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Mortality in children with kidney failure is higher in girls than boys with cardiovascular complications representing the most common causes of death. Pulse wave velocity (PWV), a measure of vascular stiffness, predicts cardiovascular mortality in adults. Here, PWV in children with kidney failure undergoing kidney replacement therapy was investigated to determine sex differences and potential contributing factors. Two-hundred thirty-five children (80 girls; 34%) undergoing transplantation (150 pre-emptive, 85 with prior dialysis) having at least one PWV measurement pre-and/or post-transplantation from a prospective cohort were analyzed. Longitudinal analyses (median/maximum followup time of 6/9 years) were performed for PWV z-scores (PWVz) using linear mixed regression models and further stratified by the categories of time: pre-kidney replacement therapy and post-transplantation. PWVz significantly increased by 0.094 per year and was significantly higher in girls (PWVz + 0.295) compared to boys, independent of the underlying kidney disease. During pre-kidney replacement therapy, an average estimated GFR decline of 4 ml/min/1.73 m(2) per year was associated with a PWVz increase of 0.16 in girls only. Higher diastolic blood pressure and low density lipoprotein were independently associated with higher PWVz during pre-kidney replacement therapy in both sexes. In girls post-transplantation, an estimated GFR decline of 4ml/min/1.73m(2) per year pre-kidney replacement therapy and a longer time (over 12 months) to transplantation were significantly associated with higher PWVz of 0.22 and of 0.57, respectively. PWVz increased further after transplantation and was positively associated with time on dialysis and diastolic blood pressure in both sexes. Thus, our findings demonstrate that girls with advanced chronic kidney disease are more susceptible to develop vascular stiffening compared to boys, this difference persist after transplantation and might contribute to higher mortality rates seen in girls with kidney failure., German Federal Ministry of Education and Research [01EO0802]; European Renal Association -European Dialysis and Transplant Association; Roche Organ Transplant Research Foundation [365520785], This study was made possible by grants from the German Federal Ministry of Education and Research (#01EO0802), the European Renal Association -European Dialysis and Transplant Association (www.eraedta.org), and Roche Organ Transplant Research Foundation (#365520785). Several coauthors are members of the European Rare Kidney Disease Reference Network (ERKNet). This study has been presented as an abstract at the TTS (The Transplantation Society) 2020 Virtual Congress on September 14, 2020.

© 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35 months (0.4–231 months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3 mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12 months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year’s follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF (− ) stones than in MRF (+) stones. However, remission rate with medical treatment was higher in cases with MRF (+) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.

Introduction. Bartter syndrome (BS) is a group of autosomal-recessive tubular disorders and it is classified into five genetic subtypes. BS can also be classified by phenotype (antenatal, classic). Patients with mutations in the same gene can present different phenotypes. In the present study, target gene sequencing was performed to evaluate the genotype-phenotype relationship. Methods. Biochemical, clinical and renal ultrasonography results were collected at presentation and the last clinic visit. Genetic analyses were performed. The findings of patients with classical BS (cBS) and antenatal BS (aBS) at presentation and the last visit were compared. Results. Our study included 21 patients (12 female, 57.1%) from 20 families with BS. The median age at diagnosis was 8 months and the median follow-up period was 39 months. The most frequent complaint was growth failure. We have found 18 different types of mutations in four genes, including nine in the CLCNKB gene, seven in the SLCA12A1 gene, one in the KCNJ1 gene and one in the BSND gene. In ten patients, nine different types of CLCNKB gene mutations were detected, five of them were novel. Seven different mutations in the SLC12A1 gene were detected in eight patients, five of them were novel. Compared to patients with aBS and cBS, prematurity was significantly higher in the group with aBS. Nephrocalcinosis was present in only one patient with cBS, all the ten hypercalciuric patients with aBS had nephrocalcinosis at the time of diagnosis and the last visit. The mean height standard deviation score (SDS) of patients with aBS were significantly lower than the cBS group at the time of presentation. The mean weight SDS at the time of presentation was worse in patients with aBS than in patients with cBS. The mean plasma potassium and chloride concentrations were significantly lower in the patients with cBS at the time of diagnosis. Conclusions. This investigation revealed the mutation characteristics and phenotype-genotype relationship of our patients and provided valuable data for genetic counseling.

Various molecular and cellular processes are involved in renal fibrosis, such as oxidative stress, inflammation, endothelial cell injury, and apoptosis. Heat shock proteins (HSPs) are implicated in the progression of chronic kidney disease (CKD). Our aim was to evaluate changes in urine and serum HSP levels over time and their relationships with the clinical parameters of CKD in children. In total, 117 children with CKD and 56 healthy children were examined. The CKD group was followed up prospectively for 24 months. Serum and urine HSP27, HSP40, HSP47, HSP60, HSP70, HSP72, and HSP90 levels and serum anti-HSP60 and anti-HSP70 levels were measured by ELISA at baseline, 12 months, and 24 months. The urine levels of all HSPs and the serum levels of HSP40, HSP47, HSP60, HSP70, anti-HSP60, and anti-HSP70 were higher at baseline in the CKD group than in the control group. Over the months, serum HSP47 and HSP60 levels steadily decreased, whereas HSP90 and anti-HSP60 levels steadily increased. Urine HSP levels were elevated in children with CKD; however, with the exception of HSP90, they decreased over time. In conclusion, our study demonstrates that CKD progression is a complicated process that involves HSPs, but they do not predict CKD progression. The protective role of HSPs against CKD may weaken over time, and HSP90 may have a detrimental effect on the disease course. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12192-021-01239-9.

Tam Metin / Full Text Q4 PMID: 34174796 WOS:000724833600003 SCI-Expanded Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. Materials and methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010–2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. Results: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5–208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. Conclusion: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease. Keywords: Bartter syndrome, hypercalciuria, nephrocalcinosis, renal tubular acidosis

Background/aim: this study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or -resistant) and the dosing regimen. Materials and methods: this multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m(2)) or high (2-4 doses of 375 mg/m(2)) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9-17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 +/- 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: the current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined., NA

Background: Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, COL4A4, or COL4A5. Associations between clinical manifestations and genotype are not yet well defined. Our study aimed to define clinical and genetic characteristics, establish genotype–phenotype correlations, and determine prognosis of AS in children. Methods: A total of 87 children with AS from 10 pediatric nephrology centers, whom had genetic analyses performed at the Hacettepe University Nephrogenetics Laboratory between February 2017 and February 2019, were included. Data regarding demographics, family history, clinical and laboratory characteristics, histopathological and genetic test results, treatments, and yearly follow-up results were retrospectively analyzed. Results: Of 87 patients, 16% presented with nephrotic syndrome. In patients with nephrotic syndrome, kidney biopsy findings showed focal segmental glomerulosclerosis (FSGS) in 79%, and COL4A3 mutations were the leading genetic abnormality (50%). Twenty-four percent of all patients progressed to chronic kidney disease (CKD). The rate of progression to CKD and the decline in the glomerular filtration rate of the patients with COL4A3 mutation were higher than other mutation groups (p ' 0.001 and p = 0.04, respectively). In kidney survival analysis, nephrotic syndrome presentation, histopathology of FSGS, COL4A3 mutations, and autosomal recessive inheritance were found as independent risk factors for earlier progression to CKD. Cyclosporin A treatment did not improve kidney survival. Conclusions: We emphasize that genetic testing is important for patients suspected as having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid-resistant nephrotic syndrome. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications. [Figure not available: see fulltext.]. © 2020, IPNA.

