Outcome Of Nephrotic Syndrome Research Articles

Preparation and Rationale for a Patient-Centered Clinical Outcome Assessment Set of Fluid Overload for Drug Development in Nephrotic Syndrome

Introduction: Fluid overload is a source of substantial morbidity for adults and children with nephrotic syndrome (NS). Preparation and Rationale for a Fluid Overload in Nephrotic Syndrome Clinical Outcomes Assessment Set for Drug Development (Prepare-NS, 5UG3FD007308) was funded by the US Food and Drug Administration to develop a core set of patient-reported and observer-reported (for young children) outcome measures of fluid overload for use in pharmaceutical trials across the lifespan. Methods: The Prepare-NS study team developed the proposed context of use with input from stakeholders. We conducted a scoping review to assess the available literature on relevant patient- and observer-reported measures and performed secondary analyses of existing qualitative and quantitative data. Results: The outcome set will aim to serve individuals 2 years of age and older with primary NS conditions (specifically focal segmental glomerulosclerosis, minimal change disease, IgM nephropathy, membranous nephropathy, and childhood-onset NS not biopsied). The existing literature describing patient-reported outcomes in NS largely relies on nonspecific measures of health-related quality of life; fluid overload has been associated with lower scores on these measures. Conclusion: To address the gap in measure availability and fluid overload content, the Prepare-NS team has launched a set of qualitative studies for concept elicitation from the population of interest to inform development of new measures. The resulting measures subsequently will undergo psychometric evaluation and validation in a survey study.

#509 Idiopathic nephrotic syndrome in children in Chad

Abstract Background and Aims The objective of our study was to describe the characteristics and outcomes of Nephrotic Syndrome (SN) in children from three major hospitals in one of the world's poorest countries, Chad. Method This observational, cross-sectional, descriptive, and multicenter study took place over a period of 36 months (1 January 2019–31 December 2021) and was carried out in three hospitals in N'Djamena, Chad. Children aged 1–15 years presenting with NS were included in the study. Results Out of 16,776 children hospitalized or followed up with in outpatient clinics, 24 cases of NS were identified, yielding a prevalence of 0.14%. The median age at presentation was 6.16 years (1–10). Nineteen children were male (sex ratio 3.8). Eight cases were classified as impure NS (33.3%). Edema was present in all patients, while oliguria was present in 29.16% (n = 7), and arterial hypertension was present in 20.83% (n = 5) of cases. Mean proteinuria, albuminemia, and total proteins were 2.86 g/L, 19.13 g/L and 30.41 g/L, respectively. The median serum creatinine was 87.3 µmol/L (75–1375 µmol/L). Three patients experienced acute renal failure upon admission. Four patients had secondary NS. All idiopathic NS patients (n = 20) who had received corticosteroid therapy had a 90% response rate to steroids. Non-responsive or relapsed patients underwent kidney biopsy (n = 7), revealing focal segmental glomerulosclerosis (FSGS; n = 4) as the most common histological lesion, followed by minimal change disease (n = 2) and membranoproliferative glomerulonephritis (n = 1). The median length of hospitalization stay was 10.67 (5–27) days. None of the patients with idiopathic NS died. At the last follow-up, sixteen patients (80%) achieved long-term complete remission with normal renal function; however, four of those had subsequent relapses. One patient with secondary NS died. Conclusion In Chad, childhood idiopathic nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function.

Risk factors and renal outcomes of AKI in children with secondary steroid-resistant nephrotic syndrome

