Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis (original) (raw)
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Non-nephrotic children with membranoproliferative glomerulonephritis: are steroids indicated?
Pediatric Nephrology, 1995
Alternate-day steroids are currently recommended to treat children with membranoproliferative glomerulonephritis (MPGN). This recommendation is based largely on uncontrolled studies demonstrating improved renal survival with steroid therapy. We reviewed the outcome of 39 children who presented with MPGN between 1968 and 1990; 27 children were treated with steroids and 12 children received no drug therapy. Life-table analysis comparing renal survival of treated versus untreated children demonstrated no difference by log rank analysis. Treated and untreated groups were compared on the basis of nine features at presentation: age, sex, type of MPGN, presence and type of hematuria, hypocomplementemia, renal insufficiency, hypertension, and nephrosis. Treated children were likely to be female (P<0.01) and nephrotic (P<0.02). Actuarial survival analyses were performed comparing the nine features with renal survival through 10 years of follow-up. Normotensive (P<0.025) and non-nephrotic (P<0.05) children had improved renal survival. The 11 non-nephrotic children demonstrated 100% long-term renal survival, including 7 who received no steroid therapy. At last follow-up, all non-nephrotic children had normal renal function, serum albumin levels >3 g/dl, and were normotensive. These data suggest that non-nephrotic children with MPGN may forego steroid treatment without compromising long-term renal function. The current common practice of treating all children with MPGN with steroids should be re-examined.
2018
OBJECTIVE To study the frequency, clinicopathological features and short-term outcome of mesangiocapillary glomerulonephritis (MCGN) in children at a tertiary care kidney center in Pakistan. METHODS A descriptive, observational study was conducted at the Paediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2011 till December 2015. A review of all paediatric (<18 years) renal biopsies during the study period was performed and cases of MCGN were enrolled. The clinical presentation, laboratory findings, histology and outcome were analyzed. RESULTS During the study period, 890 paediatric renal biopsies were performed. Of these, 63(7%) were MCGN. Among these, 34(54%) were males and 29 (46%) females. Mean age was 9.9 ± 3.2years. Thirty four (54%) presented with nephrotic syndrome (NS), and29 (46%) with rapidly progressive glomerulonephritis (RPGN).Mean duration of follow-up was 1.66 ± 1.34 years. Outcome of patients with NS with renal ...
Prognostic factors in children with membranoproliferative glomerulonephritis type I
Pediatric Nephrology, 2008
The clinical outcome of patients with membranoproliferative glomerulonephritis (MPGN) varies, with some patients progressing to end-stage renal disease. The aim of this retrospective study was to analyze the initial clinical signs and laboratory test results associated with an MPGN prognosis. The study cohort consisted of 47 patients with idiopathic MPGN Type I treated at the National Institute of Pediatrics, Mexico City, between 1971 and 2001. The median follow-up was 3 years. The three different outcomes of interest were death, renal failure, and nephrotic syndrome. The patients' ages ranged between 4 and 16 years. All patients had different degrees of proteinuria, hyperlipidemia, and microscopic/macroscopic hematuria, and 85.1% of them showed hypocomplementemia. Clinical outcomes varied, however, the most common was nephrotic syndrome, either alone or combined with other syndromes, which accounted for 74.5% of all cases. Fifteen patients died. Treatment with methylprednisolone improved the patient's condition, while the use of chloroquine or cyclophosphamide worsened it. Twenty-two patients had some degree of renal failure; glomerular filtration rate (GFR) levels and albumin values were negatively associated to renal failure, while treatment with methylprednisolone decreased the probability of renal failure. Nephrotic syndrome persisted in 18 patients; hemolytic complement and hemoglobin values were negatively associated with nephrotic syndrome, while macroscopic hematuria was positively associated with it. Signs that suggested a poor prognosis during diagnosis were low GFR, low albumin, low hemolytic complement, and macroscopic hematuria. Treatment with methylprednisolone seemed to improve prognosis, however, this needs to be confirmed with randomized studies.
The prognostic value of glomerular immaturity in the nephrotic syndrome in children
Pediatric Nephrology, 2004
The study group consisted of 16 children (9 boys and 7 girls) aged 6-52 months (mean age 27 months) with a first episode of nephrotic syndrome. Histological diagnosis (diffuse mesangial proliferation with signs of glomerular immaturity) was established by renal biopsy. The control group consisted of 47 children (26 boys and 21 girls) aged 7-58 months (mean 29 months) hospitalized with a first episode of nephrotic syndrome with diffuse mesangial proliferation, documented exclusively by histological examination. The aim of the study was to analyze the clinical course of a first episode of nephrotic syndrome in children with diffuse mesangial proliferation with and without signs of glomerular immaturity. In children with a first episode of the nephrotic syndrome and glomerular immaturity steroid resistance was more frequent (P=0.0234). Furthermore, in the study group there was a less favorable clinical course of the disease in children younger than 1 year of age and with an original serum albumin concentration lower than 1.0 g/dl. Hence, the presence of relatively rare signs of glomerular immaturity influences unfavorably the course and treatment of nephrotic syndrome in children.
