Cases Of Idiopathic Nephrotic Syndrome Research Articles

Comparison of Serum IgE Level between Initial Attack and Relapse Cases of Idiopathic Nephrotic Syndrome in Children

Background: Nephrotic Syndrome (NS) is a common and recurrent glomerular disease in childhood. Although the exact etiology of NS is not fully known, it is believed that immune system plays a pivotal role in its pathogenesis. Studies have been reported a strong association between NS and increased level of serum immunoglobulin E (IgE). In addition, children with recurrent NS, the IgE level significantly increases, and there is a higher relapse rate.
 Objective: The aim of the study was to determine the difference of Serum IgE level between initial attack and relapse cases of idiopathic Nephrotic Syndrome in children.
 Methodology: A cross sectional analytical study has been conducted on 35 initial attack and 35 relapse cases of idiopathic Nephrotic Syndrome children admitted in the Department of paediatrics, Sir Salimullah Medical College Mitford Hospital (SSMCMH), Dhaka from May 2021 to April 2022. Study subjects were enrolled by purposive sampling after confirming the diagnosis and fulfilling the inclusion and exclusion criteria. Informed written consent was taken from each patient's legal guardian before enrollment. A detailed history and thorough physical examination was done in each patient on admission. With all aseptic precaution five ml of venous blood was collected and sent for serum albumin, serum cholesterol and serum IgE level measurement. Data were analyzed using the statistical package for social sciences (SPSS) version 23.0. Independent sample t test or Mann-whitney test was used to compare data between groups. P value of less than 0.05 was considered as significant.
 Results: The mean age of the studied samples was 3.86 (ranging from 2 to 6 years). Moreover, among the studied sample male was a little bit greater than female. A significant prevalence of relapse cases were found among the children with middle income family status (P<0.05).Spot urine protein and creatinine ratio and serum cholesterol level was significantly higher in patients experience relapse attack (P<0.05). However, serum albumin level was quite similar in both cases. The mean serum IgE of patients experiencing relapse attack was 985.11+433.65 which was significantly higher than patients experiencing initial attack 470.48+227.35 (P<0.001). 
 Conclusion: This study showed that serum IgE levels are higher in relapse cases of Nephrotic Syndrome than that of initial attack. Furthermore, serum IgE is more in frequent relapse as compared to infrequent relapse. Serum IgE levels have a positive correlation with disease activity and relapse of Nephrotic Syndrome.

Idiopathic nephrotic syndrome relapse following COVID-19 vaccination: a series of 25 cases.

ABSTRACTBackgroundSeveral cases of idiopathic nephrotic syndrome (INS) relapse following the administration of coronavirus disease 2019 (COVID-19) vaccines have recently been reported, raising questions about the potential relationship between the immune response to COVID-19 vaccination and INS pathogenesis.MethodsWe performed a retrospective multicentre survey describing the clinical and biological characteristics of patients presenting a relapse of INS after COVID-19 vaccination, with an assessment of outcome under treatment.ResultsWe identified 25 patients (16 men and 9 women) presenting a relapse within 1 month of a COVID-19 vaccine injection. The glomerular disease was of childhood onset in half of the patients and most patients (21/25) had received at least one immunosuppressive drug in addition to steroids for frequently relapsing or steroid-dependent nephrotic syndrome (NS). All patients were in a stable condition at the time of injection and 11 had no specific treatment. In five patients, the last relapse was reported >5 years before vaccine injection. The Pfizer-BioNTech (BNT162b2) vaccine was used in 80% of the patients. In 18 cases, INS relapse occurred after the first injection, a mean of 17.5 days after vaccination. A second injection was nevertheless administered in 14 of these patients. Five relapses occurred after administration of the second dose and two relapses after the administration of the third dose. All but one of the patients received steroids as first-line treatment, with an additional immunosuppressive agent in nine cases. During follow-up, complete remission was achieved in 21 patients, within 1 month in 17 cases. Only one patient had not achieved at least partial remission after 3 months of follow-up.ConclusionsThis case series suggests that, in rare patients, COVID-19 vaccination may trigger INS relapse that is generally easy to control. These findings should encourage physicians to persuade their patients to complete the COVID-19 vaccination schedule.

