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Objective: The most common form of nephrotic syndrome (NS) is minimal change disease (MCD) in children and focal segmental glomerulosclerosis (FSGS) following it. As, it is important to predict corticosteroid (CS) response at the beginning of the disease, we aimed to evaluate the efficacy of some biomarkers in terms of predicting steroid response in patients with NS. Patients and Methods: Twenty patients who met the inclusion criteria for the study were divided into 3 groups and 6 healthy control participants were included in the analysis as the 4th group. Group-1 included 10 patients at the first episode of idiopathic NS (INS), group-2 included the same 10 patients in remission, group-3 included 10 patients with steroid resistant NS (SRNS) diagnosed as FSGS by renal biopsy, and group-4 included six healthy children as controls. Urinary and serum cluster of differentiation (CD) CD80, IL-17, IL-23, IL-10, TGF-β, CD86, CD28, CTLA-4 levels were measured for all groups. Results: Urinary CD80 level in INS-relapse group was significantly higher than the levels of the INS-remission, FSGS and control groups (p<0.001). Urinary CD28 and uIL-10 were significantly increased in INS-remission group than INS-relapse (p<0.05, p<0.001). Serum IL-17 was significantly higher in INS-relapse group than in INS-remission group (p<0.01). There was no difference in IL- 23,TGF-β,CD86 parameters between groups. Conclusion: In our study, urinary CD80 levels were significantly higher in the relapse group compared to the other groups. When supported by more comprehensive clinical studies, urinary CD80 level may be a good biomarker to predict CS response and to predict in favor of MCD. [ABSTRACT FROM AUTHOR]

Background: Patients with nephrotic syndrome (NS) are at a higher risk of developing invasive pneumococcal disease (IPD). Pneumococcal carriage studies are helpful tools for detecting potentially infectious serotypes and guiding immunization efforts. Pneumococcal nasopharyngeal colonization is common, and IPD can easily occur in an immunosuppressed state. Limited information is available regarding the frequency of pneumococcal carriage in individuals with NS. The aim of this study was to evaluate pneumococcal carriage and serotype distribution in children with NS. Methods: Pneumococcal carriage was detected by real-time PCR assays from nasopharyngeal swab samples from 98 children with NS, and 100 healthy controls. Isolates were serotyped by real-time PCR. Results: The pneumococcal carriage rate was 44.9% in children with NS. Regarding the recommendation about pneumococcal immunization in children with NS, the vaccination rate was low. Also, non-PCV13 serotypes have been detected in at least 25% of PCV13-vaccinated children. There is no statistically significant difference in total pneumococcal carriage rate, PCV13 serotype carriage rate, or non-PCV13 serotype carriage rate between children with NS and healthy controls (p > 0.05 for all). Conclusions: The pneumococcal carriage rate was similar between children with NS and healthy controls. However, because children with NS have an increased risk for IPD, the serotype distribution of children with NS can demonstrate the improved protection offered by new pneumococcal vaccines. Regular monitoring for IPD is crucial for assessing the evolving sero-epidemiology of pneumococcal infections and evaluating the effectiveness of vaccines for children with NS. [ABSTRACT FROM AUTHOR]

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Nephrotic syndrome progresses with various metabolic disturbances, such as proteinuria over 3.5 grams in 24 hours, hypoalbuminemia, and hypercoagulability. Patients usually complain about diffuse edema throughout the body, which is secondary to hypoalbuminemia. It has many primary and secondary causes. Patients may require a renal biopsy to confirm the diagnosis. Besides, many secondary causes of nephrotic syndrome should be examined and excluded. Although many vaccines were developed due to the COVID-19, many side effects are still reported because of the Pfizer-BioNTech COVID-19 vaccine (COVID-19 mRNA and BNT162b2), which is widely used in Turkey. This study examines a case of nephrotic syndrome with acute renal injury after Pfizer-BioNTech vaccine. [ABSTRACT FROM AUTHOR]

We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4–116.3), and median proteinuria was 6000 mg/d (IQR, 3656–9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people. [ABSTRACT FROM AUTHOR]

Background: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Methods: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Results: Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Conclusions: Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract [ABSTRACT FROM AUTHOR]

