Genetic analysis of nephrogenic diabetes insipidus patients: A study on the Iranian population.

INTRODUCTION: Nephrogenic diabetes insipidus (NDI) is a rare genetic disease that causes water imbalance. The kidneys play a crucial role in regulating body fluids by controlling water balance through urine excretion. This highlights their essential function in managing the body's water levels, but individuals with NDI may have excess urine production (polyuria), that leads to excessive thirst (polydipsia). Untreated affected individuals may exhibit poor feeding and failure to thrive. This disease is caused by mutations in the AVPR2 and the AQP2 genes which have the X-linked and autosomal recessive/dominant inheritance, respectively. Both of these genes are expressed in the kidney. METHODS: Twelve Iranian patients from 10 consanguineous families were studied in this project. DNA was extracted from the whole blood samples of the patients and their parents. All coding exons and exon-intron boundaries of the AVPR2 and AQP2 genes were sequenced in the affected individuals, and the identified variants were investigated in the parents. All variants were analyzed according to the ACMG (American College of Medical Genetics and Genomics) guidelines. RESULTS: In this study, 6 different mutations were identified in the patients, including 5 in the AQP2 gene (c.439G>A, c.538G>A, c.140C>T, c.450T>A, and the novel c.668T>C) and 1 in the AVPR2 gene (c.337C>T) in the present study. DISCUSSION: As expected, all the detected mutations in this study were missense. According to the ACMG guideline, the identified mutations were categorized as pathogenic or likely pathogenic. Unlike previous studies which showed more than 90% of mutations were in the AVPR2 gene, and only less than 10% of the mutations were in the AQP2 gene, it was found that more than 90% of our identified mutations located in the AQP2 gene, and only one mutation was observed in the AVPR2 gene, which seems it may be a result of the high rate of consanguineous marriages in the Iranian population. We observed genotype-phenotype correlation in some of our affected individuals, and some of the mutations were observed in unrelated families from same ethnicity which could be suggestive of a founder mutation.

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

Background: 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. Methods: 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. Confirmed episodes of UTI during the first year following renal transplantation were analyzed. The pattern of antibiotic resistance was determined for the causative agents of UTI. The data were analyzed using the IBM SPSS Statistics software (version 20). and the P < 0.05 was considered significant. Results: Totally, from 81 enrolled cases, 37(44.7%) cases were in the age group of 11-15 years. Overall, 19, 10, and 3 UTI episodes had occurred in the first month, from the first to sixth month, and between the sixth month and one year after transplantation, respectively. The four most common isolated bacteria were Escherichia coli (E. coli; 31.2%), Pseudomonas aeruginosa (P. aeruginosa; 25%), Enterococci (21.9%) and Klebsiella pneumoniae (K. pneumoniae; 12.5%). The highest rate of resistance was reported to trimethoprim/sulfamethoxazole (TMP/SMX), cephalosporins, and fluoroquinolones among gram-negative bacteria. However, none of the Enterococci isolates were resistant to linezolid and nitrofurantoin. Conclusion: Resistance to antibiotics is increasing among the pathogens causing UTI in pediatric renal transplanted cases. It is suggested to stop the administration of TMP/SMX and third-generation cephalosporins for empiric treatment of UTI in Iranian pediatric renal transplant recipients. Ciprofloxacin might be administered cautiously secondary to the increasing rate of antibiotic resistance in this group.

Genetic diagnosis of Bartter syndrome in Iranian patients and detection of a novel homozygous CLCNKB mutation.

An Iranian girl with clinical symptoms of Bartter syndrome like hypokalemia, polyuria, polydipsia, hyponatremia, and hypochloremic alkalosis was referred to us in whom the CLCNKB gene was genetically evaluated using Sanger sequencing. A homozygous pathogenic variant of c.1332_1335delCTCT was detected in this patient.

New Report of a Different Clinical Presentation of CD151 Splicing Mutation (c.351+2T>C): Could TSPAN11 be Considered as a Potential Modifier Gene for CD151?