Background Sclerostin and osteoprotegerin (OPG) are new markers of chronic kidney disease (CKD) mediated mineral bone disease (CKD-MBD) which were extensively evaluated in adult population. We aimed to evaluate the associations between serum levels of sclerostin/OPG and parameters of bone turnover and compare the serum levels of sclerostin/OPG in different stages of CKD in children. Methods 70 children with CKD stage 1-5, aged 2-21 years were examined. Serum levels of alkaline phosphatase (ALP), creatinine, total calcium, phosphorus , intact parathyroid hormone (iPTH) and vitamin D were measured. Serum sclerostin and OPG levels were measured in children with different levels of CKD stage and their association with bone turnover parameters were noted. Results We did not observe any significant correlation between serum levels of sclerostin and OPG and stages of CKD. A negative relationship was present between serum sclerostin and 25-OH vitamin D levels. Osteoprotegerin was positively and significantly correlated with ALP but serum sclerostin was negatively correlated with ALP. Conclusion Our study, which includes only children and adolescents with a growing skeleton under uremic conditions and excluding diabetes and atherosclerosis interference, is very valuable. We couldn't find any significant relationship between either sclerostin or OPG levels among different stages of CKD. Also our study demonstared a strong negative relationship between ALP and sclerostin levels and a strong positive relationship between ALP and OPG levels, reminding the importance of ALP levels to predict the bone-mineral status of the children with CKD.

Summary Introduction Damage to the glycosaminoglycan layer of the urothelium, which is composed of hyaluronic acid (HA), may increase the possibility of bacterial adherence and infections. Patients with neurogenic bladder (NB) who perform clean intermittent catheterization (CIC) 4–6 times a day are also under great risk for recurrent urinary tract infections (RUTIs). Objective The aim of this study was to assess the efficacy and safety of intravesical HA in reducing the frequency of RUTIs in patients with spina bifida (SB) and NB, who perform CIC. Materials and methods Ten patients (nine girls, one boy) with SB and NB affected by RUTIs received intravesical instillation of HA. Ten patients (seven girls, three boys) with SB and NB who did not accept the intravesical HA therapy were included in the control group. All patients developed symptomatic RUTIs, which occurred at least three times in the previous 12 months. The study group was treated with intravesical 40 mg HA (Hyacyst®) weekly for four weeks, then monthly for the consequent three months. Recurrence of UTIs before and after the treatment was analyzed. Results The mean age of the study group and the controls were 11.1 ± 4.8 (3.2–18.6) and 9.3 ± 5.4 (2.1–16.2) years, respectively. The mean UTIs per patient-month in the study group and the controls were 0.34 ± 0.05 and 0.35 ± 0.06, respectively. The mean follow-up time after the treatment was 16.6 ± 6.9 months in the study group and 16 ± 6.1 months in the controls. The mean UTIs per patient-month significantly decreased in the study group after the treatment (p Discussion To the best of the authors' knowledge, this study is the first one evaluating the efficacy of intravesical HA in the treatment of RUTIs in children with SB and NB. However, this study has several limitations, such as the small sample size and short follow-up time. Conclusion The findings of the present study indicate that intravesical HA is an effective and safe treatment that reduces RUTIs in patients with SB and NB, who perform CIC. Summary Table . Data of study and control groups Study Group Control Group p Age (years) 11.1 ± 4.8 9.3 ± 5.4 0.44 Gender (F/M) 9/1 7/3 0.26 UTI ppm before HA 0.34 ± 0.059 0.35 ± 0.06 0.77 UTI ppm after HA 0.11 ± 0.07 0.39 ± 0.07 Follow-up time (month) 16.6 ± 6.9 16 ± 6.1 0.83 Serum creatine (mg/dl) 0.33 ± 0.07 0.41 ± 0.14 0.13 UTI: urinary tract infection, F: female, M: male, ppm: per patient-month, HA: hyaluronic acid.

PubMedID: 30607566 Objective: Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS). Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically. Results: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ? 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ? 2, compared to patients with FFS < 2. Patients with FFS ? 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007). Conclusions: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis. © 2019, IPNA.

The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic nephrotic syndrome (NS) treated with 2 mg/kg/day prednisolone and healthy control (HC) were enrolled. Blood samples were drawn for immunologic analyses at baseline and at the first and second weeks and first, second, and third months of CS therapy in addition to first and second weeks and first, second, and third months of discontinuation. Fourteen patients (M/F, 7/7) between 1 and 8 years old were evaluated. Untreated NS exhibited high absolute lymphocyte count (ALC)(p = 0.010), absolute CD3(+) T cells (p = 0.020) and absolute CD8(+) T cells (p = 0.006) compared to HC. Suppression in ALC was observed and nadir value was noted at first month of therapy compared to baseline (p = 0.002). The CD4(+) (p = 0.036) and CD8(+) T cell (p = 0.013) counts decreased significantly at the first week of treatment compared to baseline. While baseline B cell counts was indifferent from HC, gradually increased in 2 weeks of CS initiation and decreased during the treatment with a statistical significance compared to HC (p = 0.010). However, after cessation of CS, B cell counts continued to decline and found to be significantly different than baseline at first week (p = 0.008) and at third month (p = 0.040).Apart from baseline lymphocyte subset changing observed in untreated NS patients, our data implies that T cells were suppressed very early in the CS treatment. Interestingly, depressed B cell counts were detected later but persisted even after CS cessation. Due to early decrease in T cells, it would be beneficial to assume the patients as immunosuppressed at the very beginning of CS treatment to avoid infections.• Corticosteroids (CSs) are widely used for a variety of diseases including nephrotic syndrome, which is related with complex immune disturbance including T and B cells dysfunctions. • CSs induce neutrophilic leukocytosis concomitant with lymphopenia and eosinopenia leading to immunosupression. What is New: • T cell subsets and proliferation are susceptible to CSs more than B cells; however, the reversibility is faster with dose reduction in CS. • The change of B cells and B cell subtypes (CD27 (+) memory) shows prolonged effect of CSs on B cells which may alter antibody production even after 3 months of CSs cessation.

Background/aim: We analyzed the clinical and pathological features and prognosis of 106 children with persistent asymptomatic microscopic hematuria (PAMH) with or without mild proteinuria. Materials and methods: This was a retrospective study of 106 children who were referred to our clinics from 2000 to 2013 for evaluation of PAMH. Results: Among the 106 patients, 69 (65%) were female and 37 (35%) were male. The patients were divided into two groups: 101 patients with isolated microscopic hematuria (IMH) and 5 patients with asymptomatic microscopic hematuria and mild proteinuria (AMHP). Renal biopsy was performed in all 5 children with AHMP: 2 patients had hereditary nephropathy and 2 patients had focal segmental glomerulosclerosis (FSGS). One biopsy specimen revealed nonspecific findings. Renal biopsy was performed in 9 children with IMH: 4 patients had hereditary nephropathy and 5 patients had nonspecific findings. None of the patients received any specific treatment prior to renal biopsy. During the follow-up period, none of the patients developed impaired renal function. Among all the children, only one patient with AMHP developed hypertension and 2 patients with IMH developed proteinuria. Conclusion: Long-term follow-up must be done carefully for isolated microscopic hematuria and renal biopsy should be performed in selected cases.

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. ispartof: JOURNAL OF PEDIATRICS vol:199 pages:22-+ ispartof: location:United States status: published

The study investigated a number of biomarkers for the early diagnosis of contrast-induced nephropathy (CIN), which is an important cause of acute kidney injury (AKI).The study included 91 children scheduled for elective cardiac angiography and 50 healthy controls. Biomarkers including serum (s) and urinary (u) sodium, serum and u-creatinine, s-cystatin-C, serum neutrophil gelatinase-associated lipocalin (NGAL) and urinary N-acetyl beta glucosaminidase (u-NAG)/creatinine ratio were measured 4 times sequentially in the patients and once in the controls.The patient group comprised 40 males (44%) and 51 females (56%) while the control group comprised 16 males (32%) and 34 females (68%). Age, gender, s-creatinine, estimated-glomerular filtration rate (eGFR), s-cystatin-C and fractional-excretion of sodium did not differ significantly between the groups. Serum sodium and s-NGAL were found to be lower in the patients than those of in the controls, while their u-NAG/creatinine ratio was found to be higher. Sequential data analysis revealed that s-NGAL and u-NAG/creatinine ratio increased in the first 6 h after radiocontrast media (RCM) administration and decreased at 12 and 24 h. Serum BUN and s-cystatin-C levels also showed a significant difference during the 24-h follow-up. eGFR, s-sodium and s-creatinine levels did not change in the following period. Serum cystatin-C levels revealed a significant negative correlation with eGFR. Administered RCM doses showed a positive correlation only with u-NAG/creatinine ratios.In the first 24 h, s-cystatin-C, s-NGAL and especially u-NAG/creatinine ratio showed promise as biomarkers, but eGFR is not adequate for early diagnosis of CIN. Sequential measurement of biomarkers may contribute to more accurate diagnosis of AKI.

