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)

Forty-one renal biopsies from 34 patients presenting over a 7-year period with the nephrotic syndrome or significant proteinuria and considered initially to be cases of lipoid nephrosis have been reviewed, and the results correlated with clinical findings. Lipoid nephrosis was confirmed in 25 of the patients (20 males, 5 females) 6 of whom showed a small proportion of completely sclerosed glomeruli. Twenty-two of these patients were in remission at the time of review. Nine of the patients (4 males, 5 females) showed features typical of focal glomerulosclerosis; only 4 of these were in remission, 4 showed continuing proteinuria, and one had died. The study emphasizes the importance and problems of distinguishing lipoid nephrosis from focal glomerulosclerosis.
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PMID:Lipoid nephrosis and focal glomerulosclerosis. 35 87

A microspectrophotometric study of sialomucins in human glomerular filter of the kidney in some diseases was carried out. Sialomucins were detected in paraffine sections stained after Haim, and examined on scanning microspectrophotometer. Lipid nephrosis, secondary amyloidosis, membranous-proliferative glomerulonephritis with the nephrotic syndrome were accompanied by a significant decrease in the amount of sialic acid. No reduction of sialomucins occurred in subacute glomerulonephritis with the nephrotic syndrome. A connection between the selective proteinuria and a decrease of the amount of sialomucins in the glomerulus is suggested.
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PMID:[Microspectrophotometric study of the sialomucin content of the glomerular filter in the nephrotic syndrome]. 65 13

Minimal change nephrotic syndrome has been reported to be a lymphocyte-mediated disorder. It has been suggested that the secretion of lymphokine(s) is involved in the pathogenesis of MCN and in determining proteinuria. The presence of a soluble form of IL-2 receptor (sIL-2R) has been previously described in the sera of patients with some autoimmune disorders. In this work, we report the detection of high sIL-2R levels, both in the plasma (mean value 844 +/- 436 U/ml versus normal value 276 +/- 86 U/ml) and urine of patients with MCN during the nephrotic phase alone. Instead, when the patients achieve stable remission, sIL-2R levels decrease to within normal values (mean value 332 +/- 272 U/ml). Furthermore, during the nephrotic syndrome we observed a significant inverse relationship between sIL-2R plasma levels and the mitogenic response to PHA (p less than 0.005). Since sIL-2R exerts a down-modulation on T-proliferative expansion, sIL-2R might represent one of the inhibitory serum factors extensively reported in the serum of patients with MCN-induced nephrotic syndrome.
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PMID:Lymphocyte release of soluble IL-2 receptors in patients with minimal change nephropathy. 158 55

Between November 1985 and November 1989, percutaneous renal biopsy was performed in 32 patients aged 75 or more (mean age 78). The major clinical presentations were nephrotic syndrome in 22 patients, variable amounts of proteinuria in 6 patients and unexplained severe renal insufficiency in 4 patients. In 27 cases the histological study revealed a glomerular disease. Minimal change disease (7 cases), membranous nephropathy (6 cases), amyloidosis (5 cases) and crescentic glomerulonephritis were the most frequent types. The rate of complications after biopsy was low (3 percent) with only one retroperitoneal hematoma. Owing to the atypical renal presentation of some glomerulopathies in the elderly, renal biopsy was useful to differentiate between minimal change disease, membranous nephropathy and amyloidosis. In 4 patients with minimal change disease, remission of nephrotic syndrome was obtained with corticosteroids. Moreover renal biopsy allowed to detect underlying diseases and gave indications to initiate specific therapy particularly in crescentic glomerulonephritis the outcome of which remained unfavourable. It is concluded that, even in elderly, renal biopsy is a valuable and safe diagnostic tool to obtain a rapid diagnosis, particularly in glomerular diseases, leading to a biopsy-guided therapy. It must therefore have the same indications as in younger adults with glomerular syndrome or rapidly progressive renal insufficiency.
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PMID:[Value of renal biopsy in the elderly. 32 cases]. 183 22

The clinical and histopathologic findings in 225 Irish adults with nephrotic syndrome were reviewed. Membranous nephropathy was the most common lesion found (28%), followed by proliferative glomerulonephritis (17%), and focal sclerosing glomerulonephritis (16%). Minimal change disease was the least frequent cause for idiopathic nephrotic syndrome (12%). The major secondary cause of nephrotic syndrome was amyloidosis (13%). The patients were analysed for the predictive value of the level of renal function, presence or absence of hypertension, and the degree of proteinuria. It was not possible to determine the nature of the underlying lesion giving rise to the nephrotic syndrome using any of these variables. There was also no significant difference between primary and secondary glomerular disease with regard to these factors. It is concluded that renal biopsy remains the only definitive method of establishing the underlying lesion causing idiopathic nephrotic syndrome.
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PMID:Clinical and histopathologic findings in adults with the nephrotic syndrome. 239 82

Idiopathic nephrotic syndrome (INS) is associated with a disorder of T-lymphocyte function, and an enhanced production of a vasoactive lymphokine, the vascular permeability factor (VPF). In an attempt to evaluate lymphocyte activation in various phases of INS, we measured beta 2-microglobulin (beta 2m) levels in lymphocyte culture supernatants (LCS). In 23 cases of untreated active INS, beta 2m levels in unstimulated LCS were significantly increased in comparison with those of 13 cases of untreated INS in complete remission (p less than 0.001), of 17 cases of active membranous nephropathy (p less than 0.01) and of 14 controls (p less than 0.001). In 13 patients treated with cyclosporine (Cs) (3-4.5 mg/kg/d) during 3 months, beta 2m levels were within the normal range. Although the beta 2m of 7 Cs patients without proteinuria was lower than 5 Cs patients with residual proteinuria, the difference was not statistically significant. In 15 prednisone(Pr)-treated INS patients, beta 2m levels were normalized. However their beta 2m levels were lower in 8 cases of complete remission than in 7 cases of persistent proteinuria (p less than 0.05). Concanavalin-A stimulation increased beta 2m amounts in all groups with a similar magnitude. In vitro addition of Cs (100 ng/ml) inhibited both beta 2m and VPF elevations observed in active INS. beta 2m level and VPF activity were significantly correlated (r = 0.54, p less than 0.01). High levels of beta 2m in LCS from INS are the consequence of an enhanced cellular synthesis and they are inhibited by Pr and Cs. Thus beta 2m increase in INS indeed reflects lymphocyte activation.
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PMID:Enhanced beta 2-microglobulin levels in lymphocyte culture supernatants from patients with idiopathic nephrotic syndrome: inhibition of lymphocyte activation by cyclosporine. 306 18

