Explore the vast range of titles available.

Aim: Urodynamic examination is considered the most reliable, but also a quite invasive tool for the diagnosis of voiding dysfunctions in children. In this study, we evaluated the role of invasive urodynamics in the diagnosis of lower urinary tract dysfunction in children. Methods: One thousand one hundred twenty seven patients underwent urodynamic studies in the pediatric nephrology clinic in Ege University Faculty of Medicine between March 2011 and March 2016. A retrospective analysis of data including symptoms of voiding dysfunction (urinary frequency, urgency, nocturia and/or urge incontinence) and findings of physical examination, urodynamics and ultrasonography was performed. Results: Two hundred and seventy-seven (30.8%) boys and 620 (69.1%) girls with a mean age of 7.52 (±2.6) years underwent urodynamic studies. The most common abnormality was overactive bladder detected in 630 patients (70.2%). 19.9% (n=179) of the participants had dysfunctional voiding, while 9.8% (n=88) had normal results. Conclusion: A small and frequent voiding pattern, enuresis nocturna with daytime symptoms, and postvoid urinary residual volume were the common findings seen in patients with overactive bladder. In addition, dyssynergic voiding and a bladder with large capacity but residual volume after voiding were also commonly found. To that end, we may use ultrasound, clinical examination, symptoms and voiding frequency as first-line diagnostic tools.

Insulin Resistance is common in patients with mild-to-moderate stage chronic kidney disease (CKD), even when the glomerular filtration rate is within the normal range. This study aimed to investigate the association of IR and 25-hydroxyvitamin D levels. In this study, we evaluated the frequency of IR in predialysis patients and the association with 25-hydroxyvitamin (OH)D levels. A total of 32 non-diabetic patients with predialysis were included in the study. Homeostasis model assessment for insulin resistance (HOMA-IR) index was calculated to show presence of IR. Data were analyzed with the Statistical Package for the Social Sciences (SPSS) program version 13.0 (SPSS Inc, Chicago, Ill, USA) using the Chi-square, Student t-test, and logistic regression analysis. Nineteen of 32 subjects (59.4%) were detected to have IR. There was no consistent relationship was detected between IR and glomerular filtration rate, body mass index, and blood pressure in chronic kidney disease (CKD). However; significant difference was detected in 25(OH)D levels with IR in CKD (P = 0.49). The regression analysis showed that 25(OH)D was an independent predictor of IR with an odds ratio of 1.2. In our study, we demonstrated that IR was high in different stages of CKD. Insulin-resistance in non-diabetic CKD was correlated with 25(OH)D levels. 25(OH) vitamin D levels can play a significant role in the development of IR in pediatric patients with CKD. This is important for the early detection and intervention of vitamin D deficiency thereby preventing potentially future complications related to IR.

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.

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.

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.

Knowledge of the distribution spectrum of causative organisms and their resistance patterns has become a core requirement for the rational and effective management of urinary tract infections. In the context of a prospective trial on the use of antibiotic prophylaxis in infants with underling kidney malformations, we conducted an online survey among paediatric nephrologists on positive urine cultures (July 2010–June 2012) from both hospitalized and non-hospitalized infants under 24 months of age. We collected 4745 urine cultures (UCs) at 18 units in 10 European countries. Escherichia coli was the most frequent bacterium isolated from UCs; however, in 10/16 hospitals and in 6/15 community settings, E. coli was isolated in less than 50 % of the total positive UCs. Other bacterial strains were Klebsiella , Enterococcus , Proteus and Pseudomonas not only from hospital settings. E. coli showed a high resistance to amoxicillin and trimethoprim and variable to cephalosporin. Nitrofurantoin had a good rate of efficacy, with 11/16 hospitals and 11/14 community settings reporting a resistance lower than 5 %. Conclusion : E. coli is the most common organism causing UTIs in infants; however, other bacterial strains are frequently isolated. As a result, antibiotic prophylaxis should be more elastic and adaptable over time in order to guarantee maximum efficacy.

The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort. Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group. Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score. Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity.

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

Proteinuria is an important factor for renal injury and prognosis in many diseases. The most valuable method for evaluation of proteinuria is quantitative protein analysis in appropriately 24-h collected urine. But urine collection is difficult and cumbersome especially in children and working adults. The aim of the present study is to define the usefulness of urinary protein/urine osmolality (Uprot/Uosm) ratio in quantification of proteinuria. One hundred and seventy-one patients whose age ranged between 3 and 14 years were included in the study. Uprot/Uosm ( r = 0.85, P  < 0.001) and urinary protein/creatinine Uprot/Ucrea ( r = 0.81 and P  < 0.001) ratios were significantly correlated with 24-h protein excretion. Twenty-four-hour protein excretion was correlated with Uprot/Ucrea ( r = 0.76, P  < 0.001) and Uprot/Uosm ( r = 0.79, P  < 0.001) ratios in proteinuric group. But, there was no correlation between Uprot/Uosm and Uprot/Ucrea ratios with 24-h protein excretion in non-proteinuric group. The positive and negative predictive value of Uprot/Uosm ratio of 0.28 mg/l/mOsm/kg was 89.9 and 90.8% and Uprot/Ucrea ratio of 0.24 mg/mg was 85.7 and 90% for estimating proteinuria (above 4 mg/m 2  h −1 ). The 95th percentile of Uprot/Uosm ratio was 0.25 mg/l/mOsm/kg and Uprot/Ucrea ratio was 0.27 mg/mg in normal group. The best estimate was Uprot/Uosm ratio of 1.42 (sensitivity 100%, specificity 94.9%) and Uprot/Ucrea ratio of 0.75 (sensitivity 100%, specificity 92.9%) for nephrotic proteinuria (40 mg/m 2  h −1 ). Uprot/Uosm which is a reliable and simple method can be used for quantification of proteinuria in pediatric patients with normal renal function. Using Uprot/Uosm ratio for quantification of proteinuria can remove the necessity of 24-h urine collection, urine creatinine measurement and spending additional cost and time if the center measures the urine osmolality routinely in urine analysis.