CD151, a member of the tetraspanin family, is essential for normal development of skin and kidney. To date, only 2 pathogenic variants of the CD151 gene have been identified in a related disorder with recessive inheritance. Here, in the third study of CD151 mutations, we report 3 affected siblings presenting variable degrees of renal and dermal symptoms. Whole exome sequencing (WES) was performed on the proband, followed by data analysis and in silico assessments. Confirmation of the mutation in the other patients were carried out using Sanger sequencing. The consequence of the CD151 mutation was investigated by RNA extraction and Sanger sequencing of PCR products from cDNA. Multiple computational tools were applied for protein alignment, homology modeling, and molecular interaction analysis. WES revealed the variant c.351+2T>C, NM_139029 (GRCh37) in CD151, and this was confirmed by Sanger sequencing in all patients. This variant is the result of a substitution of nucleotide T with C that changes the position +2 of the donor splice site in intron 5, leading to total loss of exon 5 from the transcript. The mentioned variant was not found in population allele frequency databases, and prediction tools concurred in its damaging effect on the protein. Based on the criteria from ACMG guidelines, this variant is pathogenic. Interestingly, in terms of clinical findings, symptoms and severity of the disease in the patients in this study were different compared to the previous report of the mutation and the disease. In addition, in silico analysis in this study appears to suggest a candidate protein, Tetraspanin-11 (TSPAN11), that could partially modify CD151 functions. This study supports the pathogenic effect of the CD151 variant c.351+2T>C, highlights the extensive variable expressivity amongst patients, reinforces the contribution of genomic content to clinical characteristics of CD151 mutations, and accentuates the importance of modifier genes.

Estrogen replacement therapy: effects of starting age on final height of girls with chronic kidney disease and short stature.

INTRODUCTION: We investigated the age of starting Estrogen replacement therapy as a key parameter for reaching near normal Final Height (FH) in Chronic Kidney Disease (CKD) girls with growth retardation. METHOD: This open label, quasi-experimental designed and matched controlled clinical trial was performed on CKD girls with short stature and later onset of puberty or delayed puberty according to clinical and laboratory investigations. Participants of group 1 and 2 had been treated with Growth Hormone (GH), and Ethinyl Estradiol (EE). EE was administered from 11 and 13 yrs. old in groups 1 and 2 respectively. Group 3 was selected from patients that did not accept to start GH or EE till 15 years old. The effect of the age of starting EE on FH, GH therapy outcomes, bone density, and calcium profile were evaluated. RESULT: Overall, 16, 22, and 21 patients were analyzed in groups 1, 2, and 3 respectively. Mean Mid-Parental Height (MPH) had no significant difference between the 3 groups. GH therapy significantly enhanced mean FH in groups 1 and 2 in comparison with group 3 (ß = - 4.29, p < 0.001). Also, multivariable backward linear regression illustrated significant negative association between FH and age of starting EE (ß = 0.26, p < 0.001). Mean Para Thyroid Hormone (PTH), mean femoral and lumbar bone density were significantly enhanced after GH and EE therapy (p value: < 0.001). CONCLUSION: We recommend starting EE from 11 yrs. old in CKD short stature girls who have no clinical and laboratory sign of sexual maturity at 11 yrs. to enhance the cost effectiveness of GH therapy.

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. MATERIALS AND METHODS: Major databases were searched, including Medline, Web of Knowledge, Google Scholar, Scopus, Cochrane, and the Iranian Scientific Information Database for all eligible studies in accordance with specific keywords. The inclusion criteria forthe retrieved studies were determination of graft survival, patient survival, and reasons for graft failure. The exclusion criteria were as follows: (1) a lack of clearresults; (2) non-English or non-Persian language format; (3) lack of access to the full-text manuscripts; and (4) case reports, case series, and review papers. A total of 115 studies were initially assessed based on the keywords; of these, 8 met inclusion criteria and were considered for final analysis; these were published between 2005 and 2017. RESULTS: According to our results, 1-year graft survival rates were overall 89.7%, and 5-year graft survival rates were 65.4%. The 1-year patient survival rates were estimated to be 97.1%, and 5-year patient survival rates were estimated to be 89.8%. Acute rejection, dialysis status before transplant, and inappropriate immunosuppression were the main risk factors. CONCLUSIONS: Our systematic review and meta-analysis indicated a high success rate of childhood kidney transplant in Iran according to long-term graft and patient survival rates.

Frequency of neurological involvement in patients with/without diarrhea hemolytic uremic syndrome: A Systematic review and meta-analysis.