WOS: 000493307500019 PubMed ID: 30215773 Background. Management of children with congenital nephrotic syndrome (CNS) is challenging. Bilateral nephrectomies followed by dialysis and transplantation are practiced in most centres, but conservative treatment may also be effective. Methods. We conducted a 6-year review across members of the European Society for Paediatric Nephrology Dialysis Working Group to compare management strategies and their outcomes in children with CNS. Results. Eighty children (50% male) across 17 tertiary nephrology units in Europe were included (mutations in NPHS1, n = 55; NPHS2, n = 1; WT1, n = 9; others, n = 15). Excluding patients with mutations in WT1, antiproteinuric treatment was given in 42 (59%) with an increase in S-albumin in 70% by median 6 (interquartile range: 3-8) g/L (P< 0.001). Following unilateral nephrectomy, S-albumin increased by 4 (1-8) g/L (P = 0.03) with a reduction in albumin infusion dose by 5 (2-9) g/kg/week (P = 0.02). Median age at bilateral nephrectomies (n = 29) was 9 (7-16) months. Outcomes were compared between two groups of NPHS1 patients: those who underwent bilateral nephrectomies (n = 25) versus those on conservative management (n = 17). The number of septic or thrombotic episodes and growth were comparable between the groups. The response to antiproteinuric treatment, as well as renal and patient survival, was independent of NPHS1 mutation type. At final follow-up (median age 34months) 20 (80%) children in the nephrectomy group were transplanted and 1 died. In the conservative group, 9 (53%) remained without dialysis, 4 (24%; P< 0.001) were transplanted and 2 died. Conclusion. An individualized, stepwise approach with prolonged conservative management may be a reasonable alternative to early bilateral nephrectomies and dialysis in children with CNS and NPHS1 mutations. Further prospective studies are needed to define indications for unilateral nephrectomy. National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children National Health Service (NHS) Foundation Trust and University College, London; National Institute for Health Research (NIHR)National Institute for Health Research (NIHR) This work was supported by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children National Health Service (NHS) Foundation Trust and University College, London. R.S. holds a Career Development Fellowship with the National Institute for Health Research (NIHR).

Background: The causative agent spectrum and resistance patterns of urinary tract infections in children are affected by many factors. Aims: To demonstrate antibiotic resistance in urinary tract infections and changing ratio in antibiotic resistance by years. Study Design: Retrospective cross-sectional study. Methods: We analysed antibiotic resistance patterns of isolated Gram (-) bacteria during the years 2011-2014 (study period 2) in children with urinary tract infections. We compared these findings with data collected in the same centre in 2001-2003 (study period 1). Results: Four hundred and sixty-five uncomplicated community-acquired Gram (-) urinary tract infections were analysed from 2001-2003 and 400 from 2011-2014. Sixty-one percent of patients were female (1.5 girls : 1 boy). The mean age of children included in the study was 3 years and 9 months. Escherichia coli was the predominant bacteria isolated during both periods of the study (60% in study period 1 and 73% in study period 2). Bacteria other than E. coli demonstrated a higher level of resistance to all of the antimicrobials except trimethoprim-sulfamethoxazole than E. coli bacteria during the years 2011-2014. In our study, we found increasing resistance trends of urinary pathogens for cefixime (from 1% to 15%, p0.05). Conclusion: In childhood urinary tract infections, antibiotic resistance should be evaluated periodically and empiric antimicrobial therapy should be decided according to antibiotic sensitivity results.

PubMedID: 28729033 Recent studies in adult chronic kidney disease (CKD) suggest that metabolic acidosis is associated with faster decline in estimated glomerular filtration rate (eGFR). Alkali therapies improve the course of kidney disease. Here we investigated the prevalence and determinants of abnormal serum bicarbonate values and whether metabolic acidosis may be deleterious to children with CKD. Associations between follow-up serum bicarbonate levels categorized as under 18, 18 to under 22, and 22 or more mmol/l and CKD outcomes in 704 children in the Cardiovascular Comorbidity in Children with CKD Study, a prospective cohort of pediatric patients with CKD stages 3-5, were studied. The eGFR and serum bicarbonate were measured every six months. At baseline, the median eGFR was 27 ml/min/1.73m2 and median serum bicarbonate level 21 mmol/l. During a median follow-up of 3.3 years, the prevalence of metabolic acidosis (serum bicarbonate under 22 mmol/l) was 43%, 60%, and 45% in CKD stages 3, 4, and 5, respectively. In multivariable analysis, the presence of metabolic acidosis as a time-varying covariate was significantly associated with log serum parathyroid hormone through the entire follow-up, but no association with longitudinal growth was found. A total of 211 patients reached the composite endpoint (ESRD or 50% decline in eGFR). In a multivariable Cox model, children with time-varying serum bicarbonate under 18 mmol/l had a significantly higher risk of CKD progression compared to those with a serum bicarbonate of 22 or more mmol/l (adjusted hazard ratio 2.44; 95% confidence interval 1.43-4.15). Thus, metabolic acidosis is a common complication in pediatric patients with CKD and may be a risk factor for secondary hyperparathyroidism and kidney disease progression. © 2017 International Society of Nephrology

The correlations between ambulatory blood pressure measurements (ABPM) and serum cystatin C (Cys C), serum creatinine (Cr), microalbumin (MA), and β2-microglobulin (β2-MG) levels in 24 h (24-h) urine were analyzed in children with solitary kidney (SK) and compared to healthy children. Fifty children with normal functioning SK and 25 controls were studied. The ABPM, serum Cys C, serum Cr, MA, and β2-MG levels in 24-h urine were measured in all children. Clinical symptoms and signs, laboratory results, urinary ultrasonography, voiding cystourethrography, and Dimercaptosuccinic acid (DMSA) scintigraphy results were recorded in the SK group. Four patients with Wilms’ tumor and two with renal scarring were excluded from the study. The mean ages of the SK group and controls were 9.6 ± 3.6 and 9.3 ± 3.3 years, respectively. The serum Cys C and Cr levels, 24-h urinary β2-MG and MA levels were similar in both groups (p > 0.05). However, 24-h urinary MA excretion was higher in patients living with SK more than 5 years (p = 0.01). Standard deviation scores of ABPM parameters showed no significant correlation with serum Cr, serum Cys C, MA, and β2-MG in 24-h urine of both groups. Children with SK have increased 24-h urinary MA excretion in the long term, and need prolonged follow-up to detect early deterioration of renal function and to prevent end-organ damage later in life.

