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)

A total of 411 children, aged from 0.3 to 18 years, suffering from glomerular diseases, were studied by renal biopsy between 1976 and 1985. The clinical presentation included nephrotic syndrome (79% of cases), renal failure (43%), and arterial hypertension (38%). In all, 177 cases presented with primary nephrotic syndrome; all had complicated courses and most were either corticosteroid-dependent or -resistant. Only 26.6% had minimal change disease on renal biopsy; 56.5% had focal-segmental sclerosis; and immunofluorescent deposits were observed in half of the group. Acute poststreptococcal (36 cases), mesangiocapillary (80 cases), and lupus (34 cases) glomerulonephritis occurred frequently; IgA glomerulopathy (10 cases) and haemolytic uraemic syndrome (6 cases) were uncommon. Glomerular crescents were observed in 71 cases. These observations illustrate the types of glomerular diseases seen in Iranian children.
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PMID:Glomerular diseases in children. "The Iranian experience". 270 71

We report on an acute glomerulonephritis with renal failure in a living-related renal allograft recipient with a cytomegalovirus infection. The clinical presentation was typical of an acute nephritis. Renal biopsy showed lesions of diffuse endocapillary proliferative glomerulonephritis. The disease resolved spontaneously. The pathogenesis of cytomegalovirus glomerulopathy is briefly discussed.
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PMID:[Acute cytomegalovirus glomerulonephritis in a renal transplant patient]. 283 87

Arterial hypertension is a frequent finding, even early in the course of diabetic nephropathy. Systemic and glomerular hypertension enhance the development of diabetic glomerulopathy and accelerate the rate of decline in glomerular filtration rate in diabetic nephropathy. Conversely, effective antihypertensive treatment reduces albuminuria and diminishes the rate of decline in glomerular filtration rate, thereby postponing end-stage renal failure in diabetic nephropathy.
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PMID:High blood pressure is a major factor in progression of diabetic nephropathy. 297 Oct 78

The sporadic concurrence of male pseudohermaphroditism and chronic glomerulopathy is associated with an extremely high risk of Wilms tumor. We report our experience with an infant who developed this triad (Drash syndrome) and review the 21 patients described in the literature, to emphasize the importance of early diagnosis and to suggest guidelines for management. The dysgenetic gonads are always intra-abdominal and carry a 20% to 30% risk for malignancy. The external genitalia are frequently ambiguous (77%); some children are phenotypically normal females. The glomerulopathy typically leads to end-stage renal failure in infancy; the subsequent death rate has, to date, been 68%. The clinical presentation of renal disease is variable and includes congenital nephrotic syndrome (14%) and infantile nephrotic syndrome (41%); 27% of patients develop proteinuria and renal insufficiency between the ages of 1 and 3 years. The high risk of Wilms tumor (55% in this review) mandates regular tumor surveillance, and prophylactic bilateral nephrectomy and gonadectomy once irreversible renal failure develops.
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PMID:Pseudohermaphroditism, glomerulopathy, and Wilms tumor (Drash syndrome): frequency in end-stage renal failure. 298 95

We report on 10 children, less than 2 years of age, who presented with a genuine type of glomerulopathy: diffuse mesangial sclerosis. In 5, the nephropathy was associated with male pseudohermaphroditism (MPH) and Wilms' tumor (WT); in 3 with MPH and in 2 with WT. The nephropathy was characterized by its very early onset, between the age of 2 weeks and 18 months. Eight patients presented with a nephrotic syndrome with (7 cases) or without (1 case) hypertension. All, but one, who is in advanced RF at 11 years of age, progressed to chronic or end-stage renal failure (ESRF) within a few months to 2 years from the onset. One additional child presented with advanced renal failure at the age of 8 months and the last one, who was hypertensive, developed an anuria related to thrombosis of renal veins at 1 year of age. Drash syndrome is characterized by the association of a "nephron disorder" with MPH and WT. We propose, on the basis of our histological findings, to extend the concept of Drash syndrome to patients who, in addition to the nephropathy, have either WT or MPH and to consider the distinctive glomerular lesions presented by all these patients as their common denominator. The pathogenesis of this glomerulopathy is obscure. Its early onset, its association with a dysembryoplastic tumor and/or with gonadal dysgenesis both suggest an antenatal dysgenetic process.
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PMID:The nephropathy associated with male pseudohermaphroditism and Wilms' tumor (Drash syndrome): a distinctive glomerular lesion--report of 10 cases. 300 Jun 66

The association of nephropathy with Wilms' tumor and male pseudohermaphroditism is known as Drash's syndrome. The absence of either pseudohermaphroditism or Wilms' tumor has also been reported. The nephropathy, characterized by proteinuria or nephrotic syndrome, leads rapidly and inevitably to renal failure. The renal findings are those of a chronic glomerulopathy with mesangial hypercellularity, glomerular sclerosis, interstitial infiltration and marked tubular dilation. This infrequent syndrome usually appears in early life, between 1 and 3 years of age. The case of nephropathy associated with Wilms' tumor we present here is characterized by age and onset: an abrupt onset of renal insufficiency without previous signs of nephropathy in a 13-year-old girl.
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PMID:Nephropathy associated with Wilms' tumor. A case of a 13-year-old girl. 303 29

