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Objectives To assess the health related quality of life of living related donors after kidney transplantation and to evaluate clinical ,social and psychological condition post donation Study design This is an observational cross sectional study that included fifty living related donors of kidney transplant. All donors were assessed after at least one year of kidney donation. Data of the study was collected between April, 2019 and March, 2020. Methods The study included 50 living related donors;All donors were assessed after at least one year of kidney donation.WHOQOL-bref questionnaire (world health organization quality of life ) was used to assess quality of life of donors. Results The mean age at time of transplantation was 35.4±7.89 years. Eight cases developed hypertension after transplantation. There was no significant difference between serum creatinine before and after transplantation while there was significant decrease in glomerular filtration rate post transplantation. There was significant increase in fasting blood glucose and cholesterol in female donors compared to males.The results of the WHOQOL-bref questionnaire showed significant difference in results of psychological, social and environmental domains pre and post donation. Conclusion Proper selection of donors is very important to avoid complications post transplantation. Follow-up of donors should be maintained after donation.

Backgrounds: Recent advances in post Kidney Transplantation (KT) care, have led to a dramatic improvement in short-term outcomes in order to achieve transplantation tolerance; including the ideal tool for clinical monitoring & new therapeutic line. This study was undertaken to analyze the CD62L in Kidney Transplant Recipients (KTRs) and to investigate its efficacy as a marker of good graft survival. Methods: Fifty pediatric KTRs and 12 healthy controls were included in the study, the frequency of T cell activation markers; CD62L was measured with flow cytometry after renal transplantation. Clinical, laboratory, immunosuppressive therapy data and graft function of transplant recipients were collected and correlated with their CD62L peripheral blood percentage. Results: The circulating CD62L% was significantly more in transplant recipients than controls (44.74% ± 17.45% vs. 33.36% ± 11.54%, p = 0.02). CD 62L% was more frequent in recipients of living related donors (p = 0.05), positively correlated with donor age (p = 0.04, r = -0.29*) and CD 4% (p = 0.000, r = 0.615). CD26L% did not show significant association with acute rejection or chronic rejection (p = 0.432, p = 0.91 respectively) or with graft function (serum creatinine or eGFR, p = 0.086, p = 0.988 respectively) or immunosuppressive medications. Conclusion: Peripheral CD62L% is increased after KT than healthy controls, however, it cannot reflect either clinical (serum creatinine and eGFR) or pathological renal graft injury. CD62L surface marker needs more analysis for its potential diagnostic and therapeutic implications as a Treg cell activation marker.

The rationale for the prescription of vitamin D analogues in patients with chronic kidney disease (CKD) is still a matter of debate. We aimed to compare native vs. active forms of vitamin D on pre-dialysis children with CKD and evaluate effects on calcium (Ca), phosphorus (P), and parathyroid hormone (PTH). Thirty children with pre-dialysis CKD were enrolled in a prospective cross-over study. Patients were randomly classified into two groups. Group A received native cholecalciferol while group B received alfacalcidol for 3 months. After 1 month (washout period), patients were switched to receive the opposite form for another 3 months. Serum Ca, P, alkaline phosphatase (ALP), PTH, and 25(OH)D3 were measured at study start (BL-1), end of first period (FU-1), before second period (BL-2), and after second period (FU-2). There was significant increase in levels of 25(OH)D3 after administration of either native or active vitamin D in the first period in both groups (p

Introduction Left ventricular hypertrophy (LVH) is the commonest myocardial response to chronic kidney disease (CKD); this response has been regarded detrimental as it impairs the blood flow to the deepest layers of the myocardium causing progressive myocardial dysfunction. The aim of these series is to assess the determinants of LVH in CKD patients and its impact on subendocardial function in such patients. Methods This study has been conducted on 40 CKD patients (Group 1) and 40 age-matched controls, both groups were assessed by transmural echocardiography to determine the subepicardial and subendocardial global longitudinal strain (GLS) as an expression of the systolic function of each of those layers. LVH was assessed by calculation of left ventricle mass index (LVMI). Both groups underwent ambulatory blood pressure monitoring. Group 1 was assessed as regards lipid profile and insulin resistance by homeostasis model assessment of insulin resistance (HOMA-IR). Results HOMA-IR proved to be a more important determinant of LV hypertrophy than SBP and DBP with a P of 0.01. Moreover subendocardial GLS was negatively correlated with LVMI with r = 0.69 and P < 0.01 denoting the negative effect. LVH plays on subendocardial function probably by impairing myocardial perfusion. Conclusion This study points toward the importance of insulin resistance in aggravation of myocardial remodeling in CKD patients; more studies are warranted to examine the role of insulin Sensitizers in reversing such remodeling and restoring subendocardial function in such important systemic disorder.