Bacterial meningitis in a known case of steroid-resistant nephrotic syndrome

Nephrotic syndrome (NS) is a kidney disorder in which, excess protein is being excreted through urine. While most cases of idiopathic nephrotic syndrome respond to steroid therapy and experience a limited number of relapses prior to complete remission, some cases suffer from frequent relapses and become steroid-dependent or are primarily steroid-resistant. Treatment options are limited to immunosuppressive drugs with significant side effect profiles. Infections in nephrotic syndrome are common and can be severe. Meningitis is one of the complications associated with nephrotic syndrome. Here, we report a child with steroid-resistant nephrotic syndrome (SRNS) with meningitis. Early detection and appropriate treatment of these complications can be lifesaving.

Thyroid Function in Children with Idiopathic Nephrotic Syndrome

Background: The underlying abnormality in nephrotic syndrome is an increase inpermeability of the glomerular membrane. Urinary loses of binding proteins such asThyroxine Binding Globulin (TBG) albumin results in a reduction in serumthyroxine(T4) and sometimes in total T3 levels. The study was done to assess thyroidhormone status of idiopathic nephrotic syndrome patients at diagnosis, comparethe thyroid function after treatment with steroid, correlate with serum albumin andcompare the thyroid function with control group.
 Materials and methods: It was an analytic type of cross sectional study, done inPaediatric ward, Chittagong Medical College Hospital, Chattogram from 01.05.11 to30.11.11. Total 58 patients in two groups were included. In group A 32 cases ofidiopathic nephrotic syndrome patients and in group B 26 control cases of same ageand sex were taken. In group A thyroid function was done at initial diagnosis and inhypothyroid patients in this group again thyroid function was done after treatmentwith steroid. Data was collected through interviewing with pre-designedquestionnaire, physical examination and investigation techniques.
 Results: Thyroid hormone level of nephrotic children were significantly lower thanthat of control group. Nephrotic children with thyroid hypofunction were foundeuthyroid after initial treatment with steroid that was statistically significant andthere was positive correlation between serum albumin and thyroid hormone level.
 Conclusion: Children suffering from nephrotic syndrome had significant thyroidhypofunction because of low serum albumin level and after treatment with steroidthey became euthyroid.
 Chatt Maa Shi Hosp Med Coll J; Vol.19 (1); January 2020; Page 51-54

A comparative study of lipid profile in first attack versus relapse cases of idiopathic nephrotic syndrome in children

A comparative study of lipid profile in first attack versus relapse cases of idiopathic nephrotic syndrome in children

Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study

Background and Aim: Steroid-resistant nephrotic syndrome (SRNS) accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.Materials and Methods: This retrospective study included 31 patients diagnosed as primary SRNS. Age at first episode, gender, parental consanguinity, and familial history of nephrotic syndrome were recorded. Demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis were analyzed, as were the number of and treatment of relapses, extra-renal manifestations, and complications of disease and treatment.Results: Mean age at first episode of nephrotic syndrome was 4,1±2,9 years. At the end of the first immunosuppressive treatment cycle, 14 (51.8%) patients achieved complete remission, 4 (14.8%) patients achieved partial remission, and 9 patients (33.3%) did not achieve remission. Analysis of the final status of the patients showed that 16 patients (51.6%) developed remission, 5 patients (16%) continued to have nephrotic range proteinuria and 10 patients (32%) developed chronic renal failure (CRF).Conclusion: The treatment of SRNS remains controversial. Early genetic testing can help the inevitable immunosuppressive treatments which may not be effective and have several side effects. Calcineurin inhibitors and mycophenolate mofetil are known to be effective immunosuppressive drugs for treating steroid resistant nephrotic syndrome .