Aim: Rituximab (RTX) is a genetically engineered chimeric monoclonal antibody which binds directly to CD20 antigen and mediates inhibition of B cell development. Although RTX has been widely used in paediatric nephrology, there is no routine protocol for its use. In this study, paediatric nephrologists in Türkiye were asked to fill out a questionnaire to understand their practice in using RTX. This study aimed to determine common practices and clarify the uncertainties regarding the use of RTX in paediatric nephrology., Methods: This was a nationwide, multicenter, retrospective cohort study based on data evaluating the use of RTX in paediatric nephrology practice. An online questionnaire was sent to all paediatric nephrology centers in Türkiye. The questionnaire forms included information about how many patients in total applied RTX treatment, for which indications they use RTX, and whether they made any preparations before using RTX., Results: According to this survey on RTX use in Türkiye, paediatric nephrologists use it most commonly in SSNS and followed by SRNS, ABMR, SLE and AAV, respectively. Dosing was highly standard but there is significant heterogeneity in pre-exposure tests and patient monitoring in the clinical practice of RTX. Also, the rate of encountering RTX-related allergic and infectious side effects at least once during the professional experience of our physicians can be quite high., Conclusion: There is an increasing need for the preparation of a guideline on the indications for RTX use for each diagnosis, posology, and the practices to be performed before and after infusion., (© 2023 Asian Pacific Society of Nephrology.)

Aim: In this study, we aimed to evaluate the demographic and histopathological characteristics and response to medications in children with idiopathic nephrotic syndrome in Turkey. Methods: We reviewed medical records of patients older than one year, who were newly diagnosed with nephrotic syndrome and had been followed for at least one year in our department between November 1994 and March, 2013. Results: A total of 289 children (169 boys) were included in the study. Fifty theree patients (18.4%) were with steroid-resistant nephrotic syndrome, 33 (11.4%) with frequently relapsing nephrotic syndrome and 53 (18.4%) were with steroid-dependent nephrotic syndrome. Cyclosporine A (CsA), cyclophosphamide, mycophenolate mofetil, levamisole, azathioprine, and rituximab were used as steroid-sparing agents in some patients. The number of patients who were responder to steroid and to CsA was similar. Majority of patients with steroid- resistant nephrotic syndrome were also resistant to mycophenolate mofetil and CsA. Conclusion: There was a high prevalence of minimal change disease based on kidney biopsy especially in boys younger than six years of age and response to steroid and CsA was almost similar. [ABSTRACT FROM AUTHOR]

Background: ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. Methods: A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Results: Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04-19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4-39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117-155) ml/min/1.73m, proteinuria was 1,008 (IQR 281-1,567) mg/m/day. After a median follow-up of 11.5 (range 4-21) months following CoQ10 administration, proteinuria was significantly decreased (median 363 [IQR 175-561] mg/m/day, P=0.025), whereas eGFR was preserved (median 137 [IQR 113-158] ml/min/1.73m, P=0.61). Conclusions: ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective. [ABSTRACT FROM AUTHOR]

Introduction: Although preventative nephrology is the effective management of childhood kidney diseases, it is hard to provide it in this undesirable conditions. In this study, we aimed to document the kidney disease profile of Syrian refugee children admitted to our hospital.Materials and Methods: One hundred and thirty Syrian refugee children were admitted to the Pediatric Nephrology Department of the University of Gaziantep from September 2012 to January 2015. Demographic data, history, symptoms, physical examination findings, laboratory investigations, diagnosis, disease outcome, and therapeutic procedures such as peritoneal dialysis and hemodialysis were obtained from patient files.Results: Of the 130 admitted children, 74 were girls (59.6%). The average age was 6.97 ± 4.2 years (range, 1 month to 17 years). Congenital abnormalities of the kidney and urinary tract were found in 34 children (26.2%). Other morbidities were chronic kidney disease in 30 (23.1%), nephrotic syndrome in 24 (18.5%), urolithiasis in 9 (6.9%), acute kidney injury in 4 (3.1%), glomerulonephritis in 5 (3.8%), enuresis in 12 (9.2%), and others in 12 (9.2%).Conclusions: Congenital abnormalities of the kidney and urinary tract and chronic kidney disease were highly prevalent in Syrian refugee children. Although free health care have been provided to all of these children, the continuation of political crisis and instability would increase the number of admissions and affect the quality of life of those children in a different environment from the home country. [ABSTRACT FROM AUTHOR]