Background Although kidney transplantation (KTX) is the treatment of choice for pediatric end stage kidney disease (ESKD); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KTX in children with ESKD secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients. Methods Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. All sporadic patients electively received 6 perioperative PE sessions. Patients with recurrence of proteinuria (n = 13; including 3 patients with genetic/familial and 10 patients with sporadic FSGS) were managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data. Results The mean follow-up duration after KTX was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KTX) in 2 patients (5%). All patients with early recurrence achieved complete remission, while patients with late recurrence developed graft failure. Current serum creatinine and proteinuria levels were not different in patients received PE (n = 31) and patients did not PE (n = 9) (p = 0.308 and 0.287 respectively). Current serum creatinine and proteinuria levels in sporadic patients (n = 28) after prophylactic perioperative PE were not different from those of genetic/ familial patients (n = 12) (p = 0.303 and 0.144 respectively). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p = 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KTX and 2 patients’ mortality (5%) occurred early postoperative (first 2 weeks). Conclusion FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KTX can be successfully managed with PE and RTX.

Background Inflammation is a major risk factor for cardiovascular (CV) complications and mortality in patients with ESRD. Patients with end stage renal disease (ESRD) strongly exhibit higher serum levels of inflammatory cytokines compared to healthy individuals. Patients & Methods The serum levels of proinflammatory cytokine IL-18, and anti-inflammatory IL-10 and of Pentraxin 3 were studied in sixty pediatric patients, aiming to compare the inflammatory status of children with end-stage renal disease on hemodialysis and those on conservative treatment; and to study the effect of vascular access and cardiac disease on those cytokines. Thirty patients had end stage renal disease on regular hemodialysis in Pediatric Nephrology Unit, Cairo University Children’s Hospital, fifteen patients had chronic kidney disease, on conservative therapy, in addition to fifteen healthy matched controls. Results The mean age of patients and duration of dialysis for the hemodialysis group was 11.93 ±3.79 and 6.03±2.46 years respectively while the other group was 8.46±3.63 years. Hemodialyzed patients had significantly higher post dialysis levels of Pentraxin3 and interleukin18 compared to pre dialysis levels with p value of 0.044 and 0.001 respectively. There was no difference regarding pre and post dialysis levels of interleukin10. Hemodialyzed patients had higher levels of Pentraxin3 and interleukin18 than conservative patients with P value of 0.006 and 0.01 respectively. Pentraxin3 and interleukin18 were higher in patients who dialyzed via central venous catheters than via Arteriovenous fistula with P value of 0.04, 0.002 respectively; while interleukin10 was lower with P value of 0.03. Conclusion Children with ESRD had higher post dialysis levels of inflammatory cytokines such as PTX3 and IL 18 than pre-dialysis levels. Also, they had higher levels than conservative patients. No significant difference as regard IL10 in both hemodialysis and conservative patients. IL10 was negatively correlated with cardio-vascular complications. Fourty percent of patients had cardio-vascular lesions proved by ECHO. The use of central venous catheter in HD patients is associated with increase in inflammatory markers. PTX3, IL18 were significantly higher in patients who dialyzed via CVC than those via A-V fistula.

Introduction Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxalate and commonly presented with radiopaque renal stones or obstructive uropathy. This study aimed to report clinical presentations, renal replacement therapy (RRT) and outcome of PH1 in end stage kidney disease (ESKD) children. Methods This is an observational cohort study. Data of 22 patients with ESKD due to PH1 were analyzed at Pediatric Nephrology Unit, Faculty of Medicine Cairo University. Results Infantile onset patients (n=10) had worst renal outcome (80% with ESRD at presentation, p=0.019) and worse patient outcome (mortality 40%, p=0.016) than juvenile (n=9) and late onset (PH1 n=3) patients. RRT modalities include peritoneal dialysis (PD) in 7 (31.8%), hemodialysis (HD) in 11(50 %) and, combined liver kidney transplantation (CLKT) in 4 (18.2%) patients. Infectious complications were encountered in 42.8% of PD patients. Better HD adequacy was observed with frequent HD (n=6) and/or HD via arteriovenous fistula (AVF) than with infrequent dialysis (n=5) and/or via central venous line (CVL) (P =0.0001 & 0.0047 respectively). Morbidity and mortality (infection related) rates of the whole cohort were 63.6% and 31.8% respectively. Conclusions Clinical presentation of PH1 varies according to the age of onset (infantile onset being the most aggressive form). Aggressive HD (better through AVF) is needed to achieve acceptable HD adequacy, PD was challenged by infection. Infection found to be the main cause of mortality even after successful CLKT. This article is protected by copyright. All rights reserved.

