Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 71-year-old man, who had been treated for hypertension, myocardial infarction and abdominal aortic aneurysm, and was admitted to our hospital because of proteinuria(3.9 g/day at the outpatient clinic and 1.5 g/day at the time of admission) and edema in the extremities. Light microscopic study of the kidney biopsy specimen revealed mesangial proliferative glomerulonephritis and glomerular paralysis. Electron microscopic findings showed endothelial damage, including widening of the subendothelial space and detachment of endothelial cells from the glomerular basement membrane. Deposition of immunoglobulins and complement was not detected by immunofluorescence studies. These pathological findings resemble the findings of thrombotic microangiopathy, but there were no clinical pictures of HUS/TTP. These findings suggest that hypertension, atherosclerosis and circulating turbulence caused by an aortic aneurysm induced severe glomerular endothelial damage leading to mesangial proliferative glomerulonephritis without an immune response.
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PMID:[A case of mesangial proliferative glomerulonephritis with endothelial damage]. 1247 92

Thrombotic thrombocytopenic purpura and adult hemolytic-uremic syndrome (TTP/HUS) have a substantial mortality rate even with currently available treatments. Although therapeutic plasma exchange is the recommended treatment of TTP/HUS, this cumbersome procedure may not be available for all patients in an emergency. In this context, plasma infusion may represent an alternative first-line therapy. We compared the effectiveness of high-dose plasma infusion (25-30 mL/kg per day) and therapeutic plasma exchange as first-line treatment of adult TTP/HUS at a single center. Two groups of patients with TTP/HUS were identified according to their initial therapy, that is, high-dose plasma infusion (19 patients) and therapeutic plasma exchange (18 patients). Clinical charts and outcomes were retrospectively analyzed. Endpoints for comparison were the duration of platelet counts below 150 x 10 /L and LDH levels above normal values; the volumes of plasma administered and the duration of treatment; complete remission, relapse, and mortality rates; and treatment-related complications. Patients of the 2 groups had comparable clinical and laboratory features on admission. Sixteen patients achieved complete remission in each group. Median times to recovery of platelet counts and LDH levels were comparable between the 2 groups. Eight patients in the high-dose plasma infusion (HD-PI) group were switched to therapeutic plasma exchange because of fluid overload (6 patients), persistent biologic disturbances (1 patient), or unresponsiveness to high-dose plasma infusion treatment (1 patient). This latter patient had severe TTP/HUS that remained refractory to therapeutic plasma exchange and vincristine, and rapidly died. All 7 remaining patients achieved complete remission with therapeutic plasma exchange. Four patients in the HD-PI group and 3 patients in the therapeutic plasma exchange (TPE) group died. In the HD-PI group, 5 patients experienced a transient nephrotic-range proteinuria during treatment. Main complications in the TPE group were collapse (1 patient) and central venous catheter infection (2 patients) or thrombosis (1 patient). Three patients in each group relapsed. High-dose plasma infusion may be an efficient treatment of TTP/HUS in patients who cannot have early plasma exchange. However, the large volumes of plasma required to reach complete remission may result in fluid overload, which may necessitate subsequent therapeutic plasma exchange.
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PMID:High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome. 1254 8

Three patients (one with idiopathic thrombocytopenic purpura [ITP] and two with thrombotic thrombocytopenic purpura [TTP]) were treated with rituximab (anti-CD20 chimeric antibody) at a dose of 325 mg/m2 administered weekly after they failed standard therapies. The patient with ITP who did not respond to steroids and anti-D antibody administration achieved augmentation of her platelet counts up to 180 x 10(3)/microL after four doses of rituximab. Six months later, when her counts started to decrease, she received maintenance therapy with an additional course of 4 standard doses of antibody that resulted in consolidation of her platelet counts around 100 x 10(3)/microL. One patient with TTP and concurrent idiopathic nephropathy who was previously treated with plasmapheresis, steroids, and vincristine improved only after 4 weekly administrations of the antibody. Moreover, his nephrotic-range proteinuria resolved after he received rituximab. The other patient with chronic TTP who still relapsed after splenectomy received 5 doses of rituximab with concomitant plasmapheresis. His thrombocytopenia improved slowly, and his platelet count stabilized at 300 x 10(3)/microL. All three patients showed evidence of response to anti-CD20 antibody with improvement in clinical outcome as well as augmentation of platelet counts to normal levels. We conclude that rituximab is a useful immunomodulating adjunct in the treatment of refractory ITP and TTP.
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PMID:Rituximab for the treatment of refractory idiopathic thrombocytopenic purpura (ITP) and thrombotic thrombocytopenic purpura (TTP): report of three cases. 1560 92

Co-existence of antineutrophil cytoplasmic antibody (ANCA)-negative pauci-immune crescentic glomerulonephritis (CGN) and thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTPHUS) is extremely rare and has a poor renal prognosis. We report a 76-year-old female that had both ANCA and anti-human lysosomal membrane protein 2 (LAMP-2) antibody-negative pauci-immune CGN with D-negative HUS. She was admitted with proteinuria and worsening renal failure with massive crescent formation on renal biopsy specimens. We initiated intravenous methylprednisolone pulse therapy followed by oral prednisolone, but she still developed D-negative HUS. We then initiated plasma exchange, which achieved remission of D-negative HUS and improved renal function. To our knowledge, this is the first report of recovery from renal failure in ANCA-negative pauci-immune CGN with TTP-HUS.
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PMID:Successful treatment with plasma exchange for ANCA-negative pauci-immune crescentic glomerulonephritis with D-negative hemolytic uremic syndrome. 2507 40