Purpose: In our study, diagnostic and demographic characteristics of patients diagnosed with minimal change disease (MCD) by biopsy, clinical and laboratory findings in our country were investigated., Methods: Data were obtained from the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database. Demographic characteristics, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented are cross-sectional and includes application data for the biopsy period., Results: Of 3875 patients, 233 patients with MCD (median age 35.0 years) were included in the study, which constitutes 6.0% of the total glomerulonephritis database. Renal biopsy was performed in 196 (84.1%) patients due to nephrotic syndrome. Median serum creatinine was 0.7 (0.6-1.0) mg/dl, mean eGFR was 104 ± 33 ml/min/1.73 m 2 and median proteinuria 6000 mg/day. The number of patients under the age of 40 years was 139 (59.7%) (Group A), and the number of patients aged 40 years and over was 94 (40.3%) (Group B). Compared to Group A, global sclerotic glomeruli (24 vs. 43, p < 0.001) interstitial inflammation (15 vs. 34, p < 0.001), interstitial fibrosis (20 vs. 31, p = 0.001, vascular changes (10 vs. 25, p < 0.001) and tubular atrophy (18 vs. 30, p < 0.001) were found to be significantly higher in Group B. There was no difference in immunofluorescent staining properties between the two groups., Conclusion: Our data are generally compatible with the literature. Chronic histopathological changes were more common in patients aged 40 years and older than younger patients. Studies investigating the effects of these different features on renal survival are needed., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) are caused primarily by mutations in genes that encode structural and regulatory proteins of the glomerular filtration barrier. The aim of this study was to determine genotype-phenotype correlations and prognosis in patients with CNS and INS. Methods: NPHS1, NPHS2, LAMB2 and the eighth and ninth exons of WT1 were sequenced in 80 and 22 patients with CNS and INS, respectively. Genotype-phenotype correlations and survival were evaluated. Results: Causative mutations were identified in 64.7 % of patients, of which NPHS1 mutations were the most common (37.4 %). The mutation detection rate was twofold higher in CNS patients than in INS patients (72.5 vs. 36.2 %). The most commonly mutated gene in CNS patients was NPHS1 (46.3 %) versus NPHS2 (13.6 %) and WT1 (13.6 %) in INS patients. NPHS2 mutations, female patients with NPHS1 mutations, and NPHS1 mutations affecting the transmembrane or intracellular domains of nephrin were associated with longer survival. Conclusions: Based on our present findings, the likelihood of identification of a genetic cause decreases with increasing age at diagnosis. The underlying genetic abnormality should be identified as early as possible, as this knowledge will facilitate clinicians in their prognostic prediction and enable patients to receive appropriate genetic counseling. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors in pediatric patients with steroid-resistant nephrotic syndrome (NS). Methods: Thirty-seven patients with normal renal function were compared with 22 healthy controls regarding the presence of subclinical CVD. Measurements included aortic pulse wave velocity (PWV), carotid intima media thickness (IMT), and left ventricular mass (LVM). Patients were additionally assessed for blood pressure (BP) pattern and the presence of hypertension by 24-h ambulatory blood pressure monitoring. Results: Compared with the controls, patients had significantly higher mean aortic PWV-standard deviation scores (SDS), mean carotid IMT-SDS, and LVM index ( p < 0.001 for all). Increased aortic PWV was noted in 5 % of patients, increased carotid IMT in 22 %, and increased LVM index in 19 %. Five patients (14 %) were hypertensive, and mean BP indexes, SDS, and BP loads during nighttime were significantly higher than those during daytime ( p < 0.001 for all). Multivariate analysis revealed a significant relationship between PWV-SDS and ferritin ( R2 = 0.269, p = 0.006) and between carotid IMT-SDS and proteinuria ( R2 = 0.141, p = 0.022). The LVM index was independently associated only with higher body mass index SDS ( R2 = 0.317, p < 0.001). In addition, six patients (16 %) had multiple abnormal subclinical CVD markers, and increased subclinical CVD risk was independently associated only with higher low-density lipoprotein cholesterol ( R2 = 0.292, p = 0.044). Conclusions: Based on these results, steroid-resistant NS children generally are at high risk of cardiovascular complications, but the increased risk is likely to be multifactorial. [ABSTRACT FROM AUTHOR]

