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Query: UMLS:C0035078 (renal failure)
 31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

IgA nephropathy is the most common form of primary glomerulonephritis worldwide. About one quarter of patients progress to terminal renal failure 10 years after the apparent clinical onset. Therefore, the disease represents a social problem in terms of number of patients requiring maintenance hemodialysis. Despite the intense research effort devolved to clarify the pathogenesis of IgA nephropathy, the exact relationship linking the several factors involved is still unknown. In this review we analyze the experimental works reported since 1979, when the first animal model of IgA nephropathy was published by Rifai et al. We also discuss the interplay between experimental data and clinical observations to maximize the information gathered from the different animal models. Finally, we report the new insights into the role played by cytokines, growth factors and autacoids.
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PMID:Primary IgA nephropathy: the relevance of experimental models in the understanding of human disease. 130 Apr 32

We have studied glomerular basal laminar thickness in biopsy material, using a simple technique involving 16 selected measurements per case. Twenty-nine biopsied cases of adult glomerular haematuria were examined together with 'diseased' controls represented by a variety of glomerulopathies including minimal-change disease and IgA nephropathy. 'Normal' control populations were provided by 13 patients with acute-onset renal failure of non-glomerular origin and nine patients undergoing nephrectomy. Analysis of groups determined by the presence or absence of haematuria, the degree of proteinuria and presence or absence of a diagnostically characteristic immunofluorescence pattern showed that the nine patients with haematuria and proteinuria of less than 200 mg/24 h represented a distinct subpopulation with a mean membrane thickness of 225 nm compared to the control mean of 343 nm (P less than 0.0001). All members of this subpopulation had mean values below an arbitrary cut-off value of 270 nm. Within other specific disease categories, sporadic cases had mean membrane thicknesses below this critical value, indicative of an overlap of pathologies. On short-term follow-up there is no evidence that the 'pure' thin-membrane population are subject to any deterioration in renal function. It is of further interest that eight of nine thin-membrane 'syndrome' cases were O Rh positive. This finding may provide a starting point for investigation of a specific genetic defect.
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PMID:Glomerular basement membrane thinning in adults: clinicopathological correlations of a new diagnostic approach. 131 88

Two female sibs aged 15 and 18 years with microcephaly, mental retardation and marfanoid habitus who developed focal segmental glomerulonephritis leading to renal failure are described. This combination of features appears to represent a unique syndrome distinct from previous reports of microcephaly in association with the nephrotic syndrome. The mode of inheritance is likely to be autosomal recessive.
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PMID:Microcephaly, focal segmental glomerulonephritis and marfanoid habitus in two sibs. 134 13

The Medical Research Council's Glomerulonephritis Registry was used to study clinicopathological correlations and renal survival in patients with IgA nephropathy reported between 1978 and 1985. IgA nephropathy was the histological diagnosis in 9.3 per cent of all renal biopsies reported to the registry during this period, and in 18.1 per cent of those with a primary glomerulonephritis. The 10-year cumulative renal survival rate accounting for censored data (Kaplan-Meier) was 83.3 per cent. Univariate analysis of survival curves (log-rank test) found the following parameters to be significantly correlated with poor renal survival: serum creatinine > 120 mumol/l (p < 0.001), hypertension (p < 0.001), serum albumin < 40 g/l (p < 0.005), proteinuria > 1 g (p < 0.025), age > 30 years (p < 0.025), and focal mesangial proliferation (p < 0.05). There was no significant difference in renal survival between males and females. Multivariate analysis (Cox's proportional hazards model) revealed that only a serum creatinine of > 120 mumol/l and a serum albumin of < 40 g/l were independently predictive of outcome. These findings indicate marked similarities between the UK experience of IgA nephropathy and the published European experience. IgA nephropathy is not a benign condition in the UK and patients with impaired renal function and/or those with a reduced serum albumin are significantly more likely to progress to end-stage renal failure within 10 years.
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PMID:Clinico-pathological correlations and long-term follow-up of 253 United Kingdom patients with IgA nephropathy. A report from the MRC Glomerulonephritis Registry. 148 40

