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Objectives Acute and chronic allograft rejection have been continuously an important obstacle in the follow-up of renal transplant recipients. During clinical management, several factors acting simultaneously result in acute rejection and chronic allograft nephropathy. Matrix metalloproteinases and tissue inhibitors of metalloproteinases are responsible for the organization of the extracellular matrix and play roles in cell proliferation and cellular invasion. Changes in matrix metalloproteinase expression levels have been reported to be associated with renal allograft rejection and interstitial fibrosis. In this study, we aimed to investigate functional polymorphisms of MMP2, MMP9, and TIMP2 genes in pediatric renal transplant recipients. Materials and methods Our study included 68 kidney transplant recipients and 58 control patients. The kidney transplant recipient group was further divided into 2 subgroups: no graft rejection (n = 47) and graft rejection (n =21). MMP2 -735C >T (rs2285053), MMP2 -1306C >T (rs243865), MMP2 -1575G >A (rs243866), MMP9 c.-1562C >T (rs3918242), TIMP2 -418G >C (rs8179090), and TIMP2 303C > T (rs2277698) polymorphisms were analyzed with the use of polymerase chain reaction and restriction fragment-length polymorphism methods. Allele prevalence was compared with reference values of the control group, and Hardy-Weinberg equilibrium was tested. Results Mean ages were 16.7 ± 3.9 years for the study group and 14.8 ± 5.6 years for the control group. The mean follow-up time after transplant was 37.7 ± 7.9 months. We compared allele frequencies in the 2 groups and calculated a statistically significant difference in rs2285053, rs243865, rs243866, rs3918242, rs8179090, and rs2277698 polymorphism frequencies between the transplant recipients and control patients. When the transplant recipient group was compared in itself with regard to allograft rejection, all investigated polymorphisms except TIMP2 -418G >C (rs8179090) revealed a statistically significant difference between those with and without rejection (P Conclusions Matrix metalloproteinases and their tissue inhibitors could be important predictive biological markers for the follow-up of kidney transplant recipients.

Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is associated with pathogenic variants in the PKHD1 gene. Autosomal dominant polycystic kidney disease (ADPKD) is mainly associated with pathogenic variants in PKD1 or PKD2. The present study aimed to identify the clinical and genetic features of Turkish pediatric ARPKD and ADPKD patients. Methods: This multicenter, retrospective cohort study included 21 genetically confirmed ARPKD and 48 genetically confirmed ADPKD patients from 7 pediatric nephrology centers. Demographic features, clinical, and laboratory findings at presentation and during 12-month intervals were recorded. Results: The median age of the ARPKD patients at diagnosis was lower than the median age of ADPKD patients (10.5 months [range: 0–15 years] vs. 5.2 years [range: 0.1–16 years], respectively, [p = 0.014]). At the time of diagnosis, the median eGFR in the ARPKD patients was lower compared to that of ADPKD patients (81.6 [IQR: 28.7–110.5] mL/min/1.73 m2 and 118 [IQR: 91.2–139.8] mL/min/1.73 m2, respectively, [p = 0.0001]). In total, 11 (52.4%) ARPKD patients had malnutrition; 7 (33.3%) patients had growth retardation at presentation; and 4 (19%) patients had both malnutrition and growth retardation. At diagnosis, 8 (16.7%) of the ADPKD patients had malnutrition, and 5 (10.4%) patients had growth retardation. The malnutrition, growth retardation, and hypertension rates at diagnosis were higher in the ARPKD patients than the ADPKD patients (p = 0.002, p = 0.02, and p = 0.0001, respectively). ARPKD patients with malnutrition and growth retardation had worse renal survival compared to the patients without (p = 0.03 and p = 0.01). Similarly, ADPKD patients with malnutrition had worse renal survival compared to the patients without (p = 0.002). ARPKD patients with truncating variants had poorer 3- and 6-year renal outcome than those carrying non-truncating variants (p = 0.017). Conclusion: Based on renal survival analysis, type of genetic variant, growth retardation, and/or malnutrition at presentation were observed to be factors associated with progression to chronic kidney disease (CKD). Differentiation of ARPKD and ADPKD, and identification of the predictors of the development of CKD are vital for optimal management of patients with ARPKD or ADPKD.