The purpose of this study was to compare the outcome of infants having antenatally detected urinary tract abnormalities (AUTAs) with respect to the presence of hydronephrosis in postnatal ultrasonography (US) examination. Between January 1999 and October 2009, 256 infants diagnosed with AUTAs were prospectively followed. Infants were divided into two groups according to the presence of hydronephrosis in postnatal US examination: Group 1, infants with hydronephrosis; Group 2, infants without hydronephrosis (including renal cyst, agenesis, ectopic kidney). The events of interest were the presence and diagnoses of uropathy, AUTA resolution, urinary tract infection (UTI), development of renal parenchymal defects (RPDs)—focal or global scarring, dysplasia—, acute kidney injury (AKI) and chronic kidney disease (CKD), and the need for surgery and dialysis treatment. The most commonly detected underlying abnormalities were ureteropelvic junction obstruction (44.8 %), vesicoureteral reflux (VUR) (30.0 %) and megaureter (9.5 %) in patients with postnatal hydronephrosis. On the other hand, multicystic dysplastic kidney (43.5 %), renal agenesis (19.4 %) and VUR (19.4 %) were mostly encountered abnormalities in patients without postnatal hydronephrosis. RPDs were significantly more common among patients with postnatal hydronephrosis compared to those without hydronephrosis (37 vs. 21 %, P = 0.02). The incidence of UTI and VUR was higher in infants with postnatal hydronephrosis than in infants without hydronephrosis. There was no statistically significant difference in terms of the development of AKI and CKD and the need for surgery and dialysis treatment between patients with hydronephrosis and those without hydronephrosis. Infants with AUTAs should be investigated postnatally. The findings from this study will help to identify the natural history and outcome of infants with AUTAs according to the postnatal US parameters.

The role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS).A total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI.Nineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI.Abnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD.

ozkaya, ozan/0000-0002-0198-1221; AKMAN, SEMA/0000-0001-7131-8911; Ozcakar, Zeynep/0000-0002-6376-9189; kavukcu, salih/0000-0002-9959-881X; WOS: 000278951200013 PubMed: 20383649 Herein the results of a multicenter study from the Turkish Pediatric Kidney Transplantation Study Group are reported. The aims of this study were to compare the quality of life (QoL) scores of Turkish children who are dialysis patients (DP), renal transplant recipients (TR), and age-matched healthy controls and to compare child-self and parent-proxy scores. The Turkish versions of the Kinder Lebensqualitat Fragebogen (KINDLA (R)) questionnaires were used as a QoL measure. The study group consisted of 211 children and adolescents with chronic kidney disease (CKD) (139 TR and 72 DP aged between 4-18 years; 13.7 A +/- 3.5 years) from 11 university hospitals, 129 parents of these patients, 232 age-matched healthy children and adolescents (aged between 4-18 years; 13.1 +/- 3.5 years) and 156 of their parents. Patients with CKD had lower scores in all subscales except for physical well-being than those in the control group. TR had higher scores in physical well-being, self-esteem, friends' subscales, and total scores than DP. Child-self scores were lower than parent-proxy scores, especially in CKD, DP, and control groups. Concordance between parent-proxy and child-self reports in the TR, DP, CKD, and control groups was only moderate for the majority of subscales (r = 0.41-0.61). It was concluded that parent-proxy scores on the QoL were not equivalent to child-self scores and that evaluating both children's and parents' perspectives were important. Additionally, psychosocial counseling is crucial not only for patients with CKD but also for their parents.

WOS: 000447379800010, PubMed ID: 30325125, To evaluate the value of Positional Installation of Contrast (PIC) and Redo-PIC cystography in patients with febrile recurrent urinary tract infection (f-UTI) where voiding cystourethrogram (VCUG) was negative. Patients with recurrent f-UTI with no reflux on VCUG referred to the outpatient clinic of Pediatric Urology, between June 2011 and June 2016 were included in the study. A PIC cystography was performed in all patients. When reflux was found, subureteric injection was performed. Urinary cultures were used for follow-up. Patients that continued having f-UTI, received redo-PIC cystography. PIC cystography was performed on 42 patients. The average age of patients was 8.0 +/- 3.6 years. Vesicoureteral reflux (VUR) was detected in 41 patients. Average follow-up time after PIC cystography was 44.6 months. Thirty-three patients (80.5%) were free of f-UTI after PIC cystography and concurrent subureteric injection. Eight patients continued to have recurrent f-UTI. Six of these patients underwent redo-PIC cystography and PIC-VUR was demonstrated in all patients. After an average follow up of 30.9 months, no f-UTI was seen in these patients. The success rate of 80.5% (33/41) after 1st PIC cystography and subureteric injection increased to 95.1% (39/41) after redo-PIC cystography in six patients. Patients with recurrent f-UTIs without VUR on VCUG are an important challenge. PIC cystography is an important tool in demonstrating occult VUR in these patients. We advise that PIC cystography is performed in all patients with recurrent f-UTI with negative VCUG and redo-PIC cystography in patients who continue to have f-UTI after 1st PIC cystography and subureteric injection.

The objective of this study was to assess the urine levels of interleukin-6 (IL-6) and interleukin-8 (IL-8) as noninvasive markers of vesicoureteral reflux (VUR) and renal parenchymal scarring (RPS) in children in the absence of a recent urinary tract infection (UTI) episode. Urine concentrations of IL-6 and IL-8 in 114 children aged 1 month to 16 years were evaluated. The children were divided into four groups: group 1, 26 children with VUR and RPS; group 2, 27 children with VUR without RPS; group 3, 34 children with RPS without VUR, group 4, 27 children without VUR and RPS, as the control group. After the first assessment, the children were divided into four larger groups for comparison purposes: group A (groups 1+2), 53 children with VUR; group B (groups 3+4), 61 children without VUR; group C (groups 1+3), 60 children with RPS; group D (groups 2+4), 54 children without RPS. Urinary IL-6 and IL-8 concentrations were determined. To avoid dilution effects and to the standardize samples, urinary levels of IL-6 and IL-8 were expressed as the ratio of cytokine to urinary creatinine (pg/mg). The median urine IL-6/creatinine was significantly higher in patients with VUR than in those without VUR (5.72 vs. 3.73). In patients with VUR, there was a significant but rather weak correlation between IL-6/creatinine concentrations and the reflux grade (p < 0.05, R = 0.305). The median urine IL-8/creatinine was significantly higher in patients with RPS than in those without RPS (43.12 vs. 16.36). In patients with RPS, there was a significant but rather weak correlation between IL-8/creatinine concentrations and the renal scar grade (p < 0.05, R = 0.251). The results of this study provide preliminary evidence that children with VUR have a high urine IL-6 concentration, whereas children with RPS have a high urine IL-8 concentration.

We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis or microlithiasis who had been referred to our pediatric nephrology clinics between 1998 and 2008 with suspected urolithiasis. The medical histories of these children (78 girls, 84 boys), who ranged in age from 2 months to 16 years (mean age 5.59±0.35 years), were reviewed retrospectively for clinical and metabolic features of urinary tract calculi. Urinary tract infections (UTI) were present in 45.9% of the cases. The most common presenting symptoms were flank pain or restlessness (25.3%) and hematuria (21.6%), followed by UTI (16%), whereas 23.5% of the cases were detected incidentally during evaluation for other medical conditions. Other symptoms at presentation included dysuria, passing stones, penile edema, enuresis, vomiting and anorexia. Urine analysis revealed metabolic abnormalities in 87% of the cases, including hypercalciuria (33.8%), hypocitraturia (33.1%), hyperoxaluria (26.5%), hyperuricosuria (25.4%), hypocitraturia + hypercalciuria (21.1%), hyperphosphaturia' (20.8%) and cystinuria (5.7%). Almost 50% of the patients had a positive family history for urolithiasis. The most frequently involved site was in the kidneys (86%). Ureters and bladder were involved in 12 and 2% of the cases, respectively. A family history of urolithiasis, presenting symptoms and underlying metabolic abnormalities were similar for microlithiasis and the patients with larger stones. However, in our study population, microlithiasis was mainly a disease of young infants, with a greater chance for remission and often not associated with structural changes. The presenting symptoms of urolithiasis show a wide spectrum, so that a high index of suspicion is important for early detection. A metabolic abnormality can be identified in 87% of cases of urolithiasis. Detection of microlithiasis may explain a number of symptoms, thus reducing invasive diagnostic procedures and allowing early recognition of metabolic abnormalities. These results draw attention to the importance of screening for UTIs in patients with urolithiasis. © IPNA 2009.