BACKGROUND: Anemia is one of the most common complications in end-stage renal disease (ESRD) patients. Hepcidin is a hormone that regulates iron homeostasis in patients with ESRD. Carnitine deficiency is commonly seen in hemodialysis (HD) patients. AIM: This study aimed to investigate the relationship between hepcidin and inflammatory and other anemia markers in children with ESRD and to evaluate the association of carnitine deficiency with anemia in these patients. SUBJECTS AND METHODS: Thirty pediatric patients with ESRD undergoing HD, and thirty healthy, age- and sex-matched children served as controls were included in the study. Serum levels hepcidin, iron status, high-sensitivity C-reactive protein, and total carnitine were measured. RESULTS: Statistically significant increases in serum levels of hepcidin (100.7 ± 0.99 ng\ml vs. 77.43 ± 0.8 ng\ml, p = 0.000), was found in HD children as compared to healthy controls. Statistically significant increase in serum levels of hs-CRP (3.94 ± 0.19 mg/l vs. 1.36 ± 0.07 mg/l, p = 0.04) was found in HD children as compared to healthy controls. However, serum levels of carnitine (29.59 ± 2.46 μmol/L vs. 36 ± 2.39 μmol/L, p = 0.000) showed statistically significant decreases in HD children as compared to healthy controls positive correlation was found between hepcidin and hs-CRP (r = 0.059, p = 0.042). Furthermore, a positive correlation was present between serum carnitine levels and serum iron levels (r = 0.651, p = 0.042). CONCLUSION: Serum hepcidin may be a more useful biomarker of functional iron deficiency in children on HD. The efficacy of carnitine treatment for children on HD with carnitine deficiency and its effect on anemia needs to be studied.

Klotho G-395-A gene polymorphism may impact children with end-stage renal disease (ESRD). We investigated the relevance of Klotho G-395-A on ESRD development and progression, and its relationship with evolution of cardiovascular complications in pediatric dialysis patients. Fifty-five children with chronic kidney disease (CKD) and seventy healthy children were genotyped for Klotho G-395A. Incidence of GA/AA genotypes and A allele were higher in ESRD patients compared with controls (54.5 vs. 7.1%, P

Background: Despite advances in immunosuppression, acute allograft rejection remains one of the key factors affecting patient and graft survival in pediatric kidney transplantation. The aim of the study is to evaluate the role of serum soluble IL2 receptor (sIL2R) level as a noninvasive assessment parameter of acute rejection (AR). Method: Serum sIL2R level was measured (using enzyme-linked immune-sorbent assay technique) in 60 pediatric kidney transplant recipients (30 recipients with AR and 30 transplant recipients with stable graft function). Results: The mean values of sIL2R level in patients experiencing AR (14.8 ± 6.54) ng/mL were significantly higher than that in patients with stable graft functions (6.44 ±1.95) ng/mL (p = 0.0001). In addition; patients with AR proved by graft biopsy had their mean values of sIL2R level (16.19 ± 7.48) ng/mL significantly higher than that of other recipients in the study population (p = 0.032). Conclusion: Serum sIL2R level may serve as a noninvasive diagnostic indicator in pediatric kidney transplant recipients experiencing AR.

BACKGROUND: Renal transplantation (RTx) is the treatment of choice for paediatric end-stage renal disease (ESRD). A major cause of morbidity and mortality after RTx is cardiovascular disease. Independent predictors of cardiovascular events were shown to constitute an endothelial dysfunction (ED). This study aims to evaluate Visfatin serum level in comparison to brachial artery flow-mediated dilatation (FMD) as a marker of endothelial dysfunction in paediatric RTx recipients.METHODS: Visfatin serum level has been evaluated in 30 patients on regular hemodialysis (HD), 36 patients post-RTx and 30 controls as a measure for ED, and has been compared to brachial artery FMD.RESULTS: Visfatin level in transplant recipients was significantly lower than the hemodialysis group as well as FMD was better in transplant recipients. In spite of marked improvement of FMD and marked reduction of visfatin in post-RTx no direct statistical correlation was found between serum Visfatin level and flow-mediated dilatation.CONCLUSION: Pediatric RTx recipients show lower serum Visfatin level and better FMD than those on regular hemodialysis, reflecting less endothelial dysfunction (ED) and less cardiovascular risk. FMD in kidney transplant recipients tends to be less than normal subjects while visfatin level of the same group is similar to controls. Pediatric RTx appears to have a positive impact on the growth development of children with ESRD.

Pediatric kidney transplantation is a multidisciplinary therapy that needs special consideration and experience. In this study, we aimed to present CUCH experience; over a 10-year period, as a specialized center of kidney transplantation in children. We studied 148 transplantations performed at a single center from 2009 to 2018. Pretransplant and follow-up data were collected and graft/patient survival rates were evaluated. A total of 48 patients developed at least one rejection episode during 688 patient-years of follow-up. Infections, recurrence of original disease, and malignancy were the most important encountered medical complications (20%, 2%, and 1.4%, respectively). One-year patient survival was 94.1%, while graft and patient survival was 91.9%. Graft/patient survival at 5, 7, and 9 years was 90%, 77%, and 58%, respectively. Infections were the main cause (69%) of mortality. Death with a functioning graft and CR were the main causes of graft loss (48% and 33%, respectively). Pediatric kidney transplantation in Egypt is still a challenging yet successful experience. Rejections and infections are the most frequent complications. Short-term outcomes surpass long-term ones and graft survival rates are similar to the international standard.

