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)

A twenty-one-year-old male was admitted to our hospital because of hypertension and proteinuria. He had felt general fatigue and low grade fever for one month. Blood pressure was 180/120 mmHg on admission. Laboratory findings showed 3+ proteinuria and 1+ occult blood in urinalysis; an accelerated erythrocyte sedimentation rate (ESR) of 39 mm/hr; elevation of LDH to 755 IU/l. Antinuclear antibody was positive with a titer of 1: 160, with a speckled pattern. Plasma renin activity and serum aldosterone were markedly elevated to 25.8 ng/ml/hr and 585.3 pg/ml, respectively. Renal function had declined mildly; endogenous creatinine clearance was 60 ml/min. Renal arteriogram demonstrated multiple intrarenal aneurysms in the bilateral kidneys. Aneurysms, 5-8 mm in diameter were located in the arteries from the interlobar to interlobular region. He was diagnosed as having polyarteritis nodosa (PN) and was then treated with 20 mg/day of prednisolone and monthly pulse therapy of cyclophosphamide. After steroid, cyclophosphamide and anti-hypertensive therapy, he became well and had normal blood pressure. The patient was considered a rare case of PN with multiple intrarenal aneurysms and accelerated hypertension. We discuss aneurysms in PN and accelerated or malignant hypertension documented in the literature.
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PMID:[A case of polyarteritis nodosa presenting with multiple intrarenal aneurysms and accelerated hypertension]. 769 55

Rapidly progressive glomerulonephritis (RPGN) is rarely associated with macroscopic polyarteritis nodosa (PAN), as seen in this series of 7 out of 235 patients. The clinical symptoms of PAN were as follows: myalgias 6; fever 1; arthralgias and mononeuropathy multiplex 5; cutaneous vasculitis 3; arterial hypertension 4, 3 of which were malignant. The mean proteinuria was 2.7 g/24 h; creatininemia 458 microM/l; microscopic hematuria was present in 4 of the 7 patients; only 1 patient was anuric. Markers of hepatitis B virus were absent in all cases. Arteriography revealed microaneurysms and renal infarcts in 6 patients and distal arterial stenosis in one. Renal biopsies from all 7 patients demonstrated extracapillary glomerulonephritis, which was associated with tubulointerstitial fibrosis in one. Necrotizing vasculitis lesions were associated in 5 cases. Immunofluorescence was positive in 5 cases. The association of RPGN and PAN exists and may be underestimated due to the lack of systematic angiographic examinations during RPGN and renal biopsies in PAN patients with renal involvement.
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PMID:[Rapidly progressive glomerulonephritis in macroscopic periarteritis nodosa. 7 cases]. 790 9

Glucocorticoids, cytostatic agents and cyclosporin are frequently employed in the treatment of glomerular diseases of immunologic origin. In order to assess the efficacy of these drugs, we retrieved--with the help of a Medline-based search--and analysed all controlled studies published since 1966 dealing with immunosuppressive therapy of glomerulonephritis. Of the 34 identified controlled studies, only 27 had a prospective and randomized design. In patients with minimal-change glomerulonephritis, proteinuria decreases and disappears during therapy with prednisone. A comparable effect can be obtained with cyclosporin. Occasionally, there is a relapse of proteinuria after cessation of the immunosuppressive therapy in some patients. These relapses can be controlled with a chlorambucil-based regimen. Chlorambucil may be successfully utilized in the treatment of focal-segmental glomerulosclerosis, a form of glomerulonephritis which is more refractory to glucocorticoid therapy and is probably pathogenetically related to minimal-change glomerulonephritis. Patients with membranous glomerulonephritis and a nephrotic syndrome seem to benefit from an alternate month prednisone and chlorambucil regimen. However, an indiscriminate treatment of all patients with this regimen is not legitimate, because some patients would be overtreated as the disease may undergo spontaneous remission. There are no well-documented valuable therapies for the IgA-associated glomerulonephritis and the membranoproliferative glomerulonephritis. The combination of prednisone with cytotoxic substances, particularly cyclophosphamide and azathioprine, seem to remarkably improve the renal prognosis of the diffuse proliferative lupus glomerulonephritis. The efficacy of cyclophosphamide and prednisone with or without plasma exchange in the treatment of the rapidly progressive glomerulonephritis due to other systemic diseases (M. Wegener, panarteritis nodosa, Goodpasture syndrome) is a widely accepted therapeutic modality, although controlled studies are lacking. Immunosuppressive therapy of glomerulonephritis bears notable risks and sometimes questionable efficacy. Thus, before prescribing any immunosuppressive therapy, it is mandatory to evaluate in every single patient the prognostic factors of the underlying disease, the probability of the onset of severe side effects and the possible acceptance of a renal replacement therapy, including renal transplantation.
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PMID:[Immunosuppressive therapy of glomerulonephritis--controlled studies]. 845 16

