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)

Five children with end-stage reflux nephropathy underwent kidney transplantation at our clinic. Reflux nephropathy was studied clinically and histologically. All children had proteinuria before starting hemodialysis, and hypertension was present in 2 cases. Three children underwent antireflux operations prior to transplantation. The original kidneys exhibiting reflux were removed during renal transplantation. All original kidneys exhibited atrophy and scarring. Focal and segmental glomerulosclerosis was found in 4 cases. PAS deposition in the interstitium, suggestive of Tamm-Horsfall glycoprotein, was found in all cases. No recurrent signs of focal and segmental glomerulosclerosis have been found in the children who have been followed up from 1 to 6 years after transplantation.
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PMID:Clinicopathological study on end-stage reflux nephropathy in renal-transplanted children. 233 Jun 60

We studied the clinical features, pathologic findings, and course of 18 patients who were found to have glomerular disease at the time of hospitalization with manifestations of acquired immunodeficiency syndrome or acquired immunodeficiency syndrome-related complex at New York University Medical Center, New York, NY, during 1984 through 1987. Focal glomerulosclerosis, characterized by segmental and/or global collapse of capillary walls, was observed in 15 of these patients; mesangial proliferation in 2, and membranous nephropathy in 1. Those with focal glomerulosclerosis typically demonstrated heavy proteinuria without edema or hypertension and progressed rapidly to renal failure in less than 1 year from the time of discovery. This form of focal glomerulosclerosis is characterized by a fulminant course, the collapse type of sclerosis, and the frequent occurrence of uremia without advanced glomerular obliteration. The absence of widespread glomerular sclerosis and the rapid course suggest that unique renal hemodynamic mechanisms may be responsible for the progression.
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PMID:Rapid renal failure in AIDS-associated focal glomerulosclerosis. 240 96

A comparative clinico-pathological study was performed on 61 children and 51 adults with IgA nephropathy. Hematuria and/or proteinuria found by chance was the most common initial clinical sign, being observed in 82.0% of the children and 52.9% of the adults (p less than 0.001). At renal biopsy, hypertension and severe proteinuria were found in 9.8% and 33.3% of the adults and 0 and 14.8% of the children (p less than 0.05, p less than 0.05). Elevations of blood urea nitrogen and serum creatinine were found at the time of biopsy in 21.6% and 9.8% of the adults but in none of the children (p less than 0.001, p less than 0.05). On histological studies, proliferative changes of the glomerulus were similar in the two groups, and diffuse mesangial proliferation was found in 62.3% of the children and 51.0% of the adults (although the difference was not significant). Focal glomerulosclerosis and tubular atrophy were found in 52.9% of the adults and 32.8% of the children (p less than 0.05). These results suggest that focal glomerulosclerosis with tubular atrophy is correlated with deterioration of renal function, hypertension and age at renal biopsy, and has an important influence on the prognosis of patients with IgA nephropathy.
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PMID:[IgA nephropathy in Japanese children and adults: a comparative study of clinico-pathological features]. 258 35

Focal segmental glomerulosclerosis and membranous glomerulonephropathy are two entities that may result in the nephrotic syndrome. Two young women exhibited concurrent focal segmental glomerulosclerosis and focal segmental membranous nephropathy on renal biopsy. Although the lesions were severe, both patients had asymptomatic proteinuria, normal renal function, and a benign clinical course. The concurrence of these glomerulopathies may portend a more benign clinical course than expected for a patient presenting with either lesion alone.
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PMID:Concurrent focal segmental glomerulosclerosis and membranous nephropathy. 232 14

The authors have studied relationships of renal structure and function in more than 100 patients with insulin-dependent diabetes mellitus (IDDM), aged 13-55 years (mean, 30 years) with diabetes for 1-30 years (mean, 19 years). The authors confirmed the unique nature of the diabetic lesions that, in constellation, occur in no other disease. It was found that increased fractional mesangial volume (Vv Mes) is strongly associated with decreased glomerular filtration rate (GFR), proteinuria, and hypertension and that all patients with overt diabetic nephropathy have Vv Mes in excess of 0.35 micron 3/micron 3. This relationship results from constriction of the capillary lumen and filtration surface as a consequence of increased Vv Mes. Global glomerulosclerosis (scarring) is common in IDDM patients and appears related to arteriolar hyalinosis. Focal segmental glomerulosclerosis is a rare lesion in these patients. Having a single kidney (transplanted IDDM patients) is not associated with accelerated lesion development. The presence or absence of microalbuminuria (MA), per se, does not predict underlying glomerular structure, which may vary from the normal range to a level of pathology bordering on that regularly associated with overt nephropathy. However, when MA is associated with hypertension, or reduced GFR or both, urine albumin excretion (UAE) generally exceeds 40 mg/24 hr, and glomerular pathology is always present. The authors concluded that diabetic nephropathy is a unique renal disorder that cannot be caused by hemodynamic factors alone. The authors further conclude that MA becomes a predictor only when other features of overt nephropathy are already present and that serious diabetic glomerular lesions can be present in patients with normal UAE.
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PMID:Some lessons from the studies of renal biopsies in patients with insulin-dependent diabetes mellitus. 297 62