The correlation between timing of the primary neurosurgical repair and urological prognosis in infants with open spinal dysraphism remains unknown. We investigated the impact of primary repair timing on neurogenic bladder prognosis in children with myelomeningocele.We retrospectively reviewed the records of 401 children with myelomeningocele followed at our multidisciplinary clinic between 1996 and 2005. Among these patients 129 were included in the study based on the availability of urological followup data at age 3 years, which was chosen as an ideal point for the standardization of urological findings. Children were assigned to 1 of 2 groups-those who underwent primary neurosurgical repair within 72 hours of delivery (group 1) and those undergoing repair after 72 hours (group 2). We compared the incidence of febrile urinary tract infections, hydronephrosis, vesicoureteral reflux and secondary tethering of the spinal cord at age 3 years, as well as cystometric bladder capacity and detrusor leak point pressure between the 2 groups.Urological followup data at age 3 years revealed significantly increased incidence of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering of the spinal cord in children in group 2 compared to group 1 (chi-square, por =0.05 for all comparisons). Urodynamic assessment also showed a significantly lower bladder capacity and a substantially higher detrusor leak point pressure in children who received primary repair at later than 72 hours (t test, p0.05). A subgroup analysis of group 1 comparing children who underwent repair before and after 24 hours following delivery failed to demonstrate any significant difference in terms of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering (chi-square, p0.05 for all comparisons), whereas mean bladder capacity was significantly higher (t test, p0.05) and mean detrusor leak point pressure was lower (t test, p = 0.08) in children undergoing repair within 24 hours of delivery.The timing of primary neurosurgical repair has a significant impact on neurogenic bladder prognosis in children with myelomeningocele. Closure of the spinal lesion on the first day of life seems to provide the best chance for favorable lower urinary tract function.

Introduction: Pyelonephritis-induced renal scarring in children is a major predisposing factor for proteinuria, hypertension, and ultimate renal failure. The aim of this study was to investigate and compare the efficacy of Tc99m dimercaptosuccinic acid (Tc-DMSA) renal scintigraphy and renal ultrasonography (USG) in detecting renal scars in children with primary vesicoureteral reflux (VUR). Materials and methods: Tc-DMSA scan and USG studies were done in 62 children who were admitted to our clinic between 1997 and 2003 because of documented urinary tract infection (UTI) and diagnosed with primary VUR. Renal scarring detection rates of Tc-DMSA scan and USG were compared according to reflux grades. Results: In the whole group, renal scars were detected by Tc-DMSA scan and USG in 55% and 38% of refluxing units, respectively. Detection rates of Tc-DMSA and USG according to reflux grades were as follows: 47% and 29 % in low-grade VUR (grades 1 and 2), 46 % and 25% in mid-grade VUR (grade 3), 76% and 65% in high-grade VUR (grades 4 and 5), respectively. Conclusion: USG was found to be an inappropriate study in the detection of renal parenchymal scars, irrespective of the reflux grade. In this study, Tc-DMSA scan detected scars in 35% of kidneys reported to be normal on USG.

Contradictory supranormal renal function (SRF) in unilateral hydronephrosis is a debatable subject resulting from the methodology of nuclear renography or the characteristics of the patient. In this study, we aimed to investigate the frequency and comparison of SRF with MAG-3 scans by 8 different technical analyses in pediatric hydronephrosis.We reviewed Tc-99m MAG-3 scans in 82 children with unilateral hydronephrosis (52 male, 30 female, mean age: 47.7 +/- 64.5 months). Of 82, 34 also had Tc-99m DMSA scans. Data were reprocessed with 4 different regions of background activity (subrenal, perirenal C-type, perirenal ring, and lateral) at 2 different time intervals (1-2 and 2-3 minute), and 8 different estimates of MAG-3 differential renal function (DRF) were obtained in 67 patients. SRF was defined as DRF greater than 55% in the hydronephrotic kidney.The routine processing protocol showed only 3 renal units with SRF, and all were on the right side (3.6%). After reprocessing, a total of 10 dilated kidneys had SRF in 1 or more of DRF estimates (5.2% of all estimations). These cases were significantly younger (8.1 +/- 6.7 vs. 42.5 +/- 52.5, P0.05) and had a larger renal area ratio (1.25 +/- .24 vs. 1.07 +/- .21, P0.05). There was no SRF with DMSA. In comparison between MAG-3 and DMSA DRF in 20 children who underwent both tests within 3 months, the best correlation was obtained when C-type correction was used for both agents at 2 time intervals (r: .86 and .84 for early and late time intervals, P0.00001, respectively).SRF in unilateral hydronephrosis is, at least, in part, technical in origin in this particular pediatric patient population with tubular immaturity (ie, physiological high background activity) and asymmetric kidney size.

Peritoneal dialysis (PD) is considered as the most common form of renal replacement therapy for newborns including preterms with acute kidney injury (AKI). Although there are several reports describing successful PD performed for AKI in preterm infants, there is no data describing the use of PD to treat AKI in preterm newborns with congenital diaphragmatic hernia (CDH), which is one of the contraindications for PD. We present a preterm newborn with CDH, truncus arteriosus and AKI treated with PD and emphasize that PD may be successfully performed with caution even in cases of contraindications when other renal replacement therapies cannot be used.

Subaşı B, Gökçe İ, Delil K, Alpay H. Vitamin D receptor gene polymorphisms in children with kidney stone disease. Turk J Pediatr 2017; 59: 404-409. Kidney stone disease has a multifactorial etiology involving the interaction of genetic and environmental factors. There is an increased risk of stone formation in the relatives of idiopathic stone patients, which can be explained up to 60% by genetic factors. This study was conducted to explore the association of vitamin D receptor (VDR) gene polymorphisms with the risk of urolithiasis (UL) in Turkish children. We investigated the VDR gene polymorphisms: ApaI, BsmI, TagI, Cdx2, FokI, in 52 children (26 boys, 26 girls) with UL and in 51 healthy children (22 boys, 29 girls) without UL. Apa I, BsmI, TagI, Cdx2, FokI genotypes were analyzed by Apa I, BsmI, TagI, Cdx2, FokI restriction enzyme digestion, respectively. The resulting alleles are designated as ABTCF (ApaI, BsmI, TagI, Cdx2, and FokI restriction site is absent), or abtcf (ApaI, BsmI, TagI, Cdx2, FokI restriction site is present), respectively. Genotype and allele frequencies were calculated, and the association with UL, hypercalciuria and hypocitraturia was investigated. Our data provide no statistically significant evidence for an association between UL and VDR ApaI, BsmI, TagI, Cdx2, and FokI genotype and allele frequencies. Patients with hypocitraturia and hypercalciuria were compared with the control group and no statistically significant difference was detected in terms of VDR gene ApaI, BsmI, TagI, Cdx2, and FokI polymorphisms and allele frequencies. Our data suggest that the VDR ApaI, BsmI, TagI, Cdx2, and FokI polymorphisms do not indicate a significant risk for UL.