We undertook this study to determine the clinical, biologic, immunologic, and therapeutic factors associated with the prognoses of polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). Three hundred forty-two patients (260 with PAN, 82 with CSS) followed from 1980 to 1993 were included in a prospective study on prognostic factors. Two hundred eighty-eight of these patients were included in the prospective studies on PAN and CSS. Items to be considered for analysis were collected at the time of diagnosis, during the acute phase of the disease. A survival curve was plotted for each clinical and biologic symptom observed in PAN or CSS. Each treatment arm of the prospective therapeutic trials was also tested: 1) prednisone (CS) + oral cyclophosphamide (CYC) + plasma exchanges (PE) versus CS E, 2) CS + PE versus CS, 3) CS + oral CY versus CS + pulse CY, 4) CS + pulse CY + PE versus CS + pulse CY in severe PAN and CSS, and 5) PE + antiviral agents after short-term CS in hepatitis B virus-related PAN. Of the parameters thus evaluated, the following had significant prognostic value and were responsible for higher mortality: proteinuria > 1 g/d (p < 0.0001; relative risk [RR] 3.6), renal insufficiency with serum creatinine > 1.58 mg/DL (p < 0.02; RR 1.86), GI tract involvement (p < 0.008. RR 2.83 for surgery). Cardiomyopathy and CNS involvement were associated with a RR of mortality of 2.18 and 1.76, respectively; these were not statistically significant. Similar survival rates were obtained with the prospectively tested therapies. The five-factors score (FFS) we established considered the prognostic factors creatinemia, proteinuria, cardiomyopathy, GI tract involvement, and CNS signs. Multivariate analysis showed that proteinuria (due to vascular or glomerular disease) and GI tract involvement were independent prognostic factors. When FFS = 0 (none of the 5 prognostic factors present), mortality at 5 years was 11.9%; when FFS = 1 (1 of the 5 factors present), mortality was 25.9% (p < 0.005); when FFS > 2 (3 or more of the 5 factors present), mortality was 45.95% (p < 0.0001 between 0 and 2, p < 0.05 between 1 and 2). We conclude that an initial assessment of PAN or CSS severity enables outcome and mortality to be predicted. The FFS is a good predictor of death and can be used to help the clinician choose the most adequate treatment. Renal and GI signs are the most serious prognostic factors.
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PMID:Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. 856 67

The authors report on 5 observations of necrotising angiitis with a specifically otological onset, namely 4 Wegener's syndromes and one periarteritis nodosa. In all 5 cases, the initial symptomatology consisted of otological signs in a feverish context, producing pictures of serous or sero-purulent otitis. Its resistance to usual forms of therapy led in 3 cases to the installation of transtympanic aerators, and in 2 cases to the performance of an antro-attico-mastoidectomy. One of the observations led to the discovery in the middle ear of a histological aspect specific to the disease, from which it can be considered that the otologic impairment could be a specific seat of the disease. The 5th observation is particular in that it was the otological signs which revealed each time a new progression of the disease, enabling the immediate implementation of an adapted treatment prior to the occurrence of the systemic signs. 3 observations proved the difficulty of diagnosis at the initial stage, when at times only the otological signs are present, together with the importance of its discovery prior to the occurrence of systemic lesions that are occasionally irreversible. Any isolated otological symptomatology in a feverish context, resisting usual therapies, should evoke the diagnosis, and lead to the performance of further duly adapted examinations (sedimentation rate, pulmonary X-ray, proteinuria, hematuria).
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PMID:[Otologic manifestations presenting and predominating in necrotizing angiitis]. 863 44