Background Acute kidney injury (AKI) is increasingly prevalent in children with nephrotic syndrome (NS). It is associated with adverse outcomes in NS, especially steroid-resistant nephrotic syndrome (SRNS). The incidence, risk factors and outcomes of AKI in secondary SRNS remain undefined. The main objectives of this study were to determine the risk factors and prognosis of AKI in hospitalized children with secondary SRNS. Material and methods This retrospective study was conducted from January 2014 to December 2019, involving 172 hospitalizations with secondary SRNS admitted to the First Affiliated Hospital of Sun Yat-sen University. AKI was defined and classified in accordance with the 2012 Kidney Disease Improving Global Outcomes (KDIGO) guidelines. Results AKI was found in 67 (39.0%) of 172 hospitalizations with secondary SRNS. Average age of onset in our group is 4.4 (3.1, 6.7) years with AKI and 3.7 (1.8, 5.6) years without AKI. Urea nitrogen level is 5.9 (4.1, 10.0) mmol/L with AKI and 5.1 (3.7, 7.0) mmol/L. Uric acid level is 446.0 (340.0, 567.0) umol/L with AKI and 401.0 (303.0, 496.0) umol/L. 24-h urinary protein level is 4.14 (2.9, 6.5) g with AKI and 2.5 (1.3, 5.3) without AKI. Multivariate logistic regression revealed that infection (OR = 5.287; 95% confidence interval, 2.349 to 11.899; p < 0.001), age at onset (OR = 1.180; 95% confidence interval, 1.032 to 1.349; p = 0.015) and uric acid level (OR = 1.003; 95% confidence interval, 1.000 to 1.006; p = 0.031) were significantly associated with the development of AKI in children with secondary SRNS. Among 72 children with secondary SRNS, six went to end-stage kidney disease (ESKD). Children in the AKI group were more likely to progress to ESKD compared with children in the non-AKI group (p = 0.017) with a median follow‐up of 48.5months. Conclusion AKI occurred in 39.0% of total hospitalizations associated with secondary SRNS. Risk factors including infection, age of onset, and uric acid level are associated with AKI in children with secondary SRNS. Furthermore, AKI was identified as a risk factor for the progression of secondary SRNS to ESKD.

Idiopathic Nephrotic Syndrome in Children in Chad: Epidemiology and Clinical Outcomes.

Nephrotic syndrome (NS) remains the most common presentation of glomerular diseases in children. Moreover, NS is primarily idiopathic, accounting for 90% of cases, with an average onset age between 2 and 10 years. The objective of our study was to describe the characteristics and outcomes of NS in children from three major hospitals in one of the world's poorest countries, Chad. This observational, cross-sectional, descriptive, and multicenter study took place over a period of 36 months (1 January 2019-31 December 2021) and was carried out in three hospitals in N'Djamena, Chad. Children aged 1-15 years presenting with NS were included in the study. Out of 16,776 children hospitalized or followed up with in outpatient clinics, 24 cases of NS were identified, yielding a prevalence of 0.14%. The median age at presentation was 6.16 years (1-10). Nineteen children were male (sex ratio 3.8). Eight cases were classified as impure NS (33.3%). Edema was present in all patients, while oliguria was present in 29.16% (n = 7), and arterial hypertension was present in 20.83% (n = 5) of cases. Mean proteinuria, albuminemia, and total proteins were 2.86g/L, 19.13g/L and 30.41g/L, respectively. The median serum creatinine was 87.3 µmol/L (75-1375 µmol/L). Three patients experienced acute renal failure upon admission. Four patients had secondary NS. All idiopathic NS patients (n = 20) who had received corticosteroid therapy had a 90% response rate to steroids. Non-responsive or relapsed patients underwent kidney biopsy (n = 7), revealing focal segmental glomerulosclerosis (FSGS; n = 4) as the most common histological lesion, followed by minimal change disease (n = 2) and membranoproliferative glomerulonephritis (n = 1). The median length of hospitalization stay was 10.67 (5-27) days. None of the patients with idiopathic NS died. At the last follow-up, sixteen patients (80%) achieved long-term complete remission with normal renal function; however, four of those had subsequent relapses. One patient with secondary NS died. In Chad, childhood idiopathic nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function.

A study of adverse maternal-foetal outcomes in nephrotic syndrome combined with preeclampsia