A case of nephrotic syndrome, showing evidence of response to saquinavir.

The treatment of primary nephrotic syndrome such as minimal change nephropathy, membranous nephropathy, and focal segmental glomerulosclerosis nephropathy remains challenging. Whilst most cases of idiopathic nephrotic syndrome respond to steroid therapy and experience a limited number of relapses prior to complete remission, some cases suffer from frequent relapses and become steroid dependent or are primarily steroid resistant. Treatment options are limited to immunosuppressive drugs with significant side effect profiles. New modalities targeting novel pathways in the pathogenesis of nephrotic syndrome are actively sought. Here we report the case of a patient with steroid dependent focal segmental glomerulosclerosis (FSGS) nephrotic syndrome with a favourable response to a novel proteasome inhibitor saquinavir.

Second-Line Immunosuppressive Treatment of Childhood Nephrotic Syndrome: A Single-Center Experience

Objective: Most cases of idiopathic nephrotic syndrome in childhood are responsive to corticosteroids. However, there is a small group of children that demonstrate steroid resistance (steroid-resistant nephrotic syndrome; SRNS), steroid dependence, or that frequently relapse (frequent-relapse steroid-sensitive nephrotic syndrome; FR-SSNS) which are more clinically difficult to treat. Therefore, second-line immunosuppressants, such as alkylating agents, calcineurin inhibitors, antimetabolites and, more recently, rituximab, have been used with varying success. The objective was to evaluate the response rates of various second-line therapies in the treatment of childhood nephrotic syndrome. Study Design: A retrospective chart review of pediatric subjects with idiopathic nephrotic syndrome was conducted at a single tertiary care center (2007-2012). Drug responses were classified as complete response, partial response, and no response. Results: Of the 188 charts reviewed, 121 children were classified as SSNS and 67 children as SRNS; 58% were classified as FR-SSNS. Sixty-five subjects were diagnosed with focal segmental glomerulosclerosis via biopsy. Follow-up ranged from 6 months to 21 years. The combined rate of complete and partial response for mycophenolate mofetil (MMF) was 65% (33/51) in SSNS and 67% (6/9) in SRNS. For tacrolimus, the response rate was 96% (22/23) for SSNS and 77% (17/22) for SRNS. Eighty-three percent (5/6) of SSNS subjects treated with rituximab went into complete remission; 60% relapsed after B-cell repletion. Eight refractory subjects were treated with combined MMF/tacrolimus/corticosteroid therapy with a 75% response rate. Conclusion: Our experience demonstrates that older medications can be replaced with newer ones such as MMF, tacrolimus, and rituximab with good outcomes and better side effect profiles. The treatment of refractory cases with combination therapy is promising.

Clinicopathological correlation in primary nephrotic syndrome

Objective: To study the clinicopathological correlation in patients suffering from primary/idiopathic nephrotic syndrome. Materials and Methods: A total of 34 cases of idiopathic nephrotic syndrome were included in the present study. All patients had undergone renals biopsy for histological diagnosis. Results: The patients were subjected to renal biopsy and on the basis of light microscopic findings they were divided into two broad groups. Group A included 25 patients whose renal biopsy showed histological picture of minimal change disease. Group B included nine patients whose renal biopsy showed features other than those seen in minimal change disease. Most patients presented in the 2 nd and 3 rd decade (range was found from 14 to 80 years). Increased serum creatinine (<1.6 mg%) wasfound in two (8%) in group A and four (44%) in group B. Thus, the number of patients having high serum creatinine was significantly higher in group B. Nine patients (33%) in group A and three (33%) in group B had low glomerular filtration rate (GFR;i.e.,<90m1/min). Low serum protein (i.e.,<6 g%) was seen in 18(72%) in group A and five (55.5%) in group B and low serum albumin (i.e.,< 3.2 g%) was found in 21 (84%) in group A and six (66%) in group B. The mean serum cholesterol was 426.76 ΁ 77.11 mg% and mean triglyceride was 269.80 ΁ 148.11 mg% in group A, while in group B the mean cholesterol was 357.33 ΁ 116.19 mg% and mean serum triglyceride was 231.55 ΁ 73.85 mg%. Conclusion: Most patients of nephrotic syndrome presented in the 2 nd and 3 rd decades. All patients in both the groups had high serum triglycerides. Seventy-three percent of nephrotic syndrome cases had histopathological diagnosis of minimal change disease, 8.8% had membranoproliferative glomerulonephritis. Membranous glomerulonephritis, focal segmented glomerulosclerosis, and diffuse proliferative glomerulonephritis accounted for 6% cases each.