Minimal change nephrotic syndrome (MCNS) which is a well defined clinical, histopathological and therapeutic entity accounts for the majority of childhood nephrosis throughout the world except Africa. We describe 15 (13%) of 115 biopsy-confirmed African children with nephrotic syndrome who had minimal change on light microscopy. These are compared to 42 biopsied Indian children with typical MCNS. The critical differences between African children and children with classical MCNS were the lack of a predictable response in eight of the former to steroids and cyclophosphamide and in a later peak age of presentation (7-8 years). These patients differed in most respects from minimal change seen in the malarial zones of North Africa except for clinical presentation. Minimal change in West and East Africa more closely approximates typical MCNS. Two of the African patients with minimal change had endemic syphilis and responded clinically to penicillin therapy. The clinical presentation in all the African children, the favourable short-term outcome in 12 of these patients, immunofluorescent (13 patients) and electron microscopic (three patients) findings, and selectivity of proteinuria (eight patients) paralleled that of classical MCNS. The implications of this study are that African children in South Africa with MCNS should not be treated with steroids or cyclophosphamide. There is no clear benefit in the use of these drugs in nephrotic children with obvious glomerular lesions and therefore it follows that such therapy should be avoided in all African children in South Africa with this disease.
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PMID:Absence of 'true' minimal change nephrotic syndrome in African children in South Africa. 667 31

In 22 (13 females, 9 males) of 224 patients presenting with nephrotic syndrome, onset occurred during the second decade of life. Lipoid nephrosis and its variants (nil, focal segmental, or focal global glomerulosclerosis and mesangial proliferative) accounted for the disease in the majority of patients (13 patients). In all with nil change (7 patients) and one of the 4 with mesangial proliferation the disease completely resolved with steroids. Forty-one percent of patients had nephrotic syndrome as the result of other forms of glomerulonephritis: 1 patient had nephrotic syndrome secondary to poststreptococcal glomerulonephritis which resolved spontaneously. Three patients had membranoproliferative glomerulonephritis and developed renal insufficiency. Proteinuria decreased or disappeared in 4 patients with systemic lupus erythematosus. One patient had membranous nephropathy that was steroid resistant. Presenting symptoms and laboratory data failed to separate nil change from the more severe glomerular lesions. Early biopsy for diagnosis is suggested for patients presenting with de novo nephrotic syndrome in the second decade of life.
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PMID:Nephrotic syndrome in the second decade of life. 730 97

Minimal change disease (MCD), mesangial proliferative glomerulonephritis (MPGN) and focal glomerulosclerosis (FGS) may be indistinguishable at some stage when examined by light microscopy, but MCD in adults has over 80% response rate to prednisolone therapy. In search of MCD, we treated 22 patients with biopsy reported MCD (1 patient), MPGN (15 patients), and FGS (6 patients), out of 84 adult patients with the nephrotic syndrome (NS) seen over 55 months, with prednisolone 60mg daily for 6 weeks (4 patients) or 8 weeks (18 patients). Two patients with MPGN showed partial remissions with proteinuria dropping to 0.8g/24 hours and 1.2g/24 hrs. All the others were non-responsive with proteinuria remaining above 2g/24 hours. Allowing that 20% of patients may not respond to prednisolone, 4 patients may have been missed. It is concluded that MCD as defined by minor glomerular changes and good response to steroids is rare in adult Nigerian patients with the NS.
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PMID:The rarity of minimal change disease in Nigerian patients with the nephrotic syndrome. 783 26

Idiopathic nephrotic syndrome encompasses two main forms of glomerular diseases, minimal change nephropathy and focal segmental glomerulosclerosis. Minimal change nephropathy is a disease of children which generally responds to corticosteroids. After remission, however, many patients show frequent relapses or steroid dependency. In these patients, cyclosporine may obtain remission of proteinuria in 80% of cases, although relapse usually occurs when the drug is stopped. Focal glomerulosclerosis is generally resistant to corticosteroids. Under cyclosporine some 40% of patients may attain complete or partial remission of the nephrotic syndrome particularly if low-dose prednisone is associated. Relapse of proteinuria usually occurs after stopping the drug. As cyclosporine may expose to chronic nephrotoxicity some guidelines should be followed to prevent this complication: - the doses should not exceed 5 mg/Kg/day - they should be adjusted whenever an increase in plasma creatinine of > or = 30% over the baseline values occurs - treatment should be stopped if there is no response within 3 months - a careful monitoring of patient under the supervision of a clinician trained with the use of cyclosporine is necessary. The term idiopathic nephrotic syndrome (INS) defines the association of a nephrotic syndrome with non specific glomerular lesions, in the absence of immune complex deposition (1). On the basis of renal histology two main types of INS are recognized: minimal change nephropathy (MCN) and focal and segmental glomerular sclerosis (FSGS).
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PMID:Cyclosporine in idiopathic nephrotic syndrome. 822 73


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