Acute paediatric kidney replacement therapies in Europe: demographic results from the EurAKId Registry.

BACKGROUND: Acute kidney injury (AKI), particularly that requiring dialysis, is a severe complication in hospitalized children that is associated with high morbidity and mortality. A prospective European AKI registry (EurAKId registry, NCT02960867) was created to describe the epidemiology and outcomes of paediatric patients treated with acute dialysis. METHODS: Children were recruited who were between 0 and 18 years of age and were treated both in and outside the paediatric intensive care unit (PICU) with peritoneal dialysis (PD), haemodialysis (HD) or continuous kidney replacement therapy (CKRT) for AKI or metabolic derangement, fluid overload (FO), sepsis or respiratory distress. Five age groups and 12 categories of primary diseases were defined. RESULTS: Data on 340 patients were analysed, of whom 86% received dialysis for AKI and 14% for reasons other than AKI. Boys accounted for 60% of the patients. Illness severity was greater in children with cardiac and haematologic diseases than those with kidney diseases. Most patients received dialysis in the PICU (84%). The most frequently used dialysis modality was CKRT (64%), followed by PD (14%) and HD (14%). The overall survival rate was 65%. Survival was significantly lower in children with three comorbidities than in children with no comorbidities (41% and 83%; P < 0.001). CONCLUSIONS: The EurAKId registry is the first prospective registry considering paediatric acute kidney replacement therapies (KRTs) in both critical and non-critical care settings, focusing on the three dialysis modalities in Europe. The clinical indications for KRT have expanded; our population was characterized by critically ill patients, primarily boys, who frequently received dialysis in the PICU with CKRT.

Interleukin-8 is increased in chronic kidney disease in children, but not related to cardiovascular disease / A Interleucina-8 é aumentada na doença renal crônica em crianças, mas não se relaciona a doença cardiovascular

Abstract Introduction: In this study, we aimed to detect the cytokine that is involved in the early stage of chronic kidney disease and associated with cardiovascular disease. Methods: We included 50 patients who were diagnosed with predialytic chronic kidney disease and 30 healthy pediatric patients in Ege University Medical Faculty Pediatric Clinic, İzmir/Turkey. Interleukin-8 (IL-8), interleukin-10 (IL-10), interleukin-13 (IL-13), and transforming grow factor-β1 (TGF-β1) levels (pg/mL) were measured by ELISA. Carotid-femoral pulse wave velocity (PWV), augmentation index (Aix), carotid intima media thickness (cIMT), and left ventricular mass index (LVMI) were evaluated as markers of cardiovascular disease. The presence of a cardiovascular disease marker was defined as an abnormality in any of the parameters (cIMT, PWV, Aix, and left ventricular mass index (SVKI)). The patient group was divided into two groups as with and without cardiovascular disease. Results: Mean Aix and PWV values were higher in CKD patients than controls (Aix: CKD 32.8±11.11%, healthy subjects: 6.74±6.58%, PWV CKD: 7.31±4.34m/s, healthy subjects: 3.42±3.01m/s, respectively; p=0.02, p=0.03). The serum IL-8 levels of CKD were significantly higher than of healthy subjects 568.48±487.35pg/mL, 33.67±47.47pg/mL, respectively (p<0.001). There was no statistically significant difference between IL-8, IL-10, IL-13, TGF-1, in CKD patients with and without cardiovascular disease (p> 0.05). Discussion: IL-8 is the sole cytokine that increases in pediatric patients with chronic kidney disease among other cytokines (IL-10, IL-13 and TGF-β1). However, we did not show that IL-8 is related to the presence of cardiovascular disease.

