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Introduction: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that can involve various organs. Renal involvement has been seen in about 60% of SLE patients, while the most common presentation of lupus nephritis (LN) is proteinuria. Objectives: This study aimed to investigate the clinical and laboratory features of LN patients, confirmed by kidney biopsy and compare these among different classes of LN. Patients and Methods: This cross-sectional study was conducted on clinical and laboratory data of patients with LN from 2001 to 2016. All patients diagnosed with definite LN by biopsy and electron microscopy (EM) were included in this study. Results: A total number of 496 patients were enrolled. The mean age of all patients was 28.32 ±11.41 years; 82.4% (409) were females. The biopsies were classified into LN classes II, III, IV, V, and VI, whose frequencies were 98 (20.6%), 93 (19.5%), 225(47.3%), 46 (9.7%), and 14 (2.9%), respectively. Tubular atrophy (P < 0.001) and interstitial fibrosis (P < 0.001) were found to be significantly associated with classes of LN. Additionally, 72.7% (8) and 48.2% (92) of patients in classes VI and IV of LN had blood urea nitrogen (BUN) levels more than the normal range (P < 0.001). Regarding serum creatinine levels, 81.8% (9) and 42.9% (81) of patients in classes VI and IV of LN had high levels (P < 0.001). Moreover, nephrotic syndrome (NS) was reported in 47.5% (47) of patients with class VI of LN followed by 38.8% (36) in class IV. Besides, edema was significantly more dominant in classes IV (74.3%, 133) and VI (75%) of LN patients (P = 0.03). Conclusion: Tubular atrophy and interstitial fibrosis were more commonly seen in LN class VI followed by class IV. Edema, hypertension, and proteinuria are common presentations in class IV. Complete assessment of renal biopsy is still helpful for the definite classification of LN. [ABSTRACT FROM AUTHOR]

Background: Nephrotic syndrome (NS), like any other chronic illness, may affect the health-related quality of life (HRQoL) of children, so do the complications related to the disease, and its treatment. A better understanding of the (HRQoL) of people with nephrotic syndrome may help to better guide their treatment. Methods: Sixty children with nephrotic syndrome were assigned as the first case group, besides 81 healthy children as the first control group and 98 children with other chronic illnesses as the second control cohort. The participant and his or her caregiver were asked to fill in the standard HRQoL questionnaire. The patient's demographic data were also collected and analyzed using SPSS version 26 software. Results: 60 patients with nephrotic syndrome (mean age 9.8 ± 3.7) scored lower grades in physical, social, educational fields as well as total scores than the healthy controls (mean age 9.5 ± 2.7) and higher than the controls with other chronic diseases (mean age 9.7 ± 3.9) (P< 0.05). The emotional QOL score was close to that of the non-healthy control group. No correlation was found between the clinical phenotype of disease regarding the response to steroids and HRQOL (P > 0.05). Conclusions: The study shows that nephrotic syndrome can affect all aspects of the quality of life of patients. We suggest that comprehensive care of patients with NS be routinely managed in a multidisciplinary clinic with filling HRQOL questionnaires integrated as a common practice. [ABSTRACT FROM AUTHOR]

Introduction: Renal biopsy is an important diagnostic procedure in pediatric nephrology. Although considered as an invasive method, numerous renal diseases cannot be definitively diagnosed and treated without it. Objectives: The aim of this study was histopathological study of renal biopsy results in children of 6 months to 18 years old. Patients and Methods: In this retrospective cross-sectional study, the available data from children who had undergone kidney biopsy between 2007 and the end of 2017 were evaluated. Demographic data, indications of biopsy, the outcome of patients, biopsy complications and histopathologic findings were collected using a checklist. Finally, data were presented as frequency and percentages. Results: The most common cause of biopsy in children was nephrotic syndrome (43.7%). Focal segmental glomerulosclerosis (FSGS) with 39 cases (32.7%) had the highest frequency in examined biopsies. In the long-term follow-up, 71 children (59.7%) cured, 7 (5.9%) died, 25 (21.0%) continued treatment, 6 (5.0%) underwent a kidney transplant surgery, and the outcome of 10 children (8.8%) was unknown. While 6.7% of patients were diagnosed with complications of biopsy, the most frequent of them was hematuria. There was no case of death or nephrectomy and only one patient needed packed RBC transfusion. Conclusion: Nephrotic syndrome was the most common indication for kidney biopsy. FSGS had the highest frequency in examined biopsies. [ABSTRACT FROM AUTHOR]