The medical histories of 27 patients with renal vein thrombosis were evaluated retrospectively. Short and long term evolution as well as prognostic factors were analysed. 24 of the patients were suffering from a nephrotic syndrome, 15 from renal failure. In the 20 biopsies performed we observed: 14 cases of extramembranous glomerulonephritis, 2 cases of minimal glomerular lesions, 3 cases of segmental focal glomerulosclerosis and 1 periarteritis nodosa. The renal vein thrombosis was bilateral (18), left (7) or right (2). In 7 patients this was associated with thrombosis of the inferior vena cava. 9 patients were treated with anticoagulants alone, 9 underwent thrombectomy, 7 were treated by thrombolysis and 2 received no treatment. Within the first six months 11 patients died as a result of complications due to hemorrhaging (5), septic infection (2) or embolism (1) and 3 patients died of undetermined causes. The progression of the remaining patients was followed up over a period of 6 months to 19 years. In 12 patients, the nephrotic syndrome regressed and renal function did not deteriorate in any of the cases. The main causes for poor prognosis are the existence of initial renal failure and glomerulonephritis which is not extramembranous. Since the evolution of the glomerulopathy does not appear to be affected by extramembranous glomerulonephritis, renal vein thrombosis associated with this complication should be treated with anticoagulants alone. The risk-benefit ratio of thrombectomy leads us to conclude that this technique should be abandoned. Thrombolysis could be considered in cases where renal vein thrombosis is associated with acute renal failure.
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PMID:[Prognosis of renal vein thrombosis in the adult: influence of treatment]. 321 41

We report a patient with polycystic kidney disease, advanced renal failure, and nephrotic-range proteinuria. Kidney biopsy revealed IgA nephropathy with lesions of focal and segmental glomerular sclerosis. This association had not been previously described and is probably coincidental. This case supports the assumption that the nephrotic-range proteinuria observed in some polycystic patients could be the consequence of another superimposed glomerular disease. This glomerulopathy can worsen the course of azotemia in these patients.
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PMID:IgA nephropathy and polycystic kidney disease. 321 62

Twelve HBsAg-negative patients with histologically documented cirrhosis of the liver of either alcoholic (8 of 12) or cryptogenic (4 of 12) origin underwent renal biopsy to investigate proteinuria, hematuria and/or renal failure. Immunofluorescence was positive for IgA in 2 patients with mesangiocapillary glomerulonephritis (MCGN) and could not be performed in 2 additional patients with the same diagnosis. However, in the remaining 8 patients, immunofluorescence was negative for IgA and frequently positive for C3, IgG, IgM and/or fibrinogen. These 8 patients without IgA were classified as follows: MCGN with subendothelial electron-dense deposits (2 cases), IgM-IgG cryoglobulinemia with diffuse endocapillary glomerulonephritis (1 case), membranous nephropathy (1 case), diffuse endocapillary proliferative glomerulonephritis (1 case), vasculitis with focal segmental necrotizing glomerulitis and crescentic glomerulonephritis (2 cases). These results show that cirrhosis of the liver can be associated with a wide variety of glomerular disorders. Contrary to previous belief, IgA is absent in two thirds of patients with cirrhosis and glomerulopathy. Therefore, the pathogenetic importance of IgA in the development of glomerular disease in such patients is doubtful.
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PMID:Glomerular disease in cirrhosis of the liver: low frequency of IgA deposits. 352 93

Accuracy of ultrasonography (US), quantitative scintigraphy, and magnetic resonance (MR) imaging in diagnosis of acute renal allograft rejection was studied in 46 patients who underwent renal biopsy. Thirty-three patients had acute rejection; six, cyclosporine nephrotoxicity, as shown by biopsy, clinical findings, and follow-up study; two, acute tubular necrosis; and five, normal biopsy findings and renal function. Accuracy in demonstrating rejection was 72% for US and 75% for scintigraphy, indicating no significant difference between the two. MR imaging was significantly more accurate, reaching a level of 98%. However, accuracy of MR in demonstrating acute tubular necrosis in a larger number of patients is not known, and its accuracy in indicating recurrent glomerulopathy or infectious disease has not been addressed. The definitive role of MR in evaluating posttransplant renal failure is currently not established, but because of its high sensitivity in detecting renal abnormality, MR can be used for cases when results of US or scintigraphy are equivocal or contradict clinical impressions or when biopsy cannot be performed for medical reasons.
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PMID:Posttransplant renal rejection: comparison of quantitative scintigraphy, US, and MR imaging. 354 32


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