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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 30 year old man developed renovascular hypertension and extreme elevation of plasma renin activity. Daily proteinuria ranged from 13 to 31 g. There were no criteria for the diagnosis of malignant hypertension. A primary glomerulopathy was excluded by a basically normal renal biopsy from the unprotected kidney. On electron microscopy the epithelial cell foot processes were not fused, thus ruling out simultaneous lipoid nephrosis. The source of renin was removed by means of a left nephrectomy. Following the procedure the patient became normotensive, the renin values normalized and the proteinuria disappeared. The results suggest that renin can cause significant proteinuria in man.
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PMID:Renin-induced massive proteinuria in man. 45 23

In the model described here the ability of cationized (basic) ferritin to bind to the glomerular basement membrane and act as a planted antigen was exploited. This planted antigen was accessible to circulating antibody resulting in in situ immune complex formation. Perfusion of cationized ferritin (isoelectric point greater than 9.5) directly into the renal arteries, followed by intravenous injection of anti-ferritin antibody resulted in induction of glomerulonephritis with heavy proteinuria lasting for about 3 weeks. Fine granular deposition of antigen, antibody and rat C3 along the glomerular capillary walls was seen by immunofluorescence. Electron microscopy revealed the presence of subepithelial deposits containing ferritin; the foot processes were fused. This demonstrates the potential role of basic antigens in the pathogenesis of in situ immune complex glomerulonephritis.
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PMID:A model of in situ immune complex glomerulonephritis in the rat employing cationized ferritin. 701 52

3 siblings--2 brothers aged 18 and 13 years, and 1 sister, aged 11 years--with adolescent or late-onset cystinosis presented with massive proteinuria. At the time their glomerular filtration rate was normal or only modestly diminished. Though glomerular injury was evident, renal tubular functional abnormalities were also present. Renal biopsy revealed histopathologic features typical of the nephropathic form of cystinosis with the Fanconi syndrome: polykaryocytosis, varying degrees of glomerular sclerosis, thickening and reduplication of basement membrane, fused foot processes, dilated tubules with altered epithelial cell features, and interstitial fibrosis. Fine granular deposits of C3 and IgM are irregularly distributed in the glomeruli, findings which have not been described in cystinosis. These deposits are possibly immune complexes being deposited in the glomeruli unrelated to the cystine-storage disease or they may represent a localized activation of the complement system induced by the glomerular injury of cystinosis.
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PMID:Glomerular lesions in patients with late-onset cystinosis with massive proteinuria. 703 94

The distribution of anionic sites within glomerular basement membranes from patients with different glomerulonephritides was examined by the dialyzed colloidal iron (DI) staining technique. The correlation of ultrastructural findings with glomerular disease, renal function, and degree of proteinuria revealed three alterations: 1) speckled DI staining in the lamina densa of patients with decreasing renal function and a proteinuria of greater than 1 g/24 hours; 2) an apparent staining disparity and diminution of DI at the lateral borders of swollen and retracted foot processes with inclination of the foot processes in the direction of the more weakly staining lateral border; and 3) heavy DI reaction on the apical of free surfaces of fused foot processes. Human subjects with a proteinuria of more than 1 g/24 hours display optimal labeling of the endothelial fenestrae, endothelial cell coat, lamina rara interna, and lamina rara externa. The staining observed may be explained either on the basis of direct DI interaction with diffusing plasma proteins secondary to a decrease in the transglomerular charge barrier consequential to a loss of intrinsic anionic sites or on the basis of "unmasking" of anionic moieties within the glomerular basement membrane. Regardless of the mechanism involved, the present study indicates that a threshold proteinuria of 1 g/24 hours is needed to effect staining in the lamina densa.
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PMID:Ultrastructural assessment by colloidal iron of the distribution and localization of anionic sites in human glomerulonephritides. 746 63

Spontaneous nephrotic (ICGN) mice develop proteinuria, hypoproteinemia and hypercholesterolemia. These symptoms steadily progress to chronic renal failure. Details of the changes of the kidney, in the late stage (more than 5 months old) were investigated by both light and electron microscopy. The kidney exhibited a slightly whitish, granular surface and the cortex became thinner and contained fibrous lesions, in which clusters of unaffected and occluded renal tubules were randomly scattered. In the juxtamedullary and outer medullary zone, there were highly dilated renal tubules, which sometimes contained urinary casts. The glomerulus exhibited basement membrane thickening in the capillary loops and the capillary lumen was narrowed in size and sometimes occluded. No detachment of the podocyte from the basement membrane was observed and the podocyte foot-processes were extensively fused, causing their characteristic slits to be lost. The thickened basement membranes were found both in the glomerulus and around the occluded renal tubules, while the basement membrane in the dilated renal tubule appeared normal. Therefore, the basement membranes of the glomerulus and renal tubules appear to react differently in the pathogenesis of the condition. In conclusion, ICGN mice are a good model for not only the nephrotic syndrome but also for chronic renal failure.
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PMID:Morphological studies on the kidney of the spontaneous nephrotic (ICGN) mice in the late stage. 778 18

The change of anionic sites on GMB was studied in both adriamycin induced nephrosis and nephrotoxic serum nephritis in rats by means of calculating the PEI stained particles under electron microscope. The results showed that the number of anionic sites within lamina rara externa of GMB was reduced significantly in both kinds of proteinuria rats and the size of anionic sites was also changed, especially in the segments of GBM with fused foot processes. The results demonstrate that the loss of anionic sites on GBM and the functional damage to the charge selective barrier of glomeruli may play an important role in proteinuria of these animal models.
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PMID:[Anionic site changes on glomerular basement membrane (GBM) in proteinuria rats]. 803 79