Introduction: Idiopathic Nephrotic Syndrome is a multifactorial disease that accompanying with immune system dysfunction. Cytokines as potent immunomodulators have a key role in pathogenesis of the disease. We aimed to evaluate the association between TNFα -308G > A polymorphism with Idiopathic Nephrotic Syndrome and its effect on the response to steroid therapy.Methods: This case-control study was performed on 168 patients with Nephrotic Syndrome and 153 healthy children. Genotyping of TNF-α rs1800629 (-308G > A) variant was detected by polymerase chain reaction restriction fragment length polymorphism method (PCR-RFLP).Results: The results revealed that there was no significant difference in allele (P > .05, OR = 0.92, 95% CI: 0.59 to 1.43) or genotype (P > .05, OR = 1.00; 95% CI: 0.62 to 1.65) frequency of TNF-α rs1800629 (-308G > A) between childhood cases of nephrotic syndrome and healthy controls. Also no association was found between genotype (P > .05, OR = 2.28; 95% CI: 1.03 to 5.04), and allele frequency (P > .05, OR = 1.93; 95% CI: 0.97 to 3.87) among the SSNS and SRNS groups.Conclusion: Our results did not support any association between the TNF polymorphism and the risk of nephrotic syndrome in a sample of southeast Iranian population. [ABSTRACT FROM AUTHOR]

Background: Electron microscopy (EM) is a valuable tool in the diagnosis of renal amyloidosis, particularly in the early stages of the disease. In Iran, studies on EM and the clinical features of renal amyloidosis are scarce. The objective of the present study was to survey EM investigations, pathological classifications, and clinical features of renal amyloidosis. Methods: This cross-sectional study was performed in Shiraz, Iran, during 2001-2016. Out of 2,770 kidney biopsies, 27 cases with a diagnosis of renal amyloidosis were investigated. EM investigation and six staining procedures for light microscopy (LM) were performed. Two pathological classifications based on glomerular, peritubular, perivascular, and interstitial involvement were made. Finally, the association between these classifications and the clinical features was assessed. Chi-square, Fisher's exact, Independent t test, and Multiple logistic regression analysis were used. P values<0.05 were considered statistically significant. Results: In 51.9% of the cases, the clinical diagnosis was nephrotic syndrome. Proteinuria and edema were the most prevalent clinical manifestations. The role of EM investigation for diagnosis was graded "necessary" or "supportive" in 48.2% of the patients. In the classification based on glomerular classes, variables such as the mean blood pressure (P=0.003), history of hypertension (P=0.02), creatinine >1.5 (P=0.03), and severe tubular atrophy (P=0.03) were significantly higher in class B (advanced amyloid depositions). Conclusion: EM plays an important role in the diagnosis of renal amyloidosis. EM in conjunction with LM investigation with Congo red staining is recommended, to prevent misdiagnosis of patients with a clinical suspicion of renal amyloidosis. Among different pathological features of renal amyloidosis, the severity of glomerular amyloid depositions had a clear relationship with clinical presentations. [ABSTRACT FROM AUTHOR]

