Clinical presentation and management challenges of sphingosine-1-phosphate lyase insufficiency syndrome associated with an SGPL1 variant: a case report.

Background: This case report describes a unique presentation of sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) caused by a rare SGPL1 variant, highlighting the diagnostic and management challenges associated with this condition.

Case Presentation: A 2-year-old Iranian female presented with steroid-resistant nephrotic syndrome (NS), primary adrenal insufficiency (AI), growth delay, seizures, and hyperpigmentation. Laboratory evaluation revealed hypoalbuminemia, significant proteinuria, hyperkalemia, and elevated adrenocorticotropic hormone (ACTH) levels.

From designing minimum data set to developing kidney transplantation registry in Iran.

Objective: Currently, population growth and increasing life expectancy are becoming one of the biggest public health challenges in the world, which has increased the prevalence of chronic diseases such as end-stage renal disease and the need for kidney transplantation. The use of a variety of registries has the potential to determine the effectiveness of clinical care and costs and improve the quality of patient care. The aim of this study is to design minimum data set to develop a kidney transplantation registry in Iran to improve the quality of care for people with end-stage renal disease.

Etiology and Antimicrobial Susceptibility Pattern of Urinary Tract Infection in the First Year after Pediatric Renal Transplantation: A Preliminary Study.

Urinary tract infection (UTI) is common after pediatric renal transplantation, and the emergence of multidrug-resistant (MDR) bacteria causing UTI is a therapeutic challenge in this regard. The main purpose of this study was to determine the UTI frequency, its etiologic agents, and the antibiotic susceptibility pattern in the first year following renal transplantation in Iranian pediatric recipients. In a retrospective cohort study, all of the 81 children who had undergone renal transplantation in Hazrat Rasoul Akram Hospital between 2012 and 2017 were enrolled.

Early Postoperative Kidney Transplant Complications Related to Immunomodulator Regimen in Pediatric Recipients.

Objectives: Calcineurin inhibitors (cyclosporine and tacrolimus) are widely used in kidney transplant to prevent acute transplantrejection; however,the effects of these medications on graft sequelae after transplant remain unclear. We aimed to compare early complications, including graftrejectionandinfectionrates after kidney transplant, in childrenbetween the cyclosporine and tacrolimus immunomodulator regimens.

Materials And Methods: In this prospective cohort study, 105 pediatric patients who were candidates to receive kidney transplant in the age range of 4 to 18 years were included.

Long-Term Outcome of Kidney Transplant Among Iranian Children: A Systematic Review and Meta-Analysis.

Objectives: End-stage kidney disease has dramatic health effects and life consequences in children. Presently, kidney transplant has been globally accepted as a treatment of choice for end-stage kidney disease in both children and adults, leading to better quality of life and longer patient survival. Because of lack of comprehensive information on the outcome of kidney transplant among children in Iran, we aimed to present a proper vision of pediatric kidney transplant in Iran by systematically reviewing the current literature.

Evaluation of Serum Level of Carnitine in Children with Acute Pyelonephritis (APN) Compared to Healthy Children.

Aim: This cross-sectional case-control study evaluated the serum carnitine level in children with urinary tract infection (UTI).

Background: Acute pyelonephritis (APN) is a common bacterial infection of the upper urinary tract in children which may also lead to renal damage and tubular atrophy. Activation of inflammatory mediator bedside alterations in the cytokines and generation of reactive oxygen species (ROS) play a striking role in the development of tissue damage after pyelonephritis.

Efficacy and Safety of Rituximab in Children With Steroid- and Cyclosporine-resistant and Steroid- and Cyclosporine-dependent Nephrotic Syndrome.

Introduction: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome.

Materials And Methods: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks.

Comparison of tuberculin skin test and interferon gamma release assay for diagnosis of latent tuberculosis infection in pediatric candidates of renal transplantation.

IGRA has been approved as an alternative in vitro test to diagnose Mycobacterium TB infection. This study aimed to assess the diagnostic value of TST in comparison with QFT assay to detect LTBI among Iranian children candidate for renal transplantation. This cross-sectional study was performed on 31 children who were candidate for renal transplantation admitted to Ali Asghar Children's Hospital and Rasoul Akram Hospital, Tehran, Iran, from 2013 to 2014.

Comparison the Serum STREM1 Levels Between Children with Upper and Lower UTI.

Background: Pyelonephritis is the most common and important infection among Iranian pediatric population. Differentiation between upper and lower Urinary Tract Infection (UTI) is often difficult based on clinical data. Therefore, definite diagnosis is helpful for choosing appropriate antibiotic and decision for hospital admission.