Resumo Introdução: Neste estudo, o objetivo foi detectar a citocina envolvida no estágio inicial da doença renal crônica e associada à doença cardiovascular. Métodos: Incluímos 50 pacientes diagnosticados com doença renal crônica pré-dialítica e 30 pacientes pediátricos saudáveis na Clínica Pediátrica da Faculdade de Medicina, Universidade de Ege, İzmir/Turquia. Níveis de interleucina-8 (IL-8), interleucina-10 (IL-10), interleucina-13 (IL-13), fator de transformação do crescimento -β1 (TGF-β1) (pg/mL) foram medidos por ELISA. Velocidade de onda de pulso carotídeo-femoral (VOP), índice de amplificação (AIx), espessura da camada íntima-média da carótida (cIMT), índice de massa do ventrículo esquerdo (IMVE) foram avaliados como marcadores de doença cardiovascular. A presença de marcador de doença cardiovascular foi definida como uma anormalidade em qualquer dos parâmetros (cIMT, VOP, AIx, índice de massa do ventrículo esquerdo (IMVE)). Os pacientes foram divididos em dois grupos como com e sem doença cardiovascular. Resultados: Valores médios de AIx e VOP foram maiores em pacientes com DRC que nos controles (AIx: DRC: 32,8±11,11%, indivíduos saudáveis: 6,74±6,58%, VOP: DRC: 7,31±4,34m/s, indivíduos saudáveis: 3,42±3,01m/s, respectivamente; p=0,02, p=0,03). Níveis séricos de IL-8 de DRC foram significativamente maiores que de indivíduos saudáveis 568,48±487,35pg/mL, 33,67±47,47pg/mL, respectivamente (p<0,001). Não houve diferença estatisticamente significativa entre IL-8, IL-10, IL-13, TGF-1, em pacientes com DRC com e sem doença cardiovascular (p> 0,05). Discussão: IL-8 é a única citocina que aumenta em pacientes pediátricos com doença renal crônica entre outras citocinas (IL-10, IL-13 e TGF-β1). Entretanto, IL-8 não se associou à presença de doença cardiovascular.

Interleukin-8 is increased in chronic kidney disease in children, but not related to cardiovascular disease.

INTRODUCTION: In this study, we aimed to detect the cytokine that is involved in the early stage of chronic kidney disease and associated with cardiovascular disease. METHODS: We included 50 patients who were diagnosed with predialytic chronic kidney disease and 30 healthy pediatric patients in Ege University Medical Faculty Pediatric Clinic, Izmir/Turkey. Interleukin-8 (IL-8), interleukin-10 (IL-10), interleukin-13 (IL-13), and transforming grow factor-ß1 (TGF-ß1) levels (pg/mL) were measured by ELISA. Carotid-femoral pulse wave velocity (PWV), augmentation index (Aix), carotid intima media thickness (cIMT), and left ventricular mass index (LVMI) were evaluated as markers of cardiovascular disease. The presence of a cardiovascular disease marker was defined as an abnormality in any of the parameters (cIMT, PWV, Aix, and left ventricular mass index (SVKI)). The patient group was divided into two groups as with and without cardiovascular disease. RESULTS: Mean Aix and PWV values were higher in CKD patients than controls (Aix: CKD 32.8±11.11%, healthy subjects: 6.74±6.58%, PWV CKD: 7.31±4.34m/s, healthy subjects: 3.42±3.01m/s, respectively; p=0.02, p=0.03). The serum IL-8 levels of CKD were significantly higher than of healthy subjects 568.48±487.35pg/mL, 33.67±47.47pg/mL, respectively (p<0.001). There was no statistically significant difference between IL-8, IL-10, IL-13, TGF-1, in CKD patients with and without cardiovascular disease (p> 0.05). DISCUSSION: IL-8 is the sole cytokine that increases in pediatric patients with chronic kidney disease among other cytokines (IL-10, IL-13 and TGF-ß1). However, we did not show that IL-8 is related to the presence of cardiovascular disease.

What is The Best Choice in Steroid-Dependent Nephrotic Syndrome: Mycophenolate Mofetil Plus Dexamethasone or Cyclosporine A.