Background. In steroid-resistant nephrotic syndrome (SRNS), a considerable number of patient progress to end-stage renal disease (ESRD), despite aggressive therapy. The latest advance in familial focal segmental glomerulosclerosis (FSGS) has been the discovery of a mutant form of canonical transient receptor potential channel 6 (TRPC6) leading to FSGS through unclear mechanisms. The aim of this study is to screen for TRPC6 mutations in familial and sporadic SRNS patients. Methods. Twenty-five children with SRNS originating from Turkey were included in this study. Nine patients had familial and 16 patients had sporadic SRNS. Mutation analysis was performed in all 13 coding exons of the TRPC6 gene with the direct DNA sequencing method. The control group consisted of 50 normal healthy children originating from Turkey. Results. No mutation was detected in nine children (four familial, five sporadic). A variant (L395A) in one patient, intronic nucleotide substitution (c.171 + 16 A>G and c.171 + 86 G>C) in six patients and previously described missense (A404V; rs36111323) and synonymous (N561N; rs12366144) aminoacid variants in nine patients were found. Among patients with intronic, missense and synonymous aminoacid variants, 5 patients had familial and 11 patients had sporadic SRNS. Their mean age at onset of proteinuria was 2.6 ± 1.7 years. Seven cases (three familial, four sporadic) progressed to ESRD with a mean time of 10.2 ± 2.9 years. Conclusions. In conclusion, analysis of TRPC6 gene mutations in FSGS will provide new insights into the pathogenesis of nephrotic syndrome. Previous works have emphasized that the patients with only hereditary familial FSGS carried a missense mutation in the TRPC6 gene. Our findings suggest that TRPC6 mutations may also have an important role in the pathogenesis of sporadic SRNS. [ABSTRACT FROM PUBLISHER]

The podocin ( NPHS2) gene encodes podocin protein, which has an important role in glomerular ultrafiltration and controlling slit membrane permeability. The detection of an NPHS2 mutation affects the treatment plan for children with nephritic syndrome (NS). The frequency and spectrum of podocin mutations in the Turkish population have remained largely unknown. The aim of this study was to screen for podocin mutations in Turkish patients with steroid-resistant NS (SRNS) and to compare it with other published series. There were 295 children with SRNS, originating from Turkey, included in this study. Forty-one patients (13.8%) had familial NS and 254 patients (86.2%) had sporadic NS. Mutation analysis was performed in all eight exons of the NPHS2 gene with the direct DNA sequencing method. There were 53 different pathogenetic NPHS2 mutations detected, including 37 novel mutations. The mutation detection rate was 24.7% for all patients, 29.2% for familial, and 24% for sporadic SRNS. The most common mutated exon was exon 5 (52 allele). The presence of mutations in exon 4 was found to increase the risk of end-stage renal disease (ESRD). Among patients with mutations, the rates of renal failure and/or ESRD (26%) were significantly higher than in those without mutations (12.6%). The mean time of progression to renal failure and ESRD in patients with mutations (1.8 ± 2.5 years) was significantly shorter than in patients without mutations (3.7 ± 4.0 years). Additionally, in patients with heterozygote mutations, fewer cases (13.6%) progressed to renal failure and/or ESRD than in with patients who had homozygote/compound heterozygote mutations (31.3%). In conclusion, podocin mutations are responsible for some of both familial and sporadic SRNS cases in Turkey. The mutations in this gene should be searched for in every child after presentation with the first episode of NS. [ABSTRACT FROM AUTHOR]