Configuring a Better Estimation of Kidney Size in Obese Children and Adolescents.

Background: Obesity ignites numerous health and psychosocial problems and is associated with various comorbidities. Body mass index (BMI) is also independently associated with improved risk for numerous kidney disorders. As renal length is considered a vital parameter in the clinical assessment of renal patients, normal renal length has to be defined in accordance to BMI.

Serum procalcitonin level for prediction of high-grade vesicoureteral reflux in urinary tract infection.

Introduction: Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis.

Pretransplant Epstein-Barr virus serostatus and incidence of posttransplant lymphoproliferative disorder in pediatric renal transplants.

Objectives: Pediatric renal transplant recipients are at increased risk of Epstein-Barr virus infection which may be due to the high percentage of Epstein-Barr virus seronegative recipients at the time of transplant in the pediatric age group. We aimed to assess the Epstein-Barr virus serostatus of recipients and donors and the incidence of posttransplant lymphoproliferative disorder in pediatric renal transplant at the Labafinejad Hospital in Tehran, Iran.

Materials And Methods: We reviewed the clinical records of 183 children who had a renal transplant at the Labafinejad Hospital in Tehran, Iran, between 2003 and 2011.

Rituximab in the treatment of nephrotic syndrome: a systematic review.

Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some researchers. It has also been shown that the anti-CD20 antibody, rituximab, may be an option in the treatment of these patients. In this systematic review, we performed extensive search and identified studies on rituximab use in children with nephrotic syndrome.

Treatment update of sensitized pediatric kidney transplant recipients: a review.

Sensitization of recipients is an increasing problem in children. Some case series in children exist comparing the diverse desensitizing protocols. These protocols include intravenous immunoglobulin, cytomegalovirus immune globulin, plasmapheresis, and some adjunctive therapies such as rituximab.

Are serum and urine neutrophil gelatinase-associated lipocalin predictive of renal graft function in short term?

NGAL is a member of the lipocalin protein family that has diverse function but similar structure. The functions of NGAL are not clear, but it appears to be expressed in stress conditions and in tissues undergoing involution. Varied studies have shown increased levels of plasma or urinary NGAL in diverse renal damages.

Glomerular function in neonates.

Glomerular filtration rate is low in fetal and neonatal life. It increases after birth and reaches approximately 20 mL/min/1.73 m2 at 1 month of age in term and preterm neonates.

Prevalence and outcome of focal segmental glomerulosclerosis in Iranian children with nephrotic syndrome.

Introduction: Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children.

Outcome of renal transplantation in children: a multi-center national report from Iran.

The outcome of pediatric renal transplantation was previously reported by a single-center study at the year 2006. Therefore, we aimed to evaluate and report the characteristics and outcome of renal pediatric renal transplantation in a multi-center nationwide study. In this nationwide report, medical records of 907 children (≤18yr) with renal transplantation in eight major pediatric transplant centers of Iran were recorded.

Effect of levamisole in steroid-dependent nephrotic syndrome.

INTRODUCTION. Childhood idiopathic nephrotic syndrome is characterized by frequent relapsing courses or steroid dependency. Levamisole is a popular drug for treatment of these patients.

Enalapril induced reversible acute renal failure detected by (99m)Tc-DMSA renal scan in a patient with bilateral renal artery stenosis: a case report.

The authors report and discuss a case of bilateral renal artery stenosis in a hypertensive 9 year-old girl that was first suspected with (99m)Technetium-dimercaptosuccinic acid renal scintigraphy. The scintigraphy showed signs of acute renal failure while the patient was on enalapril for controlling her hypertension. Bilateral renal artery stenosis was confirmed with computed tomography angiography.

Posterior urethral valve in a child presenting as recurrent epididymo-orchitis.

Epididymo-orchitis is an uncommon complication of posterior urethral valve. A four-year-old boy was admitted because of right-sided epididymo-orchitis. Scintigraphy of the scrotum showed increased uptake of radiotracer on the right side.

Pulmonary hypertension and predisposing factors in patients receiving hemodialysis.

Introduction: The aim of this study was to evaluate the frequency of unexplained pulmonary hypertension (PHT) among patients on hemodialysis at 2 centers and to evaluate possible predisposing factors.

Materials And Methods: In this cross-sectional study, PHT was screened by Doppler echocardiography on the day after dialysis in 62 patients with end-stage renal disease receiving maintenance hemodialysis via arteriovenous access. Pulmonary hypertension was defined as a systolic pulmonary arterial pressure (PAP) higher than 35 mm Hg, and the systolic PAP was calculated using the modified Bernoulli equation.