Frequent-relapsing, steroid-dependent minimal change disease: is rituximab the answer?

In patients with minimal change disease, development of steroid-dependency or frequent relapses pose difficult therapeutic problems. Prolonged administration of corticosteroids or the use of additional immunosuppressive therapy can result in significant toxicity. Recent data point to the use of rituximab as an important treatment option to induce long-term remission in patients with minimal change diseases who are either frequent relapsers or require significant immunosuppression to remain in remission. Minimal change disease (MCD) accounts for the majority of cases of idiopathic nephrotic syndrome (NS) in children and up to 20% of cases of idiopathic NS in white adults [1]. On kidney biopsy, MCD is characterized by widespread effacement of epithelial cell foot processes on electron microscopy, while glomeruli appear normal on light microscopy and immunoglobulin and complement deposition are absent on immunofluorescence. Most cases of MCD are idiopathic, although drugs (such as non-steroidal anti-inflammatory drugs), hematological malignancies (mainly Hodgkin lymphoma) and thymoma are well-recognized causes of secondary MCD. While most patients respond to corticosteroid therapy, up to 25% of treated patients have frequent relapses and up to 30% of patients become steroid dependent [2, 3]. In these patients, alternative therapies aimed at minimizing corticosteroid toxicity have been used, including alkylating agents, antimetabolites and calcineurin inhibitors. While these agents may be beneficial, some patients respond poorly or not all, and their use may be complicated by the development of serious adverse effects, e.g., infertility and malignancy with the use of cyclophosphamide. With calcineurin inhibitors, steroid de

Peritubular capillaries and renal function in pediatric idiopathic nephrotic syndrome

Nephrotic syndrome (NS) is a common renal disorder with significant tubulo-interstitial damage due to the combined effects of proteinuria and obstruction of efferent blood flow. Peritubular capillary (PTC) loss has also been correlated with interstitial fibrosis. This study included 30 pediatric cases of idiopathic NS. Clinical details, including biochemical parameters, were recorded and renal biopsy slides were reviewed for histological features. PTCs were highlighted using anti-CD34 antibody and quantified with the help of image analysis software. Postmortem kidney biopsies from seven children were taken as controls for quantification of PTCs and interstitial fibrosis. Wherever possible, as ultrastructural examination of the renal biopsy was performed. Appropriate statistical methods were applied. Patients with minimal change disease (MCD) had lower serum creatinine as compared with those with focal and segmental glomerulosclerosis (FSGS). Similarly, tubular atrophy and interstitial fibrosis were significantly lower in MCD than in FSGS. PTC density was lower in all groups of NS as compared with the controls. Biopsies with FSGS had a lower PTC density compared with both MCD and mesangioproliferative glomerulonephritis. PTC density showed a negative correlation with serum creatinine and degree of proteinuria. PTC loss appears to play an important role in the development of renal biopsy changes in pediatric NS. This aspect of the renal vasculature requires further study in idiopathic NS.

Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study

Some difficult cases of idiopathic nephrotic syndrome (NS) have been treated with a HIV protease inhibitor provided with proteasome-inhibiting activity. The objective of this study was to limit nuclear factor κB (NF-κB) activation which is up-regulated in these patients, aiming at decreasing proteinuria and prednisone need. Ten cases with long-lasting (up to 15 years) history of NS with steroid dependence (six cases, of which three with secondary steroid resistance) or resistance to steroids (four cases) unsuccessfully treated with multiple immunosuppressive drugs, accepted a treatment with the protease inhibitor saquinavir. p50/p65 NF-κB nuclear localization and immunoproteasome/proteasome messenger RNA (mRNA) were monitored in peripheral blood mononuclear cells (PBMCs). The effects of saquinavir on NF-κB nuclear localization in cultured PBMCs and in immortalized human podocytes were assessed. After a median follow-up of 14.7 months (6-68.7), 1/4 primary steroid-resistant NS (SRNS) and 5/6 steroid-dependent NS or secondary SRNS became infrequent (5) or frequent (1) relapsers, with 63% prednisone reduction (from 25.3 to 8.4 mg/kg/month, P = 0.015). Saquinavir was effective in association with low doses of calcineurin inhibitors (cyclosporine 2 mg/kg/day or tacrolimus 0.01-0.06 mg/kg/day). No side effects were observed apart from transitory mild diarrhoea. In PBMCs, NF-κB was down-regulated, while MECL-1 immunoproteasome/beta2 proteasome mRNA ratio was reversed to normal values. In culture, saquinavir blunted NF-κB activation in human podocytes and in PBMCs. In this pilot study, a HIV antiprotease drug reduced proteinuria and had a steroid-sparing effect in some multidrug-resistant/-dependent NS. This observation warrants further investigation.

Syndrome néphrotique idiopathique de l’enfant : incidence, présentation clinique et devenir dans le département de la Gironde, France

To estimate the incidence and describe the clinical presentation and outcome (steroid responsiveness, clinical course, complications) of idiopathic nephrotic syndrome in children in a population-based retrospective study. Using local registries and the hospital discharge diagnosis system from two centers, all new cases of idiopathic nephrotic syndrome were identified in Gironde (France) between January 1992 and May 2008. To estimate incidence, population-based denominators were obtained from the National Institute for Statistics and Economic Studies (INSEE). Clinical data were collected from medical charts. Ninety-nine cases of idiopathic nephrotic syndrome were reported (66 boys, 18 non-Caucasians) with an incidence of 2.3/100,000 (CI, 1.8-3.0) children less than 15 years. Ninety patients (91%) had steroid-sensitive nephrotic syndrome (SSNS) and nine (9%) were steroid-resistant (SRNS). The median time to remission in SSNS was 11 days. Relapses occurred in 75 (83%) children with SSNS with a median of four relapses (range, 1-32). The cumulative relapse-free incidence was 60% at 10 years after diagnosis in SSNS and 13% of patients aged 18 years old or over still had active disease. In SSNS, the only significant factor associated with steroid dependency or use of non steroid drugs was the time to initial response to steroids greater than 14 days. Nineteen children (19%) experienced severe complications of nephrotic syndrome including 11 bacterial infections and two thromboembolic complications. Two children with SRNS, of whom one was initially steroid-responsive, developed end-stage renal failure. The incidence and outcome of idiopathic nephrotic syndrome in Gironde are comparable to the rates found in other studies. The disease may have a long course and the time for response to steroids at disease onset is the main predictor of steroid dependency and of use of non steroid agents.