A 86 year-old woman was re-admitted because of purpura of her upper and lower extremities, abdominal pain and blood stools. Seven weeks previously, she underwent a gastrectomy for gastric cancer. After re-admission, proteinuria and hematuria were noted, and the serum creatinine level increased. Two months, after the onset of purpura, she died of pneumonia. On autopsy examination, fibrinoid vasculitis of acute inflammatory stage (II) at small arteries and/or arterioles in the bladder, rectum, lungs, spleen and crescentic glomerulonephritis without immune deposits were observed. A diagnosis of microscopic polyarteritis nodosa (M-PN) was made based on these clinical and histological findings. M-PN refers to systemic vaculitis with segmental necrotizing glomerulonephritis. However, this condition may be difficult to diagnose because vasculitis such as Scholein-Henoch purpura (SHP) and/or hypersensitivity angitis, diseases in which the small arteries and arteroles are mainly affected, occasionally bears a clinical and histological resemblance to M-PN. Because differential diagnosis from SHP was required, this case provided abundant suggestions with regard to the entity of M-PN.
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PMID:[An autopsy case of microscopic polyarteritis nodosa resembling Schoenlein-Henoch purpura]. 872 Feb 67

A patient with a history of drug abuse and histologically diagnosed hepatitis B-related polyarteritis nodosa was admitted for severe hypertension. After a cesarean section because of worsening of her clinical status with severe proteinuria and edema, she improved dramatically. No sign of acute polyarteritis nodosa occurred.
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PMID:Severe preeclampsia superimposed on polyarteritis nodosa. 906 53

We encountered two patients with pulmonary hemorrhage who had high levels of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Patient 1 was a 69-year-old woman. Both were admitted to our hospital complaining of hemoptysis. Microscopic hematuria was detected in patient 1, and proteinuria and renal insufficiency were detected in patient 2. Chest X-ray films showed bilateral patchy infiltrates in patient 1, and right middle-lower infiltrates in patient 2. In both patients the levels of MPO-ANCA were high and the results of tests for anti-basement membrane antibodies were negative. These patients were suspected to have pulmonary-renal vasculitic syndrome with a high level of MPO-ANCA. In patient 1, because the level of MPO-ANCA decreased after treatment with steroid therapy, we believe that measuring the level of MPO-ANCA was useful in the management of the disease. Rapidly progressive glomerulonephritis developed in patient 2, and was exacerbated despite hemodialysis, steroid therapy, and plasma exchange therapy. Use of the term microscopic polyangiitis (MPA) was first proposed by yhe Chapel Hill Consensus Conference in 1993. MPA, which was formerly called microscopic polyarteritis nodosa, connotes pauci-immune necrotizing vasculitis affecting arterioles, venules, or capillaries, and this condition is strongly associated with ANCA. Patients with pulmonary-renal vasculitic syndrome who have MPO-ANCA may be given a diagnosis of MPA. Therefore, we diagnosed MPA in these two patients. Testing for ANCA may be useful in patients with pulmonary hemorrhage and renal involvement.
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PMID:[Two patients with pulmonary hemorrhage associated with myeloperoxidase-antineutrophil cytoplasmic antibody]. 907 Nov 67