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

During the past 11 years, the Metro Toronto Glomerulonephritis Registry has prospectively followed all cases of glomerulonephritis starting from the time of biopsy. Focal segmental glomerulosclerosis was diagnosed by strict histologic criteria in 103 patients. Exclusion of patients with follow-up of less than 12 months reduced the number to 93 (55 adults and 38 children). Mean length of follow-up from the time of biopsy was 61 months. Ninety percent of children, but only 33 percent of adults received treatment with steroids, with or without cytotoxic drugs (p less than 0.001). Complete remission, defined as daily proteinuria of less than 250 mg, was not different in adults (39 percent) from that in children (44 percent), with a mean remission duration for all patients of 38 months. Chronic renal insufficiency, defined as a creatinine clearance of less than 0.8 ml/second/1.73 m2 for more than 12 months, was similar in adults (40 percent) and children (34 percent). Five-year renal actuarial survival, defined as the absence of chronic renal insufficiency, was 96 percent for patients with a history of complete remission, and 55 percent for those without (p less than 0.0002). Logistic regression analysis showed treatment to be the only significant factor for complete remission (p less than 0.001). Complete remission, in turn, was important for renal preservation, defined as the absence of chronic renal insufficiency (p less than 0.001). Age did not affect the treatment response or long-term renal outcome in focal segmental glomerulosclerosis. yet, the percent of adults treated was much lower than that of children, despite the fact that the majority of the untreated adults had the same clinical parameters as the treated adults and children. Thus, a judicious course of treatment is as much indicated in adults as in children with this disorder.
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PMID:Evidence suggesting under-treatment in adults with idiopathic focal segmental glomerulosclerosis. Regional Glomerulonephritis Registry Study. 357 62

The pattern of glomerulonephritis (GN) consisting of 1057 renal biopsies is presented. Primary GN accounted for 91% and secondary GN 9% of which the commonest is lupus nephritis. Asymptomatic haematuria and proteinuria was the commonest mode of presentation (41%), gross haematuria 9%, nephrotic syndrome 29% while 5.5% presented with renal impairment and 4.3% with hypertension. Mesangial proliferative GN is the commonest histopathological lesion forming 66% of all primary GN. Minimal Lesion, Focal Global Sclerosis and Focal Segmental Glomerulosclerosis accounted for 7% each. Membranous GN was uncommon (3%) while Mesangiocapillary GN, Diffuse Endocapillary GN and Crescentic GN were even rarer. If the presenting feature was asymptomatic haematuria and proteinuria the likely diagnosis was IgA nephritis, and, if nephrotic syndrome it was likely to be Idiopathic Mesangial Proliferative GN but with negative staining on immunofluorescence. The course and prognosis of the various forms of GN are next discussed. Nephrotic syndrome with Minimal Lesion has an excellent prognosis while Crescenteric GN usually carries a grim prognosis. Finally, factors affecting the progression of IgA nephritis, the commonest form of GN occurring in Singapore are examined. Patients who developed renal failure ran two different courses; one was a slowly progressive course over an average of 7.7 years before reaching end stage renal failure (ESRF), while the other was a more rapid decline to ESRF within an average of 3.3 years where severe uncontrolled hypertension seemed to be the major adverse factor. 9% had renal impairment at the end of a follow up of 50 + 1/2 - 34 months while 5% progressed to ESRF. The cumulative renal survival was 91% after 6 years with no further loss up to 14 years. Unfavourable long term prognostic indices were proteinuria of more than 2 gms, hypertension, crescents on renal biopsy, severe segmental sclerosis and medial hypertrophy of blood vessels.
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PMID:Glomerulonephritis in Singapore: an overview. 370 31

We studied members of three generations of a family presenting a nephropathy characterized by proteinuria, occasional microscopic hematuria, progressive deterioration of renal function and an autosomic dominant hereditary pattern. In seven percutaneous needle biopsies, examination by light microscopy showed findings compatible with Focal Segmental Glomerulosclerosis (FSGS) in six patients and Focal Global Glomerulosclerosis (FGG) in one case. Deposits of immunoglobulins IgM, IgA and C3 following mesangial and peripheral distribution were observed. According to electron microscopy, the basal membrane was unchanged though electron dense deposits were found at subendothelial, subepithelial and mesangial locations.
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PMID:Focal segmental glomerular sclerosis in three generations of a single family. 390 92

Focal, segmental glomerulosclerosis is frequently associated with chronic proteinuria and progressively declining renal function in humans as well as in experimental models of glomerular disease. Although little is known regarding the pathogenesis of this lesion, persistent, massive proteinuria has been associated with a poor prognosis. The administration of adriamycin to rats results in proteinuria of glomerular origin. We used this model to study the glomerular functional and structural alterations associated with proteinuria of 4 to 5 weeks duration. Studies of single nephron function revealed a 34% reduction in nephron plasma flow and a 50% decline in the glomerular ultrafiltration coefficient in rats given adriamycin. Single nephron glomerular filtration rate, however, was only modestly reduced (27%), because of an 8.0 mm Hg elevation of mean transcapillary hydraulic pressure difference (P less than 0.05). Morphologically, glomeruli of adriamycin-treated rats demonstrated significantly increased mesangial matrix and cellularity. In addition, glomerular capillaries frequently appeared enlarged, and epithelial cell bleb formation was evident. Focal glomerulosclerosis, however, was only rarely seen. The functional and morphologic characteristics of chronic adriamycin nephrosis are different from those associated with chronic proteinuria induced by repetitive administration of aminonucleoside of puromycin. Comparison of the two models suggests that the development of focal glomerulosclerosis can be dissociated from proteinuria and elevations of intraglomerular hydraulic pressures.
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PMID:Adriamycin-induced chronic proteinuria: a structural and functional study. 400 23


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