Objectives: Nephrotic syndrome is a glomerular disease characterised by a loss of albumin and high-molecular-weight proteins such as thyroxine-binding globulin and thyroid hormones, potentially resulting in subclinical or even overt hypothyroidism. This study aimed to compare thyroid hormone levels between nephrotic children and healthy controls as well as between nephrotic children in the active phase of the disease and those in remission. Methods: This case-control study was conducted between March 2016 and 2018 at a paediatric hospital in Qazvin, Iran. A total of 73 nephrotic children comprised the case group--including 49 with active disease and 24 in remission--while the control group included 74 healthy children. Thyroid function was assessed according to levels of thyroid-stimulating hormone (TSH), free triiodothyronine (T3), free thyroxine (T4), total T4, total T3 and anti-thyroid peroxidase. Results: All of the controls had normal total T4 levels. Elevated TSH levels were more frequent in nephrotic children compared to controls (34.2% versus 10.8%; P = 0.001). A significantly lower number of patients with active disease were euthyroid compared to those in remission (51% versus 95.8%; P = 0.001). Moreover, 7 (9.5%) of patients in the active and no patient in remission phase had abnormal total T4 levels (P <0.001), while 14.3% and 0% had highly elevated TSH levels (P = 0.002). Conclusion: Due to the prevalence of subclinical and even overt hypothyroidism, thyroid screening tests may be required for nephrotic children. However, further research is needed to confirm these findings. [ABSTRACT FROM AUTHOR]

Background and Aim: Despite the spread of Henoch-Schonlein purpura (HSP) in all societies, especially in Asian children, no comprehensive study on HSP has been conducted in Iranian children and most of these reports are limited to disease cases or exclusively to patients with HSP. Therefore, this study was conducted to describe the clinical, diagnostic, and therapeutic approaches in children with HSP in Iran. Methods: This historical cohort study was performed in all children suffering from HSPN hospitalized at Ali-Asghar Children's Hospital, Tehran between April 2006 and March 2017. The patients' baseline characteristics including demographics, clinical symptoms and laboratory parameters were all collected from hospital files. The patients were followed up for at least six months of initiating treatment and also for 12 to 120 months after treatment. Results: Of 100 patients with HSP, 18 (11 boys and 7 girls) had indications for biopsy that were included in the study. The mean age of the participants was 7.72 ± 2.69 years. Nephrotic syndrome was found in 44.4% and nephritic syndrome in 61.1% of the patients. Hematuria was found in 66.7%, proteinuria in 66.7%, and hypertension in 38.9% of the patients. The mean serum creatinine was 1.0 ± 0.6 mg/dl with a mean GFR of 95 ± 5ml/min. Regarding pathological classification, 33.3% had class II and 66.7% had class III. With respect to therapeutic regimen, 61.1% were treated only with steroids while others were treated with a combination of steroids and immunosuppressant drugs. During the follow-up time, all patients were treated successfully with the mentioned regimens. In all treated subjects, proteinuria disappeared in all urine samples. Due to complete improvement in all patients, repeated renal biopsy was not indicated. Conclusion: Kidney involvement occurs as nephritic syndrome in about two thirds of patients and as nephrotic syndrome in the remaining cases. In the majority of patients, treatment with steroids alone is successful although combined therapy with immunosuppressant drugs is required in the remaining patients. In summary, the therapeutic protocols are associated with a significant long-term recovery (a five-year recovery of 87.5% in our study). [ABSTRACT FROM AUTHOR]

Background: The aim of this report is to highlight the importance of considering nephrotic syndrome as a potential underlying cause of bilateral central serous retinal detachment in a patient with systemic lupus erythematosus and to underscore the significance of a comprehensive systemic workup in these patients., Case Presentation: A 19-year-old Iranian female patient with history of systemic lupus erythematosus presented with progressive vision loss and bilateral macular elevation. Ophthalmic examination revealed periorbital edema, chemosis, and subretinal fluid at the macula of both eyes. Optical coherence tomography confirmed the existence of subretinal fluid and serous detachment located at the macula of both eyes. On fluorescein angiography, there were no signs of subretinal leakage such as smoke stack sign or expansile dot in late phases. Laboratory tests detected hypoalbuminemia and significant proteinuria, leading to the diagnosis of nephrotic syndrome. Treatment with prednisolone and albumin infusion resulted in improved visual acuity and resolution of subretinal fluid., Conclusion: Nephrotic syndrome can be a rare underlying cause of bilateral central serous retinal detachment, and its association with systemic lupus erythematosus should be considered. Hypoalbuminemia in nephrotic syndrome alters fluid dynamics in the retina, contributing to bilateral central serous retinal detachment. Early recognition and management of nephrotic syndrome are essential for vision recovery and preventing long-term complications., (© 2023. The Author(s).)

