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)

Camostat mesilate, a developed derivative of gabexate mesilate for oral use, was administered in a daily dose of 600 mg for 4 weeks to 17 patients with heavy proteinuria due to various nephropathies. Five patients had glomerulonephritis (3 patients with IgA nephropathy, one each with membranoproliferative GN and membranous nephropathy) and 3 had systemic vasculitis. These patients had been treated with glucocorticoid, cyclophosphamide, anticoagulants, and dipyridamole. Five patients had diabetic nephropathy and had been treated with conventional therapy including angiotensin converting enzyme inhibitors. Two cases with benign nephrosclerosis, one with Alport syndrome, and the rest with end-stage renal failure of undetermined cause were also included in this study. Urinary protein decreased promptly within 2 weeks (from 5.2 +/- 0.7 to 3.5 +/- 0.5, mean +/- SE, p less than 0.005), and serum total protein and albumin levels increased significantly. Serum creatinine levels did not change. Decreases in urinary protein excretion of more than 50% were observed in five out of eight patients with glomerulonephritis or systemic vasculitis, two out of five with diabetic nephropathy, and one with chronic renal failure. However, urinary protein excretion values remained at the same level in two patients with benign nephrosclerosis and a patient with Alport syndrome. We suggest that camostat mesilate caused a change in glomerular capillary permeability for macromolecules through its inhibitory effects on the kallikrein-kinin system, complement system, coagulation system, and platelet function, which contributed to the treatment of the various nephropathies.
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PMID:Effect of camostat mesilate on heavy proteinuria in various nephropathies. 279 62

Primary IgA mesangial nephropathy was first described in adults by Berger, and has been increasingly recognized in children. IgA nephropathy is a frequent type of glomerulonephritis in 3 to 15 year-old children in France. Clinical features and outcome have been defined and the progression to renal failure is possible. The pathogeny of IgA nephropathy remains unclear and is under multifactorial control and, at present, no satisfactory specific treatment is available.
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PMID:[Berger's disease or primary IgA nephropathy in children]. 281 62

IgA nephropathy (IgAN) leads to renal failure in up to 30% of affected children and adults. There is currently no consensus on therapy in IgAN. Six patients with risk factors for disease progression were identified based on clinical or histologic findings. These patients were treated with alternate-day prednisone for 12 to 60 (mean, 36) months and followed for 28 to 60 (mean, 54) months. Follow-up biopsies were available in four patients. At last examination all treated patients had normal urinalysis and serum creatinine level. Follow-up biopsies showed stable or improved glomerular histology in three of four patients. One patient had a slight worsening of the interstitial disease. No steroid toxicity was observed. The outcome of these treated patients was compared with that of 15 comparable patients from another center who received no treatment and with patients from two published clinical pathology series. A normal urinalysis was found at follow-up in all treated patients, compared with one of 15 untreated patients (P = 0.003). None of the patients in the published series with comparable disease had normal urinalysis at follow-up. End-stage renal disease or renal insufficiency occurred in seven of 15 untreated and no treated patients (P = 0.19). The data strongly support the need for a prospective control trial of prednisone therapy in IgAN.
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PMID:Alternate-day prednisone therapy in children with IgA-associated nephritis. 291 65

We describe a patient who developed terminal renal failure of two HLA-identical renal allografts due to crescentic IgA nephropathy. The first graft contained IgA deposits at the time of donation, suggesting that transmission of IgA deposits may have contributed to the nephritis of the first allograft. The second graft was free of IgA deposits at the time of donation, but the recipient developed a similar, rapidly progressive nephritis. This case points up the malignant potential of IgA nephropathy and the complex nature of transplant planning for patients with end-stage renal disease (ESRD) secondary to IgA nephropathy. Living-related donor (LRD) transplants seem to be associated with a higher rate of recurrence than cadaveric grafts. This higher rate may partly reflect the inadvertent transmission of subclinical IgA deposits from donor to recipient and a genetic susceptibility of certain HLA types (specifically B35 and DR4) to recurrent disease. Cadaveric transplants may be preferable in the setting of high-risk HLA types or familial patterns of IgA nephropathy.
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PMID:Recurrent IgA nephropathy in living-related donor transplantation: recurrence or transmission of familial disease? 304 1

The diagnosis of recurrent renal disease after transplantation is dependent on an accurate and complete diagnosis of the initial cause of renal failure and a similar determination of the cause of graft failure. To be classified as recurrent, the disease in the renal graft must be identical to that seen in the native kidneys. Recurrence of disease accounts for less than 2% of all graft failures, but the overall incidence of recurrent disease is probably 5 to 10 times more common. The most frequent cause of recurrent disease is glomerulonephritis, which was first recognized to recur soon after renal transplantation was introduced. It was then recognized that a variety of metabolic disorders would recur, but it has taken 25 years of experience for a clear picture to emerge of recurrence in most conditions. No initial cause of renal failure poses a contraindication to at least one attempt at transplantation, although with Fabry's disease and oxalosis, a special assessment of the risks for the individual recipient is warranted. In some patients, experience has shown the need for a delay in the commitment to transplantation (eg, in those with anti-glomerular basement membrane [GBM] antibody glomerulonephritis or Henoch Schonlein purpura), the need for the choice of a particular immunosuppressive regimen (eg, in hemolytic uremic syndrome [HUS]), the need for avoidance of primary nonfunction (eg, in oxalosis), and the desirability of avoiding live kidney donation (eg, in heterozygote donors in Fabry's disease, high-risk recipients with focal glomerulosclerosis, and in recipients with HUS). Probably all types of glomerulonephritis recur, but with great variation in frequency and severity. In some forms of glomerulonephritis, recurrence may be frequent and definite on histopathological criteria but may only have a minor clinical expression (eg, dense deposit disease, anti-GBM antibody glomerulonephritis, IgA nephropathy), but in others, recurrence is less predictable yet it is clearly associated with premature graft failure (eg, focal glomerulosclerosis, membranous nephropathy). A common theme emerging is that where the initial glomerulonephritis is aggressive and causes kidney failure over a short time, recurrence is more likely, and when present, it will lead to graft failure with an increased frequency. Clinical manifestations, the frequency of recurrence, and the prognosis of the graft are now identified for most conditions. Unexpected observations have included the rarity of recurrent systemic lupus erythematosus (SLE), the immediate return of heavy proteinuria in focal glomerulosclerosis, and the predictable return of dense deposit disease.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Recurrence of disease following renal transplantation. 304 3