Clinicohistopathologically, we observed 109 patients with asymptomatic urinary abnormalities found via the Japanese school medical screening process. Follow-up was for a mean period of 9.3 +/- 4.0 years. More than 80% of the patients had either IgA nephropathy (IgAN, 47 cases, 43.1%), thin membrane disease (TMD; 21 cases, 19.3%) or normal glomerulus (NG; 20 cases, 18.3%). Complete remission appeared in 60.0% of the NG cases, 14.3% of the TMD cases and in 19.1% of the IgAN cases, and remission was significantly high in the NG group (p less than 0.01). No patient with TMD and NG ever progressed to the extent of pronounced proteinuria or renal failure. One patient deteriorated and required hemodialysis, and 2 patients developed renal insufficiency in IgAN. All of these cases possessed severe glomerular sclerotic change when the initial biopsies were performed. All IgAN cases that went into remission, however, had minor glomerular abnormalities. A positive family history of urinary abnormality was observed in 14.1% of both the IgAN group and the NG group, whereas we observed 71.4% in the TMD group, which was significantly high (p less than 0.01). Other cases included 4 each with non-IgA proliferative glomerulonephritis, focal segmental glomerular sclerosis, membranoproliferative glomerulonephritis and Alport's nephritis. It was concluded that the majority of patients (80.7%) with urinary abnormalities found via the school screening program had IgAN, NG or TMD. 74.5% of the IgAN group and 85.7% of the TMD group had long histories of urinary abnormalities extending into adulthood with no deterioration of the renal function.
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PMID:Asymptomatic urinary abnormalities found via the Japanese school screening program: a clinical, morphological and prognostic analysis. 152 46

Proliferation of mesangial cells and expansion of mesangial matrix are common histologic features of proliferative glomerular disease, a frequent cause of renal failure. Proliferation of glomerular mesangial cells occurs in response to platelet-derived growth factor (PDGF), and these cells release PDGF and express PDGF A and B chain mRNAs. However, all studies relating PDGF to potential changes in glomerular structure and function to date have been performed in vitro. To explore the role of PDGF in proliferative glomerulonephritides, we studied the expression of PDGF in vivo in two animal models of IgA nephropathy with different histologic patterns of glomerular injury: either predominant mesangial proliferation or expansion of mesangial matrix. Increased expression of PDGF and PDGF B-chain mRNA in whole kidneys from diseased mice was demonstrated by immunohistochemical techniques and by solution hybridization assay, respectively. Immunohistochemically, PDGF was localized primarily within the mesangial area of glomeruli and to a much lower extent in interstitium. The increased PDGF expression correlated with the degree of hypercellularity and clinical features of the disease. In addition, PDGF expression was increased in some forms of human glomerulonephritis, characterized by mesangial proliferation. These findings suggest that PDGF may be a major contributor to mesangial cell proliferation seen in proliferative glomerulonephritides.
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PMID:Platelet-derived growth factor expression in mesangial proliferative glomerulonephritis. 171 46

Leukocytoclastic vasculitis, immune complex disorder (type III), is a skin disease with both an acute form characterized by bullae, vesicles and ulcerations, and a chronic form characterized by petechiae, macules and ulcerations. The disease presents certain systemic features including diffuse or focal glomerulonephritis and renal failure. The histopathologic characteristics of leukocytoclastic vasculitis in the skin appear primarily in small blood vessels and consist of an infiltration of inflammatory cells, leukoclasis, swelling of endothelial cells, occlusion of blood vessels, accumulation of fibrin and fibrinoid degeneration, as well as the presence of immune complexes in and around blood vessel walls. Although leukocytoclastic vasculitis is described as several diseases which can spread systemically, including the gastrointestinal tract and the kidneys, the manifestations of the disease in the oral cavity have not yet been reported. The present paper reports unique oral lesions in a 38-yr-old woman, diagnosed as leukocytoclastic vasculitis, without any accompanying skin or systemic lesions.
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PMID:Leukocytoclastic vasculitis (anaphylactoid purpura): a unique occurrence in the oral cavity. 175 54