Schimke Immuno-osseous Dysplasia (SIOD) is a rare autosomal recessive disease caused by a biallelic mutation in SMARCAL1 gene. Typical findings in SIOD include spondylo-epiphyseal dysplasia, steroid resistance nephrotic syndrome, progressive renal failure, T-cell immunodeficiency, bone marrow failure, and cerebral infarction. In this case report, we describe a 9-yr-old girl who presented with failure to thrive in infancy. Nephrotic syndrome was diagnosed at the age of four years. She had three episodes of admission with cerebral stroke due to moyamoya syndrome. In the last admission at Namazi Hospital, Shiraz, southern Iran, in October 2016, she had new cerebral ischemia, developed seizure, and finally died. [ABSTRACT FROM AUTHOR]

Introduction: Nephrotic syndrome is the most common chronic renal disease in children. Mostly, it is controlled by steroids. Many underlying pathologies exist in patients with steroid-resistant nephrotic syndrome (SRNS). Among them are 'focal segmental glomerulosclerosis (FSGS) and 'minimal change disease' (MCD). Examining patients' clinicopathologic characteristics can be helpful by giving an insight into the etiology of steroid resistance and determining patient prognosis. Material and Methods: This cross-sectional study was performed in 'Children's Medical Center' between 2001 and 2011. From 150 patients biopsied, seventy-one children with SRNS, aged 1-14 years, were included. Results: Among 150 patients biopsied, 71 children (47.3%) had steroid-resistant nephrotic syndrome. Forty-four (62%) of these were boys. Upon pathologic investigation of SRNS cases, FSGS came in first, with the highest prevalence at a rate of 32.4%, and MGN came in last, at a rate of 5.6%. The mean age of disease onset was 4.7 years and the mean age of undergoing biopsy was six years. Conclusions: In this study, the predominant pathologic pattern of steroid-resistant nephrotic syndrome was FSGS, a finding similar to that of most studies conducted in this field. MCD was observed in 21.1% of patients, which indicates the variety in reporting renal lesions, particularly, regarding the diagnoses of MCD, mesangioproliferative glomerulonephritis and early stages of FSGS. [ABSTRACT FROM AUTHOR]

Introduction. Mutations in podocin (NPHS2) gene have the key role in the pathogenesis of steroid-resistant nephrotic syndrome (SRNS) in children, but data is scarce regarding their prevalence and natural course among different all ethnic groups. This study was aimed to demonstrate the spectrum of NPHS2 mutations in children with SRNS and to compare the clinical course of disease in patients with and without mutation. Materials and Methods. All 8 exons of NPHS2 were sequenced in 99 children, including 49 with SRNS and 50 with steroid-sensitive nephrotic syndrome (control group) by DNA sequencing. Results. The prevalence rates of NPHS2 gene mutation among children with SRNS and SSNS were 31% and 4%, respectively. The prevalence rates of mutation among familial and sporadic forms were 57% and 26%, respectively. Thirty-three percent of the children experienced recurrence of primary disease after kidney transplantation, none of whom had a mutation. The clinical response to treatment was poorer in children with mutation in comparison with patients without mutation (12% versus 32%, respectively; odds ratio, 3.29, 95% confidence interval, 0.40 to 25.64). Patients with and without mutation could not be differentiated by demographic and histological features, glomerular filtration rate at onset, hypertension, progression to end-stage renal disease, and proteinuria. Conclusions. Mutations of NPHS2 gene are frequent among Iranian children with SRNS. Regarding similar clinical features in patients with and without mutation and poor response to pharmacotherapy in patients with mutation, a molecular approach might be necessary for different treatment plans and prediction of prognosis. [ABSTRACT FROM AUTHOR]