Delayed graft function is a common adverse outcome after renal transplant. Attempts for early prediction and prevention of delayed graft function are often challenging and misleading. Herein, we investigated for the first time the correlation between delayed graft function and preoperative noninvasive hematologic parameters to predict the possible adverse outcomes for renal transplant in pediatric patients.In this study, preoperative hematologic parameters of 51 pediatric renal transplant recipients followed between 2015 and 2021 were analyzed retrospectively. The selected 16 renal transplant patients with delayed graft function and 35 patients without delayed graft function had no concomitant comorbidities. The cutoff values for platelet-to-lymphocyte ratio of5 and neutrophilto- lymphocyte ratio of175 were considered low.We retrospectively evaluated a total of 51 (male/female, 33/18) pediatric kidney transplant recipients with a median age of 12 (interquartile range, 8-18) years. Median level of circulating lymphocytes was significantly higher in patients with delayed graft function compared with patients without delayed graft function (2 vs 1, P = .040). The preoperative low values for platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio were more prevalent in recipients who developed delayed graft function versus those who did not develop delayed graft function (68.8% vs 31.4% [P = .014] and 68.8% vs 34.3% [P = .023], respectively).Pretransplant low platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte were associated with increased number of delayed graft dysfunction. These novels and noninvasive inflammatory biomarkers may contribute to an early prediction of delayed graft function in pediatric kidney transplant recipients.

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent attacks of fever and serositis. Diagnosis is made according to clinical findings and supported by genetic analysis. The most commonly used adult diagnostic criteria are the Tel-Hashomer criteria. Pediatric criteria for FMF diagnosis were described in 2009, but their reliability should be supported by additional reports. In this study, we aimed to compare the pediatric criteria and the Tel-Hashomer and 2019 Eurofever/PRINTO classification criteria using our FMF cohort. A total of 113 patients diagnosed with FMF were included. Demographic features and laboratory findings were retrospectively collected from the patients' files. The patients were evaluated with the Tel-Hashomer, pediatric and Eurofever/PRINTO classification criteria. At least two of five new pediatric criteria were as sensitive (89%) and specific (85%) as the Tel-Hashomer criteria (sensitivity 70%, specificity 96%). We also evaluated the Eurofever/PRINTO classification criteria using our cohort and found a sensitivity of 94% and specificity of 91%. Conclusion: Using pediatric criteria for the diagnosis of FMF in children is a feasible and simple approach that can diagnose the disease based on at least two criteria. Therefore, our study supports the use of pediatric criteria in FMF diagnosis of children. Our results also confirm that the Eurofever/PRINTO classification criteria can be successfully applied for the diagnosis of FMF due to their high sensitivity (94%) and specificity (91%). What is Known: • The FMF diagnosis is made according clinical findings and supported by genetic analysis. • The use of adult diagnostic criteria in pediatric FMF patients is controversial since classical clinical presentation is often absent in children. What is New: • Our study supports both the use of pediatric criteria and Eurofever/PRINTO classification criteria in clinical practice.

Background/aim Growth differentiation factor (GDF)-15 is related to inflammation and mortality in many conditions. We aimed to determine if an elevated serum GDF-15 level is related to nutritional status of patients on hemodialysis (HD) and mortality. Materials and methods Routine HD patients (n = 158) were included in the study and followed for 18 months. Some malnutrition/ inflammation scoring indexes (malnutrition/inflammation score (MIS), controlling nutritional status (CONUT) score, and prognostic nutritional index (PNI)), biochemical parameters, and GDF-15 were used to build Cox regression multivariate models to study the association with mortality. Results Among the patients, 90 (57 %) had a high MIS (≥8), which associates with worse status. The serum GDF-15 level was higher in the same group (p = 0.003). The serum GDF-15 level differentiated malnutrition/inflammation according to the MIS (p = 0.031). Age, GDF15, and C-reactive protein (CRP) were significantly associated with higher all-cause mortality risk. Patients with both age and GDF-15 above the mean had a hazard ratio of 2.76 (p = 0.006) when compared with those both Conclusion In HD patients, the GDF-15 level is increased in worse nutritional status. Beyond the MIS, age, GDF-15 and CRP would be used together to estimate the worse clinical outcome in these patients.