BackgroundAlthough the majority of pregnancies with preeclampsia are characterised by elevated blood pressure, preeclampsia is often associated with nephrotic syndrome with similar symptoms such as high proteinuria and bilateral lower limb oedema. In this study, we compared the maternal–foetal outcomes of pregnant women with preeclampsia in a population with nephrotic syndrome and explored the factors that contribute to the corresponding outcomes and disease development.MethodsA total of 90 pregnant women were included in this study, of whom 30 had nephrotic syndrome and were diagnosed with preeclampsia during pregnancy, and 60 had nephrotic syndrome alone. Descriptive statistical analyses of baseline data were performed to analyse the effect of combined preeclampsia on maternal and foetal pregnancy outcomes using unadjusted and adjusted logistic regression models.ResultsIn this study, the baseline data of the two study populations demonstrated no differences except for the history of caesarean section and 24-h proteinuria results, which were significantly different (P < 0.05). The risk of preterm birth in the nephrotic syndrome with preeclampsia group was 8.25 (95% CI:3.041–22.084 P < 0.05); for a low birth weight, the risk was 6.00 (95% CI:2.302–15.638 P < 0.05); for foetal distress,the risk was 5.667 (95% CI:2.070–15.514 P < 0.05); and the risk of foetal birth restriction was 7.429 (95% CI: 2.642–20.885 P < 0.05). A risk-based analysis of adverse maternal outcomes yielded a risk of miscarriage of 2.200 (95% CI: 0.584–8.291; P > 0.05). After adjusting the model for each outcome, significant risks of preterm labour, foetal birth restriction, and low birth weight were revealed (P < 0.05).ConclusionCombined preeclampsia has a significantly higher risk of adverse pregnancy outcomes for the foetus.Therefore, the prevention and control of eclampsia in pregnant women should be improved to ensure maternal and neonatal health.

Clinical Profile and Outcomes of Nephrotic Syndrome With Acute Kidney Injury in Adults: A Prospective Cohort Study.

Nephrotic syndrome (NS) is characterized by various clinicopathological conditions like proteinuria, hypoalbuminemia, and anasarca. Patients with NS are prone to experience associated problems like acute kidney injury (AKI). The present study aimed to investigate the clinical profile and outcomes of NS with AKI in adults. This prospective, observational study was conducted over a period of one year. Adult patients with NS diagnosed with AKI were enrolled in the study. Data were collected at baseline and patients were followed up for at least three months. A total of 60 patients were enrolled. The majority of the patients (78.3%) were aged between 18 and 30 years. Anemia was observed among 96.7% of the patients. A significant improvement was observed in the mean levels of proteinuria (5.80 vs. 1.70 gm/dL; P < 0.001), total cholesterol (270.00 mg/dL vs. 160.00 mg/dL), serum creatinine (2.18 mg/dL vs. 1.68 mg/dL; P < 0.001), and serum albumin (1.86 gm/dL vs. 3.29 gm/dL; P < 0.001) at baseline to three months. Pre-renal AKI was diagnosed in 95% of patients. According to histological classification, 19 patients had minimal change disease, whereas focal segmental glomerulosclerosis was observed in 23 patients. It was observed that 96.7% of the patients did not necessitate the need for renal replacement therapy. The present study successfully examined the clinical profile and outcomes of adults with NS and AKI. The findings provide valuable insights into the characteristics and prognosis of this patient population, contributing to a better understanding of NS with AKI in adults.

WCN23-1118 Incidence and outcomes of Nephrotic syndrome in IgA Nephropathy : A tertiary care centre study

WCN23-1118 Incidence and outcomes of Nephrotic syndrome in IgA Nephropathy : A tertiary care centre study

Psychosocial Functioning and Health-Related Quality of Life in Children with Nephrotic Syndrome: Preliminary Findings

Background: Nephrotic syndrome (NS) is one of the most commonly diagnosed kidney diseases in childhood, with a high relapse rate. Aim: This study was designed to assess emotional and behavioural problems, temperament, family environment, and evaluate the health-related quality of life in children with NS. Methods: This cross-sectional study was conducted at a tertiary care hospital in South India. A purposive sampling technique was used. A sample of 32 children between ages 6 and 12 years with NS—steroid-sensitive (infrequent relapsers, frequent relapsers, or those with steroid-dependent) and steroid-resistant—for more than 6 months duration and managed by the Pediatric Nephrology department were recruited. The control group of 30 healthy children was matched for age and gender. The assessment was conducted using the Strengths and Difficulties Questionnaire (SDQ), Pediatric Quality of Life Inventory™ (PedsQL 4.0), Malhotra Temperament Schedule, and Family Environment Scale. Data obtained was examined using descriptive and inferential statistics. Results: On the SDQ, 34.4% of children with NS indicated a total score within the clinical range. Children with NS had an overall lower score on the QOL scale ( P &lt; .003). It was found that the SRNS subtype had poor scores on SDQ ( P &lt; .023) and QOL ( P &lt; .017). The temperament of children with NS did not differ from those of healthy children. The families with NS scored lower on organization ( P &lt; .042) and control ( P &lt; .006) subscales. Conclusion: Children with NS have a risk of emotional and behavioural problems. It appears that children with NS have a poor quality of life. Families of children with NS were less organized and low in control. Findings indicate the need for routine psychological evaluation and intervention as they will impact the course and outcome of NS.