Introduction. Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the first year of life and is a diagnostic and therapeutic challenge for the pediatricians as well as pediatric nephrologists. Our study is the first study of Iranian children with CNS in two pediatric nephrology centers in Tehran, Iran. Materials and Methods. We reviewed medical charts of 30 infants diagnosed with CNS from 1990 to 2005. Results. There were 15 boys and 15 girls with CNS (mean age, 1.7 months). The presentation of the disease was nephrotic syndrome in 96.6% of the patients. Eighty percent of the patients presented within 3 months of life and 16 in the neonatal period. The Finnish type of CNS was seen in 43.3% and diffuse mesangial sclerosis in 50%. Preterm labor and low birth weight was seen in 20%. A family history of nephrotic syndrome in infancy was noted for 8 children (26.7%). Numerous complications of nephrotic syndrome occurred in 73.3%. Seventy percent of the patients had 27 episodes of infections. Sepsis was seen in 43.3% of the children, of which 61.5% were caused by gram-negative bacteria and 38.6% were caused by Staphylococcus aureus. Thrombotic complications and hypertension developed in 6.6% and 23.3% of the patients, respectively. The mortality rate of patients was 86.6%. Conclusions. Diffuse mesangial sclerosis is an important cause of CNS. The outcome of our patients was poor and most of our patients died before reaching the age of 5 years old. [ABSTRACT FROM AUTHOR]

Introduction. Glomerulonephritis is the third most common cause of end-stage renal disease. Epidemiological data of kidney disease is population-based and has great geographic variability. The aim of this study was to assess the results of all kidney biopsies in a 5-year period in the Guilan province. Materials and Methods. In a retrospective study of 336 kidney biopsies recorded in the Department of Nephrology in Razi Hospital of Rasht, capital city of Guilan province, from August 2001 to September 2006, data consisting of age, gender, indication of kidney biopsy, and histopathological diagnosis were collected and analyzed. Results. A total of 336 kidney biopsies were reviewed (73.8% males; mean age, 40.12 ± 16.78 years). Nephritic syndrome (42.5%) and nephrotic syndrome (38.7%) were the most frequent indications of biopsy. Overall, pathologic examinations were indicative of glomerulonephritis in 272 (81.0%) biopsies and nonglomerular diseases in 64 (19.0%). The most common cause of secondary glomerulonephritis was lupus nephritis (82.6%). Focal and segmental glomerusclerosis (20.5%) was the most common pathologic diagnosis, followed by membranous glomerulonephritis (14.9%), minimal change disease (11.6%), tubulointerstitial nephritis (8.9%), and IgA nephropathy (3.6%). The most common pathologic finding among glomerular diseases was focal segmental glomerusclerosis (25.4%), while tubulointerstitial nephritis (46.9%) was the most common among nonglomerular diseases, followed by diffuse glomerulosclerosis, interstitial fibrosis, and tubular atrophy indicative of end-stage renal disease (23.4%). Conclusions. In our study, FSGS was the most common pathologic finding in kidney biopsies, and the frequency of IgA nephropathy was much lower than that in other studies. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to determine steroid dependent and independent ocular abnormalities in children with nephrotic syndrome (NS). Due to the relapsing nature of NS prolonged usage of corticosteroid puts the patients at the risk of ocular side effects of prednisolone. Since published data evaluating both steroid dependent and independent ophthalmic findings in children with NS is scarce, we evaluated ophthalmic findings in this group of patients according to the response to steroid. Methods: The study population consisted of 35 patients with steroid-sensitive NS (SSNS) and 40 patients with steroid-resistant NS (SRNS). The patients who aged 2-18 years underwent ophthalmologic examination for steroid dependent and independent ocular abnormalities. Results: The median age of patients was 7.2 years (ranged 1.2-19 years). Forty-seven subjects were men and 28 were women. Patients with SRNS had significantly higher systolic and diastolic blood pressure than patients with SSNS (p < 0.05). Forty-five out of 75 patients (60%) had either steroid dependent or independent ophthalmic symptoms. Twenty percent of the patients had steroid dependent and 29% had steroid independent ocular abnormalities. Posterior subcapsular cataract and myopic astigmatism were the most common steroid dependent and independent ophthalmologic symptoms, respectively Conclusions: Steroid independent eye involvements are not uncommon in NS children and should be considered especially in SRNS. [ABSTRACT FROM AUTHOR]

