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Objective: We aimed to present the characteristics, genetic analysis results, long-term prognosis of our patients with distal kidney tubular acidosis, and the relationship between hyperammonemia and distal kidney tubular acidosis. Materials and Methods: Biochemical, clinical, and imaging findings were collected at presentation and the last clinic visit, and results of the genetic analysis were recorded. Results: Our study included 9 patients (3 female, 33%). The median age at diagnosis was 3 months, and the median follow-up period was 111 months. Height standard deviation scores were less than −2 in 4 (44%) patients at presentation and in 3 (33%) at the last clinic visit. The median estimated glomerular filtration rate was 98 mL/min/1.73 m2 at presentation and 126 mL/min/1.73 m2 at the last clinic visit. We have found 8 different types of mutations of 2 genes, including 6 in the ATP6V0A4 gene, 2 in the SLCA4A1 gene, and 2 of them were novel. At the time of presentation, nephrocalcinosis and hypercalciuria were present in all our patients, but at the last visit, only 1 patient had hypercalciuria. Sensorineural hearing loss was found in 4 of our patients with a mutation in the ATP6V0A4 gene. Serum ammonia levels were found to be high in 3 patients with mutations in the ATP6V0A4 gene. Conclusion: Adequate metabolic control is essential for optimal growth and preserved kidney function in distal kidney tubular acidosis patients. Distal kidney tubular acidosis may be associated with hyperammonemia. We recommend keeping potassium levels at high-normal levels to reduce ammonia levels, especially in the absence of acidosis.

Introduction: We aimed to evaluate cardiovascular risks and influencing factors by measuring aortic elasticity parameters and carotid intima thickness in children with cronic renal failure (CRF), and also evaluated the hydration status of patients with Bioimpedance spectroscopy (BIS) measurements and investigated the effect of hydration status on vascular functions. Metods: The study group consisted of 38 patients with CRF, their ages between 6 to 20 years (mean ± SD= 13.34 ± 3.74), of which 16 were girls and 22 were boys. The control group, which consisted of 31 healthy children between 7 and 16.5 years of age (mean ± SD= 12.1± 2.92), 16 girls and 15 boys). Left ventricular end-diastolic, end-systolic, left atrial diameters, interventricular septum, and left ventricular posterior wall thickness were measured. Systolic and diastolic diameters of the aortic annulus and aorta at each level were obtained; z-scores, aortic strain, distensibility, stiffness index were calculated. Carotid intima-media thickness and flow- mediated dilatation were studied. Bioimpedance spectroscopy was performed to all patients. Results: Interventricular septum and left atrial (p=0,002, p=0,013), sinus valsalva and sinotubular junction z scores (p=0,009, p=0,012) were found to be higher and distensibility and strain decreased, stiffness index increased in the abdominal aorta of patients with CRF (p=0,007, p=0,002, p=0,004). Patients with CRF had statistically significant over-hydration. Conclusion: Vascular wall changes that affect the elastic properties of the aortic wall begin to develop in childhood in patients with CRF. This is related to the severity of the disease and the patient’s hydration status.

There are various oral symptoms related to the disease and its management in individuals with chronic kidney disease (CKD). The aim of the study was to investigate the oral health status of children with different stages of CKD, kidney transplant recipients (KTR), and healthy children.A total of seventy-one children diagnosed with CKD and fifty-two healthy children were included in the study. Each patient was examined for dental caries by the decayed-missing-filled-teeth (DMFT/dmft) index and the International Caries Detection and Assessment System (ICDAS-II), developmental defects of enamel (DDE) by the DDE index, and oral hygiene by the debris (DI), calculus (CI), and simplified oral hygiene (OHI-S) indices.The median number of DMFT/dmft was 1.00 (interquartile range (IQR):1.00-4.00) in children with stage 1-3 CKD, 0.00 (IQR: 0.00-2.50) in stage 4-5 children, 0.00 (IQR: 1.00-3.00) in KTR, and 8.00 (IQR: 1.00-13.00) in healthy children. According to ICDAS-II categories, the percentage of children with severe caries was 53.8% in healthy children, while it was 44.4% in KTR, 25.9% in stage 1-3, and 11.4% in stage 4-5 children. While the percentage of children with DDE was 88.8% in KTR, 80% in stage 4-5, and 66.7% in stage 1-3 children, this rate was 44.2% in healthy children. The highest mean OHI-S score was observed in stage 4-5 children (2.10 ± 1.08), followed by KTR (1.46 ± 1.19), stage 1-3 (1.27 ± 0.61), and healthy children (0.45 ± 0.44), respectively.Compared to healthy children, children with CKD had more debris accumulation, calculus formation, and more DDE but a lower severity of dental caries. A higher resolution version of the Graphical abstract is available as Supplementary information.