Background An essential milestone in pediatric transplantation is to find noninvasive biomarkers to monitor acute rejection (AR). In this retrospective (Case-control) study, we examined the role of Fas − 670A/G and Fas Ligand (FasL) − 843C/T gene polymorphisms in allograft nephropathy in pediatric renal transplant recipients. Methods In 47 pediatric kidney transplant recipients and 20 healthy controls, Fas − 670A/G and FasL − 843C/T gene polymorphisms as well as serum soluble Fas Ligand level (sFasL) were measured. Results Serum sFasL levels were significantly higher in transplant recipients children than that in controls (548.25 ± 298.64 pg/ml vs 143.17 ± 44.55 pg/ml, p = 0.0001). There was no significant difference between patients with AR and those without AR in regards to serum sFasL levels (567.70 ± 279.87 pg/ml vs 507.85 ± 342.80 pg/ml, p = 0.56). Fas − 670A/G genotypes or alleles were not significantly different between controls and transplant recipients and among transplant recipients with and without AR. (P > 0.05 for all). FasL − 843C/T genotypes were not different between transplant recipients and controls and among transplant recipients with and without AR (P > 0.05 for all). However, Frequency of C allele in transplant patients was significantly higher than that in the control group (44.68% vs 25%, P = 0.03). FasL − 843C/T alleles were significantly different between patients with and without AR (P = 0.03). The percentages of C allele were higher in children with AR (58.82% vs 36.67%). We found that serum FasL and serum creatinine were variables that were independently associated with AR. Conclusion This study suggests that FasL gene polymorphisms in peripheral blood might be accurate in detecting cellular AR.

Background : End stage renal disease (ESRD) is a common cause of morbidity and mortality among children. Interleukin 4 is a cytokine that might influence the progression of chronic kidney disease (CKD) to end stage renal disease. There are variable number of tandem repeats (VNTRs) in IL4 gene that could play major roles in genetic predisposition to some diseases. Aim of the study: The purpose of this study is to detect the association of allelic variant in intron 3 VNTR-IL4 gene with the end stage renal disease and if these variants could be considered as risk markers for this disease. Subjects and methods : The study was conducted on fifty-five children with CKD and fifty healthy children served as controls. All participants were genotyped for intron 3 VNTR by Polymerase Chain Reaction. Results : The frequency of intron 3 VNTR-IL4 P1P2 + P2P2 genotypes was significantly higher in ESRDchildren than those with P1P1 genotype (88.7% vs. 15.4%, OR 43; 95% CI 13–134, P value < 0.001). Also, the frequency of P2 allele was significantly higher in ESRD-children compared with healthy controls (70.9% vs. 8%, OR 28; 95% CI 12–64, P value < 0.001). Furthermore, a significantly higher frequencies of P1P1 genotype and P1 allele among the control group were demonstrated (84.6% vs. 11.3%, P < 0.001 and 92% vs. 29.1%, P < 0.001, respectively). Conclusion : we concluded that the P2 allele is an allelic variant predisposing to ESRD in children with CKD and it could be considered a risk factor for the development of ESRD. Keywords : VNTR-IL4 ESRD PCR Children Gene polymorphism

Objectives To compare outcomes of renal transplantation (RTx) in children with end-stage renal disease (ESRD) resulting from lower urinary tract dysfunction (LUTD) vs other causes. Patients and Methods A database of children (

The increasing demand for solid organs has necessitated the use of ABO and Rhesus (Rh) D minor mismatched transplants. The passenger lymphocyte syndrome (PLS) occurs when donor lymphocytes produce antibodies that react with host red blood cell (RBC) antigens and result in hemolysis. Our aim was to evaluate prospectively the role of PLS in post transplant anemia and hemolysis in ABO and RhD minor mismatched recipients of liver and kidney grafts and to study the association of PLS with donor lymphocyte microchimerism. We examined 11 liver and 10 kidney recipients at Day +15 for anemia, markers of hemolysis, direct antiglobulin test and eluates, and serum RBC antibodies. Microchimerism was determined in peripheral blood lymphocytes by genotyping of simple sequence length polymorphisms encoding short tandem repeats. Immune hemolytic anemia and anti-recipient RBC antibodies were observed in 2 out of 11 liver (18.2%) and 2 out of 10 kidney (20%) transplants. RBC antibody specificity reflected the donor to recipient transplant, with anti-blood group B antibodies identified in 2 cases of O to B and 1 case of A to AB transplants while anti-D antibodies were detected in 1 case of RhD-negative to RhD-positive transplant. Donor microchimerism was found in only 1 patient. In conclusion, passenger lymphocyte mediated hemolysis is frequent in minor mismatched liver and kidney transplantation. Recognizing PLS as a potential cause of post transplant anemia may allow for early diagnosis and management to decrease the morbidity and mortality in some patients.