The use of mycophenolatemofetil (MMF) in the treatment of steroid-dependent nephrotic syndrome (SDNS) is beneficial in decreasing the relapse rate and/or steroid dose. The effectiveness and long-term results of MMF/dexamethasone (DEX) in the treatment of SDNS are not well known. In this study, we aimed to determine the efficiency, safety, and long-term results of MMF/DEX in patients with SDNS in comparison with cyclosporine A (CsA) in a retrospective single-center trial. Between January 2009 and December 2015, 54 SDNS patients were treated with either MMF/DEX (n = 29) or CsA (n = 25). Relapse rates, relapse-free time, cumulative exposure to corticosteroids, proteinuria, and estimated glomerular filtration rate (eGFR) were retrospectively evaluated at 0, 3, 6, 12, 24, and 36 months after the initiation of treatment. The mean cumulative exposure to corticosteroids for the MMF/DEX and CsA groups was 72.40 ± 71.85 mg/kg/year and 122.31 ± 74.35 mg/kg/year, respectively. There was a significant decrease in the cumulative exposure to corticosteroids in the MMF/DEX group (Z = 3.869; P <0.001). While the mean annual relapse for the MMF/DEX group was 1.07 ± 0.25, it was 1.70 ± 1.01 in the CsA group, and this difference was statistically significant (Z = 1.968; P = 0.049). Relapse-free time for the 1st, 2nd, and 3rd years compared between the MMF/DEX and CsA groups was 9.57 ± 2.58 versus 6.38 ± 2.43, 10.27 ± 1.98 versus 8.28 ± 2.28, and 9.67 ± 2.06 versus 6.52 ± 3.04, respectively. The difference was significantly higher in favor of MMF/DEX (between-subject effects F = 48.352; P<0.001). Both eGFR and proteinuria significantly changed over time. However, there was no significant difference between the groups until the later time points of the follow-up. The difference became evident only at the 2nd-and 3rd-year measurements. MMF/DEX seems superior to CsA in preventing relapses and reducing cumulative exposure to cortico-steroids. Thus, it may be considered a treatment option in children with SDNS.

Regulatory T-cell Changes in Patients with Steroid-Resistant Nephrotic Syndrome after Rituximab Therapy.

The aim of this study was to investigate the effect of rituximab (RTX) treatment on serum immunoglobulin (Ig) A, G, M levels, and B and CD4+CD25+FoxP3+ [T regulatory (Treg)] cell numbers in children who received RTX therapy with steroid-resistant nephrotic syndrome (SRNS). Twenty-three SRNS children who received RTX and 20 healthy children in the control group were included. In this cross-sectional cohort study, 23 children with SRNS levels were determined before and one month after RTX treatment by serum IgA, IgG, IgM, and percentages of CD4+CD25+ FoxP3+ cells and B CD19+ cells by flow cytometry (FASCalibur). RTX was administered at a total of four doses of 375 mg/m2/week. Before RTX treatment, percentages of Treg and IgG values were significantly lower in the SRNS group compared to the control group, respectively (P = 0.001). B-cells were significantly lower one month after RTX treatment than before RTX treatment, respectively (P = 0.001). One month after RTX treatment percentages of Tregs, it was found to be significantly higher than before treatment level (P = 0.001). Seventy percent (11/23) remission was achieved with RTX treatment. RTX treatment not only depletes the number of B-cells in SRNS patients but also causes an increase in the number of percentages of Treg cells.

Active vitamin D is cardioprotective in experimental uraemia but not in children with CKD Stages 3-5.

BACKGROUND: Uraemic cardiac remodelling is associated with vitamin D and Klotho deficiency, elevated fibroblast growth factor 23 (FGF23) and activation of the renin-angiotensin system (RAS). The cardioprotective properties of active vitamin D analogues in this setting are unclear. METHODS: In rats with 5/6 nephrectomy (5/6Nx) treated with calcitriol, the cardiac phenotype and local RAS activation were investigated compared with controls. A nested case-control study was performed within the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study, including children with chronic kidney disease (CKD) Stages 3-5 [estimated glomerular filtration rate (eGFR) 25 mL/min/1.73 m2] treated with and without active vitamin D. Echocardiograms, plasma FGF23 and soluble Klotho (sKlotho) were assessed at baseline and after 9 months. RESULTS: In rats with 5/6Nx, left ventricular (LV) hypertrophy, LV fibrosis and upregulated cardiac RAS were dose-dependently attenuated by calcitriol. Calcitriol further stimulated FGF23 synthesis in bone but not in the heart, and normalized suppressed renal Klotho expression. In the 4C study cohort, treatment over a mean period of 9 months with active vitamin D was associated with increased FGF23 and phosphate and decreased sKlotho and eGFR compared with vitamin D naïve controls, whereas LV mass index did not differ between groups. CONCLUSIONS: Active vitamin D ameliorates cardiac remodelling and normalizes renal Klotho expression in 5/6Nx rats but does not improve the cardiac phenotype in children with CKD Stages 3-5. This discrepancy may be due to further enhancement of circulating FGF23 and faster progression of CKD associated with reduced sKlotho and higher serum phosphate in vitamin D-treated patients.

Membranous nephropathy in a child with crescentic glomerulonephritis: Coincidence or comorbidity?