Objective: Idiopathic Nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children with the potential of progression to end stage renal disease (ESRD). INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Children's Medical Center since 1995 to 2007. Methods: In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5 steroid dependent NS and group 6 steroid resistant NS. Findings: A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL) was the most common (36.7%) pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones), 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. Conclusion: Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy. [ABSTRACT FROM AUTHOR]

This study was conducted to determine the prognostic value of some clinical, laboratory, histopathologic and therapeutic factors in 62 children with focal segmental glomerulosclerosis. There were no significant differences between the factors studied, except for severe interstitial fibrosis, which was more frequent in patients with chronic kidney disease (P=0.03). The prevalence of chronic kidney disease in non-responder groups was significantly higher (P<0.05). We found therapy with cyclophosphamide to be promising in patients with focal segmental glomerulosclerosis. [ABSTRACT FROM AUTHOR]

Nephrotic syndrome (NS) is a clinical entity characterized by massive loss of urinary protein leading to hypoproteinemia and edema. This prospective cross sectional study was performed on 44 children with idiopathic nephrotic syndrome (INS). The objectives were to study the clinical and biochemical parameters at the time of diagnosis of nephrotic syndrome and to study the histopathological distribution of different subtypes of INS and drug response pattern. There were 29 (66%) males and 15 females (34%). The mean age of NS was 4.87±3.24 years. Facial edema was found in 42 (95%), microscopic hematuria in 10 (23%), gross hematuria in 2 (4.5%), and hypertension in 5 (11.2%) of patients. In 17 children who underwent biopsy, focal segmental glomerulosclerosis was the most common pathologic finding (41%). Other subtypes included minimal change in three (18%), membranoproliferative glomerulonephritis in 1(5.8%), diffuse proliferative glomerulonephritis in 2 (11.6%), membranous glomerulonephritis in 1 (5.8%), and diffuse mesangial proliferation in 3 (17.5%) of cases. At the time of hospital admission, peritonitis were present in five (11.4%), pneumonia and upper respiratory infection (sinusitis) in eight (18%), cellulitis in two (4.5%). Among 44 children with NS, 29 (66%) were steroid sensitive cases, nine (20.5%) were steroid resistant and six (13.5%) were steroid dependent. Among patients with steroid sensitive NS, 37% were without relapsers, 38.8% frequent relapsers and 26.4% were infrequent relapsers. These results suggest that there are differences between season of incidence, response to treatment with corticosteroid and pathologic findings in our study and other studies in Iran and other countries. [ABSTRACT FROM AUTHOR]

Introduction. Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. Materials and Methods. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 ± 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. Results. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively (P = .03). Conclusions. We showed that patients with late steroid resistance had better response to immunosuprressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD. [ABSTRACT FROM AUTHOR]

