Minor Urinary Abnormalities Research Articles

Predicting the prognosis of renal dysfunction by renal expression of α-smooth muscle actin in children with MPGN type 1

Background: To clarify whether renal α-smooth muscle actin (α-SMA)-positive cell and macrophage accumulation can predict the prognosis of renal dysfunction, we evaluated them by means of multivariate analysis and compared them with other clinical predictors in patients with membranoproliferative glomerulonephritis (MPGN) type 1 with normal renal function. Methods: We enrolled 35 patients with MPGN type 1 who had normal creatinine clearance at the time of renal biopsy. These patients were divided into 2 groups based on clinical status at the last examination. Group 1 consisted of 12 patients with normal urine and 12 patients with minor urinary abnormalities at the latest observation, whereas group 2 consisted of 7 patients with persistent nephropathy and 4 patients with renal insufficiency. The first and second renal biopsy findings, including α-SMA and CD68-positive staining, were investigated for both groups. Results: Mean scores for glomerular and interstitial α-SMA staining in group 2 were significantly higher than those in group 1. At the second biopsy, mean scores for interstitial CD68-positive staining in group 2 were higher than those in group 1. Mean scores for glomerular and interstitial α-SMA at the first biopsy correlated with the chronicity index at the second biopsy in both groups. Conclusion: Our results suggest that glomerular and interstitial α-SMA expression at the first biopsy can predict the prognosis of children with MPGN type 1.

Clinical and pathological features of children with Henoch-Schoenlein purpura nephritis: risk factors associated with poor prognosis

To clarify the risk factors related to prognosis in patients with Henoch-Schoenlein purpura nephritis (HSPN), we investigated the cases with HSPN on long-term observation. We enrolled 114 patients who had been diagnosed with HSPN from 1974-1997. These patients were divided into 2 groups based upon features at last follow-up. One group, designated "favorable", consisted of 69 patients with normal urine and 25 patients with minor urinary abnormalities, and the second group, designated "unfavorable", consisted of 15 patients with active renal disease and 5 patients with renal failure. The clinical features, laboratory data and pathological findings were investigated in 2 groups. Nephrotic syndrome, decreased factor XIII activity, hypertension and renal failure at onset were more frequent in "unfavorable" than in "favorable". The rate of glomeruli with crescents, macrophage infiltrations, tubulointerstitial changes and acute exacerbation in "unfavorable" were higher than those in "favorable". There were 5 cases with renal insufficiency, and renal survival rate was 95.6% for over 15 years. These results suggest that the above mentioned risk factors play an important role in prognosis of the patients with active renal disease and renal failure.

IgM nephropathy: Clinical picture and long-term prognosis

Background: Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis with mesangial hypercellularity and diffuse IgM deposits. Methods: We studied clinical presentation, morphological findings, and prognostic factors in 110 patients with IgM nephropathy without systemic diseases. The series included both pediatric and adult patients with nephrotic syndrome (NS) or minor urinary abnormalities. Results: Mean postbiopsy follow-up was 8 years. During 15 years of follow-up, 36% of patients developed renal insufficiency and 23% reached end-stage renal failure. In multivariate analysis, hypertension at the time of renal biopsy was the only significant risk factor for renal insufficiency. Of histological parameters, interstitial fibrosis had the strongest prognostic value. Hypertension was diagnosed in 50% of patients with a postbiopsy follow-up of 15 years. Twenty-nine percent of nephrotic patients had disease resistant to corticosteroids, whereas 80% of patients with steroid-sensitive disease were steroid dependent. Eleven patients, 8 patients with NS and 3 patients with asymptomatic proteinuria, underwent repeated renal biopsy. In five samples, typical morphological characteristics of focal and segmental glomerulosclerosis were seen. Conclusion: We propose that IgM nephropathy can be divided into two subgroups with similar renal biopsy findings, but differences in sex distribution and initial presentation. Am J Kidney Dis 41:343-350.

Multiple combined therapy for severe Henoch-Schönlein nephritis in children.

From 1980 through 1992, 14 children with Henoch-Schönlein nephritis (HSN) showing severe glomerular changes (grade IV or V) were given a multiple combined therapy with prednisolone, cyclophosphamide, heparin/warfarin, and dipyridamole, and were followed for 7.5+/-0.9 years. The period between the onset of nephritis and the start of therapy was 0.8+/-0.4 years. Ten patients underwent follow-up biopsy after therapy. The percentage of glomeruli having crescents/segmental lesions was significantly reduced after therapy (70%+/-5% vs. 42%+/-7%, P <0.01), due mainly to the resolution of crescents (51%+/-8% vs. 13%+/-5%, P <0.01). Thus, histological grade was significantly improved (5 grade IV and 5 grade V vs. 7 grade III and 3 grade IV, P <0.01). After an average follow-up period of 7.5 years, 9 patients showed normal urine and renal function, 4 showed minor urinary abnormalities, and 1 heavy proteinuria. No patient developed chronic renal insufficiency. These findings suggest that the multiple combined therapy could be effective. for histologically severe HSN, although a prospective controlled study should be performed.