Background: Diarrhea-associated-hemolytic-uremic-syndrome (D+HUS) is a common from of HUS. Central-nervous-system (CNS) involvement is one of the most common extrarenal organ involvements in children with D+HUS. This systematic review and meta-analysis aim to recognize the frequency of neurological complications in pts with HUS. Methods: Databases of PubMed, Embase, and Web of Science were searched systematically to find the papers on neurological involvement in HUS pts. Two researchers independently assessed the papers' quality and extracted data. CMA v. 2.2.064. was used for data analysis. Heterogeneity was evaluated using the I-squared (I2) test, and a fixed/random-effects model was used when appropriate. Results: In this review, 21 studies including 2,189 participants with a median age between 1.3-40-year-old, entered the meta-analysis. The meta-analysis in D+HUS patients indicated 27.0% with neurological complications (95% CI, 22.0%-32.6%), 25.5% of symptoms weren't categorized (95% CI, 15.9%-38.3%), 20.8% of them developed the seizures (95% CI, 2.3%-74.4%). In D-HUS pts, 20.8% of them were presented neurological symptoms (95% CI, 17.9%-24.0%), of which 29.0% weren't categorized (95% CI, 19.2%-41.2%), 17.5% of pts got into coma (95% CI, 9.6%-29.7%), 5.6 % showed hemiparesis (95% CI, 2.8%-10.9%), 17.2% experienced lethargy (95% CI, 5.2%-44.1%), 30.5% developed the seizures (95% CI, 18.2%-46.2%), 7.4% manifested speech abnormalities (95% CI, 0.2%-7.22%), 6.4% of D-HUS pts presented visual-disturbances (95% CI, 3.4%-11.6%). Conclusion: This systematic review and meta-analysis indicated more than one-fourth of both D+HUS and D-HUS patients were presented with neurological symptoms, and the most prevalent symptoms were seizures, which can lead to an epilepsy sequel.

Chronic Kidney Disease in Iran: First Report of the National Registry in Children and Adolescences.

PURPOSE: Knowing the epidemiological aspects of chronic kidney disease (CKD) in children is crucial for early recognition, identification of reversible causes, and prognosis. Here, we report the epidemiological characteristics of childhood CKD in Iran. MATERIALS AND METHODS: This cross-sectional study was conducted during 1991 - 2009. The data were collected using the information in the Iranian Pediatric Registry of Chronic Kidney Disease (IPRCKD) core dataset. RESULTS: A total of 1247 children were registered. The mean age of the children at registration was 0.69 ± 4.72 years (range, 0.25 -18 years), 7.79 ± 3.18 years for hemodialysis (HD), 4.24 ± 1.86 years for continuous ambulatory peritoneal dialysis (CAPD), and 3.4±1.95 years for the children who underwent the renal transplantation (RT) (P < .001). The mean year of follow-up was 7.19 ± 4.65 years. The mean annual incidence of CKD 2-5 stages was 3.34 per million age-related population (pmarp). The mean prevalence of CKD 2-5 stages was 21.95 (pmarp). The cumulative 1-, 5-, and 10-year patients' survival rates were 98.3%, 90.7%, and 84.8%, respectively. The etiology of the CKD included the congenital anomalies of the kidney and urinary tract (CAKUT) (40.01%), glomerulopathy (19.00%), unknown cause (18.28%), and cystic/hereditary/congenital disease (11.14%). CONCLUSION: The incidence and prevalence rate of pediatric CKD in Iran is relatively lower than those reported in Europe and other similar studies. CAKUT was the main cause of the CKD. Appropriate management of CAKUT including early urological intervention is required to preserve the renal function. Herein, the long-term survival rate was higher among the children with CKD than the literature.

Whole exome sequencing revealed a novel homozygous variant in the DGKE catalytic domain: a case report of familial hemolytic uremic syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia caused by small vessel thrombosis, thrombocytopenia, and renal failure. The common cause of aHUS is a dysregulation in the alternative complement pathway. Mutations in none complement genes such as diacylglycerol kinase epsilon (DGKE) can also result in this syndrome. CASE PRESENTATION: Here, we report on a 19-year-old female with the clinical diagnosis of aHUS, who has unaffected consanguineous parents and an older sibling who was deceased from aHUS when she was seven months old. We performed whole exome sequencing (WES) followed by evaluation of detected variants for functional significance, using several online prediction tools. Next, in order to confirm the detected pathogenic variant in proband and segregation analysis in her family, Sanger sequencing was done. The novel variant was analyzed in terms of its impact on the protein 3-dimensional structure by computational structural modeling. The results revealed that the proband carried a novel homozygous missense variant in DGKE located in exon 6 of the gene (NM_003647.3, c.942C > G [p.Asn314Lys]), and in silico analysis anticipated it as damaging. Protein computational study confirmed the influence of potential pathogenic variant on structural stability and protein function. CONCLUSION: We suggest that some variations in the catalytic domain of DGKE like p.Asn314Lys which can cause alterations in secondary and 3-D structure of protein, might lead to aHUS.

Changes in body mass index after pediatric renal transplantation.