There are very few published reports on the causes of chronic kidney disease (CKD) in Egyptian children. We reviewed the records of 1018 (males 56.7%, age ranged from 1 to 19 years) Egyptian patients suffering from CKD and followed-up at the pediatric nephrology units (outpatient clinics and dialysis units) of 11 universities over a period of two years. The mean of the estimated glomerular filtration rate was 12.5 mL/min/1.73 m 2 . Children with CKD stage I and stage II comprised 4.4% of the studied group, while those with stage III, IV and V comprised 19.7%, 18.3% and 57.6%, respectively. The most common single cause of CKD was obstructive uropathy (21.7%), followed by primary glomerulonephritis (15.3%), reflux/urinary tract infection (14.6%), aplasia/hypoplasia (9.8%) and familial/metabolic diseases (6.8%); unknown causes accounted for 20.6% of the cases. Of the 587 patients who had reached end-stage renal disease, 93.5% was treated with hemodialysis and only 6.5% were treated with peritoneal dialysis.

Vascular access represents a lifeline for children undergoing hemodialysis. A failure of vascular access among patients receiving regular hemodialysis is associated with increased morbidity, mortality and costs. We assessed the possibility of using soluble adhesion molecules as reliable predictors of vascular access failure in children on hemodialysis. Moreover, we evaluated whether there is an association among the different studied adhesion molecules in hemodialysis patients with thrombosed and non-thrombosed arteriovenous fistula fistulas (AVFs). This study included 55 hemodialysis children, 36 with good access and 19 with access failure, and 20 healthy volunteers. Forty-four patients had native AVFs and 11 patients had tunneled permanent catheter (11with thrombosed and 33 with non-thrombosed AVFs). Serum-soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble intercellular adhesion molecule-1 (sICAM-1), soluble E-selectin (sE-selectin) and soluble P-selectin (sP-selectin) were measured using ELISA technique. A significant increase was found in the levels of sVCAM-1, sICAM-1, sE-selectin and sP-selectin versus controls and all hemodialysis patients, hemodialysis patients with good access and hemodialysis patients with access failure (P=0.001 for sVCAM-1 and sICAM-1 and P=0.0001 for sE-selectin and sP-selectin). A significant increase was found in the levels of sVCAM-1, sE-selectin and sP-selectin in both chronic hemodialysis patients with thrombosed and non-thrombosed native AVFs versus controls (P=0.0001 for all parameters). There was significant difference between both chronic hemodialysis patients with thrombosed and non-thrombosed native AVFs as regard to sVCAM-1 (54.64±30.82 versus 25.69±27.96ng/ml, P=0.04). Both sICAM-1 and sP-selectin were positively correlated with the erythropoietin (EPO) dose in hemodialysis children (r=0.31, P=0.04 and r=0.32, P=0.04, respectively). A significant positive association was found between E-selectin and sP-selectin in hemodialysis patients with thrombosed AVFs (r=0.83, P=0.04). There was a significant correlation between sVCAM-1 and EPO dose in thrombosed AVF group (r=0.84, P=0.01). The assessment of serum sVCAM-1 might be useful for the identification of the chronic hemodialysis patients at an increased risk for native AVFs thrombosis. The role of EPO in vascular access failure should be taken into consideration. The clinical relevance of these observations warrants further investigations.

Introduction: Serum procalcitonin (PCT) levels are known to be low in healthy individ­uals in healthy subjects but are increased in patients with a severe bacterial infection. It has not been extensively studied in children with chronic kidney disease (CKD), treated either with hemodialysis (HD) or with renal transplantation. Material and methods: During a 6-month period, blood samples were taken from 102 (55 HD children and 47 renal transplant recipients) children with a strong clinical suspicion of infection. Procalcitonin levels were measured by ELISA. Results: Thirty-four/102 cases had proven infections as defined previously. Children with proven infections had a significantly higher PCT (0.920 ±0.24 ng/ml) than those without (0.456 ±0.53 ng/ml), p = 0.04. The ideal cutoff value derived for serum PCT was 0.5 ng/ml. This threshold value established a sensitivity of 94.1% and a specificity of 87.9%. Conclusions: This study indicates that significantly increased PCT concentration is a promising predictor of systemic bacterial infection in children with CKD, with good sensitivity and specificity. This study proposes that serum PCT is a convenient index of infection in CKD children at a cutoff value of 0.5 ng/ml.

Pediatric kidney transplant recipients are at special risk of infection with opportunistic fungi, such as Aspergillus spp., which is uncommon but can be fatal. We report a 16 year male renal transplant recipient, who suffered from acute graft dysfunction five months post transplantation. Imaging of the graft revealed sever back pressure and increased echogenic contents with a distinct pelviureteric ill-defined small mass. Percutaneous nephrostomy was done to relieve the obstruction and microscopic examination and fungal culture of the nephrostomy urine were done which revealed the organism (Aspergillus fumigatus). He developed initial improvement subsequent to relief of obstruction; graft function partially regained and received voriconazole for six months. His radiological finding gradually disappeared and graft function resumed to an acceptable level 4 weeks later. As of September 2016 (6 years later), despite the graft injuries, graft function had been conserved. This case serves to reinforce the concept that high index of suspicion of such infection and repeated examination with specific culture media are mandatory for transplant recipients particularly being potentially treatable and if neglected might be fatal infection.