Background: As the coronavirus disease 2019 (COVID-19) outbreak continues to evolve, it is crucially important for pediatricians to be aware of the differences in demographic and clinical features between COVID-19 and influenza A and B infections.Purpose: This study analyzed and compared the clinical features and laboratory findings of COVID-19 and influenza A and B infections in children.Methods: This retrospective study evaluated the medical data of 206 pediatric COVID-19 and 411 pediatric seasonal influenza A or B patients.Results: COVID-19 patients were older than seasonal influenza patients (median [interquartile range], 7.75 [2–14] years vs. 4 [2–6] years). The frequency of fever and cough in COVID-19 patients was lower than that of seasonal influenza patients (80.6% vs. 94.4%, P

Late antibody-mediated rejection triggered by donor-specific antibodies is a leading cause of kidney allograft failure. Effective treatment options for late antibody-mediated rejection are limited in renal transplant recipients. Here, we report 2 pediatric cases of severe late antibody-mediated rejection resistant to conventional immunosuppressive therapy who were successfully treated with eculizumab. Two patients who fulfilled the late antibody-mediated rejection diagnostic criteria (positive donor-specific antibodies, elevated mean fluorescence index, acute and/or chronic morphological lesions in the microvasculature, and abnormal kidney function test) were included in this study. Both patients were previously unsensitized with negative panel-reactive antibody. Case 1 was a 12-year-old male patient with kidney failure secondary to vesicoureteral reflux who underwent related-living donor kidney transplantation 2 years ago. Eleven months later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After the patient was treated with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 3.8 to 1.5 mg/dL. Case 2 was an unsensitized 16-year-old male patient with kidney failure secondary posterior urethral valve who underwent related-living donor kidney transplantation 4 years ago. Two years later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After treatment with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 2.1 to 1.01 mg/dL. In both patients, eculizumab therapy effectively reduced the markers of late antibody-mediated rejection and improved the kidney function.

Acute kidney injury (AKI) after cardiac surgery in children with congenital heart disease (CHD) is a serious complication closely associated with high morbidity and mortality. Despite numerous studies on AKI in children, most studies have excluded neonates. We sought to characterize AKI associated with cardiac surgery in neonates, determine its incidence, perioperative and postoperative risk factors, and short-term results.This retrospective study included 177 neonates who were operated on for CHD in our hospital between January 2015 and December 2019. Data of the patients were analyzed according to nKDIGO (neonatal Kidney Disease Improving Global Outcomes) and nRIFLE (neonatal Risk, Injury, Failure, Loss of function, End-stage kidney disease) criteria for evaluating AKI retrospectively. Data of groups with and without AKI were analyzed.The average age of 177 neonates were 8.2 ± 6.1 (1-28) days. Twenty-two (12.4%) neonates had CS-AKI defined according to nKDIGO criteria. Four (2.3%) neonates reached nKDIGO stage I, 1 (0.6%) reached stage II, 17 (9.6%) reached stage III. Thirty-eight (21.5%) neonates had CS-AKI defined according to nRIFLE criteria. Twenty-four (13.6%) neonates reached nRIFLE stage risk(R), 6 (3.4%) reached stage injury(I), 8 (4.5%) reached stage failure (F). The incidence of cardiac surgery-associated acute kidney injury (CS-AKI) in neonates was 12.5% and 21.5% for nKDIGO and nRIFLE, respectively. The percentage difference between nKDIGO and nRIFLE for AKI assessment was due to the criteria for nRIFLE stage risk(R) urine output1.5 mL/kg/h for 24 h. In both classifications, the duration of cardiopulmonary bypass, operation, inotropic treatment, and mechanical ventilation, length of intensive care unit (ICU), and hospital stay were significantly higher in the AKI group than those without AKI group (AKI and mortality rates were similar between groups according to the nKDIGO and nRIFLE criteria. For early prediction of AKI and adverse outcomes, diagnostic reference intervals might be specified in more detail in neonates undergoing cardiac surgery for CHD.