Prognostic Indicators in Childhood IgA Nephropathy

A number of clinical, laboratory and pathologic parameters were assessed for their prognostic significance in 200 children aged less than 15 years with IgA nephropathy, who had shown normal renal function at the time of initial biopsy and were followed for more than 2 years thereafter. After a mean follow-up period of 5.0 years from the initial biopsy, 93 patients had no demonstrable abnormality, 76 had minor urinary abnormalities, 21 had persistent heavy proteinuria and 10 had developed chronic renal impairment. A poor outcome was found to be correlated with heavy proteinuria at biopsy, diffuse mesangial proliferation, a high proportion of glomeruli showing sclerosis, crescents or capsular adhesions, the presence of moderate or severe tubulointerstitial changes, and the presence of subepithelial electron-dense deposits and lysis of the glomerular basement membrane by electron microscopy. The percentage of glomeruli displaying crescents, sclerosis and adhesions appeared to be the most reliable prognostic indicator. Nine of the 27 patients (33%) in whom greater than or equal to 30% of glomeruli showed crescents, sclerosis and adhesions developed chronic renal impairment, and only 14% of these patients had normal urine at follow-up. In contrast, only 1 of the 173 patients in whom less than 30% of glomeruli showed such lesions developed chronic renal impairment (p less than 0.001) and 51% of these patients showed complete remission at follow-up (p less than 0.001). These results demonstrate that an accurate prediction of the outcome based on the initial renal biopsy findings is possible early in the course of children with IgA nephropathy.

Les indices de progression de la glomérulopathie à IgA

A retrospective and prospective study of 58 patients with IgA glomerulonephritis was undertaken between the 1st April 1975 and the 1st May 1982, to determine the clinical, biological and histological features associated with the onset of renal failure and a poor prognosis. At the end of this study the patients were classified into 4 groups according to the clinical stage of their disease: a) normal, b) minor urinary abnormalities, c) active disease and d) terminal renal failure. The renal biopsies were reviewed and an index of histological activity defined. The significant features associated with progression of the disease to renal failure were: persistent proteinuria, a transient nephrotic syndrome, hypertension, diffuse proliferative glomerular changes with epithelial crescent formation, tubulo-interstitial changes and vascular lesions on renal biopsy. When confronted with the clinical outcome, the histological results of the first renal biopsy performed when the disease was first diagnosed and quantified in an index of activity, had a good predictive value.

Prognosis of Henoch-Schönlein nephritis in children.

All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.

Renal involvement in Schölein-Henoch purpura.

Abstract. Koskimies, O., Rapola, J., Savilahti, E. and Vilska, J. (The Children's Hospital, University of Helsinki, Helsinki, Finland). Renal involvement in Schonlein‐Henoch purpura. Acta Paediatr Scand, 63: 357, 1974.–During the period 1964–71 Schönlein‐Henoch syndrome was found in 91 children. Fifty‐four showed no signs of renal disease and recovered rapidly. Eighteen patients with haematuria or proteinuria lasting less than 4 weeks also made full recovery. Nineteen patients had persisting urinary abnormalities and were followed‐up clinically and with biopsies. Immunotluorescent and electron microscopic studies were performed in 6 patients.Histopathological changes correlated well with the severity of the disease. Generalized proliferative glomerulonephritis, sometimes with glomerular fibro‐epithelial crescents, signified severe disease with protracted course. Most of these cases had heavy proteinuria and often nephrotic syndrome. The outcome after a follow‐up of 2 years was good, however. Twelve patients recovered completely and 6 were clinically symtomless but showed minor urinary abnormalities. Only one died and she had showed fulminant clinical and histopathological glomerulonephritis from the beginning of the disease.Immunotluorescent studies showed positive reactions in the mesahgium and glomerular capillaries in 4 patients. In the electron microscopy dense deposits were found at the corresponding sites. In two late biopsies immunotluorescence was negative despite definitive histological changes suggesting disappearance of the immunopathogenetic effectors of the nephritis.

Schönlein—Henoch Nephritis

We report the clinical and laboratory findings of 88 patients with renal manifestations of Schonlein-Henoch syndrome, all of whom were studied by renal biopsy. Sixty-six were referred to us from other centres because of diagnostic or therapeutic problems. The patients' ages ranged from two to 19 years. Evidence of recent streptococcal infection was obtained in 33 children, but their illness was indistinguishable from that of the remainder. Serum C3 levels were normal in all patients. Differential renal clearances of plasma proteins showed moderately or poorly selective proteinuria in most patients and appeared to have little prognostic value. The clinical manifestations of renal involvement were usually apparent early in the course of the illness, occurring within one month of onset in 75 patients. They ranged in severity from microscopic haematuria alone to a nephrotic syndrome which often followed an acute nephritic onset. Children with severe renal involvement tended to be older and to have more prolonged systemic manifestations, as well as troublesome alimentary symptoms. Light microscopy of renal biopsy specimens revealed minimal changes in 15 patients, the remainder showing proliferative glomerulonephritis ranging in severity from minor lesions of focal distribution to diffuse involvement, including crescents. The Electronmicroscopic appearance in specimens from 33 patients is also described. The duration of follwup ranged from three months to 16 years from onset. Three children died within nine months of onset; of 59 survivors who were followed up for more than two yfiirs, 34 were normal when last seen, 17 had minor urinary abnormalities, six had heavy proteinuria with or without hypertension, and two had deteriorating renal function. It was not possible to demonstrate any favourable effect of corticosteroids or cytotoxic drugs, used singly or in combination. There was a fairly good correlation between the severity of clinical presentation and the morphological appearances. No child with microscopic haematuria alone showed more than minor focal lesions while, on the other hand, the occurrence of epithelial crescents in more than 45 per cent of glomeruli was invariably associated with a nephrotic syndrome. A clinical presentation which included both nephritic and nephrotic features, and the involvement of more than 45 per cent of glomeruli with crescents were identified as the two factors of greatest prognostic significance.