Introduction Cardiopulmonary bypass (CPB) surgery is considered one of the most frequent surgical procedures in which acute kidney injury (AKI) represents a frequent and serious complication. The aim of the present study was to evaluate the efficiency of neutrophil gelatinase-associated lipocalin (NGAL) as an early AKI biomarker after CPB in pediatric cardiac surgery. Material and methods The study included forty children aged 2 to 78 months undergoing CPB. They were divided into group I: patients who suffered AKI grades II and III; and group II: patients who did not develop AKI or at risk. Peripheral venous blood was withdrawn pre- and post-operatively for serial measurements of NGAL and creatinine. Statistical analysis was performed using Statistical Package for Social Sciences version 14. Results Mean plasma NGAL levels showed highly significant elevations in group I patients at 2, 12, and 24 h after surgery (p < 0.0001) compared to group II. Significant correlations were found between NGAL and creatinine at different time intervals. Highly significant correlations (p < 0.0001) were found between plasma NGAL and AKI at 2, 12 and 24 h after surgery. A cut-off level of 100 ng/ml at 2 h, and 125 ng/ml at 12 h post-operatively both recorded the highest accuracy, being 95% accurate, with sensitivity of 100% and 89.5% respectively, and specificity of 90.5% and 100% respectively. Conclusions This study showed that plasma NGAL could be used as an early biomarker for detection of AKI following CPB. We recommend further studies on a wider scale to validate the current study results.

INTRODUCTION AND OBJECTIVES: Patients with obstructive uropathy and voiding dysfunction (OUeVD), such as posterior urethral valves, neurogenic bladder dysfunction and vesicoureteral reflux represent 20% to 30% of kidney transplantation (KT) population. Historically, these patients were denied KT due to suboptimal lower urinary tract (LUT) and frequent UTI that may compromise graft survival. A number of series reported acceptable KT outcomes in these patients compared to patients with normal LUT. We aim to evaluate the outcome of KT in pediatric patients with ESRD due to OUeVD compared to patients with ESRD due to medical renal disease (MRD) METHODS: A retrospective review of patients’ records who underwent KT at Cairo University Pediatrics Hospital from January 2007 through January 2014. Outcomes of KT done for ESRD due OUeVD (Group I) were evaluated and compared with outcomes for KT done for ESRD due to MRD (Group II). Multiple perioperative parameters were examined including ancillary procedures performed to optimize the UT for KT in addition to graft survival. RESULTS: 103 patients underwent KT in the reviewed period with 74/103 (71.8%) males and 29/103 (28.2%) females. Mean patient age at KT was 5.05 12.4 (2.2e18) years. Group I patients included 29 patients while Group II included 74 patients. All patients in Group I needed ancillary procedure prior to KT with 55.2% in Group II. Mean follow up was 3.34 1.63 (1e7) years and 2.12 1.08 (1e5) years for Group I and II, respectively. Mean creatinine at 12 months was 0.94 0.24mg/dL and 0.97 0.36mg/dL for Group I and II respectively, with no statistically significant difference between the 2 groups (p1⁄40.8). All Group I patients were maintaned on TMPeSMX antibiotic prophylaxis. There was no signifant difference in posteKT UTI rates (7/29 (24%) in Group I and 9/74 (12.3%) in Group II (p1⁄40.05). The incidence of backepressure was comparable in both groups, 6/29 (20.69%) in Group I and 16/74 (21.62%) in Group II (P value1⁄40.92). CONCLUSIONS: KT can be safely performed in pediatric patients with OUeVD with/without reconstructed LUT, with results comparable to patients with ESRD due to MRD. Antibiotic prophylaxis helps maintain acceptable UTI rates.

The cardiovascular disease is an important cause of morbidity and accounts for almost 50% of deaths in patients undergoing maintenance dialysis. Many harmful molecules of the uremic milieu, such as the middle molecules, are difficult to remove by conventional hemodialysis (HD). On-line hemodiafiltration (OL-HDF) can achieve a considerable clearance of middle molecules and, together with its sterile ultrapure infusate, may have favorable effects on inflammation and cardiovascular complications. We aimed in this study to assess the effect of OL-HDF on improving the chronic inflammatory state associated with chronic kidney disease and the possible impact of these changes on myocardial function in chronic HD children. Thirty pediatric patients [12 (40%) males and 18 (60%) females with a mean age of 11.3 ± 3.2 years] on conventional HD for at least six months were switched to OL-HDF for six months. Variables for comparison at the end of each period included the levels of serum C-reactive protein and Kt/V as well as electrocardiography and echocardiographic measurements, including left ventricular mass index (LVMI). On changing from HD to OL-HDF, there was a significant decrease in hs-CRP (from 7.9 ± 8.9 to 3.4 ± 3 μ g/mL) (P = 0.01) and frequency of diastolic dysfunction (P = 0.04), while systolic function (FS and EF) improved significantly (P = 0.007 and 0.05, respectively), while LVMI did not change. We conclude that OL-HDF was well tolerated in children with improvement of the systolic function of the myocardium and the overall frequency of diastolic dysfunction.