Significant weight gain following renal transplantation is common in adult and pediatric recipients and mostly depends on receiving higher doses of steroids, changes in mood and feelings, as well as their level of physical activities. This study was performed to evaluate body weight and body mass index (BMI) before and after kidney transplantation in children and adolescents. In this cross-sectional study, 71 pediatric renal transplant recipients (42 boys and 29 girls) were included. World Health Organization criteria were used for comparing Z-score BMI for age in our cases. Overweight was defined as Z-score BMI >+1 SD (standard deviation) and obesity as >+2 SD. At the time of transplantation, the mean age was 10.8 ± 3 years (5-16 years) and based on BMIZ-score, the patients were found to be thin (BMIZs <-2 SD) in 16.9%, normal (BMIZs = -2 to +1 SD) in 67.6%, overweight (>+1 SD to +2 SD) in 9.9%, and obese (BMIZs >+2 SD) in 5.6%.The mean follow-up duration after transplantation was 3.57 ± 1.68 years (1-7 years) and at the time of reevaluation after transplant, their mean age was 14.4 years (6-18 years). The mean BMI was 22 ± 5.3 kg/m2, and for BMI grouping, the patients were thin in 7%, normal in 54.9%, overweight in 21.1%, and obese in 17%. Pretransplant thinness (BMIZs <-2 SD) was found in 12 patients (16.9%), equally in boys and girls, and in most of them (83.3%), BMIZs changed to normal or even >+1 SD after transplant. Chronic continuous decrease of glomerular filtration rate (CCD/GFR) was found in 27 cases (38%); 74.1% were male (P = 0.045), hypertriglyceridemia was found in 74.1% (P = 0.023%), hypercholesterolemia in 63% (P = 0.032),and obesity in 18.5% (p = 0.5). The incidence of obesity has tripled after kidney transplantation. It was not a risk factor for graft or patient survival in our experience, whereas pretransplant obesity had some effects on long-term graft outcome.

Chronic Neurological Complications in Hemolytic Uremic Syndrome in Children.

INTRODUCTION: Central nervous system (CNS) involvement is the most common extrarenal involvement in hemolytic uremic syndrome (HUS). There are limited reports on clinical cause of chronic neurologic problems in HUS. We evaluated residual neurologic involvement in children with HUS. MATERIALS AND METHODS: This cross-sectional study was conducted on 58 patients with a diagnosis of HUS referred to 2 tertiary pediatric centers. Neurological examinations was performed on all of the patients and they were followed up between 2001 and 2015. Data including demographic variables, type of HUS, neurological symptoms, and other complications were recorded. Neurological involvements that occurred after 6 months from the acute phase of HUS were considered as chronic neurological involvement. RESULTS:  Among 58 patients who were included in the study, 31 (53.4%) had neurological manifestations (31 with acute and 19 with chronic complications). There was no significant difference in acute neurological manifestations between typical and atypical HUS, while chronic neurological manifestations were more frequents in patients with atypical HUS (P = .05). The most common presentations were seizure and decreased level of consciousness. Chronic neurologic problems were found in follow-up visits of 11 patients with acute and 8 without acute involvement. Hypertension was associated with chronic manifestations (P = .01). CONCLUSIONS: According to our results, residual neurological problems were not infrequent in HUS and they were more related with atypical form of disease. Evidence of hypertension is a significant variable for persistence of neurologic problems.

The Value of Urinary Cystatin C Level to Predict Neonatal Kidney Injury.

BACKGROUND: The role of urinary cystatin C to early predict acute kidney injury (AKI) in children and neonates remains uncertain. The present study aimed to assess and compare the level of urinary cystatin C in neonates with and those without AKI. METHODS: This cross-sectional study was performed on 55 available neonates who were involved by AKI and admitted to the neonatal department at Ali-Asghar hospital in Tehran in 2016. 97 neonates with jaundice and normal serum creatinine level were randomly selected as the control group. In both groups and on admission, the urine levels of cystatin C and creatinine were measured. RESULTS: The average urinary level of cystatin C was 162.87 ± 56.50 mmol/mole creatinine in the group with AKI and 68.06 ± 57.16 mmol/mole creatinine in the control group that was significantly higher in former group (p < 0.001). The measurement of cystatin C level in urine could predict kidney injury with a sensitivity of 98.2%, a specificity of 39.2%, a positive predictive value of 47.8%, a negative predictive value of 97.4%, and an accuracy of 60.5%. Assessment of the area under the receiver operating characteristic (ROC) analysis showed that measuring urinary cystatin C level could effectively discriminate kidney injury from normal kidney condition in neonates (AUC = 0.868, 95CI: 0.811 - 0.925, P < 0.001). The best cutoff value of urinary cystatin C level to predict kidney injury was shown to be 41.5 mmol/mole creatinine yielding a sensitivity of 98.2% and a specificity of 46.4%. CONCLUSION: Measurement of cystatin C in urine is an early sensitive method to diagnose neonatal kidney injury.

