Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035078 (renal failure)
 31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study was undertaken to learn the significance of focal glomerular sclerosis in children who have nephrotic syndrome. Tissue obtained by percutaneous renal biopsy 10-15 years previously was re-examined. Initially, two of the 29 biopsy specimens contained focal segmental hyalinosis or sclerosis and five of the 29 had focal glomerular obsolescence. The paraffin blocks were serially sectioned and examined. Following this procedure, seven of the 29 biopsies had focal segmented hyalinosis and 16 of the 29 had focal glomerular obsolescence. The percentages of focal segmental hyalinosis and focal glomerular obsolescence were recorded. Only those patients whose focal segmental hyalinosis exceeded 2% progressed to renal failure. Age-matched autopsy material from patients dying without renal dysfunction was used as a control. Focal glomerular sclerosis was seen in 75.8% of the control specimens, although few glomeruli within each specimen were involved. Focal glomerular sclerosis may be found normally; it may be found in nephrotic children who do not develop renal failure. The quantification of sclerotic lesions may be of prognostic significance in childhood nephrosis.
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PMID:The significance of focal glomerular sclerosis in children who have nephrotic syndrome. 96 34

Focal and segmental glomerulosclerosis accounted for 4.1% of all primary glomerulopathies. Typical signs of focal and segmental glomerulosclerosis were striking arteriolosclerosis on histological examination, the urinary syndrome of high proteinuria with mild haematuria and frequent nephrotic syndrome. The ten-year cumulative percentage of remissions was 30%. Patients with remission had at the time of biopsy a normal serum creatinine level and no or minimal regressive tubulointerstitial changes. The ten-year cumulative percentage of renal failure was 40%. Presence of nephrotic syndrome, extracapillary proliferation and advanced tubulointerstitial changes, tended to shorten a kidney survival. In one third of the patients the serum creatinine level rose by more than 50 mol/1/year.
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PMID:[Focal segmental glomerulosclerosis]. 175 2

Focal and segmental glomerulosclerosis (FGS) is an important cause of the nephrotic syndrome and renal failure in children and adults. This review will present a detailed description of the pathological and clinical features of FGS and discuss in depth the modern concepts of its pathogenesis, which recent studies indicate is multifactorial and analogous to that of atherosclerosis.
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PMID:Ideas in pathology. Focal and segmental glomerulosclerosis. 206 66

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

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

Focal segmental glomerulosclerosis is an important cause of the nephrotic syndrome in children and adults. This paper reviews the pathogenesis, clinical manifestations, morphology, and treatment of focal glomerulosclerosis. In addition, it considers the recently described association of focal glomerulosclerosis with nonglomerular renal diseases and the possible role of this glomerular lesion in progressive renal failure.
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PMID:Focal segmental glomerulosclerosis. 637 66

A girl with idiopathic steroid-resistant nephrotic syndrome was studied at onset of the disease at 6 years of age and during recurrences of the nephrotic syndrome after successive transplantations of three renal allografts. Focal glomerulosclerosis was demonstrated shortly after onset of the original disease with progression to renal insufficiency over 14 months. Recurrence of the nephrotic syndrome was documented 4 weeks following transplantation of the first renal allograft and a biopsy demonstrated focal segmental glomerulosclerosis without evidence of rejection. By 13 months, recurrent disease had caused renal failure requiring dialysis. After transplantation of the next long-functioning graft, the nephrotic syndrome rapidly recurred with urinary protein excretion of 8 g/day and normal glomerular filtration rate at 8 weeks. After the 5th month, progressive renal insufficiency developed and dialysis was resumed at 10 months after transplantation. The nephrectomy specimen showed advanced focal segmental glomerulosclerosis and chronic rejection. Early after the last renal allograft, she had mild persistent proteinuria and then developed nephrotic syndrome at 10 months which was attributed to chronic rejection. The graft was lost to delayed accelerated rejection at 17 months. The rapid recurrence of nephrotic syndrome after transplantation of each of the first two long-functioning allografts to this patient is most consistent with recurrence of original disease. However, observations in a subsequent graft demonstrated that rapid recurrence of the original disease is not inevitable.
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PMID:Recurrent nephrotic syndrome with three successive renal allografts. 675 72

Renal transplantation was performed in 25 patients for renal failure secondary to nephrotic syndrome and histologically proven segmental glomerulosclerosis (FSGS). They received 33 allografts, 13 from cadaveric (CAD) and 20 from living related donors (LRD) including 6 HLA-identical siblings. All have been followed for at least 1 year with none lost to follow-up. Overall, functional graft survival of the 33 grafts was 68.7% at 12 months and 60.5% at 48 months, similar to controls matched for age, sex, time of transplant, and donor source. Recurrent FSGS was documented histologically in 9 (5 CAD and 4 LRD) of 33 grafts (27.3%) and resulted in loss of graft function in 3 (9.9%). The presence and extent of mesangial proliferation (MP) in conjunction with or preceding typical lesions of FSGS in native kidneys was predictive of recurrent disease. Age at onset, duration of disease, and donor source were not. Sixteen patients with only FSGS on multiple biopsies of native kidneys received 22 allografts and recurrent disease occurred in 4 (18%) but did not cause loss of graft function in any. Six patients with focal areas of MP as well as FSGS underwent eight transplants, in which recurrent FSGS developed in two (25%) and caused graft loss in one. All three grafts transplanted to the patients with diffuse MP and FSGS developed recurrent disease, this resulting in graft failure in two. This study demonstrates the importance of a thorough histological evaluation by multiple biopsies of all patients with steroid-nonresponsive nephrotic syndrome. Only in this manner does it appear possible to define that subgroup of patients with FSGS who are at greatest risk of clinically significant recurrent disease.
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PMID:Incidence and significance of recurrent focal segmental glomerulosclerosis in renal allograft recipients. 704 54

In a retrospective analysis, 51 patients with focal segmental glomerulosclerosis and idiopathic nephrotic syndrome, who were treated with steroid or cyclophosphamide therapy, were divided into three clinical groups according to the remission profile of their nephrotic syndrome. Group 1 patients (19.37%) consistently responded to medication; none has progressive renal failure (mean follow-up 10.6 years). Group 2 patients (25, 40%) failed to respond to medication; terminal renal failure has occurred in 12 of them. Group 3 patients (7, 14%) initially appeared to be responsive to medication and continued to respond for up to 18 months, but subsequently became unresponsive to any therapy; five of them have required dialysis or transplantation. This third group of patients could not be separated clinically or pathologically from group 1 patients, all of whom have an excellent prognosis. One should, therefore, be cautious about predicting the outcome of steroid-responsive nephrotic patients, especially those with FSGS, until at least 18 months after the onset of illness.
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PMID:Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. 704 16

Focal segmental glomerulosclerosis, nephrotic syndrome and chronic renal failure were associated with spondyloepiphyseal dysplasia, growth failure, lymphopenia and transient ischemic attacks leading to severe neurological symptoms in three children. Two boys and one girl developed the full syndrome at the age of 5, 6 and 10 years. Positron emission tomography revealed perfusion defects of both cerebral and cerebellar arteries. A variant of the disease was found in two other children who had a nephrotic syndrome and terminal renal failure with only mild spondyloepiphyseal dysplasia, impaired growth and a normal cerebral function. It is concluded that there may be a close association between focal segmental glomerulosclerosis and spondyloepiphyseal dysplasias.
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PMID:Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia. 773 84


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