To investigate the effect of intravesical hyaluronic acid on Escherichia coli-induced cystitis and cystitis-induced hypercontractility in rats.Bacterial cystitis was induced in Wistar female rats by intravesical inoculation of E. coli. Isotonic saline was instilled in the control group (n = 6). The rats were either non-treated, treated with gentamycin (4 mg/kg, 5 days) or treated intravesically with hyaluronic acid (0.5 mL, 0.5%). On the eighth day, the bladder tissues were excised for histological examination, and the measurements of myeloperoxidase, superoxide dismutase and catalase activities. Contraction/relaxation responses to carbachol, isoprotrenol and papaverine were studied.Tissue myeloperoxidase activity was increased, but superoxide dismutase and catalase activities were decreased in bacterial cystitis, while hyaluronic acid treatment reversed these changes. In the hyaluronic acid-treated group, healing of the uroepithelium was observed, while decreased inflammatory cell infiltration was obvious in gentamycin-treated group. E. coli-induced cystitis in all rats resulted in increased contraction responses to carbachol compared with controls (P 0.01). Treatment with hyaluronic acid, but not gentamycin, significantly (P 0.05) depressed hypercontractility at maximum carbachol concentrations. In all rats with cystitis, papaverine-induced relaxation was increased, whereas isoproterenol-induced relaxation curves were not different between the studied groups.Gentamycin treatment, despite its ameliorative effect on inflammation, had no impact on the contractile dysfunction of the injured bladder. Intravesical hyaluronic acid, in addition to its supportive role in the healing of the epithelium, seems to lower the increased threshold for contraction and to reduce oxidative stress. These findings support a potential role for hyaluronic acid in the treatment of bacterial cystitis.

The aim of this retrospective multicenter study was to define the epidemiological and clinical features and prognostic factors of the first diarrhea-related hemolytic uremic syndrome (D+HUS) outbreak in Turkey in 2011. All pediatric nephrology centers in Turkey were asked about D+HUS patients via e-mail. Seventy D+HUS patients (median age: 5.7 years) participated. The seasonal peak was around the 7th, 8th and 9th months with 44 cases, centered in the east Marmara region. No causative agent could be identified. The rate of neurological complications and mortality was 21.4% and 4.2%, respectively. Eculizumab was used in four cases. Two of them had severe neurological complications despite plasma exchange. Elevated polymorphonuclear leukocyte count during hospital admission was the predictor of both severe disease and poor outcome. Duration of prodrome was the predictor of poor outcome (p0.05). In conclusion, the median age of the affected children was greater than in the previous reports, while clinical features and outcome were similar.

Late-preterm birth is associated with higher rates of neonatal morbidity and mortality and higher health care utilization, but its impact on later life is not well known. In this study, we aimed to evaluate whether late-preterm birth affects blood pressure, renal function, and urinary protein excretion in children later in life. Sixty-five children aged 4 to 13 years born as late-preterm and 65 age- and sex-matched children born full term were evaluated with 24-h ambulatory blood pressure monitoring (ABPM), urinary microalbumin excretion (UAE), and glomerular filtration rate (GFR). All subjects underwent ABPM prospectively. For each gender, daytime, nighttime, and 24-h systolic and diastolic and mean blood pressures (SBP, DBP, and MAP) were transformed to standard deviation scores (SDS). Blood pressure profiles (SBP DBP, and MAP) were considered abnormal when the corresponding SDS values exceeded 1.63. Urinary microalbumin excretion was expressed as milligrams per day, and the value between 30 and 300 mg/day was defined as microalbuminuria (MA). There was no significant difference in the mean GFR and MA levels between late-preterm and term children. 24-h systolic BP SDS, daytime systolic BP SDS, nighttime systolic BP SDS, 24-h diastolic BP SDS, nighttime diastolic BP SDS, 24-h MAP BP SDS, daytime MAP BP SDS, and nighttime MAP BP SDS were found to be significantly higher in late-preterm children compared to term children. Conclusion: We conclude that late-preterm children have higher BP levels, so those children should be followed up carefully by the pediatrician regarding probable hypertension in their future life.

We have evaluated the clinical, radiological and metabolic features of infantile urolithiasis (UL).We have reviewed the medical records of 93 children who were diagnosed as having UL before 1 year of age. We recorded patient demographics, the age at diagnosis, presenting symptoms, family history, the localizations and dimensions of stones, urinary metabolic examinations, as well as physical, laboratory, and radiologic findings. Our secondary objective was to compare some features of this group with those of older children with UL followed-up in the same clinic which were previously reported.We evaluated 93 children referred to our pediatric nephrology clinics. A family history of UL was 56.2 % in the study group. Resolution of stones was observed in 30.1 % of the cases. Urinary tract infections (UTIs) were detected in 65.9 % of females and 46.2 % of males. At least one urinary metabolic abnormality was found in 79.5 % of all the children. Most commonly seen metabolic abnormality was hypercalciuria. In all patients stones were located in kidneys except one infant who had an ureteral stone together with a kidney stone. Fifteen (16.1 %) children had an accompanying systemic disorder.Among pediatric urinary stone diseases infantile UL can be regarded as a separate clinical entity. Coexistence of systemic disorders and anatomic anomalies at high frequencies may indicate a role of distinct pathogenetic mechanisms. In addition, high rates of UTIs and metabolic abnormalities in this age group justify screening for these parameters during follow-up of these children.

The clinical course of focal segmental glomerulosclerosis (FSGS) is heterogeneous in children. To evaluate the clinical course and the predictors of outcome in Turkish children with primary FSGS, a retrospective study was conducted by the Turkish Pediatric Nephrology Study Group in 14 pediatric nephrology centers. Two hundred twenty-two patients (92 boys, 130 girls, aged 1-16 years) with biopsy-proven primary FSGS were included. One hundred forty-eight patients were followed-up for a median of 51 months (range: 0.26-270). The clinical course was characterized by complete remission in 50 (33.8%), persistent proteinuria in 50 (33.8%) and progression to renal failure in 48 (32.4%) patients. Progression to end-stage renal disease (ESRD) was significantly higher in patients who did not attain remission. Complete remission, partial remission and progress to renal failure were recorded in 37%, 32% and 28%, respectively, of the patients (n = 73) treated with prednisone combined cyclophosphamide/cyclosporine A. However, in patients (n = 33) treated with pulse methyl prednisolone plus oral prednisone (up to 20 months) combined with cyclophosphamide, complete remission in 51.5% and partial remission in 27.3% of the patients were noted. Progression to renal failure was observed in 9.1% of this group of patients. Multivariate analysis showed that only plasma creatinine at presentation was an independent predictive value for outcome. Patients with serum creatinine level higher than 1.5 mg/dl had 6.6 times increased rate of progression to renal failure. Failure to achieve remission is a predictor of renal failure in children with primary FSGS. The use of immunosuppressive treatment in conjunction with prolonged steroid seems beneficial in primary FSGS in children.

ozkaya, ozan/0000-0002-0198-1221; BAYAZIT, AYSUN K/0000-0002-2644-5628; Ozcakar, Zeynep/0000-0002-6376-9189; DUZOVA, ALI/0000-0002-4365-2995 WOS: 000278951200009 PubMed: 20512652 The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.6% were newborns (median age 3 days, range 1-24 days), and 67.4% were children aged > 1 month (median 2.99 years, range 1 month-18 years). The most common medical conditions were prematurity (42.2%) and congenital heart disease (CHD, 11.7%) in newborns, and malignancy (12.9%) and CHD (12.3%) in children aged > 1 month. Hypoxic/ischemic injury and sepsis were the leading causes of AKI in both age groups. Dialysis was performed in 30.3% of newborns and 33.6% of children aged > 1 month. Mortality was higher in the newborns (42.6 vs. 27.9%; p < 0.005). Stepwise multiple regression analysis revealed the major independent risk factors to be mechanical ventilation [relative risk (RR) 17.31, 95% confidence interval (95% CI) 4.88-61.42], hypervolemia (RR 12.90, 95% CI 1.97-84.37), CHD (RR 9.85, 95% CI 2.08-46.60), and metabolic acidosis (RR 7.64, 95% CI 2.90-20.15) in newborns and mechanical ventilation (RR 8.73, 95% CI 3.95-19.29), hypoxia (RR 5.35, 95% CI 2.26-12.67), and intrinsic AKI (RR 4.91, 95% CI 2.04-11.78) in children aged > 1 month. Turkish Academy of SciencesTurkish Academy of Sciences [A.D/TUBA-GEBIP/2006-6] Dr. Duzova is supported by The Turkish Academy of Sciences (Program to Reward Successful Young Scientists, A.D/TUBA-GEBIP/2006-6).