Cardiovascular outcomes of Nephrotic syndrome in childhood (CVONS) study: a protocol for prospective cohort study

BackgroundNephrotic syndrome (NS) is characterized by dyslipidemia which is a well-known risk factor for atherogenesis. Atherosclerosis in childhood is mostly subclinical and endothelial dysfunction is known to precede this. Evidence for screening for endothelial dysfunction and cardiovascular risk factors and early identification of premature onset of atherosclerosis in childhood NS remains tenuous in the absence of well-designed prospective studies addressing cardiovascular comorbidity in NS. The objective of our study is to examine endothelial dysfunction and short-term cardiovascular outcomes in a carefully phenotyped cohort of patients with Nephrotic syndrome as compared to healthy controls.MethodsIn a multi-centric prospective cohort study, 70 Steroid Resistant NS (SRNS), 70 Steroid Sensitive (SSNS) patients along with 70 Healthy Controls are being recruited. After a baseline assessment of functional and structural status of heart (2D Echocardiography), arteries (Carotid Doppler and Intima Media Thickness measurements) and microcirculation [a combination of 2D Echocardiography, Laser Doppler Flowmetry (LDF) and Brachial Artery Flow mediated dilation (FMD) and Nail Fold Capillaroscopy (NFC)], the patients are being investigated for endothelial dysfunction. Venous blood sample (15 ml) is being collected for routine investigations and assay of biochemical endothelial markers through Flow Cytometry. The patients will be followed up at 12 months and 24 months after the recruitment to look for any change from baseline period.DiscussionThis study will able to provide a better understanding of the epidemiology of endothelial dysfunction and associated subclinical cardiovascular co-morbidity in childhood NS. Findings on characterization of prevalence of endothelial dysfunction and subclinical markers may be used to design future randomized controlled trials for evaluating the efficacy of preventive and therapeutic interventions in reducing the incidence of cardiovascular disease.

The impact of IgM deposits on the outcome of Nephrotic syndrome in children

BackgroundThe significance of IgM deposits in glomerular mesangium has been controversial since they were first described due to the variations in the both the definitions used and described impact on clinical outcome. The aim of our study was to evaluate the significance of the IgM deposits in the glomerular mesangium for outcomes of nephrotic syndrome (NS) in children.MethodsForty-five children with NS who underwent renal biopsy at tertiary pediatric hospital from January 1st, 2000 to December 31st, 2015 and the pathology diagnosis of minimal change disease, focal segmental glomerulosclerosis and mesangial hypercellularity (MH) were retrospectively analyzed. IgM positivity was defined as ≥1+ imunofluorescence with predominantly mesangial distribution. The patients were stratified into IgM-positive (n = 18) and IgM-negative (n = 27).ResultsAt the end of the median follow-up 4.5 years (range 0.17–13.14), the IgM-positive group was represented by 11 patients (61.1%) in remission, 3 patients (16.7%) with active disease and normal kidney function, 2 (11.1%) patients with active disease and impaired kidney function, 2 (11.1%) patients on renal replacement therapy. Accordingly, the IgM-negative group included 13 patients (48.1%) in remission, 12 (44.4%) with active disease and normal kidney function, 1 (3.7%) with active disease and impaired kidney function, 1 (3.7%) on renal replacement therapy, with no statistical significance between groups (p = 0.186).ConclusionsThis study did not reveal significant differences of the disease outcomes between IgM-positive and IgM-negative groups.