Nephrotic syndrome (NS) is a clinical entity characterized by massive loss of urinary protein (primarily albuminuria) leading to hypoproteinemia (hypoalbuminemia) and its result, edema. Hyperlipidemia, hypercholesterolemia, and increased lipiduria are usually associated. Although not commonly thought of as part of the syndrome, hypertension, hematuria, and azotemia may be present. This prospective cross-sectional study was performed on 44 children (with age of onset up to 14 years) with idiopathic nephrotic syndrome (INS) in our center during 2000-2007. The objectives were to study the clinical and biochemical parameters and the histopathological distribution of different subtypes of INS and drug-response pattern in these patients.The study group included 44 children. There were 29 (66%) boys and 15 girls (34%). The mean age was 4.87 ± 3.24 years. Facial edema was found in 42 (95%), microscopic hematuria in 10 (23%), gross hematuria in 2 (4.5%), and hypertension in 5 (11.2%) patients. In children who underwent biopsy, focal segmental glomerulosclerosis was the most common pathologic finding (41%). Other subtypes included minimal change disease in three (18%), membranoproliferative glomerulonephritis in one (5.8%), diffuse proliferative glomerulonephritis in two (11.6 %), membranous glomerulonephritis in one (5.8%), and diffuse mesangial proliferation in three (17.5%) cases. At the time of hospitalization peritonitis was present in five (11.4%), pneumonia and upper respiratory infection (sinusitis) in eight (18%), and cellulites in two (4.5%) patients. Twenty nine patients (66%) were steroid sensitive, 9 (20.5%) steroid resistant, and 6 (13.5%) steroid dependent. Among patients with steroid-sensitive NS, 37% were nonrelapsers, 38.8% frequent relapsers, and 26.4% were infrequent relapsers. Differences seems to exist between season of incidence, suitable response to treatment with corticosteroids, and pathologic findings of biopsy in our study and other studies from Iran and other countries. [ABSTRACT FROM AUTHOR]

Background: There are controversy results in the optimal management of children with steroid-dependent and steroid-resistant nephrotic syndrome (SDNS, SRNS). This study aimed to determine the efficacy and safety of rituximab (RTX) in these pediatric patients., Methods: Medical records of 1-18-year-old Iranian children with SDNS (n = 26) and SRNS (n = 22) with a follow-up for at least 24 months were included from 2009 to 2019. The short- and long-term responses to RTX were respectively evaluated to determine the random protein-to-creatinine ratio after 6 and 24 months and classified as complete (CR) and partial (PR) remission or no response., Results: Male patients (n = 26) were slightly predominate. The median age of patients at the time of RTX therapy was 8.6 ± 4.01 years. At the end of the 6-month follow-up, CR and PR occurred in 23 (47.9%) and 12 (25%) patients, respectively. Of 23 patients with CR, 18 (69.2%) and 5(22.7%) had SDNS and SRNS, respectively (p < 0.005). However, only 18 (37.5%) of patients after 24 months had been in CR. No significant difference in the CR rate was found between the two groups. RTX was more effective when administered during the proteinuria-free period (p = 0.001)., Conclusion: In the short term, RTX significantly was efficient in inducing complete or PR in SDNS and SRNS patients. However, the favorable response rate in a long-term follow-up was insignificantly lower between the two groups., (© 2022. The Author(s).)

Steroid-resistant nephrotic syndrome (SRNS) is a clinical challenge with variable clinical outcomes. In patients with SRNS, unsuccessful anti-inflammatory and anti-proteinuric effects of steroids lead to end-stage renal disease (ESRD). Our objective was to define the expression pattern of the glucocorticoid receptors (GR) α and β and their epigenetic regulators (miR-24, miR-30a, and miR-370) in a group of adults with SRNS. In this regard, sixty primary NS patients with focal segmental glomerulosclerosis (FSGS, N = 30) and membranous glomerulonephritis (MGN, N = 30) and also healthy volunteers (N = 24) were enrolled. Real-time PCR was performed to evaluate the expression levels of the aforementioned genes in peripheral blood mononuclear cell (PBMC) samples. Furthermore, an in-silico analysis was performed to understand the signaling pathways and biological procedures that may be targeted by these microRNAs in NS. The decreased and increased levels of GRα and GRβ were not significant, respectively. Statistically significant reduced miR-24 levels were observed between control/MGN (p = .022) and MGN/FSGS (p = .032) groups. Additionally, a decrease was detected in miR-30a between MGN and FSGS (p = .049) groups. There was a significant increase in miR-370 expression level between control and NS groups (p = .029), as well as control/MGN (p = .008), and MGN/FSGS (p = .046). Bioinformatics analysis predicted the possible targets of the studied genes including genes involved in TGF-β, Notch1, and p53 signaling pathways, regulation of gene expression, intracellular signal transduction, negative regulation of response to the stimulus, cell-cell signaling, and cell activation in the pathogenesis of SRNS. Taken all together, dysregulated levels of GRα, GRβ were not attributed to SRNS in our patients. It seems that pharmacokinetics and the genetic variations in podocyte-related genes may be associated with the steroid-resistance in our adult patients with NS rather than GR expression., (© 2020 International Union of Biochemistry and Molecular Biology.)