Methods. We aimed to clarify arteriosclerotic risk and to document possible relationships between cardiovascular risk factors and echocardiographic parameters in paediatric peritoneal dialysis (PD) patients. M-mode/Doppler/tissue Doppler echocardiographic studies and lipid/lipoproteins, homocysteine, high-sensitivity C-reactive protein (HS-CRP) levels and carotid intima-media thickness (CIMT) were determined in 59 patients (age: 14.2 +/- 4.5 years) and in 36 healthy subjects. Results. Structural and functional cardiac abnormalities were observed in patients on maintenance dialysis. Increased left ventricular mass index (LVMI, P = 0.000), relative wall thickness (P = 0.000), myocardial performance index (MPI, P = 0.000) were documented in the patients. Lipoprotein (a) (P = 0.000), homocysteine (P = 0.001), HS-CRP (P = 0.000) and CIMT (P = 0.000) were significantly elevated in the patients. Left ventricular hypertrophy (LVH) was prevalent in 68\% of the patients. Patients with LVH had higher levels of HS-CRP (P = 0.001) and CIMT (P = 0.028) than those without LVH. Haemoglobin was an independent predictor of LVMI (beta: -8.9, P = 0.001), while residual diuresis and CIMT were independent predictors of diastolic dysfunction (beta: -0.45, P = 0.034 and beta: 5.90, P = 0.008, respectively). Albumin (beta: -0.72, P = 0.018) and Kt/V urea (beta: -0.48, P = 0.012) were significant predictors of CIMT. There were positive correlations between LVMI and CIMT. HS-CRP was positively correlated with LVMI as well as CIMT. Conclusions. Elevated levels of atherosclerotic/inflammatory risk factors, low haemoglobin levels and loss of residual renal function and their negative effects on heart are of remarkable importance in paediatric patients on maintenance peritoneal dialysis. Achieving recommended targets for haemoglobin, blood pressure and Kt/V urea, preserving residual renal function as well as managing inflammation and subsequent arteriosclerosis is obviously essential to improve the patients' prognosis.

39th Annual Meeting of the European-Society-for-Paediatric-Nephrology -- SEP 10-13, 2005 -- Istanbul, TURKEY ozkaya, ozan/0000-0002-0198-1221; Cetinyurek Yavuz, Aysun/0000-0003-4024-8775 WOS: 000266882400012 PubMed: 19439991 Background/Aim: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schonlein purpura (HSP) nephritis in children. Methods: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). Results: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. Conclusion: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely. Copyright (C) 2009 S. Karger AG, Basel European Soc Paediat Nephrol

Coeliac disease is an autoimmune enteropathy characterised by chronic inflammation of the small intestinal mucosa and the presence of typical autoantibodies. Coeliac disease may be a risk factor for renal disease. Immunoglobulin A (IgA) nephropathy is reported in the majority of these cases. Only one adult patient had been reported with membranoproliferative glomerulonephritis (MPGN) and coeliac disease. Here, we report a case in a 12-year-old girl with coeliac disease who presented with severe anaemia and later developed nephrotic syndrome. Renal biopsy of the patient was consistent with MPGN type 1, which has not been previously reported in children with coeliac disease. A gluten-free diet was started. After 6 months of this diet, her nephrotic syndrome resolved completely. This case is presented to draw attention to the rare association of coeliac disease and MPGN type 1.

The aim of this study was to compare ambulatory blood pressure monitoring (ABPM) parameters in offspring with at least one hypertensive parent (HP) to offspring with normotensive parents (NP) and to determine whether gender of parent or child might influence the association between parental hypertension and blood pressure (BP). Eighty-nine healthy children (mean age 11.1 +/- 3.9 years) with HP and 90 controls (mean age 10.5 +/- 3.1 years) with NP were recruited. Age, gender, and height did not differ between the two groups, whereas children of HP had higher weight, body mass index (BMI), and waist circumference compared with healthy controls. No difference was found in casual BP between the two groups. In contrast, during ABPM daytime and nighttime mean systolic and diastolic BP and mean arterial pressure (MAP) standard deviation scores (SDS) were significantly elevated in children with HP. The mean percentage of nocturnal BP decline (dipping) was not significantly different between the two groups. Children with hypertensive mothers had higher daytime systolic and MAP SDS than controls; no such difference was detected for children with hypertensive fathers. Daytime systolic and MAP SDS were significantly elevated in boys with HP compared with boys with NP but failed to be significant in girls. Multiple linear regression analysis showed that parental history of hypertension (B = 0.29) and BMI (B = 0.03) were independently correlated with increase of daytime MAP SDS. Early changes in ambulatory BP parameters were present in healthy children of HP. BP in HP offspring was influenced by the gender of the affected parent and the offspring.

Schimke immunoosseous dysplasia (SIOD), which is characterized by prominent spondyloepiplayseal dysplasia, T-cell deficiency, and focal segmental glomerulosclerosis, is a panethnic autosomal recessive multisystem disorder with variable expressivity. Biallelic mutations in switch/sucrose nonfermenting (swi/snf) related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) are the only identified cause of SIOD. However, among 72 patients from different families, we identified only 38 patients with biallelic mutations in the coding exons and splice junctions of the SMARCAL1 gene. This observation, the variable expressivity, and poor genotype-phenotype correlation led us to test several hypotheses including modifying haplotypes, oligogenic inheritance, or locus heterogeneity in SIOD. Haplotypes associated with the two more common mutations, R820H and E848X, did not correlate with phenotype. Also, contrary to monoallelic SMARCAL1 coding mutations indicating oligogenic inheritance, we found that all these patients did not express RNA and/or protein from the other allele and thus have biallelic SMARCAL1 mutations. We hypothesize therefore that the variable expressivity among patients with biallelic SMARCAL1 mutations arises from environmental, genetic, or epigenetic modifiers. Among patients without detectable SMARCAL1 coding mutations, our analyses of cell lines from four of these patients showed that they expressed normal levels of SMARCAL1 mRNA and protein. This is the first evidence for nonallelic heterogeneity in SIOD. From analysis of the postmortem histopathology from two patients and the clinical data from most patients, we propose the existence of endophenotypes of SIOD.

Background: Fluoroscopic voiding cystourethrography (VCUG) is a widely used imaging test for the diagnosis of vesicoureteral reflux (VUR). However, high gonadal radiation and intermittent imaging are the main disadvantages of VCUG. Direct radionuclide cystography (DRC) has been advocated for the detection of VUR with increased sensitivity and low radiation doses, however, having the disadvantage of providing less anatomical details for urethral evaluation. In this study, DRC has been compared with standard fluoroscopic VCUG for detection of VUR. Methods: A total of 41 children (82 kidney ureter units, KUU) aged 1 month–126 months (median, 15 months) were studied sequentially using DRC and VCUG. The indications of VUR studied were urinary tract infection in 29 children, VUR follow up in eight children and antenatal dilatation history in four children. Results: A total of 18 refluxing ureters were detected by DRC, 22 refluxing ureters by VCUG and 14 refluxing ureters by both methods. The two methods were concordant for the detection and exclusion of VUR in 85% of KUU. VUR was missed by VCUG in four KUU (three severe, one mild) whereas VUR was missed by DRC in eight KUU (four grade I, four grade III). Conclusions: There was a good correlation between DRC and VCUG in the evaluation of VUR. DRC provides continuous monitoring and low gonadal radiation exposure. DRC can be used in the diagnosis of VUR as an alternative to VCUG in selected cases.