Serum Beta-Trace Protein for Assessment of Kidney Function in Neonates.

INTRODUCTION: Compared to the conventional methods, serum beta-trace protein (BTP) has been shown to be more helpful for estimating glomerular filtration rate; however, its value is remained unclear in neonates. The present study aimed to investigate the range of serum BTP level in healthy term neonates and its value to estimate glomerular filtration rate. MATERIALS AND METHODS: This cross-sectional study was conducted on 50 healthy term neonates without underlying cardiovascular or kidney disorders who were admitted to Ali Asghar hospital in 2013. Serum BTP was measured using an automated nephelometric immunoassay. Glomerular filtration rate was assessed using the Schwartz equation based on serum creatinine level. RESULTS: The mean age of the neonates was 6.2 ± 3.6 days (range, 2 to 17 days), their mean gestational age was 38.02 ± 0.20 weeks, and their mean height was 49.8 ± 1.7 cm. The mean serum BTP level was 0.41 ± 0.11 mg/L (range, 0.19 mg/L to 0.92 mg/L). The mean serum creatinine level was 0.49 ± 0.16 mg/dL (range, 0.3 mg/dL to 1.0 mg/dL). The mean estimated GFR was 48.90 ± 15.88 mL/min. A positive correlation was observed between the reciprocal concentrations of BTP and GFR (r = 0.383, P = .006). Furthermore, the reciprocal concentrations of BTP was associated with the reciprocal concentrations of serum creatinine level (r = 0.365, P = .009). CONCLUSIONS: Measurement of serum BTP can be a reliable tool for detecting kidney function in neonates. Further studies are warranted to design a suitable formula for GFR estimation based on serum BTP in neonates.

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. The children were followed up for 2 years. Effectiveness was defined as remission of proteinuria in response to rituximab. Side effects of rituximab were monitored. RESULTS: Overall, 23 (57.1%) of the children had steroid- and cyclosporine-resistant nephrotic syndrome, of whom 8 (34.8%) revealed complete response and 3 (13%) revealed partial response. Seven children (16.7%) had late-resistant nephrotic syndrome, of whom 6 (85.7%) revealed complete response and none revealed partial response. Ten children (26.2%) had steroid- and cyclosporine-dependence all of whom revealed complete response to rituximab. Complete response rate was significantly higher in those with drug-dependent pattern than the other groups (P = .002). There was no association between response to rituximab and pathological basis of disease. Side effects were found in 4 patients as leukopenia in 2, alopecia in 1, and eosinophilia in 1. CONCLUSIONS: Rituximab is effective for children with nephrotic syndrome with high efficacy and well tolerability, especially in those with steroid- and cyclosporine-dependent nephrotic syndrome.

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. TST and QFT were performed for all patients. QFT was negative in all patients, while TST was positive only in one case. Both tests results were negative in 30 patients, yielding an accuracy rate of 96.7% for TST to diagnose LTBI when compared to QFT. In conclusion, compared to QFT, TST is still a valuable diagnostic tool with high accuracy rate for diagnosis of LTBI in children candidates for renal transplantation and can still be used as an accurate test for screening Mycobacterium TB infection.

The relationship between serum and urine NGAL and graft function in pediatric renal transplant recipients / Relación entre niveles de NGAL en suero y orina y función del injerto en pacientes pediátricos trasplantados renales

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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. MATERIALS AND METHODS: Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment. RESULTS: There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR. CONCLUSIONS: We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.

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. RESULTS: Of all the patients, 61.2% were Epstein-Barr virus seropositive at the time of transplant. Graft survival rate and the rate of acute rejection episodes were not statistically different between the seropositive and seronegative recipients. Three patients (0.005%) had posttransplant lymphoproliferative disorder after transplant. CONCLUSIONS: We showed that the rate of seronegative recipients in our cohort is similar to other studies, but the rate of posttransplant lymphoproliferative disorder was low in our recipients.

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. There are some case reports as well as larger series in this regard. The majority of these case reports and series have demonstrated the success of rituximab in the treatment of nephrotic syndrome, especially in pediatric patients with steroid-dependent and frequent-relapsing nephrotic syndrome. Nevertheless, the treatment strategies before and after rituximab infusion are not clear to date. On the other hand, it is believed that positive results on rituximab use in nephrotic syndrome are much more reported by researchers than the negative results and this is an important bias. Although most reports on rituximab use in pediatric patients have not recognized significant side effects, the long-term adverse events of rituximab are not known. Thus, controlled long-term studies are required to be done to assess the risk-benefit profile of rituximab in childhood nephrotic syndrome.