Rapidly progressive glomerulonephritis (RPGN) is rare syndrome in children, characterized by clinical features of glomerulonephritis and rapid loss of renal function, and is associated with crescentic glomerulonephritis. Primary membranous nephropathy (MN) is an immune-complex-mediated cause of the adult nephrotic syndrome but occurs less frequently in children. RPGN is rarely observed in adults with primary MN. In this article, we report a case of MN, which developed during long-term follow-up of previously treated RPGN. Our case may be the first to demonstrate primary MN and crescentic glomerulonephritis in a child. We would like to underline the importance of not dropping the long-term follow-up of cases with primary RPGN (not accompanied by other glomerulonephritis and vasculitis symptoms) who had improved with treatment.

Genetics and outcome of atypical hemolytic-uremic syndrome in Turkish children: a retrospective study between 2010 and 2017, a single-center experience.

INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. In the recent years several studies have been published describing these mutations. In this study, the initial clinical findings, treatments and long-term follow-up results of 19 patients who were hospitalized with the diagnosis of aHUS were presented. MATERIALS AND METHODS: Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. Disease causing complement factor H (CFH) mutations were determined.  Results. CFH mutations were detected in 5 of 19 aHUS cases. Of these, one was novel and 4 were previously reported. We reported here the clinical course of aHUS patients with CFH mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys) which caused  previously defined aHUS. Two of the CFH mutation cases developed end stage kidney disease that required hemodialysis, 1 case developed chronic kidney disease. Two cases were in remission, one of them with supportive therapy and the other case was in remission with eculizumab treatment. CONCLUSIONS: Morbidity rate is higher in children with aHUS. The renal prognosis and morbidity rate is higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS children with CFH mutation depends on the genetic background.

Determinants of Statural Growth in European Children With Chronic Kidney Disease: Findings From the Cardiovascular Comorbidity in Children With Chronic Kidney Disease (4C) Study.

Failure of statural growth is one of the major long-term sequelae of chronic kidney disease (CKD) in children. In recent years effective therapeutic strategies have become available that lead to evidence based practice recommendations. To assess the current growth performance of European children and adolescents with CKD, we analyzed a cohort of 594 patients from 12 European countries who were followed prospectively for up to 6 years in the 4C Study. While all patients were on conservative treatment with a mean estimated glomerular filtration rate of 28 ml/min/1.73 m2 at study entry, 130 children commenced dialysis during the observation period. At time of enrolment the mean height standard deviation score (SDS) was -1.57; 36% of patients had a height below the third percentile. The prevalence of growth failure varied between countries from 7 to 44% Whereas patients on conservative treatment showed stable growth, height SDS gradually declined on those on dialysis. Parental height, pubertal status and treatment with recombinant growth hormone (GH) were positively, and the diagnosis of syndromic disease and CKD stage were negatively associated with height SDS during the observation period. Unexpectedly, higher body mass index (BMI) SDS was associated with lower height SDS both at enrolment and during follow up. Renal anemia, metabolic acidosis, and hyperparathyroidism were mostly mild and not predictive of growth rates by multivariable analysis. GH therapy was applied in only 15% of growth retarded patients with large variation between countries. When adjusting for all significant covariates listed above, the country of residence remained a highly significant predictor of overall growth performance. In conclusion, growth failure remains common in European children with CKD, despite improved general management of CKD complications. The widespread underutilization of GH, an approved efficacious therapy for CKD-associated growth failure, deserves further exploration.

Acute dialysis in children: results of a European survey.

The number of children with acute kidney injury (AKI) requiring dialysis is increasing. To date, systematic analysis has been largely limited to critically ill children treated with continuous renal replacement therapy (CRRT). We conducted a survey among 35 European Pediatric Nephrology Centers to investigate dialysis practices in European children with AKI. Altogether, the centers perform dialysis in more than 900 pediatric patients with AKI per year. PD and CRRT are the most frequently used dialysis modalities, accounting for 39.4% and 38.2% of treatments, followed by intermittent HD (22.4%). In units treating more than 25 cases per year and in those with cardiothoracic surgery programs, PD is the most commonly chosen dialysis modality. Also, nearly one quarter of centers, in countries with a gross domestic product below $35,000/year, do not utilize CRRT at all. Dialysis nurses are exclusively in charge of CRRT management in 45% of the cases and pediatric intensive care nurses in 25%, while shared management is practiced in 30%. In conclusion, this survey indicates that the choice of treatment modalities for dialysis in children with AKI in Europe is affected by the underlying ethiology of the disease, organization/set-up of centers and socioeconomic conditions. PD is utilized as often as CRRT, and also intermittent HD is a commonly applied treatment option. A prospective European AKI registry is planned to provide further insights on the epidemiology, management and outcomes of dialysis in pediatric AKI.