Clinical outcomes of nephrotic syndrome in immunoglobulin a nephropathy

Immunoglobulin A (IgA) nephropathy can be complicated by the nephrotic syndrome in rare cases. Although corticosteroid therapy should be recommended in such cases, the response to steroid treatment has been variable, and spontaneous remission also has been reported without steroid treatment in some cases. We report a retrospective analysis of our experience on the clinical outcomes of nephrotic syndrome in patients with IgA nephropathy, in the nephrology department of a provincial hospital in South Korea. Thirty-three patients with biopsy-proven IgA nephropathy with nephrotic syndrome were enrolled between March 1990 and March 2013. We analyzed data according to demographic, clinical, and laboratory records. The mean follow-up duration was 62 ± 45 months (10-204) in 33 patients. Complete remission occurred in 10 steroid-users and two steroid-nonusers. Partial remission occurred in seven steroid-users, and eight steroid-nonusers. During follow-up, six patients showed progressive deterioration of renal function. Among the IgA nephropathy patients with nephrotic syndrome, 36% and 45% of patients had complete and partial remission, respectively. Steroid treatment may effectively reduce proteinuria. However, spontaneous remission occurs in some cases.

Outcomes of primary nephrotic syndrome in elderly Japanese: retrospective analysis of the Japan Renal Biopsy Registry (J-RBR).

There are very little data available regarding nephrotic syndrome (NS) in elderly (aged ≥65 years) Japanese. The aim of this study was to examine the causes and outcomes of NS in elderly patients who underwent renal biopsies between 2007 and 2010. From July 2007 to June 2010, all of the elderly (aged ≥65 years) Japanese primary NS patients who underwent native renal biopsies and were registered in the Japan renal biopsy registry (J-RBR; 438 patients including 226 males and 212 females) were identified. From this cohort, 61 patients [28 males and 33 females including 29, 19, 6, 4, and 3 patients with membranous nephropathy (MN), minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS), membranoproliferative glomerulonephritis (MPGN), and other conditions, respectively] were registered from the representative multi-centers over all districts of Japan, and analyzed retrospectively. The treatment outcome was assessed using proteinuria-based criteria; i.e., complete remission (CR) was defined as urinary protein level of <0.3 g/day or g/g Cr, and incomplete remission type I (ICR-I) was defined as urinary protein level of <1.0-0.3 g/day or g/g Cr, and renal dysfunction was defined as a serum creatinine (Cr) level of 1.5 times the baseline level. In this elderly primary NS cohort, MN was the most common histological type of NS (54.8 %), followed by MCNS (19.4 %), FSGS (17.4 %), and MPGN (8.4 %). Of the patients with MN, MCNS, or FSGS, immunosuppressive therapy involving oral prednisolone was performed in 25 MN patients (86.2 %), 18 MCNS patients (94.7 %), and all 6 FSGS patients (100 %). CR was achieved in all 19 (100 %) MCNS patients. In addition, CR and ICR-I were achieved in 16 (55.2 %) and 18 (62.1 %) MN patients and 4 (66.7 %) and 5 (83.3 %) FSGS patients, respectively. There were significant differences in the median time to CR among the MCNS, FSGS, and MN patients (median: 26 vs. 271 vs. 461 days, respectively, p < 0.001), and between the elderly (65-74 years, n = 7) and very elderly (aged ≥75 years, n = 12) MCNS patients (7 vs. 22 days, p = 0.037). Relapse occurred in two (6.9 %) of the MN and nine (47.4 %) of the MCNS patients. Renal dysfunction was observed in five (7.2 %) of the MN patients. Serious complications developed in eight (14.8 %) patients, i.e., two (3.7 %) patients died, four (7.4 %, including three MCNS patients) were hospitalized due to infectious disease, and two (3.7 %) developed malignancies. The initiation of diabetic therapy was necessary in 14 of the 61 patients (23.0 %) with much higher initial steroid dosage. Renal biopsy is a valuable diagnostic tool for elderly Japanese NS patients. In this study, most of elderly primary NS patients respond to immunosuppressive therapy with favorable clinical outcomes. On the other hand, infectious disease is a harmful complication among elderly NS patients, especially those with MCNS. In future, modified clinical guidelines for elderly NS patients should be developed.