Children and adolescents with chronic diseases have more screen exposure time compared with their healthy peers. In this study, we investigated screen exposure time of children who received renal replacement therapy, which included kidney transplant and dialysis treatment, versus a healthy control group.Our study included 55 children and adolescents between the ages of 8 and 18 years. Although 28 participants did not have any chronic disease, 27 had chronic diseases and received renal replacement therapy. Among these patients, 17 had kidney transplant and 10 were receiving dialysis. A sociodemographic information form and the Conners Short-Form Parent Rating Scale were given to parents. Pediatric and adolescent patients completed the Children's Depression Inventory and Spielberger State-Trait Anxiety Scale-2. We analyzed differences between the groups with and without renal replacement therapy and examined relations between continuous variables.Duration of television screen time was significantly higher in children and adolescents receiving renal replacement therapy. Patients in the renal replacement therapy group showed a positive correlation between the Conners Short-Form Parent Rating Scale anxiety subscores and duration of smartphone use. In the kidney transplant recipient group, smartphone and computer durations were positively correlated and television duration was negatively correlated with the Conners Short-Form Parent Rating Scale behavioral problems subscores.Children on renal replacement therapy may be at risk in terms of excessive television exposure. Children who are on dialysis and have had a kidney transplant may be more prone to the negative effects of screen exposure than healthy peers who do not have chronic illnesses. These children and adolescents should be closely monitored to avoid the negative effects of excessive screen exposure.

Nutcracker syndrome related to the left kidney vein compression is a cause of orthostatic proteinuria during childhood. Some studies have shown that the ratios between maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions must be more than 4 in order to make a Nutcracker syndrome diagnosis. Our aim was to investigate whether the use of a decrease in aortomesenteric angle between upright and supine positions in the presence of isolated orthostatic proteinuria can be a criterion for the diagnosis of Nutcracker syndrome. Relevant patient information, which included demographic data, clinical examination findings, laboratory data, urinary system ultrasound, and kidney color flow Doppler ultrasound results, were prospectively collected. Thirty-nine pediatric patients with orthostatic proteinuria were included in the study. Left kidney vein compression findings were demonstrated in 31 patients. The ratio of maximum velocities of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions was above 4 in only 7 of our patients. Ratio of aortomesenteric angle between upright and supine positions was significantly decreased for patients with left kidney vein compression findings. Conclusion: The use of a decrease in the ratio of aortomesenteric angle between upright and supine positions in the presence of orthostatic proteinuria, instead of the ratios for maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments, can be more helpful for the diagnosis of Nutcracker syndrome in the differential diagnosis of orthostatic proteinuria. What is Known: • Proteinuria may be a sign of an impending kidney disease • Nutcracker syndrome is a cause of orthostatic proteinuria. What is New: • Ratio of aortomesenteric angle between upright and supine positions0.6 can be used for Nutcracker syndrome diagnosis.

Eculizumab is effective treatment of pediatric atypical hemolytic uremic syndrome (aHUS). However, the optimal duration of treatment is not clearly defined. The aim of this study was to retrospectively analyze the outcome of pediatric patients with aHUS, who started eculizumab treatment but discontinued it during the follow-up period.The clinical and laboratory findings of the pediatric patients with aHUS were recorded on a web-based, national registry system, known as the Turkish aHUS Registry. The study included 63 patients who had to have received more than four doses of eculizumab during the acute phase of the disease.The median age at diagnosis was 3.62 (IQR: 1.29-6.14) years. During the follow-up period, 39 patients continued to receive standard eculizumab treatment (standard treatment group, treatment every 2 weeks) while 24 received an extended dose of eculizumab at three-four-week intervals (non-standard treatment group). There was no significant difference between both groups in terms of clinical and laboratory parameters. Eculizumab treatment was discontinued in 18 patients (30.7%, F/M:11/7), and the median age of these patients at diagnosis and their median follow-up duration were 4.0 (IQR:2.7-10.2) and 4.2 (IQR:2.2-7) years respectively. The median eGFR at the last visit was 110 (84.7-146.1)ml/min/1.73 mEculizumab is a successful treatment option in pediatric patients with aHUS and it can be safely discontinued with close monitoring in a selected group of patients. In case of recurrence, eculizumab should be restarted immediately to achieve complete remission.