Urine protein electrophoresis can be performed by capillary electrophoresis using spun unconcentrated urine diluted with running buffer. The separation which utilises the excellent sensitivity of capillary electrophoresis gives electropherograms similar to those obtained by diluting concentrated urine with buffer. A 72 cm x 50 microns internal diameter (ID) fused silica capillary was used for the analysis which took less than 15 min. Bence Jones protein can be detected from unconcentrated urine over urine protein concentrations ranging from 9.7 g/L to 0.04 g/L. Other clinical patterns, such as glomerular proteinuria, the presence of intact immunoglobulin and Tamm Horsfall protein can also be detected. The benefit of using unconcentrated urine is the time and cost saved by not concentrating the urine.
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PMID:Clinical application of capillary electrophoresis to unconcentrated human urine proteins. 937 78

Nephrin and NEPH1, the gene products of NPHS1 and NEPH1, are podocyte membrane proteins of the Ig superfamily. Similar to the nephrin knockout, mice lacking NEPH1 show severe proteinuria leading to perinatal death. To identify the ligand of NEPH1, the extracellular domain of NEPH1 was fused to human IgG. This NEPH1-Ig fusion protein labeled the glomerular capillary wall of mouse kidneys in a staining pattern identical to NEPH1 and nephrin, prompting speculation that that NEPH1 might form homodimers and/or heterodimers with nephrin. In coimmunoprecipitation and pull-down assays, the NEPH1-Ig fusion protein precipitated wild-type NEPH1 from overexpressing HEK 293T cells. Truncational analysis revealed that the adhesive properties were not confined to a single Ig domain of NEPH1. Fusion proteins containing two Ig domains of NEPH1 were sufficient to immobilize NEPH1, but they failed to interact with control protein containing the phylogenetically related PKD repeats of polycystin-1. NEPH1 also precipitated nephrin, a protein with eight Ig domains and a fibronectin-like domain. Truncational analysis of nephrin revealed a very similar mode of interaction, i.e., two nephrin Ig domains fused to human IgG precipitated either nephrin or NEPH1, but not the control protein. Both NEPH1 and nephrin interactions were strictly dependent upon posttranslational glycosylation, and bacterially expressed protein failed to bind NEPH1. These findings demonstrate that the Ig domains of NEPH1 and nephrin form promiscuous homodimeric and heterodimeric interactions that may facilitate cis- and trans- homodimerizations and heterodimerizations of these molecules at the glomerular slit diaphragm.
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PMID:Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1. 1266 Mar 26

Podocyte stress precedes proteinuria in hypercholesterolemic rats. Molsidomine, a nitric oxide (NO) donor, prevented podocyte stress and proteinuria in long-term hypercholesterolemia, suggesting that podocyte stress was due to NO deficiency. Podocytes express the angiotensin II type 1 receptor, which influences their function. Because NO counteracts angiotensin II, it was hypothesized that in a setting of impaired renal NO availability, angiotensin II receptor inhibition could prevent podocyte stress. For determining the effect of NO deficiency on podocyte stress, one group of female rats were fed 2% cholesterol and another group the arginine analogue N-omega-nitro-L-arginine (L-NNA; 40 mg/kg food) for 2 wk. Another group of rats that were fed 2% cholesterol also received the NO donor molsidomine (120 mg/L water) for 2 wk before and during cholesterol feeding. For determining the influence of angiotensin II in the setting of decreased renal NO availability, rats that were treated with cholesterol or L-NNA received the angiotensin II type 1 antagonist losartan (200 mg/L water) for 2 wk before and during cholesterol or L-NNA administration. Desmin staining and electron microscopy were used to monitor podocyte activation. Glomerular caveolin was quantified by immunohistochemistry. Renal cortical NO synthesis, NO synthase isoforms, and caveolin-1 protein mass were also measured. Both short-term cholesterol and L-NNA induced podocyte stress as evidenced by enhanced desmin staining and electron-dense fused foot processes. Podocyte stress was prevented by molsidomine in short-term hypercholesterolemia. Furthermore, losartan prevented podocyte stress in rats that were treated with cholesterol or with L-NNA. Finally, hypercholesterolemia decreased renal cortical NO synthase activity and increased caveolin-1 protein mass and glomerular caveolin staining, and these changes were also prevented by losartan. It is suggested that podocyte stress in these models of early injury results from angiotensin II, unopposed by the action of endogenous NO. This underscores the strategic role of angiotensin II blockers in early kidney disease.
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PMID:Hypercholesterolemia in rats induces podocyte stress and decreases renal cortical nitric oxide synthesis via an angiotensin II type 1 receptor-sensitive mechanism. 1503 97

Noonan syndrome is characterised by short stature, typical facial dysmorphology and congenital heart defects. Urogenital abnormalities are reported in 10% of the cases. We present a 14-year-old girl with characteristic features of Noonan syndrome and nephrotic-range proteinuria. She had crossed fused ectopic kidneys. Renal biopsy showed focal segmental glomerulosclerosis. Oral steroids were instituted and she responded well. The case highlights this novel renal presentation of Noonan syndrome.
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PMID:Noonan syndrome: crossed fused ectopic kidneys and focal segmental glomerulosclerosis-a rare association. 1943 94


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