American Journal of Medicine, 1983
Dl 104 patients with idiopathic mesangiocapillary gkmeruknephritk studied for at least two years, 69 patients had type I disease and 35 had type II. Forty-five patients were children, and 59 were adults. Type II mesangiocapillary glomerulonephritis was more common in children than in adults, but no other clfnkal feature distinguished the two types at onset. Complement studies revealed that patients with type II had lower serum C3 concentrations and more frequently showed CB-splitting activity (C3 nephritk factor) in the serum. Children had hypertension or a lowered gkmerular filtratton rate less frequently at onset than did adults, but children had a higher incidence of a hematuric onset; C3 nephritic factor was also more frequent in the children.
[Nephrotic syndrome in childhood]
Orvosi hetilap, 2006
Minimal change nephrosis, the main pediatric form of idiopathic nephrotic syndrome is usually a benign condition responsive to standard steroid treatment. However, relapses occur frequently leading to secondary steroid resistance in a small proportion of cases. Steroid resistant nephrotic syndrome presents mainly as focal segmental glomerulosclerosis. It represents about 8-10% of cases of pediatric idiopathic nephrotic syndrome. The prognosis of focal segmental glomerulosclerosis is bad, with the majority of cases evolving to terminal renal insufficiency within several years. Among the causes of idiopathic nephrotic syndrome are changes of cellular immunity, circulating plasma factors not fully identified yet and mutations of podocyte proteins. Podocyte mutations are responsible for the development of about one third of cases steroid resistant focal segmental glomerulosclerosis. Treatment of idiopathic nephrotic syndrome is determined by international guidelines. If initial steroid ...
Kidney International Reports, 2020
The reclassification of membranoproliferative glomerulonephritis (MPGN) into immunecomplex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) has provided insights into 2 distinct diseases. Although outcomes in adults are poor in both diseases, the pediatric literature is scarce and limited to small, single-center cohorts. Methods: We conducted a retrospective analysis of 165 pediatric patients across 17 hospitals to compare outcomes between children with IC-MPGN and C3G. Results: Forty-two percent of patients initially diagnosed with MPGN were reclassified as C3G after a review of renal biopsy reports. There was a trend toward higher serum creatinine levels in patients with C3G compared with IC-MPGN both at diagnosis (mean 168.9 [range 45.4-292.4] vs. 93.7 [range 70.7-116.6] mmol/l, P ¼ 0.25) and after a mean follow-up time of 4 years (mean 145.0 (range À8.1 to 298.1) vs 99.1 (range 46.3-151.9) mmol/l, P ¼ 0.47), although the estimated glomerular filtration rate (eGFR) was not significantly different. Steroid treatment was associated with a significant improvement in eGFR versus no steroids in C3G (mean þ43.0 (range 12.9-73.0) vs. À3.0 (range À23.1 to 17.2) ml/min per 1.73 m 2 , P ¼ 0.02) but not in IC-MPGN. Overall kidney function was preserved in both groups although hypertension remained prevalent in 42.5% of the cohort at the last follow-up, and the urine protein/creatinine ratio remained elevated (mean 253.8 [range 91.9-415.7] mg/mmol). This large pediatric IC-MPGN/C3G cohort revealed nearly half of the patients were misclassified, and there may be a trend toward worse renal prognosis in C3G although they may have greater steroid responsiveness. The overall prognosis appears to be more favorable than in adults; however, persistent hypertension and proteinuria suggest suboptimal disease control.
Long-term prognosis of idiopathic nephrotic syndrome in children
Renal Failure, 2015
Background: To investigate the demographic, clinical and laboratory data of the children with idiopathic nephrotic syndrome (INS), and to determine prognostic factors that affect the clinical outcome of the patients. Methods: Medical charts of 372 patients diagnosed to have INS and followed up at least 5 years between January 1990 and December 2008 were evaluated, respectively. After initial demographic, clinical and laboratory findings of the patients were documented, therapeutic protocols, prognosis and prognostic factors were investigated. Results: 299 of the patients (80.4%) were steroid responsive and 73 (19.6%) were not. Focal segmental glomerulosclerosis (FSGS) was observed in 57%, minimal change disease (MCD) in 20.6% and diffuse mesengial proliferation in 21.9% renal biopsy materials. Steroid sensitivity was higher in patients with MCD and under the age of five years. Resistance to steroids was higher in children with FSGS. Complete remission was achieved in 96% of patients who were sensitive to steroids and in 46.6% who were resistant. 15% of patients who were steroid resistant developed chronic kidney disease (CKD). Conclusion: Intercurrent infections and response to steroid therapy are the most important factors affecting the prognosis of the disease.