Effects of Hemodiafiltration versus Conventional Hemodialysis in Children with ESKD: The HDF, Heart and Height Study.

BACKGROUND: Hypertension and cardiovascular disease are common in children undergoing dialysis. Studies suggest that hemodiafiltration (HDF) may reduce cardiovascular mortality in adults, but data for children are scarce. METHODS: The HDF, Heart and Height study is a nonrandomized observational study comparing outcomes on conventional hemodialysis (HD) versus postdilution online HDF in children. Primary outcome measures were annualized changes in carotid intima-media thickness (cIMT) SD score and height SD score. RESULTS: We enrolled 190 children from 28 centers; 78 on HD and 55 on HDF completed 1-year follow-up. The groups were comparable for age, dialysis vintage, access type, dialysis frequency, blood flow, and residual renal function. At 1 year, cIMT SD score increased significantly in children on HD but remained static in the HDF cohort. On propensity score analysis, HD was associated with a +0.47 higher annualized cIMT SD score compared with HDF. Height SD score increased in HDF but remained static in HD. Mean arterial pressure SD score increased with HD only. Factors associated with higher cIMT and mean arterial pressure SD-scores were HD group, higher ultrafiltration rate, and higher ß2-microglobulin. The HDF cohort had lower ß2-microglobulin, parathyroid hormone, and high-sensitivity C-reactive protein at 1 year; fewer headaches, dizziness, or cramps; and shorter postdialysis recovery time. CONCLUSIONS: HDF is associated with a lack of progression in vascular measures versus progression with HD, as well as an increase in height not seen in the HD cohort. Patient-related outcomes improved among children on HDF correlating with improved BP control and clearances. Confirmation through randomized trials is required.

Association of Vitamin D Deficiency with Increased Pulse Wave Velocity and Augmentation Index in Children With Chronic Kidney Disease.

INTRODUCTION: It is known that in children with chronic kidney disease (CKD), cardiovascular damage starts in the form of arterial stiffness. There are risk factors other than the traditional ones such as arterial stiffness hypertension, obesity, hypercholesterolemia, and insulin resistance. Vitamin D deficiency is rather common in CKD, and it was introduced as a risk factor for atherosclerosis; however, its relationship with arterial stiffness is not known completely. The purpose of this study was to research the relationship between 25-hydroxyvitamin D levels and arterial stiffness. MATERIALS AND METHODS: Arterial stiffness was evaluated by measuring augmentation index (AI) and pulse wave velocity (PWV) from the radial and carotid arteries with a Vicorder. The 25-hydroxyvitamin D levels were measured by an immunoassay method. RESULTS: In the 81 CKD patients (mean age, 13.21 ± 6.02 years; mean body mass index, 19.42 ± 5.12 kg/m2; and 56.8% male), the mean vitamin D level was 60.71 ± 39.52 ng/mL, the mean AI was 7.93 ± 7.77%, and the mean PWV was 9.79 ± 4.36 m/s. Serum levels of 25-hydroxyvitamin D was correlated with AI (r = -0.482, P = 0.001) and PWV (r = -0.57, P = .001). CONCLUSIONS: In this study, it was proven that vitamin D deficiency in children was related to nondiabetic and nondialysis CKD.

Effect of haemodiafiltration vs conventional haemodialysis on growth and cardiovascular outcomes in children - the HDF, heart and height (3H) study.