Long-Term Outcome of Nephrotic Syndrome in an Allogeneic Hematopoietic Stem Cell Recipient Without Typical Features of Graft Versus Host Disease

Nephrotic syndrome (NS) is one of the chronic kidney diseases in long-term survivors of allogeneic hematopoietic stem cell transplantation (HSCT) and it has been an increasingly reported complication. Recently, Colombo et al. suggested that careful monitoring of renal function in myeloablative HSCT recipients with chronic graft-versus-host disease (cGVHD) may document the incidence of NS (1). Six cases of NS together with extensive cGVHD were reported in that retrospective study. The cumulative incidence of NS was reported to be 8% at day +1681. Additionally, Srinivasan and co-workers reported NS with a cumulative incidence of 6.1% in nonmyeloablative HSCT recipients from a related human leukocyte antigen (HLA)-compatible donor (2). In contrast to previous reports, three of seven NS cases were not associated with cGVHD. It was usually observed that NS is not improved with the reinitiation of aggressive immunosuppression and it results in progressive renal failure necessitating dialysis. We would like to present our experience regarding the incidence and outcome of NS in patients who underwent allogeneic HSCT. We analyzed 70 adult patients with a minimum follow-up of 100 days who underwent myeloablative allogeneic HSCT. Thirty patients were identified as having cGVHD and one patient NS without evidence of cGVHD. This patient was a-34-year-old woman. She received unmanipulated blood HSCT for acute myeloid leukemia from her HLA full-matched brother. The conditioning was with busulfan and cyclophosphamide. Cyclosporine and short course methotrexate were given as GVHD prophylaxis. The major early posttransplant complication was severe hemorrhagic cystitis. She did not experience GVHD. After 4 months of GVHD prophylaxis cessation, pedal edema was detected during routine follow-up. The serum albumin level was 1.9 g/dL with urinary protein excretion rate 6 g/day. Creatinine clearance was within normal limits, but serum lipid levels were increased. She was in hematological remission and DNA analysis revealed complete chimerism. Renal biopsy showed normal architecture of the glomerulus with normal thickness of the basement membrane. Immunofluorescence microscopy revealed a characteristic pattern of membraneous nephropathy (MN); thin subepithelial granular deposits of immunoglobulin G and C3 along the glomerular basement membrane. Hydroxy-methylglutaryl coenzyme A reductase inhibitor and angiotensin-converting enzyme inhibitor treatment were initiated to reduce serum lipid levels and urinary protein excretion, respectively. Prednisone was administered as immunosuppressant. The patient de-veloped bilateral avascular necrosis of the femoral head and refused further therapies. Steroid treatment was withdrawn. Eight years after the diagnosis of NS, the disease did not progress. The proteinuria persisted but decreased to 0.5–1.5 g/day and the serum albumin level rose to 2.8 g/dL. She has still denied any symptom signifying an underlying disorder or GVHD. As conclusion, the risk of NS as a postHSCT MN should be considered and renal functions should be carefully monitored. The evolution of NS does not usually parallel in the clinical course of cGVHD (2–4). MN—the most common finding on renal biopsy—may be underrecognized until it is clinically symptomatic (5). In all series reported to date, the clinicopathologic features of NS demonstrated that there is an immune-mediated process (1–6). We consider that there may be an association between cGVHD and postHSCT MN, but the treatment should be individualized, especially in cases without manifestations of cGVHD. Sevgi Kalayoglu-Besisik Department of Internal Medicine Division of Hematology Istanbul University Istanbul School of Medicine Istanbul, Turkey Alper Yurci Department of Internal Medicine Division of Gastroenterology Erciyes University School of Medicine Kayseri, Turkey Halil Yazici Department of Internal Medicine Division of Nephrology Istanbul University Istanbul School of Medicine Istanbul, Turkey Ipek Yonal Department of Internal Medicine Istanbul University Istanbul School of Medicine Istanbul, Turkey Deniz Sargin Department of Internal Medicine Division of Hematology Istanbul University Istanbul School of Medicine Istanbul, Turkey

Nephrotic Syndrome after Allogeneic Hematopoietic Stem Cell Transplantation as a Late Complication of Chronic Graft-versus-Host Disease

