schonlein purpura (HSP) is usually a mild condition involving the skin, gut, joints, and kidneys and has a good prognosis. We present a 63-year-old Hispanic man who had an unusually severe form of HSP with a fatal outcome attributable to vasculitis causing myocardial necrosis. There is only one citation in the literature of HSP-related myocardial vasculitis, which involved the right ventricle and was successfully treated with steroids. Our patient had severe HSP-related myocardial necrosis, tracheobronchitis, and nephritis. The bronchial lesions resolved, presumably because of steroid therapy. This probably is the first case of fatal myocardial necrosis related to HSP. We conclude that HSP can, in some cases, have an aggressive course. It becomes imperative to recognize the involvement of the other organ systems, such as the heart, so that appropriate therapy may be initiated. immunosuppression may have a beneficial effect on extrarenal lesions. Controlled clinical trials are needed to establish the efficacy of such treatment.
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ranking = 0.49989495652669
keywords = nephritis
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8/100. Non-Q wave acute myocardial infarction in acute meningococcemia in a 10-year-old girl.

    INTRODUCTION: Children with acute meningococcemia may have impaired myocardial function resulting in low cardiac output despite normal intravascular volume. Severe meningococcal infection has been associated with acute interstitial myocarditis, endocarditis, and pericarditis, but not with myocardial infarction. CASE: We present the case of a 10-year-old girl with positive family history for premature myocardial infarction who sustained an acute myocardial infarction temporally related to meningococcemia. DISCUSSION: This is the first pediatric case of non-Q wave acute myocardial infarction associated with purpura fulminans in meningococcemia. Similarly, the association of high troponin i levels and meningococcemia has not been described previously. Although, the patient's genetic predisposition for myocardial infarction might have been a potential contributing factor, there was no angiographic evidence of coronary artery disease in this patient. Thereby, other factors related to shock, endotoxin, microthrombi of meningococcemia, and their treatment might have been also contributing. We propose possible mechanisms for this rare but serious complication of meningococcemia and review the literature.
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ranking = 0.0001050434733136
keywords = interstitial
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9/100. IgA myeloma presenting as Henoch-Schonlein purpura with nephritis.

    IgA nephropathy (IgAN) and Henoch-Schonlein purpura (HSP) are both characterized by IgA-mediated tissue injury, including mesangial proliferative glomerulonephritis. Abnormalities of IgA1 glycosylation are described in IgA nephropathy and HSP nephritis. IgA-antineutrophil cytoplasmic antibodies (ANCA) have been inconsistently described in the serum of patients with HSP. In IgA myeloma, the paraprotein-mediated renal lesion is typically cast nephropathy; IgAN or HSP have only rarely been reported in myeloma even when an IgA paraprotein is circulating in large concentrations. We report the case of a 50-year-old man with IgA myeloma who presented with HSP including nephritis and rapidly progressive renal failure. His IgA1 had altered O-glycosylation in the pattern seen in IgAN and also contained an IgA-ANCA. This case adds further weight to the evidence that IgA1 O-glycosylation abnormalities predispose to mesangial IgA deposition and also that IgA-ANCA may have a pathogenic role in the development of HSP.
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ranking = 3.4992646956868
keywords = nephritis
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10/100. Cerebral vasculitis in Henoch-Schonlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone.

    Neurological complications are rare during the course of Henoch-Schonlein purpura (HSP). We report a 7-year-old girl with HSP who presented with seizures, loss of vision and disturbance of consciousness. magnetic resonance imaging (MRI) showed high signal intensity in the gray and white matter over the left parietal and both occipital lobes, compatible with MRI findings of cerebral vasculitis. The eye fundi revealed multiple branches of retinal artery occlusion. Intravenous pulse methylprednisolone (MTP) followed by oral steroid therapy was initially administered for HSP nephritis. Cerebral vasculitis developed 10 days post-MTP treatment, with progressive worsening of consciousness. Oral steroid was discontinued and plasmapheresis was performed alone. Her level of consciousness dramatically improved after plasmapheresis. The brain MRI and eye fundi findings were consistent with her clinical improvement. To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP.
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ranking = 0.49989495652669
keywords = nephritis
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