BACKGROUND: Cardiovascular disease is prevalent in children on dialysis and accounts for almost 30% of all deaths. Randomised trials in adults suggest that haemodiafiltration (HDF) with high convection volumes is associated with reduced cardiovascular mortality compared to high-flux haemodialysis (HD); however paediatric data are scarce. We designed the haemodiafiltration, heart and height (3H) study to test the hypothesis that children on HDF have an improved cardiovascular risk profile, growth and nutritional status and quality of life, compared to those on conventional HD. We performed a non-randomised parallel-arm intervention study within the International Paediatric Haemodialysis Network Registry comparing children on HDF and conventional HD to determine annualised change in cardiovascular end-points and growth. Here we present the 3H study design and baseline characteristics of the study population. METHODS: 190 children were screened and 177 (106 on HD and 71 on HDF) recruited from 28 centres in 10 countries. There was no difference in age, underlying diagnosis, comorbidities, previous dialysis therapy, dialysis vintage, residual renal function, type of vascular access or blood flow between HD and HDF groups. High flux dialysers were used in 63% of HD patients and ultra-pure water was available in 52%. HDF patients achieved a median convection volume of 13.3 L/m2; this was associated with the blood flow rate only ((p = 0.0004, r = 0.42) and independent of access type (p = 0.38). DISCUSSION: This is the largest study on dialysis outcomes in children that involves deep phenotyping across a wide range of cardiovascular, anthropometric, nutritional and health-related quality of life measures, to test the hypothesis that HDF leads to improved cardiovascular and growth outcomes compared to conventional HD. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02063776 . The trial was prospectively registered on the 14 Feb 2014.

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

OBJECTIVE: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. STUDY DESIGN: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. RESULTS: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. CONCLUSIONS: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

Urine Flow Cytometry in the Diagnosis of Urinary Tract Infection.

OBJECTIVES: To determine the cut-off value of bacteria with urine flow cytometer in diagnosing urinary tract infection. METHODS: From a total of 546 patients admitted to the hospital with urinary tract infection (UTI), two urine samples were obtained for each patient. Urine samples in sterile containers were divided into two; first for urine culture and second for simultaneous measurement with UF-1000i flow cytometry device. The presence of a single type of bacteria over 103 CFU/mL in urine culture was accepted as UTI. RESULTS: Of 546 patients, 210 (38.5%) were boys and 336 (61.5%) were girls. There was no growth in 489 (89.5%) of the urine specimens and 57 (10.4%) samples showed growth (>103 CFU/ml). A threshold of 10 bacteria/µL in flow cytometry (sensitivity = 100%) showed the best compatibility with culture. Diagnostic values in terms of sensitivity (100%), specificity (43.5%), negative predictive value (100%) and positive predictive value (17.7%) were satisfactory. CONCLUSIONS: The UF-1000i flow cytometer can give results quickly and exclude UTI using the determined number of cut-off bacteria at low cost. Thus, if Sysmex UF-1000i is used, quick and accurate results can be obtained and unnecessary laboratory tests can be prevented. Also, patient convenience can be increased.

Comparing different estimated glomerular filtration rate equations in assessing glomerular function in children based on creatinine and cystatin C.

BACKGROUND: Glomerular filtration rate (GFR) is the best marker used to assess renal function. Estimated GFR (eGFR) equations have been developed, and the ideal formula is still under discussion. We wanted to find the most practical and reliable GFR in eGFR formulas. We compared serum creatinine (Scr)- and cystatin C (cysC)-based eGFR formulas in the literature. We also aimed to determine the suitability and the reliability of cysC for practical use in determining GFR in children. METHODS: We have enrolled 238 children in the study. Measurement of 24-hour creatinine clearance was compared with eGFR equations which are based on Scr, cysC, and creatinine plus cysC. RESULTS: Of the patients (n = 238), 117 were males (49.2%), and 121 (50.8%) were females with a median age of 9.0 years. The areas under the ROC curves of Counahan-Barratt and Bedside Schwartz were equal and 0.89 (with a 95% CI 0.80-0.97). The areas under the ROC curves were not significantly different in all cystatin C-based eGFR equations. The highest AUC values for differentiating normal vs abnormal renal functions according to CrCl24 were for the CKiD-cysC and CKiD-Scr-cysC equations. CONCLUSIONS: In our study, compared with creatinine-based ones, the cystatin C-based formulas did not show much superiority in predicting eGFR. Still, we think Bedside Schwartz is a good formula to provide ease of use because, in this equation, the constant k is same for all age groups. However, the most valuable equations in determining chronic kidney disease are the CKiD-cysC and CKiD-Scr-cysC equations.

A novel mutation of interleukin-1 receptor antagonist (IL1RN) in a DIRA patient from Turkey: Diagnosis and treatment.