In 191 patients with mesangial IgA nephropathy, GFR was determined as clearance of 51Cr-EDTA. 86 (45%) of them had subnormal renal function 7.3 +/- 4.6 years after renal biopsy. The change in GFR was followed in 153 patients with repeated determinations of 51Cr-EDTA clearance. 50.3% of the patients had a loss of more than 1.1 ml/min/year, which we regard as pathological. The markers of progressive disease were: male sex, high output of urinary protein, severe histological lesions and presence of hypertension. Even patients lacking these markers had a significantly increased incidence of progressive disease. Of 93 patients, with initially normal GFR, 32% will have a subnormal GFR within five years and 25% will develop end-stage renal failure within 20 years. In 38 patients with six or more determinations of 51Cr-EDTA clearance, the predictive value of the first four determinations was calculated. Of 26 with a decrease of more than 1.1 ml/min/year, 13 (50%) developed subnormal GFR during follow-up, while 11 of 12 (91.7%) with a decrease of less than 1.1 ml/min/year (P less than 0.05) remained normal. This shows that repeated determinations of GFR with an accurate method will predict the final outcome early in the disease. We also confirmed that single or repeated determinations of clearance of creatinine are of little value in separating a normal GFR from a slightly decreased one, but more reliable in detecting a markedly reduced GFR.
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PMID:Deterioration of GFR in IgA nephropathy as measured by 51Cr-EDTA clearance. 176 5

The impacts of IgA nephropathy and pregnancy on each other were evaluated in 118 women who conceived 168 times between 1970 and 1988. Rates of spontaneous abortion, normal delivery, live birth and perinatal death were 9, 66, 87 and 4%, respectively. Infants born to women with glomerular filtration rates (GFR) lower than 70 ml/min prior to conception had a higher perinatal mortality rate (14% vs. 3%, P less than 0.001). This was also true if pre-pregnancy blood pressures were consistently higher than 140/90 mm Hg (33% vs. 1%, P less than 0.001). These were the figures for the whole 18 year period, but stratification of the data revealed that most adverse results occurred in the 1970's, during which the perinatal death rate was 9%, while it was 0% in the 1980's. Eighty-five women were followed for three years or more. At final follow-up, the rates of decrease in GFR, and increases in blood pressure and proteinuria were 19, 11 and 7%, respectively. In most patients the natural history of IgA nephropathy was similar to that of women who had not experienced pregnancy, but there were five instances where gestation seemed to accelerate functional loss, with rapid development of end-stage or near end-stage renal failure. Most women with IgA nephropathy should anticipate few problems with pregnancy, if they are normotensive and their preconception GFR exceeds 70 ml/min. The gestation in such instances should have little influence on the natural history of their nephropathy.
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PMID:Pregnancy in IgA nephropathy. 176 10

Angiotensin-1 converting enzyme inhibitors (ACEI) have been shown to reduce proteinuria in azotaemic diabetics and in other glomerulopathies, and such treatment has also slowed the development of experimentally-induced glomerulosclerosis in animals. We have treated 13 patients with focal segmental glomerulosclerosis (FSGS) and IgA nephropathy (IgAN) with Captopril 12.5 mg twice daily for six months and assessed their response in terms of 24 hour urinary protein excretion, blood pressure, glomerular filtration rate, effective renal plasma flow and derived values for filtration fraction and renal vascular resistance. A mean fall of 29 per cent in urinary protein excretion was observed over the six months treatment schedule. No significant changes were observed in other parameters of renal haemodynamics measured. We conclude that Captopril therapy in patients with FSGS and IgAN reduces urinary protein excretion consistently over a six month period, and that this may in the longer term retard the progression of their renal failure.
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PMID:Reduction of proteinuria with captopril therapy in patients with focal segmental glomerulosclerosis and IgA nephropathy. 181 Aug 99


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