Familial Mediterranean Fever (FMF) is an autoinflammatory disease characterized with recurrent attacks of fever and serositis. The diagnosis is made according to clinical findings and supported by genetic analysis. The most used adult diagnostic criteria are the Tel-Hashomer criteria. The pediatric criteria for the FMF diagnosis of children were described in 2009, but their efficacy should be supported with further reports. In this study, we planned to compare the pediatric criteria and the Tel-Hashomer criteria in our FMF patients. We also aimed to evaluate the importance of the 2019 Eurofever/PRINTO classification criteria in this patient group. A total of 113 patients diagnosed with FMF were included in our study. Demographic features and laboratory findings were retrospectively recorded from the patients’ files. The patients were evaluated with the Tel-Hashomer, pediatric and Eurofever/PRINTO classification criteria. At least two of five new pediatric criteria were as sensitive (88.6%) and specific (84.62%) as the Tel-Hashomer criteria (sensitivity 69.9%, specificity 95.7%). We also evaluated the Eurofever/PRINTO classification criteria in our patients and found its sensitivity 93.8% and specificity 90.6%. Conclusion: Using pediatric criteria in the diagnosis of FMF in children is a feasible and simple method that can diagnose the disease based on at least two criteria. Therefore, our study supports the use of pediatric criteria in the diagnosis of FMF in children. Our results also confirm that the Eurofever/PRINTO classification criteria can be successfully used in the diagnosis of FMF due to their high sensitivity (93.8%) and specificity (90.6%).

Purpose: To report the manifestations, patterns of disease, treatment strategies and outcomes in pediatric patients with noninfectious uveitis.Methods: Demographic information of 76 cases was recorded. Symptoms, anatomic location, laterality, visual acuity (VA), intraocular pressure, associated systemic diseases, therapeutic strategies, side effects, complications were reviewed.Results: Thirty-one patients were diagnosed as uveitis on routine surveillance because of underlying systemic disease. The most common anatomic location was intermediate uveitis (34.2%). Juvenile idiopathic arthritis (JIA) was the most common underlying systemic disease (25%). Glaucoma was the most common complication (7.7%). The patients with refractory uveitis received adalimumab (26.5%), infliximab (4.6%) and tocilizumab (3.1%). The mean first-year VA was between 20/32 and 20/20 in 116/140 eyes.Conclusion: Most pediatric noninfectious uveitis cases have bilateral intermediate uveitis. JIA was the most common systemic association. The first-year VA was good in most eyes which may be due to early use of corticosteroid-sparing agents.

Introduction Rejection is the most important problem for renal graft function and survival. Complement system plays a key role in immune responses from host to graft. It was demonstrated that complement system activation is related with renal fibrosis. We evaluate clinical impact of complement deposition findings on biopsies in acute rejection episodes of pediatric renal transplant patients. Method Demographics of the patients, graft functions, acute rejection episodes and graft loss were recorded from data files of 165 pediatric renal transplant patients. Findings of 98 renal biopsies were retrospectively evaluated. Results Thirty three patients with kidney transplant had 44 acute rejection episodes (32 pure cellular acute rejection episodes / 1 pure humoral acute rejection episode / 11 combined acute cellular and acute humoral rejection episodes) proven by biopsy. C1q staining was positive in 7 biopsies, C3 staining in 15 biopsies and, C4d staining in 15 biopsies. 26 patients had graft fibrosis. All patients with a rejection history had a significant decrease in GFR value during follow-up. Patients who did not have fibrotic changes in first biopsy had same level of deterioration of GFR when compared with patients who had fibrotic changes in first biopsy. Conclusion We could not demonstrate a significant relation between complement deposition and renal fibrosis, and between complement deposition and GFR values. Our data demonstrated that graft outcomes and graft loss after acute rejection episodes cannot be predicted only with complement deposition on graft or only with graft fibrosis.