Introduction: Focal segmental glomerulosclerosis (FSGS) accounts for 20% of nephrotic syndromes among children as well as 75% of the steroid resistant nephrotic syndrome (SRNS). The aim of the present study was to evaluate the influence of parental consanguinity on clinical course and outcome of FSGS in children., Methods: This historical cohort was carried out on 69 children affected by steroid resistant FSGS. Patients' data were recorded at the initial and the final analyses and response to therapeutic measures. Subjects were also questioned about the history of parental consanguinity., Results: Forty-four participants (63.8%) were male with a male to female proportion of 1.76:1. Mean baseline age was 5.69 ± 2.39 (range: 1 to 10). Fifty-one patients (73.9%) reported consanguinity. A more significant resistance to cyclosporine A and cyclophosphamide was observed in participants denoting parental consanguinity than those with no kinship. The average renal survival time obtained significantly lower among those reporting consanguinity compared to the others (8.33 vs. 10.44 years, P < .05). According to univariate analysis results, parental consanguinity was a risk factor for developing chronic kidney disease (HR = 4.56, 95% CI: 1.06 to 19.47; P < .05)., Conclusion: Patients with FSGS plus parental consanguinity presented less renal survival time with more resistance to cures being more predisposed to the development of CKD.

Objective: This study aims at determining the relationship between prednisolone cumulative dose and linear growth in pre-pubertal children with idiopathic nephrotic syndrome., Method: This cross-sectional study was conducted on all children with idiopathic nephrotic syndrome registered to the pediatric nephrology department at the main referral children's hospital in Southwestern Iran. Inclusion criteria included age (males <12 years; females <10 years), >6 months of use, and the minimum prednisolone cumulative dose of 152mg/kg. The exclusion criteria were individuals who had entered puberty or had other diseases affecting linear growth. Based on the prednisolone cumulative dose of ≥550mg/kg (four or more relapses), the children were divided into two groups. All data regarding age, height, and weight at disease onset and the last visit, bone age, and the parents' height were collected. Secondary variables including mid-parental target height and predicted adult height were also calculated. Height data were compared between the different rates of relapse., Results: A total of 97 children (68% male) were enrolled. Their post-treatment mean height Z-score was less than that obtained before treatment (-0.584 vs. -0.158; p=0.001). Subjects with higher prednisolone cumulative doses were found to have more reduction in height Z-score (p=0.001). Post-treatment height prediction also showed less growth potential compared to pre-treatment target height (p=0.006). Thirty-three children (34.4%) had four or more relapses, among whom more mean-height Z-score decreases were found compared to those with less-frequent relapses (-0.84 vs. -0.28; p=0.04)., Conclusion: This study showed the negative effect of cumulative dosages of prednisolone on linear growth, which was greater in children with four or more relapses., (Copyright © 2018. Published by Elsevier Editora Ltda.)

The author discusses aspects of glomerulopathies that cause end-stage renal diseases in younger populations in Iran which call for different treatment options to prevent outcomes. He cites studies that compare steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome related to gene mutations in idiopathic nephrotic syndrome. The author believes that histologic features and clinical findings do not differentiate genetic mutations which may require more studies.

Of 231 children with nephrotic syndrome, 87% were steroid sensitive and 13% steroid resistant. Of patients with steroid sensitive nephrotic syndrome, 38.8% were non-relapsers, 34.8% frequent relapsers and 26.4% infrequent relapsers. Among those with steroid resistant nephrotic syndrome, 37.5% had focal segmental glomerulosclerosis with a high mortality rate of 44%. [ABSTRACT FROM AUTHOR]