The aim of this study was to evaluate the incidence of urinary tract infection (UTI) in newborns with asymptomatic, unexplained indirect hyperbilirubinemia in the first two weeks of life. Jaundiced infants, otherwise clinically well, less than two weeks of ages, with a total bilirubin level above 15 mg/dl were eligible for the study. A bilirubin work-up including glucose-6-phosphate dehydrogenase (G-6 PD) level, as well as urinalysis and a urine culture were performed in all patients. Patients with UTI, defined as more than 10,000 colony-forming units per milliliter of a single pathogen obtained by bladder catheterization, were evaluated for sepsis. Renal function tests and renal ultrasound were performed in cases with UTI. During follow-up, voiding cystourethrogram (VCUG) and dimercaptosuccinic acid scintigraphy (DMSA) were performed as well. A total of 102 patients were enrolled. The bilirubin work-up of patients did not demonstrate any significant underlying disorder. None of the infants had a high direct bilirubin level. UTI was diagnosed in eight (8%) cases [Enterobacter aerogenes (3/8:38%), Enterococcus faecalis (2/8:25%), Klebsiella pneumoniae (2/8:25%) and Escherichia coli (1/8:12%)]. Of those eight patients, only four (50%) had pyuria. Bacteriuria was present in seven (88%) patients. The sepsis screen was negative in all but one case with a high C-reactive protein (CRP) level. None of the patients had a positive blood culture. Renal function tests were within normal levels in all patients. Renal ultrasound showed urinary tract abnormalities in three (38%) patients (hydronephrosis, n=1 and pelviectasis, n=2). VCUG was performed in all patients during the study period and one had unilateral grade 3-4 reflux, while only one patient had a diverticulum of the bladder. DMSA was performed in seven patients and none had renal scars. It is of importance that UTI can occur in asymptomatic, jaundiced infants even in the first week of life. Although it is well known that UTI is a common cause of prolonged jaundice, urine culture should be considered in the bilirubin work-up of infants older than three days of age with an unknown etiology.

ozkaya, ozan/0000-0002-0198-1221; Kasapcopur, Ozgur/0000-0002-1125-7720; WOS: 000243032300011 PubMed: 16586044 Aim: The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population. Patients and methods: A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schonlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed. Results: Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener's granulomatosis 0.4%, and Beh et disease 1.9%. There was no clear gender dominance. The mean age was 11.05 +/- 4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series. Conclusion: This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well.

Human paraoxonase 1 (PON1) is a serum enzyme related to high-density lipoprotein which has a major role in preventing oxidative modification of low-density lipoprotein. Due to its amino acid substitution PON1 has two genetic polymorphisms. These polymorphisms are characterized by the location of glutamine (A genotype) and arginine (B genotype) at position 192, and leucine (L genotype) and methionine (M genotype) at position 55. Hyperlipidemia and increased lipid oxidation in nephrotic syndrome may lead to glomerulosclerosis and progression of the glomerular disease. In this study we aimed to investigate PON1 192 and PON1 55 polymorphisms in children with focal segmental glomerulosclerosis (FSGS) and control subjects. The study included 25 children with biopsy-proven FSGS and 30 healthy controls. We demonstrated a statistically significant difference between FSGS patients and control subjects with respect to the distribution of the PON1 polymorphism. The AA genotype was less frequent and the AB+BB genotype was more frequent in FSGS patients than in controls (48 versus 73% for AA genotype and 52 versus 27% for AB+BB genotype, p

Hypercalciuria is an important and common risk factor in the formation of renal stones. In this study we evaluated the incidence and the clinical presentation of hypercalciuria in 75 children over 5 years of age with the diagnosis of recurrent urinary tract infection (UTI). We measured random urinary calcium/creatinine value (three times), 24-h urinary calcium excretion, serum calcium, phosphorus, electrolytes, blood gas, blood urea nitrogen and creatinine levels. Hypercalciuria was found in 32 patients (43%). The mean urinary calcium/creatinine ratio for hypercalciuric patients was 0.50+/-0.21 mg/mg (min: 0.24, max: 2.60). The mean urinary calcium/creatinine ratio for the rest of the study population--those without hypercalciuria--was 0.10+/-0.04 mg/mg (min: 0.01, max: 0.18). Presenting symptoms of the hypercalciuric patients and normocalciuric patients were similar. History of familial urolithiasis was positive in 19 patients (59%). Predisposing urinary tract abnormalities in recurrent UTI was shown in 12 of the hypercalciuric patients (12/32, 37.5%) and 8 of the normocalciuric patients (8/43, 19%) without a statistically significant difference between. We conclude that hypercalciuria is not a rare finding among recurrent UTI cases in Turkish children. Hypercalciuria does not modify the clinical presentation of UTI, and we suggest the investigation of urinary calcium excretion in children with recurrent UTI.

Background: Early diagnosis and proper treatment, including long-term follow up, are very important for neonatal urinary tract infections (UTI). Methods: The present study reports the analysis and long-term follow-up results of 71 newborns treated for UTI. Results: Forty-one per cent of patients were preterm babies. Suspected sepsis and hyperbilirubinemia were the main presenting features. Community-acquired and nasocomial UTI accounted for 63% and 37% of cases, respectively. The leading causative agents were Escherichia coli for community-acquired UTI and Klebsiella pneumoniae for nasocomial UTI. The urosepsis rate was 5%. Abnormal ultrasonography findings were present in 23% and vesicoureteral reflux was present in 15% of babies. A total of 23% of patients showed renal photopenic areas on dimercaptosuccinic acid scan. The recurrence rate was 28% occurring between 1.5 and 12 months, in particular in the first 6 months. Most of the recurrences developed in patients with no predisposing abnormalities. Conclusion: Pediatric nephrologic follow-up of babies experiencing UTI in the neonatal period is very important to identify the predisposing congenital abnormalities and scarred kidneys, to diagnose and to treat the recurrences earlier.

Bilateral fungal obstruction of the renal collecting system is rare in infancy. Treatment options include medical or surgical procedures. Reports of successful medical treatment with liposomal amphotericin B have been published but the duration of treatment is controversial. We report a 3-week-old preterm baby with myelomeningocele who had experienced acute renal failure related to bilateral renal fungus balls, which improved with percutaneous nephrostomy and 12 weeks of liposomal amphotericin B intravenously combined with 5-fluorocytosine orally for 9 weeks.

Propionic acidemia is an inherited disorder of organic acid metabolism characterized by a spectrum of clinical and biochemical findings. The usual presentation is life-threatening ketoacidosis and hyperammonemia. In this report we present a neonate with propionic acidemia presenting with prominent neurologic problems without ketoacidosis. The patient had a serum ammonia level of 3,500 microg/dl which was effectively lowered to normal values in 48 hours by peritoneal dialysis, with remarkable improvement in neurologic status. However, she developed Candida albicans peritonitis, and sepsis and died of cardiorespiratory failure. Infants who have an early onset propionic acidemia have a high mortality and morbidity rate. In conclusion, propionic acidemia should be in the differential diagnosis of patients with neurologic symptoms and hyperammonemia with or without acidosis.

We have studied serological and clinical response to live, attenuated varicella zoster virus (VZV) vaccine (Varilrix, SmithKline Beecham) in 20 patients with steroid-sensitive nephrotic syndrome (SSNS) in remission and 22 normal controls who had no history of varicella and no detectable antibody to VZV. Nephrotic patients included 15 boys and 5 girls, with a mean age of 4.7 years (range 2-11.4 years). The controls were healthy age-matched children (13 girls and 9 boys). Seventeen patients with SSNS (85%) and 19 healthy controls (86%) seroconverted 8 weeks after vaccination. One patient with SSNS had a relapse 20 days after vaccination, and 1 child in the control group had a rash. Two years after vaccination, antibodies to VZV were detected in 12 of 17 responders, 2 of 3 non-responders, and 13 of 22 controls. Within 2 years of vaccination, 3 of the vaccine responder children with SSNS had a mild varicella infection. Two responder and 1 non-responder nephrotic children and 9 controls were lost to long-term follow-up. Our results show that immunization with a single dose of VZV vaccine is safe and effective in children with SSNS in remission.