This study aims to determine the incidence and outcome of nephrotic syndrome in patients who underwent allogeneic stem cell transplantation in a single center. Records of 279 adult patients with hematological diseases who underwent allogeneic hematopoietic stem cell transplantation were analyzed to evaluate the incidence and outcome of nephrotic syndrome. The diagnosis of chronic graft-versus-host disease was based on clinical evidence with histological confirmation whenever possible. Of the 279 patients, 105 with a minimum follow-up of 100 days developed chronic graft-versus-host disease: six of these had nephrotic syndrome. The cumulative incidence of nephrotic syndrome was 8% at day +1,681. Patients grafted with peripheral blood stem cells had a higher probability of developing nephrotic syndrome than did those grafted with bone marrow: 24% and 3%, respectively. The pathological diagnosis was membranous glomerulonephritis in four patients, and minimal change disease in one; the diagnosis could not be histologically confirmed in the sixth patient. All patients had extensive chronic graft-versus-host disease and were receiving treatment with cyclosporine A and steroids (four patients). Response to immunosuppressive therapy with cyclosporine A and steroids was achieved in all patients at a median time of 12 weeks after transplantation. Patients with chronic graft-versus-host disease may be considered to be at risk of nephrotic syndrome: careful monitoring of renal function is advisable, particularly in patients receiving allogeneic peripheral stem cell grafts.

Improved outcomes in nephrotic syndrome.

Nephrotic syndrome can now be treated effectively in most cases. All patients should be treated with a low-salt diet, diuretics to reduce edema, and statins to normalize serum lipid concentrations. Patients with nephrotic syndrome are prone to deep vein thrombophlebitis, renal vein thrombosis, and pulmonary emboli. Depending on the condition, additional treatment may include corticosteroids, angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs), cyclosporine, cytotoxic agents, or mycophenolate.

Implication of serum IgE in childhood nephrotic syndrome.

Elevated serum IgE levels have been related to glomerular diseases. We investigated the relationship between serum total and specific IgE levels and their modulating factors [interleukin-4 (IL-4) and sCD23] and the outcome of childhood nephrotic syndrome (NS) after steroid treatment. We found that children with NS had significantly higher serum IgE levels than age-matched allergic patients and normal controls. Patients with steroid-resistant nephrotic syndrome (SRNS) had higher serum IgE levels than patients with steroid-sensitive nephrotic syndrome (SSNS) both pre and post treatment. Elevated initial serum IgE levels appeared to be associated with poor outcome. Although one-half of nephrotic children had detectable specific IgE to common allergens (dust mites or milk), the presence of specific IgE was not correlated with disease outcome. Serum IL-4 levels were not different among normal controls and patients with SRNS or SSNS. However, serum sCD23 levels in NS patients were significantly higher than in normal controls both pre and post treatment. Serum sCD23, but not IL-4, levels were correlated with serum total IgE levels. Our results suggest that regulation of total IgE production correlates with the disease activity and outcome of NS, although the presence of common specific IgE may not be linked to the pathogenesis.

Papillary pore counts: a method of studying developmental aberrations in diseased juvenile kidneys.

Counts of the number of pores of primary collecting tubules (ducts of Bellini) on renal papillae, and values calculated by adjusting the counts of compound papillae to those of "virtual" single papillae, were determined for kidneys of patients with end-stage renal failure. Values for chronic glomerulonephritis, Alport's disease, infantile polycystic disease, trisomy 18, and trisomy 13 were not abnormal. Kidneys of patients with CUTO showed significantly low pore counts, indicating that this process in some cases is a true hypoplasia, with mean reduction of number of ducts of Bellini of 26%. FGS showed a high proportion of single papillae (80% vs. normal 60%) with high virtual pore counts, suggesting that a developmental abnormality underlies this disorder (or this outcome of nephrotic syndrome). Cystinosis showed a high proportion of compound papillae (80% vs. 40%) but low virtual pore counts, implying that this genetic disorder causes both maldevelopment and postnatal functional abnormality of the kidneys. A Jeune syndrome kidney produced very low pore counts (mean 8 vs. 16.6 for virtual pore counts), and Down's syndrome also showed low pore counts (mean VPC 15.1 vs. normal 16.6), indicating that the low kidney weights demonstrated by others with Down's syndrome reflect a true hypoplasia.