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 13-year-old boy was admitted to this hospital for evaluation of pitting edema of both legs. Three years ago, he had been diagnosed to have nephrotic syndrome. Two and half years ago, because of persistent heavy proteinuria, poor response to steroids and frequent relapse of disease, a renal biopsy was done; characteristics of IgM nephropathy was shown. About a year previously, the patient felt dizziness and weakness of the left side of his body upon awakening one morning. Neurologic examination showed loss of muscle tone, muscle power and deep tendon reflexes. Sensory and cranial nerve function were intact. Blood pressure was normal. The CT scan of brain showed a patch of low attenuation area in the right temporal region, obliteration of the right cortical sulci and mild compression of right lateral ventricle. A diagnosis of nephrotic syndrome with right cerebral infarction was made. The patient's condition became stable two days later after mannitol infusion, correction of electrolytes, and supportive therapy. According to literature, most cases of nephrotic syndrome complicate with renal thrombosis, pulmonary emboli, and deep vein thrombosis. Few cases complicate with cerebral thrombosis and infarction. If patient have low plasma albumin and anti-thrombin III level, hyperfunction of platelet aggregability and use long-term diuretic therapy, they may be at higher risk of thromboembolic complications. If thromboembolic complications exist, anticoagulation treatment should be instituted. Prophylactic therapy with aspirin or dicumarol is not currently recommended.
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PMID:[Nephrotic syndrome complicated with cerebral infarction: report of one case]. 182 17

In INS, the histological appearance constitutes a classical prognostic element: minimal-change nephropathy (MCN) responds better to treatment than focal glomerulosclerosis (FGS) or IgM nephropathy (IgMN). However, this criterion is not consistent. We evaluated the prognostic value of the proteinuria selectivity index (SI): the ratio of IgG clearance to transferrin (Tf) clearance. Proteinuria was selective for an SI less than or equal to 0.01. In the 39 MCN, the SI ranged from 0.01 to 0.39 (median 0.10) and proteinuria was selective in 21 cases. In the 13 FGS and IgMN, the SI varied from 0.05 to 0.40 (median 0.22) and proteinuria was selective in 1 case (p less than 0.01 between these two groups). The SI ranged from 0.01 to 0.17 (median 0.07) for the 25 corticosensitive (CS) forms and from 0.08 to 0.40 (median 0.20) for the 27 corticoresistant (CR) ones (p less than 0.001). Twenty-four of the 30 MCN patients and 19 of 22 cases of selective proteinuria were CS. Multivariant analysis enabled the identification of variables predictive of the response to steroids. Age, sex and level of proteinuria had no such value. The predictive value of the SI was greater than that of the histological appearance (McFadden's R-square, 47 versus 22%, p less than 0.001). When the histological aspect was known, the SI provided additional precision, but the reverse situation was not true. The predictive curve of CS as a function of the SI was sigmoidal, therefore reflecting a homogeneous distribution, despite their different histological types.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Proteinuria selectivity index: prognostic value in idiopathic nephrotic syndromes]. 192 48

We report here the cases of 15 children in whom nephrotic syndrome developed, from among 164 children (55% male, 90% black) followed in our acquired immunodeficiency syndrome clinic from 1984 through 1990. Mean age at onset of nephrotic syndrome was 4.9 +/- 2.6 years. Fourteen patients were black and one was Hispanic. Seventy-three percent of our patients with nephrotic syndrome were girls. The mean duration of clinical acquired immunodeficiency syndrome before development of nephrotic syndrome was 1.7 +/- 1.1 years. In eight patients, nephrotic syndrome appeared between 3 and 11 months after intravenous infusions of immune globulin or albumin were administered as part of a research protocol; this incidence (8/47) was higher than the incidence of nephrotic syndrome among those who did not receive intravenous infusions (7/117, p less than 0.05). Tissue for histologic examination was available for 80% of the patients, and histologic examination demonstrated mesangial hypercellularity (5 patients), focal segmental glomerulosclerosis (4 patients), minimal change disease (2 patients), and IgM nephropathy (1 patient). Deposition of one or more immunoglobulins was noted in all but one patient studied with immunofluorescence. Corresponding electron-dense deposits were seen by electron microscopy in 78% of specimens. Prednisone did not induce a remission of nephrotic syndrome in the 13 patients treated, whereas cyclosporine did so in the 3 patients to whom it was administered. Five patients were in the end stage of renal disease within 8 months. Successful maintenance peritoneal dialysis was performed in three patients, but 80% of patients have died of human immunodeficiency virus-related complications; one patient was lost to follow-up. We conclude that immune-complex deposition is consistently seen in children with human immunodeficiency virus-associated nephrotic syndrome. This nephrotic syndrome is resistant to steroid therapy, but we observed a remission of the proteinuria with cyclosporine therapy in three patients. For patients with end-stage renal disease, maintenance peritoneal dialysis may improve the quality of life.
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PMID:Nephrotic syndrome associated with acquired immunodeficiency syndrome in children. 194 75

The renal biopsy material of Tampere University Central Hospital comprises 1992 renal biopsy specimens, accessioned during the years 1978-1989. Among these, there were three cases of mesangial glomerulonephritis with a peculiar type of immunofluorescent reactivity. Striking mesangial deposits of both IgA and IgM were found in glomeruli, whereas C3 deposits were absent or present in slight amounts. The light microscopic findings ranged from mild mesangial glomerulonephritis to more advanced forms of sclerosing glomerulopathy. Electron microscopic examination disclosed an increase of mesangial matrix, together with mesangial and paramesangial electron-dense deposits. Two of the patients had microscopic hematuria associated with proteinuria, and one had isolated proteinuria. The authors propose that this group of cases may represent a new subgroup of primary mesangial glomerulonephritis that has not been described previously. They differ immunohistologically from both IgA nephropathy and IgM nephropathy, and therefore could be designated as IgA-IgM nephropathy.
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PMID:IgA-IgM nephropathy. A subgroup of primary mesangial glomerulonephritis. 204 96