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

Primary focal segmental glomerulosclerosis (FSGS) in children accounts for 7-20% of all cases of idiopathic nephrotic syndrome (NS). To evaluate the clinical course of children with primary FSGS, the records of 72 patients with biopsy-proven FSGS admitted between 1995 and 2008 were retrospectively analysed. Forty-eight patients were male (66.7%). The mean age at presentation was 76.5 +/- 54 (range 12-192) months. The mean duration of follow-up was 76.3 +/- 42 (range 9-156) months. Seventeen patients (23.6%) presented with gross haematuria at initial presentation, 15 (20.8%) presented with hypertension and 10 patients (14%) presented with chronic renal insufficiency (CRI). The initial response to oral prednisolone was steroid resistance in 63 patients (87.5%) and steroid dependence in 4 patients (5%), while 5 patients (6.9%) were biopsied from the start because of atypical presentation. According to pathological classification, there were the tip variant (2%), collapsing variant (6%), perihilar variant (7%) and NOS (not otherwise specified; 85%). At the last clinical visit, 12 patients (16.7%) were in complete remission and 11 (15.3%) had progressed to CRI. Renal survival rates at 5 and 10 years were 93% and 68%, respectively. In conclusion, in this study we had a low percentage of FSGS, and fewer patients presenting with gross haematuria and hypertension compared with previous reports. In addition, the short-term overall renal survival seems to be better in our cohort.

Low birth weight, but not postnatal weight gain, aggravates the course of nephrotic syndrome

Clinical and animal studies have shown a higher risk of an aggravated course of renal disease in childhood after birth for babies small for gestational age (SGA). In addition relative "supernutrition" and fast weight gain in early infancy seem to support the development of later disease. In a retrospective analysis of 62 cases of idiopathic nephrotic syndrome treated between 1994 and 2004 at a university centre for paediatric nephrology, we related the course of disease to birth weight and to the weight gain in the first 2 years of life. Six children were born SGA (birth weight <-1.5 standard deviation score), and 56 were born as appropriate for gestational age (AGA). In all SGA children renal biopsy was performed, while only 55% of the AGA children underwent renal biopsy (P = 0.07), showing no difference in renal histology. In the SGA group, four of six patients developed steroid resistance (vs 12/56 AGA, P < 0.05). Of the SGA children, 83% needed antihypertensive treatment in the course of the disease compared to 39% of the AGA children (P = 0.07). The extent of weight gain between birth and 24 months of age did not influence the course of disease. In conclusion, we were able to find evidence for an aggravated course of idiopathic nephrotic syndrome in former SGA children. Independently of birth weight, weight gain in the first 2 years of life did not influence the course of disease.

Posttransplantation relapse of FSGS is characterized by glomerular epithelial cell transdifferentiation.

This study examined six cases of idiopathic nephrotic syndrome with primary lesions of focal segmental glomerulosclerosis (FSGS) that relapsed after renal transplantation. The glomerular lesions comprised the cellular, the collapsing, and the scar variants of FSGS and showed shedding of large round cells into Bowman's space and within the tubular lumens. Immunohistochemistry and confocal laser microscopy carried out on kidneys with FSGS relapse disclosed several phenomena. (1) Some podocytes that expressed podocalyxin, synaptopodin, and glomerular epithelial protein-1 were detached from the tuft and were free in the urinary space. (2) In the cellular variant, most podocytes had lost podocyte-specific epitopes (podocalyxin, synaptopodin, glomerular epithelial protein-1, Wilm's tumor protein-1, complement receptor-1, and vimentin). In the scar variant, these podocyte markers were absent from cobblestone-like epithelial cells and from pseudotubules. (3) Podocytes had acquired expression of various cytokeratins (CK; identified by the AE1/AE3, C2562, CK22, and AEL-KS2 monoclonal antibodies) that were not found in the podocytes of control glomeruli. Parietal epithelial cells expressed AE1/AE3 CK that were faintly, if ever, found on the parietal epithelial cells of normal glomeruli. (4) Numerous cells located at the periphery of the tuft or free in Bowman's space and within tubular lumens expressed macrophagic epitopes (identified by PGM1 [CD68], HAM56, and 25F9 monoclonal antibodies). These macrophage-like cells expressed the activation epitopes HLA-DR and CD16. (5) A number of these cells coexpressed podocalyxin + AE1/AE3 CK, podocalyxin + CD68, and CD68 + AE1/AE3. These findings suggest that in primary FSGS relapsing on transplanted kidneys, some "dysregulated" podocytes, occasionally some parietal epithelial cells, and possibly some tubular epithelial cells undergo a process of transdifferentiation. This process of transdifferentiation was especially striking in podocytes that acquired macrophagic and CK epitopes that are absent from normal adult and fetal podocytes.

Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies: A 20-year renal biopsy study

Studies and textbooks from the 1970s and early 1980s list focal-segmental glomerulosclerosis (FSGS) as accounting for 10% to 15% of cases of idiopathic nephrotic syndrome in adults, although a recent review by D'Agati ( Kidney Int 46:1223–1241, 1994) reported an approximately sevenfold increase in the incidence of FSGS from 1974 to 1993 in an active renal biopsy practice. To investigate possible changes in the incidence of FSGS in our renal biopsy practice, we reviewed reports from all nontransplant, adult (≥ 18 years) renal biopsies received in our laboratory from 1974 to 1993, which comprised 7,420 cases. All diagnoses of membranous nephropathy (MN), minimal change nephropathy (MCN), and FSGS made in each year were compiled; cases clearly or suspicious of being secondary to an underlying systemic disease, glomerulonephritis, or drug reaction were excluded. Relative frequencies of MN, MCN, and FSGS among these three diseases and among all biopsies were calculated for each year of the study. Regression analysis showed a significant ( P < 0.001) increase in the odds of a diagnosis of FSGS over the study period: 7.6% per year among all biopsies and 6.8% per year among cases of MN, MCN, and FSGS only. Among all biopsies, the yearly incidence of FSGS increased from 4.0% ± 0.6% (mean ± SD) during the period between 1974 and 1979 to 12.2% ± 2.0% during the period from 1987 to 1993. The odds of a diagnosis of MN (mean yearly incidence, 9.5% ± 1.9%) did not vary significantly over the study period while the odds of a diagnosis of MCN (mean yearly incidence, 4.0% ± 1.2%) declined at a rate of 2.2% per year ( P < 0.03). Frequencies of diagnosis of MN, MCN, and FSGS by two pathologists were almost identical. Review of available slides from cases of FSGS revealed 21 (none before 1980) with characteristic histologic features of the collapsing glomerulopathy (CG) variant of FSGS. No more than four cases of CG were observed in any year of the study, and CG accounted for 4.7% of total FSGS cases for which diagnostic slides were available. Compared with 42 patients with non-CG FSGS, the CG cohort showed a greater percentage of black patients (86% v 38%), significantly higher mean levels of serum creatinine (3.8 ± 2.7 mg/dL v 1.9 ± 1.5 mg/dL) and urinary protein (14.3 ± 9.6 g/24 hr v 7.7 ± 5.8 g/24 hr) at the time of renal biopsy, and a greater likelihood of and more rapid progression to end-stage renal failure. Our findings confirm an increase in the incidence of FSGS over the 20 years between 1974 and 1993, both overall and among primary nephropathies leading to the nephrotic syndrome. In agreement with others, we find that CG appears to represent a particularly “malignant” variant of FSGS, although in our experience this variant is observed relatively infrequently.

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

Thirty-three children with idiopathic nephrotic syndrome received an eight-week course of 2.5 to 3.0 mg/kg of body weight of cyclophosphamide. Twenty-five (76%) were in remission 12 to 28 months la...

Forms of Nephrotic Syndrome More Likely to Progress to Renal Impairment

In this article, emphasis is placed on those conditions that appear as the idiopathic nephrotic syndrome but that are more likely to result in progressive renal failure. Four conditions, membranoproliferative glomerulonephritis (mesangiocapillary glomerulonephritis), mesangial proliferative glomerulonephritis, membranous nephropathy, and focal segmental glomerulocosclerosis, account for 12 to 15 per cent of cases of idiopathic nephrotic syndrome during childhood. The further aim of this article is to focus on recent progress in our understanding of these conditions and to remind readers that little is known regarding the etiology and pathogenesis of each, and that effective, clearly proven forms of treatment for each are not apparent currently.