Abstract Background : It has been reported that there are racial and regional differences in peak incidence age, histopathological features and response to steroid therapy in childhood idiopathic nephrotic syndrome. Methods : One hundred and thirty-eight patients with a diagnosis of idiopathic nephrotic syndrome, followed up in 1994-2000, were assessed retrospectively. The aim of this study was to assess the patients’ response pattern to steroid therapy, to determine whether the duration of the initial steroid therapy alters the steroid response pattern of the disease and to assess renal biopsy results. Results : One hundred and fourteen patients who initially received only steroid therapy and were followed up regularly were classified according to response pattern. Of the 114 patients, 30 children had an initial response, 25 children had infrequent relapse, 19 had frequent relapse, 25 had steroid dependence and 15 children had steroid resistance. The 99 patients with steroid responsive nephrotic syndrome were divided into two groups with respect to duration of the initial steroid therapy. There was no statistically significant difference between standard and short therapy groups with respect to the steroid response patterns. Percutaneous renal biopsy was performed in 43 of the 138 patients. Mesengioproliferative glomerulonephritis was the most common histopathological lesion, followed by membranoproliferative glomerulonephritis. The proportions of membranous glomerulonephritis, focal segmental glomerulosclerosis and minimal change nephrotic syndrome were low in our group. Conclusions : Our study group is similar to one reported from Saudi Arabia with respect to the steroid response pattern and to Saudi Arabian and Nigerian reports with respect to the histopathology. Although it has been reported that short initial steroid therapy was followed by a higher rate of relapses, there was no statistically significant difference between standard and short... [ABSTRACT FROM AUTHOR]

Background: Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children. Methods: This is a retrospective study in which 392 Turkish children who were diagnosed with NS during the last 10 years and were followed for at least 2 years, were evaluated. Mean age of the study group was 4.6 ± 3.4 years (range 0.9−16 years) and 232 were male and 160 were female. Results: In total, 280 patients were diagnosed as MCNS with their initial presentations, laboratory features, and clinical course. Kidney biopsy was performed in the remaining 112 children according to current recommendations. The results showed that membranoproliferative glomerulonephritis (MPGN) was the most common histopathologic diagnosis, 38 (34%) of the 112 patients were found to have MPGN. The number diagnosed as FSGS was 26 (23%). A significant difference was found between the age groups for both MPGN and FSGS, the former being more common in children >6 years of age and the latter more frequent in children ≤6 years. MCNS was found in only 19% of all biopsies performed and the total incidence of MCNS (presumptive + biopsy proven) was 76%. There was no significant difference between the incidence of different histopathological subtypes before and after 1995. Conclusion: Minimal change nephrotic syndrome is still the most common primary NS in childhood and MPGN is found to be the most common histopathologic subtype in the present patient population who underwent biopsy. [ABSTRACT FROM AUTHOR]

Tertian malaria is endemic in south-eastern Anatolia. As in Europe and America, in south-eastern Anatolia, an etiological agent is seldom identified in nephrotic syndrome. Two patients with Plasmodium vivax and nephrotic syndrome are described here. The possible relationship between Plasmodium vivax and nephrotic syndrome should be explored in children in endemic malarial regions. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to describe the incidence and contributory risk factors for thromboembolic complications in children with nephrotic syndrome (NS) and thrombosis., Methods: Among 188 children with the diagnosis of NS (80 girls; mean age: 12.6 ± 5.4 years) followed up in our hospital for the last 5 years, 17 (9.0 %) children (16 boys) identified as having thromboembolic complications. All 17 children with NS and thrombosis were screened for laboratory risk factors for thrombosis. The diagnosis was confirmed by cranial magnetic resonance imaging, doppler ultrasonography, and echocardiography., Results: Among 17 children with thrombosis, 14 (82.3 %) were found to have focal segmental glomerulosclerosis (FSGS) as underlying pathology by renal biopsy. The mean age of the thrombotic children was 4.5 ± 3.2 years at the diagnosis of NS and that was 7.1 ± 4.9 years at the time of thrombosis. The mean time from NS diagnosis to the first thrombosis development was 2.6 ± 2.3 years. Thrombosis occurred during the first year of NS in 9/17 (52.9 %) children. Most of the children (88.2 %) had venous thrombosis. Among the screened risk factors, high factor VIII level (64.7 %) was the leading factor followed by decreased antithrombin III level (29.4 %). Furthermore, 4 children had central venous catheters and 2 had infection as clinical risk factors for thrombosis., Conclusion: In this case series, subtype of FSGS, active disease state of NS, central venous catheters, and some inherited and acquired thrombotic risk factors have been identified as contributory factors for the development of thrombosis in children with NS.