It is becoming evident that IgA nephropathy (IgAN) is the most common glomerular disease, and a frequent cause of end-stage renal failure in both white and Asian populations. Its significance as a public health problem is not known since little epidemiologic research is available in most countries. The apparent geographic variations in the percentage of IgAN in kidney biopsy specimens may reflect different clinical policies for diagnostic tests. As a consequence, the frequency of IgAN cannot be accurately extrapolated from these data for any given population. The highest percentages, reported in Singapore and Japan, may be influenced by the systematic screening of urines in both countries. By contrast, IgAN has been detected rarely in blacks either from the United States or from Africa. However, biopsies are performed infrequently in African patients with only microscopic hematuria. Such ethnic differences may suggest a possible role of genetic factors in the etiology of IgAN. As shown recently in France and Italy, antibiotic therapy of streptococcal infections apparently has not influenced the percentage of IgAN in kidney biopsy specimens. These facts and the rarity of the glomerulonephritis in blacks suggest that infections may not be responsible for the etiology of IgAN. The traditional search for causal agents should be approached more vigorously. It will require innovative epidemiologic efforts to understand the mechanisms by which multiple factors (environmental and genetic) acting together influence the risk of disease.
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PMID:Worldwide perspective of IgA nephropathy. 305 55

Two different clinical syndromes might be observed at presentation in most patients with IgA nephropathy (IgAN): (1) an acute reversible episode of macroscopic hematuria or (2) asymptomatic urinary abnormalities. Patients in these groups differ by genetic markers, the severity of their histologic lesions, and the rate of progression to renal insufficiency. Macroscopic hematuria is more common in children, and its frequency decreases with increasing age. In our experience, most patients presenting in adulthood with macroscopic hematuria did not have proteinuria or microscopic hematuria prior to the episode of macroscopic hematuria, suggesting the onset of disease was indeed in adulthood. IgAN is not a benign disease. About 20% of patients reach end-stage renal failure after 20 years of clinical disease. Features generally associated with a poor prognosis include older age at onset, no history of recurrent macroscopic hematuria, hypertension, and consistent proteinuria. In some studies, men progressed more rapidly than women. Using the regression of Cox in the present study, the magnitude of proteinuria was the only clinical parameter that independently predicted progressive renal impairment.
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PMID:Clinical features and natural history in adults with IgA nephropathy. 305 56

C3 and Bf alleles were examined in the general population, in 67 patients with biopsy-confirmed mesangial IgA nephropathy and 81 patients with other types of glomerulonephritis, from the Heidelberg and Leiden renal programmes respectively. In both populations, a significant excess of homozygous phenotype C3FF (3.4% in controls; 10.4% in IgA nephropathy) and a deficit of C3FS heterozygous phenotype (35.8% in controls; 19.4% in IgA nephropathy) were observed in patients with IgA nephropathy, but not in other types of glomerulonephritis. No difference of C3 gene frequencies was found. C3FF was associated with an adverse clinical outcome (a higher prevalence of renal failure and hypertension). A significant excess of Bf-F gene frequency was noted (0.20 in controls; 0.33 in IgA nephropathy). In addition, an excess of phenotype BfFF was found (none in controls; 10.4% in IgA nephropathy). BfFF homozygotes also carried a higher risk of an adverse outcome (renal failure and hypertension). The data suggest a role for genetically coded (presumably) immunological factors in the genesis and course of IgA nephropathy.
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PMID:Genetic polymorphism of C3 and Bf in IgA nephropathy. 311 58

This article discusses the feasibility of fitting a straight line to the reciprocal serum creatinine (1/SCr) values obtained from 32 patients with IgA mesangial nephropathy and established renal insufficiency. Three models were tested for fit: linear, logarithmic and quadratic. The decline of renal function, once renal failure has been established, is neither constant nor regular in all patients with this fairly well-diagnosed glomerular disease. Therefore we cannot rely on simple mathematical models to compare sequences of serum creatinine values or to forecast decline in renal function in patients with IgA nephropathy.
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PMID:Renal function in IgA nephropathy with established renal failure. 313 34

A retrospective study of 67 patients with IgA nephropathy carried out at the Glasgow Royal Infirmary revealed an overall 10-year actuarial renal survival of 77.4%. At the time of presentation, 27 patients (40.3%) were hypertensive and 40 (59.7%) were normotensive. As expected, the survival was worse in the hypertensive group. However, when the effect of control of blood pressure was assessed, a significantly worse survival was found in those whose hypertension was inadequately controlled, compared to those whose hypertension was well controlled, in whom survival was not significantly different from that of the normotensive group. The differences in survival could not be explained by increased patient age nor by longer duration of disease. Good control of hypertension may prevent progression to end-stage renal failure in IgA nephropathy.
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PMID:Progressive IgA nephropathy: the role of hypertension. 314 79


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