Sözeri B, Gerçeker-Türk B, Yildiz-Atikan B, Mir S, Berdeli A. A novel mutation of interleukin-1 receptor antagonist (IL1RN) in a DIRA patient from Turkey: Diagnosis and treatment. Turk J Pediatr 2018; 60: 588-592. Autoinflammatory diseases can cause severe inflammation in bone and skin such as neonatal-onset multisystem inflammatory disease (NOMID), Majeed syndrome, interleukin-36 receptor antagonist deficiency (DITRA) and deficiency of interleukin-1 (IL-1) receptor antagonist (DIRA) syndrome. Here we report a five-year old boy who was admitted to the hospital with pustular skin lesions and fever in the first month of his life. Molecular analysis of IL1RN gene revealed a single homozygous C nucleotide deletion at nucleotide position 396 (p.Thr133Profs*118). The novel p.Thr133Profs*118 mutation found in our study caused frameshift mutation and as a result, the respective protein is most likely non-functional. The patient, who received a variety of treatments for various preliminary diagnoses until the final diagnosis (DIRA), was treated with recombinant IL-1Ra, anakinra, and experienced significant clinical improvement.

Evaluation of development of subclinical atherosclerosis in children with uveitis.

Uveitis is a chronic inflammatory disease. Chronic inflammation has been shown to have a role in pathogenesis of atherosclerosis. Atherosclerosis is the most important risk factor of cardiovascular diseases and is shown to start as early as childhood. In this study, we investigated the presence of subclinical atherosclerosis in children with uveitis. Seventy five patients who were diagnosed as having uveitis in ophthalmology and pediatric rheumatology clinics were included in the study. Patients with hypertension, obesity, dyslipidemia, diabetes, and with history of early cardiovascular disease were excluded. Arterial stiffness, carotid-femoral pulse wave velocity (PWV), augmentation index (AIx), and carotid artery intima-media thickness (cIMT) were measured for each patient. These measurements were compared with 50 healthy children with similar age and sex as controls. The mean age of patients in this study was 12.24 ± 2.69 years, and the mean age of controls was 11.32 ± 4.52 years. PWV and AIx values were higher in the patient group (p = 0.04, p = 0.03). cIMT levels were not different in patient and control groups. When patients were grouped as having uveitis for more than 5 years or not, patients with longer duration of uveitis had higher PWV, AIx, and cIMT levels (p values were 0.01, 0.02, and 0.04 respectively). Vascular functions deteriorate first with endothelial damage in children with uveitis and as disease continues, increase in cIMT is added. We think that for follow-up of the disease and evaluation of the treatment, non-invasive subclinical atherosclerosis markers should be used along with activation criteria of primary diseases.

Early Effects of Renal Replacement Therapy on Cardiovascular Comorbidity in Children With End-Stage Kidney Disease: Findings From the 4C-T Study.

BACKGROUND: The early impact of renal transplantation on subclinical cardiovascular measures in pediatric patients has not been widely investigated. This analysis is performed for pediatric patients participating in the prospective cardiovascular comorbidity in children with chronic kidney disease study and focuses on the early effects of renal replacement therapy (RRT) modality on cardiovascular comorbidity in patients receiving a preemptive transplant or started on dialysis. METHODS: We compared measures indicating subclinical cardiovascular organ damage (aortal pulse wave velocity, carotid intima media thickness, left ventricular mass index) and evaluated cardiovascular risk factors in 166 pediatric patients before and 6 to 18 months after start of RRT (n = 76 transplantation, n = 90 dialysis). RESULTS: RRT modality had a significant impact on the change in arterial structure and function: compared to dialysis treatment, transplantation was independently associated with decreases in pulse wave velocity (ß = -0.67; P < 0.001) and intima media thickness (ß = -0.40; P = 0.008). Independent of RRT modality, an increase in pulse wave velocity was associated with an increase in diastolic blood pressure (ß = 0.31; P < 0.001). Increasing intima media thickness was associated with a larger increase in body mass index (ß = 0.26; P = 0.003) and the use of antihypertensive agents after RRT (ß = 0.41; P = 0.007). Changes in left ventricular mass index were associated with changes in systolic blood pressure (ß = 1.47; P = 0.01). CONCLUSIONS: In comparison with initiating dialysis, preemptive transplantation prevented further deterioration of the subclinical vascular organ damage early after transplantation. Classic cardiovascular risk factors, such as hypertension and obesity are of major importance for the development of cardiovascular organ damage after renal transplantation.