Background and Aims Cystic kidney diseases are a heterogeneous group of chronic renal disease. Autosomal recessive polycystic kidney disease (ARPKD) is generally diagnosed in utero or at birth due to mutations in PKHD1 gene. Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease commonly associated with mutations in PKD1 or PKD2. In this study, we aimed to investigate clinical and genetic features of ARPKD and ADPKD in a group of Turkish patients. Method A total of 69 children with genetically confirmed ARPKD (10 females, 11 males) or ADPKD (28 females, 20 males) from seven pediatric nephrology centers were included in this study. Demographic features, family history, clinical and laboratory findings at presentation and during 12 months intervals were collected. Results For ARPKD patients, the median age at diagnosis was 10.5 (IQR; 0.75-58.5) months. Consanguinity between parents was present in 11 patients (52.4%). At the time of diagnosis, 14 (66.7%) patients had eGFR For ADPKD patients, the mean age at diagnosis was 5.5±4.6 years. At the time of diagnosis 11 (22.9%) patients had eGFR Conclusion Cystic kidney diseases are one of the most clinically and genetically heterogenous diseases. Differentiating them and establishing the predictors for CKD development is important to provide appropriate management including choosing appropriate donor in renal transplantation.

OBJECTIVES Hypomagnesemia is considered as an independent risk factor for new-onset diabetes mellitus after kidney transplant. New-onset diabetes mellitus is an important comorbidity associated with allograft failure. In this study, our aim was to investigate the correlation between hypomagnesemia and preva-lence of posttransplant diabetes mellitus. MATERIALS AND METHODS We retrospectively evaluated 189 pediatric renal transplant recipients who underwent their first renal transplant. A patient with type 1 diabetes mellitus before transplant was excluded from the analysis. Hypomagnesemia was defined as having serum magnesium levels lower than 1.7 mg/dL. Diabetes was defined according to American Diabetes Association criteria. Serum magnesium and glucose levels at month 1 after transplant were recorded. RESULTS We evaluated the records of 188 patients. New-onset diabetes mellitus was diagnosed in 7 patients (3.7%). Hypomagnesemia was shown in 50% of patients (64/120). Mean glucose levels were higher in those with hypomagnesemia than in patients without hypomagnesemia (119.2 vs 91.56 mg/dL, respectively; P = .01) A significant negative correlation was observed between serum magnesium and glucose levels (r = -0.53; P < .05). Mean serum magnesium level in patients with new-onset diabetes mellitus was lower than in patients without diabetes (1.56 ± 0.21 vs 1.75 ± 0.26 mg/dL; P = .052). CONCLUSIONS Hypomagnesemia is a common problem in pediatric renal transplant. The relationship between serum magnesium and glucose levels suggests that hypomagnesemia plays a role in the development of new-onset diabetes mellitus after transplant. Compre-hensive studies are needed to support this association.

OBJECTIVES Lower urinary tract abnormalities have been considered to be contraindications for renal transplant. However, advancements in diagnosis and treatment in this area have allowed renal transplant as a treatment choice in selected cases. We evaluated clinical outcomes of pediatric renal transplant in patients with lower urinary tract dysfunction. MATERIALS AND METHODS We retrospectively evaluated data from 165 pediatric renal transplant patients, and data were compared between patients with and without lower urinary tract dysfunction. Patient demographics, cause of chronic renal failure, acute rejection episodes, and graft loss were included in our analyses. RESULTS Seventeen patients had lower urinary tract dysfunction, and the remaining 148 patients had functional lower urinary tracts. Patients with lower urinary tract dysfunction were younger than the other patient group at diagnosis of chronic renal failure. The mean follow-up after renal transplant in the 2 groups was similar. Differences with regard to donor type, immunosuppressive treatment, and acute rejection episodes were not significant between the 2 groups. Eight patients had lost their grafts during follow-up; however, only 1 of these patients was in the lower urinary tract dysfunction group. Graft loss rate was similar between the groups. CONCLUSIONS Pediatric patients with lower urinary tract dysfunction had similar graft outcomes versus other pediatric renal transplant patients. Careful evaluation and preparation of the lower urinary tract are important factors for renal transplant success.

Background There are few reports about parathyroidectomy due to secondary hyperparathyroidism in patients with end-stage renal failure in the literature. We aimed to evaluate the surgical treatment methods and the results of patients who were operated for secondary hyperparathyroidism with end-stage renal disease in our center. Method Sixteen patients with the diagnosis of secondary hyperparathyroidism were treated surgically in our center. Demographical data, laboratory findings, and imagining methods were all examined. The effect of the Technetium 99m methoxyisobutylisonitrile (Tc-99m-MIBI) probe sensitive to gamma rays detection was also evaluated to locate and identify all the parathyroid glands during the operation. Results Eleven of the patients underwent intravenous (IV) Tc-99m MIBI preoperatively and a gamma probe was detected during surgery. The gamma probe was not used in five patients. Four parathyroid glands were removed in eight (72.7%) out of 11 patients with gamma probes and three parathyroid glands were found in three patients. Total parathyroidectomy and parathyroid autoimplantation were made to eight patients who had removed four glands, subtotal parathyroidectomy was done for the other patients. On a comparison of laboratory findings before and after the surgery, there was a significant relationship between the decrease of serum parathyroid hormone and calcium levels (p

aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow-up period of 58.5 (min-max, 4-94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow-up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti-CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti-C5 therapy and none of those patients experienced aHUS recurrence during a median follow-up period of 21 (min-max, 9-42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow-up. © 2020 Wiley Periodicals LLC

WOS: 000412042200025, PubMed ID: 28566477, We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and, E-Rare (German Ministry of Education and Research); Polish Ministry of Science and Education grant [N402631840]; German Research FoundationGerman Research Foundation (DFG) [Scha 477/11-1]; Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) [108S417]; European Union (EU) Seventh Framework Programme (EURenOmics) grant [2012-305608], The PodoNet project has been made possible by support received from E-Rare (German Ministry of Education and Research), European Union (EU) Seventh Framework Programme (EURenOmics) grant 2012-305608, Polish Ministry of Science and Education grant N402631840, German Research Foundation grant Scha 477/11-1, and Scientific and Technological Research Council of Turkey grant 108S417.

Item does not contain fulltext BACKGROUND: We explored the variation in country mortality rates in the paediatric population receiving renal replacement therapy across Europe, and estimated how much of this variation could be explained by patient-level and country-level factors. METHODS: In this registry analysis, we extracted patient data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry for 32 European countries. We included incident patients younger than 19 years receiving renal replacement therapy. Adjusted hazard ratios (aHR) and the explained variation were modelled for patient-level and country-level factors with multilevel Cox regression. The primary outcome studied was all-cause mortality while on renal replacement therapy. FINDINGS: Between Jan 1, 2000, and Dec 31, 2013, the overall 5 year renal replacement therapy mortality rate was 15.8 deaths per 1000 patient-years (IQR 6.4-16.4). France had a mortality rate (9.2) of more than 3 SDs better, and Russia (35.2), Poland (39.9), Romania (47.4), and Bulgaria (68.6) had mortality rates more than 3 SDs worse than the European average. Public health expenditure was inversely associated with mortality risk (per SD increase, aHR 0.69, 95% CI 0.52-0.91) and explained 67% of the variation in renal replacement therapy mortality rates between countries. Child mortality rates showed a significant association with renal replacement therapy mortality, albeit mediated by macroeconomics (eg, neonatal mortality reduced from 1.31 [95% CI 1.13-1.53], p=0.0005, to 1.21 [0.97-1.51], p=0.10). After accounting for country distributions of patient age, the variation in renal replacement therapy mortality rates between countries increased by 21%. INTERPRETATION: Substantial international variation exists in paediatric renal replacement therapy mortality rates across Europe, most of which was explained by disparities in public health expenditure, which seems to limit the availability and quality of paediatric renal care. Differences between countries in their ability to accept and treat the youngest patients, who are the most complex and costly to treat, form an important source of disparity within this population. Our findings can be used by policy makers and health-care providers to explore potential strategies to help reduce these health disparities. FUNDING: ERA-EDTA and ESPN.

Complement dysregulation is related to different glomerular pathologies. Patients with complement dysregulation have high recurrence risk after transplant; however, with trough-effective therapeutics, renal transplant can be an option for these patients. Here, we present 2 boys with renal disease related to complement dysregulation and their outcomes after renal transplant. Patient 1 had atypical hemolytic uremic syndrome, which was treated with eculizumab before renal transplant; eculizumab therapy was also continued after transplant as preventive therapy. Eculizumab therapy was stopped at year 2 post-transplant. At year 4 post-transplant, his serum creatinine level was 0.87 mg/dL. Patient 2, who had chronic renal disease related to C3 glomerulopathy, was not responsive to eculizumab before renal transplant. At month 4 posttransplant, C3 glomerulopathy recurrence was demonstrated with biopsy, and serum creatinine level was 1.96 mg/dL at this time. Eculizumab was started as a rescue therapy. At year 4 posttransplant, his serum creatinine level was 2.07 mg/dL. In our 2 patients with complement dysregulation, eculizumab was an effective and preventive therapy after renal transplant. However, more studies are needed to understand the long-term efficacy and safety of eculizumab after renal transplant.