Background: Various pathogenic factors were blamed for the neurologic deficits in renal patients. Fumonosin (FB1) is an occasional mycotoxin that was shown to be responsible for neurologic disturbances in horses by disturbing the sphingolipid metabolism. It was detected as a contaminant among several foodstuffs in Egypt, but was not studied as a risk factor in humans. Objectives: To study the cross-sectional associations between serum FB1, sphinganine and sphingonsine and the prevalence of neurologic deficits among other related factors including serum aluminium (Al), serum parathyroid hormone (PTH) levels and other hematological and chemical parameters in a group of children and adult renal patients. Methods: Visual evoked potential (VEP) was tested in 40 end stage renal disease patients (20 children and 20 adults) on regular dialysis treatment (RDT), 40 patients with different glomerulonephritis (GN) (20 children and 20 adults) and 10 healthy controls. They were studied for serum FB1, sphinganine, sphingosine, calcium, phosphorus, PTH levels, Al, albumin, blood urea, serum creatinine, blood gases and hemoglobin percentage. Results: FB1 with elevation of sphinganine/sphingosine ratio was found in 12/40 of ESRD patients and 12/40 GN patients but in none of controls (p ˂ 0.05). Of the GN group, 9/40 had delayed VEP and 3 of these 9 had positive FB1, but showed no significant correlation with neither FB1 nor PTH (p ˃ 0.05). Of the ESRD group, 13/40had delayed VEP. This delay was detected in 8/12 cases with positive FB1 and in 10/19 cases with more than double the high serum PTH and in 13/34 cases with Al more than 20 ug/dl. Al was a sole risk factor in 2/13 ESRD patients with delayed VEP, but neither high PTH nor positive FB1 was encountered solely in any of these cases. In adults ESRD group, VEP abnormalities showed significant correlation with PTH (p ˂ 0.05) but not with Al or FB1. Yet, in children it was significantly correlation with the serum PTH, Al and FB1 (p ˂ 0.004, 0.03, 0.0001 respectively). Conclusions: We conclude that long duration of hyperparathyroidism is a risk factor causing neurologic deficits in ESRD patients and may be aggravated by high serum Al and/or a positive FB1, particularly in children.

The aim of this study was to detect possible risk factors for UC and UTI following pediatric renal Tx and effect of these complications on outcome. One hundred and eight children who underwent living donor Tx between 2009 and 2015 were retrospectively included. Extraperitoneal approach was used with stented tunneled extravesical procedure. Mean recipient age was 9.89 ± 3.46 years while mean weight was 25.22 ± 10.43 kg. Seventy-three (67.6%) recipients were boys while 92 (85.2%) were related to donors. Urological causes of ESRD were present in 33 (30.6%) recipients (14 [13%] posterior urethral valve, 16 [14.8%] VUR, and 3 [2.8%] neurogenic bladder). Augmentation ileocystoplasty was performed in 9 (8.3%) patients. Mean follow-up was 39.3 ± 17.33 months. UC were detected in 10 (9.3%) children (leakage 4 [3.7%], obstruction 3 [2.8%], and VUR 3 [2.8%]) while UTIs were reported in 40 (37%) children. After logistic regression analysis, UC were significantly higher in children with cystoplasty (44.4% vs 6.1%; P = .001). UTIs were significantly higher in girls (51.4% vs 30.1%; P = .001) and in children with urological causes of ESRD (51.5% vs 30.7%; P = .049). UC and UTI were not significantly associated with increased graft loss or mortality. UC were significantly higher in children with cystoplasty while UTIs were significantly higher in girls and children with urological causes of ESRD. Presence of UC did not affect the rate of graft loss or mortality due to its early detection and proper management.

Achieving dry weight after hemodialysis (HD) is critical as chronic fluid over-load can result in left ventricular hypertrophy, while small fluid shifts may result in intra-dialytic morbid events (IME). In the pediatric population, estimating dry weight can be difficult due to growth while on dialysis. Continuous non-invasive monitoring of the hematocrit (NIVM) has been proposed as a more accurate method of estimating dry weight. Fifteen pediatric patients on chronic HD (6 males and 9 females; mean age 11.4 ± 2.28 years) were included in an uncontrolled prospective study involving three phases. In phase 1, patients were observed for one month for their dry weight and frequency of IME. Phase 2 consisted of using NIVM-guided ultrafiltration algorithm for rate of blood volume (BV) reduction and post-dialysis refill, recommending an intra-dialytic reduction in BV of 8% in the first hour and 0.1). In phase 1, 40% of patients experienced no IME, 33% experienced one or two IME while 27% experienced more than two IME; during phase 3, 80% experienced no IME, 20% experienced one or two IME while no one experienced more than two IME. NIVM can serve as an objective method for determining dry weight as well as predicting and preventing IME in the pediatric population on maintenance HD.