Eight patients belonging to 3 unrelated families had biopsy-proven IgM mesangial nephropathy. In the first family, the mother and the 2 daughters were affected; in the second, the mother and the son; in the third, 2 sisters and the brother. Two additional sisters of the third family showed a clinical picture consistent with chronic glomerulonephritis. The clinical picture was that of hematuria and/or proteinuria. No patients had nephrotic syndrome. Genealogic investigation enabled us to discover 2 additional affected members in the kindred of the first family (1 with IgA nephropathy, 1 with clinical glomerulonephritis) and 3 other affected members in the pedigree of the third family (1 with IgA nephropathy, 1 with sclerosing glomerulonephritis, 1 with clinical glomerulonephritis). Immunogenetic studies showed the recurrence of an extended haplotype bearing DR beta 11-DQ beta 3B-DQ alpha 2-C4A3-C4B1-BfS in 9 of 10 affected members. Our data suggest that genetic factors may be involved in the mechanism of the disease and support the hypothesis that IgM nephropathy is a distinct disease entity.
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PMID:Familial IgM mesangial nephropathy: a morphologic and immunogenetic study of three pedigrees. 224 52

Three patients with a long-standing history of familial Mediterranean fever (FMF) were found to have both microscopic hematuria and proteinuria during the acute attacks. Kidney biopsies from all patients revealed diffuse mesangial proliferative glomerulonephritis with intense IgM and C3 deposits; all were negative for amyloidosis. To our knowledge this is the first time the development of IgM nephropathy in patients with FMF is described.
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PMID:IgM nephropathy associated with familial Mediterranean fever. 235 58

Two cases of rheumatoid arthritis with renal lesions are presented. In one of the patients a mesangial IgM nephropathy and in the other patient a mesangiocapillary glomerulonephritis were proved clinically and by biopsy. In the first patient proteinuria was found only while in the second patient the proteinuria was combined with nephrotic syndrome and arterial hypertension. The various renal lesions in rheumatoid arthritis and their causes are discussed.
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PMID:[Kidney involvement in rheumatoid arthritis]. 263 86

The effect of cyclosporin on proteinuria was studied in 11 patients with steroid-responsive nephrotic syndrome (10 minimal change nephropathy, one IgM nephropathy) and in four patients with steroid-resistant nephrotic syndrome from focal segmental glomerulosclerosis. Cyclosporin (mean initial dose 7.7 mg/kg per day) produced a complete remission of proteinuria in 15 nephrotic episodes in the ten patients with minimal-change nephropathy after a mean 14.3 days (range 7-23 days) of therapy. All patients remained in remission while receiving cyclosporin (mean duration of follow-up 147 days; range 40-230 days). However, when cyclosporin was discontinued on nine occasions in five patients, all relapsed after a mean 47.8 days (range 7-180 days). Four of the five patients were subsequently rechallenged with cyclosporin and all responded. Maintenance cyclosporin therapy to prevent relapse was not associated with any adverse effects, and there was no significant difference between the creatinine clearance before and after 30 days of therapy (86.9 +/- 19.3 and 81.7 +/- 23.5 ml/min respectively, P greater than 0.1). The patient with steroid-responsive IgM nephrotic syndrome did not respond to cyclosporin, and there was no significant effect of cyclosporin on proteinuria in the four patients with FSGS. Cyclosporin is an effective agent for the treatment of patients with frequently relapsing minimal-change nephropathy who became steroid dependent when cyclophosphamide is contraindicated. However, unlike cyclophosphamide, long-term remissions which persist after treatment is withdrawn are not obtained, and patients may be said to be cyclosporin dependent.
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PMID:Cyclosporin in the treatment of steroid-responsive and steroid-resistant nephrotic syndrome in adults. 314 13

Total serum IgE was measured in 119 cases of primary glomerular diseases and 33 normal healthy persons. Statistically significant higher levels were noted in minimal change disease (MCD; median: 630 U/ml), IgM nephropathy (IgMN; 618 U/ml), focal glomerulosclerosis (FGS; 373 U/ml) and membranous glomerulonephritis (MGN; 144 U/ml). A higher level of serum IgE was noted in association with more frequent relapse or steroid resistance in MCD and IgMN and in FGS with nephrotic syndrome. A small group of IgA nephropathy with nephrotic range proteinuria was also noted to have extraordinarily high serum IgE. These findings suggest that IgE may play an important role in the pathogenesis of MCD, IgMN, and FGS and may serve as a prognostic indicator in terms of steroid responsiveness in MCD and IgMN.
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PMID:Serum IgE in primary glomerular diseases and its clinical significance. 338 Feb 16

It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. In 16 affected children, only 4 (25%) had minimal-change lesion, 7 had IgM nephropathy, 3 had diffuse mesangial hypercellularity, and 2 had focal segmental glomerulosclerosis. Fourteen of these patients have required immunosuppressive therapy with cyclophosphamide. Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.
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PMID:Paucity of minimal-change lesion in children with early frequently relapsing steroid-responsive nephrotic syndrome. 357 68


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