Background: Vesicoureteral reflux is the most common urinary congenital anomaly in children. Given the risk associated with radiation exposure there has been an increasing need for radiation-free method in the diagnosis and follow-up of the vesicoureteral reflux. The aim of our study is to compare conventional urosonography without contrast enhancement and x-ray voiding cystourethrography.Patients and Methods: Children with recurrent urinary tract infection with suspected vesicoureteral reflux were included to the study. Vesicoureteral reflux is demonstrated and graded by x-ray voiding cystourethrography. DMSA is used for the evaluation of renal scar. Conventional sonographic procedure was performed in all patients. Ureterovesical junction insertion angle was evaluated. The diameter and length of the ureterovesical junction were also measured.Results: 268 children enrolled to the study. Vesicoureteral reflux was demonstrated in 62 children by x-ray voiding cystourethrography. Ureterovesical junction insertion angle measurement had a statistically significant relation for right and left vesicoureteral reflux presence (right: r: .646, p: .01 and left: r: .446, p: .01). Diagnosis sensitivity of vesicoureteral reflux with conventional ultrasonography is 95.10% and specificity is 81% (Youden’s index 76.1%) for the cutoff value of the ureterovesical junction insertion angle is 28.6 degrees. Positive predictive value is 87.2%, negative predictive value is 94.73% and diagnostic accuracy is 86.29% with conventional ultrasonography.Conclusions: Measurement of ureterovesical junction insertion angle, length and diameter by conventional urosonography is an easy accessible and cheap technique with high sensitivity and specificity for the diagnosis and followup of the vesicoureteral reflux without exposure to ionizing radiation.

PubMedID: 29610995 Background: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. Methods: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. Results: The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. Conclusions: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians. © 2018, IPNA.

Amac: Idrar yolu enfeksiyonu, cocukluk caginin en sik gorulen enfeksiyonlarindan biridir. Piyelonefritin uzun donemde gorulen sekelleri nedeniyle sistitten ayirimi onemlidir. Bu calismanin amaci, cocuklarda 99mTc-Dimerkaptosuksinik Asid ile sintigrafik goruntuleme yapilmaksizin piyelonefrit ayirimini yapmakta prokalsitonin ve C-Reaktif Protein gibi belirteclerin tanisal degerini saptamak, 99mTc-Dimerkaptosuksinik Asid ile sintigrafik goruntuleme yapilacak hastalar konusunda yol gosterici olup olmayacagini belirlemektir. Materyal ve Metod: Bu calismaya, 3 ay ile 16 yas arasinda, ilk kez idrar yolu enfeksiyonu geciren 50 cocuk dahil edilmistir. Retrospektif gozleme dayali bir calismadir. Piyelonefrit tanisi icin serum C-Reaktif Protein ve prokalsitonin duzeylerinin ortanca degeri, duyarlilik, ozgulluk ve cut-off degerleri saptandi. Bulgular: Hastalarin 32’si (%64) piyelonefrit tanisi alirken, 18 (%36) hasta sistit tanisi aldi. Piyelonefrit tanisi icin CReaktif Protein cut-off degeri %69 duyarlilik ve %61 ozgulluk ile 34 mg/L iken, prokalsitonin icin bu deger %69 duyarlilik ve %66 ozgulluk ile 0,23 ng/mL saptandi. Her iki belirtecin birlikte kullanilmasi ise %63 duyarlilik ve %78 ozgulluk ile piyelonefrit tanisini destekledi. Sonuc: Cocuklarda piyelonefrit tanisini koymakta serum prokalsitonin ve C-Reaktif Proteinin birlikte degerlendirilmesi, 99mTc-Dimerkaptosuksinik Asid ile sintigrafik goruntuleme yapilacak hastalarda yol gosterici olabilir. Boylece cocuklar 99mTc-Dimerkaptosuksinik Asid ile sintigrafik goruntulemenin dezavantajlarindan uzak tutulabilir.