To evaluate the effect of patient, surgical, and medical factors on surgical complications and graft function following renal transplantation (Tx) in children weighing ≤ 20 kg, because the number of this challenging group of children is increasing.Between June 2009 and October 2013, 26 patients received living donor renal allotransplant using the extraperitoneal approach (EPA). The immunosuppression regimen was composed of prednisolone, mycophenolate mofetil, and ciclosporin or tacrolimus.The mean weight was 16.46 ± 2.61 kg. Mean cold ischemia time was 53.85 ± 12.35 min. The graft survival rate (GSR) and patient survival rate (PSR) were 96% at 3 years. Acute rejection episodes (AREs) occurred in eight patients (30%). Postoperative surgical complications were ureteral leakage (3), vesicoureteric reflux (2), and renal vein thrombosis (2) (with one graft nephrectomy). Mean follow-up was 37.5 ± 7.4 months.Excellent PSR and GSR can be achieved in low weight (20 kg) recipients. Even in very low weight patients, the EPA was used. No cases were reported with primary graft non-function due to use of living donors, increasing pre-Tx body weight to at least 10 kg and maintaining adequate filling pressure before graft reperfusion. The presence of related donors and use of induction therapy and tacrolimus decreased the rate of ARE while the presence of pre-Tx lower urinary tract surgical interventions increased the rate of ureteric complications, but this was statistically insignificant.

The use of hemodialysis catheters is an essential component of dialysis practice. Children are particularly likely to require multiple courses of dialysis over their lifetime, hence the repeated need for vascular access. These catheters remain a significant source of morbidity and mortality. All catheters inserted for hemodialysis at the Center of Pediatric Nephrology and Transplantation, Cairo University over a period of 40 months were studied. Patient data as well as data of catheter insertion, dwell, cause of removal and complications were reported. A total of 195 uncuffed central venous catheters were used for temporary access in 131 patients for a mean duration of 35.7 days. Of attempted insertions, 87.4% achieved successful access, of which 56% remained for the required period, 8.9% were accidentally dislodged, and 35.1% were removed due to complications—mostly infection. The overall rate of possible catheter-related bacteremia was 9.6 episodes/ 1,000 catheter days. Infection increased with longer catheter dwell. Nineteen cuffed tunneled catheters were surgically inserted and used for up to 11 months (mean 117 days). Loss of these catheters was attributed mainly to infection (ten episodes) and catheter thrombosis (six episodes). During the study, 317 femoral catheters were inserted. Uncuffed central venous catheters are both needed and useful for short-term hemodialysis. Vascular access for extended durations may be provided by cuffed tunneled catheters. Infection is the major serious concern with both uncuffed and cuffed catheters.

Background Control of metabolic derangements related to bone disease is one of the important goals in management of patients with chronic kidney disease. Traditional treatment using calcium containing phosphate binders and active vitamin D 3 analogues can lead to hyperphosphatemia and hypercalcemia with increase in calcium phosphorus product and metastatic calcifications. Other methods of therapy such as calcimimetics and hemodiafiltration are additional options of treatment that can lead to better control of metabolic bone disease with avoidance of these complications. Methods Our study was a case-control study which was performed over 6 months on 33 children with end stage renal disease on regular hemodialysis. They were randomly divided into 3 groups: The first group (11 cases) on regular hemodialysis and received Cinacalcet (Mimpara) 30 mg/day in addition to conventional care (vitamin D and phosphorus binders) as needed, the Second group (13 cases) on hemodiafiltration (HDF) for at least 6 months and received conventional care only, and the third group (9 cases) [control group] on regular hemodialysis received conventional care only. Monthly follow up of serum calcium, phosphorus and alkaline phosphatase was done in addition to baseline and 3-monthly parathormone level (intact PTH). Results The mean age of whole study population was 10.6 ± 3.9 years (range 3-17 years), and the mean weight was 20.2 ± 7.3 kg (range 8-39.1 kg). 􀀥oth groups I and II, but not group III, showed a statistically significant increase in hematocrit level and a statistically significant decrease in alkaline phosphatase and PTH after the study period. There was a significant percent reduction of PTH level in group I patients compared to those in group III. There was no statistically significant difference between percentage of achievement of the NFK-K/DOQI recommended goals in the three groups. Conclusions On spite of the costs of cinacalcet and hemodiafiltration, patients with resistant hyperparathyroidism should use one of these modalities to avoid complications of the metabolic bone disease. The cost of cinacalcet and HDF should be calculated in the context that they will save in other items, as decreasing the dose of erythropoietin and vitamin D analogues and decreasing the complications, and consequently therapy of bone disease.