Microscopic polyarteritis may involve medium-sized and small blood vessels as well as arterioles, venules and capillaries. We have compared the clinical and laboratory features in patients with microscopic polyarteritis and medium vessel involvement, with the features found in patients with polyarteritis nodosa affecting medium vessels alone. In a 9-year period, 21 patients presented to our hospital with a form of polyarteritis. Seven had microscopic polyarteritis demonstrated histologically (6/7, 86%) and associated with dysmorphic urinary red cells (7/7, 100%), as well as medium vessel vasculitis demonstrated histologically (7/7) or by angiography (1/7, 14%). Five patients had polyarteritis nodosa with medium vessel vasculitis demonstrated histologically (3/5, 60%) or by angiography (2/5, 40%); and no evidence of a glomerular vasculitis on biopsy (2/7, 29%) or in the urinary sediment (0/7, 0%). The remaining 9 patients had microscopic polyarteritis but medium vessel involvement was not excluded by angiography. All patients with microscopic polyarteritis and medium vessel involvement had glomerular hematuria (> 100,000 glomerular RBC/ml), proteinuria > 0.5 g/24 hours), and an elevated serum creatinine (0.166 to 0.811 mmol/l). Other symptoms included fever (6/7, 86%), night sweats (5/7, 71%), gastrointestinal bleeding (4/7, 57%), proximal myopathy (3/7, 43%) and peripheral neuropathy (3/7, 43%). One patient (1/7, 14%) had hypertension. Anemia (6/7, 86%), a raised ESR (6/7, 86%), thrombocytosis (6/7, 86%), hypoalbuminemia (6/7, 86%) and abnormal liver function tests (6/7, 86%) were common. Two patients (29%) had an eosinophilia. All 5 individuals who were tested for ANCA were positive (2cANCA, 2pANCA and one pattern not described). In contrast, in patients with polyarteritis nodosa and medium vessel involvement alone, an elevated ESR was common (4/5, 80%) but fever (1/5, 20%), night sweats (0/5, 0%), proximal myopathy (1/5, 20%) and peripheral neuropathy (1/5, 20%) were seen infrequently; hypertension (1/5, 20%) and eosinophilia (1/5, 20%) were also uncommon; and ANCA were not demonstrated (0/3, 0%). Medium-sized vessel involvement is common in patients with microscopic polyarteristis, and these patients are more likely to have renal involvement and systemic symptoms, and be ANCA-positive, than patients with polyarteritis nodosa alone. Gastrointestinal symptoms are often seen in both groups.
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PMID:Classical polyarteritis nodosa and microscopic polyarteritis with medium vessel involvement--a comparison of the clinical and laboratory features. 910 64

Here we report a case of microscopic polyangitis (MPA) with anti-neutrophil cytoplasmic antibodies against myeloperoxidase (MPO-ANCA) accompanied by perceptive dominant hearing loss as the initial sign of the disease. A 52-year-old woman was admitted to Tokyo Metropolitan Ohtsuka Hospital in April 1996 because of bilateral progressive hearing loss. On admission, she had presented bilateral perceptive hearing loss, fever, myalgia, and weight loss. Laboratory data showed accelerated erythrocyte sedimentation rate, proteinuria, microscopic hematuria, leukocytosis, hypoproteinemia, and elevated creatinine level. Serum level of MPO-ANCA was elevated. Chest X-ray and computed tomography showed mild pleural effusion and interstitial shadow in bilateral lung fields. The biopsy findings detected that focal glomerulonephritis, and necrotizing angitis of small artery in kidney. The diagnosis of MPA was made with clinical course, laboratory findings, radiographic findings, and biopsy finding. Treatment was initiated with 45 mg of prednisolone, followed by marked improvement of hearing loss and decreased titer of serum MPO-ANCA. There are only seven reports showing hearing loss as the initial sign of the disease occurred in polyarteritis nodosa and MPA. The present case suggests the possibility that hearing loss may be the symptom of MPO-ANCA associated vasculitis.
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PMID:[A case of microscopic polyangitis accompanied by hearing loss as the initial sign of the disease]. 949 68


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