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 International Pediatric Nephrology Association.Background: Automated peritoneal dialysis (APD) is increasingly preferred worldwide. By using a software application (Homechoice with Claria sharesource system (CSS)) with a mod-M added to the APD device, details of the home dialysis treatment become visible for PD nurses and physicians, allowing for close supervision. We aimed to evaluate the perceptions of patients/caregivers, PD nurses, and physicians about the advantages and disadvantages of CSS. Methods: Three different web-based questionnaires for patients/caregivers, nurses, and physicians were sent to 15 pediatric nephrology centers with more than 1 year of experience with CSS. Results: Respective questionnaires were answered by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses. Most of the nurses and physicians (87% and 73%) reported that CSS improved patient monitoring. A total of 73% of nurses suggested that CCS is not well known by physicians, while half of them reported reviewing CSS data for all patients every morning. Sixty-eight percent of physicians thought that CSS helps save time for both patients/caregivers and healthcare providers by reducing visits. However, only 20% of patients/caregivers reported reduced hospital visits. A total of 90% of patients/caregivers reported that being under constant monitoring made them feel safe, and 83% stated that the patient’s sleep quality improved. Conclusions: A remote monitoring APD system, CSS, can be successfully applied with children for increased adherence to dialysis prescription by giving shared responsibility and may help increase the patient’s quality of life. This platform is more commonly used by nurses than physicians. Its potential benefits should be evaluated in further well-designed clinical studies with larger patient groups. Graphical abstract: A higher resolution version of the Graphical abstract is available as Supplementary information. [Figure not available: see fulltext.]

Objective: As increasing number of young people with renal diseases are reaching adulthood and issues related to their transition to adult units are getting more important. Failure to transfer adolescents under a well-designed transfer program can lead to a decline in attendance to an adult unit. We aim to share our experience and the views of the patients who went through a transition program. Material and Methods: We conducted a telephone questionnaire with 88 patients, who were asked to evaluate their con cerns before their transition and their experience during the visit in the adult nephrology unit after the transition process. Results: The mean age of participants was 19.21±0.97 years. Thirty patients (34%) had chronic kidney disease, and three of them were on dialysis. Three patients (3.4%) had a renal transplant at the time of transition. Fifty-one patients (58%) were felt anxious before the transition process. After their transfer to the adult unit, although 84 patients (95.5%) were satisfied to be in the adult unit, there were still 65 patients (74%) ready to return to the pediatric unit, if it was possible. Conclusion: Transition to the adult unit is still a cause of concern for adolescent patients, even if they are transferred under a well-designed program. It is important to raise awareness about transition programs among pediatricians and to develop a pedi atric-adult transition program for the medical and psychological well-being of patients.

Objective: Classical Bartter syndrome (cBS) and Gitelman syndrome (GS) are genotypically distinct, but there is a phenotypic overlapamong these two diseases, which can complicate the accurate diagnosis without genetic analysis. This study aimed to evaluate thecorrelation between clinical and genetic diagnoses among patients who have genetically defined cBS and GS.Patients and Methods: The study included 18 patients with homozygous/compound heterozygous CLCNKB (NM_000085) (n:10/18)and SLC12A3 (NM_000339) (n:8/18) mutations. Biochemical, clinical and radiological data were collected at presentation and at thelast visit.Results: In cBS group age at diagnosis, median plasma potassium and chloride concentrations were significantly lower and medianplasma HCO3 and blood pH values were significantly higher. Patients with GS had significantly lower median plasma magnesiumconcentrations and urinary calcium/creatinine ratio. One child with GS had normocalciuria, two children with cBS had hypocalciuriaand hypomagnesemia. Low estimated glomerular filtration rate (eGFR) (ml/dk/1.73m2) and growth failure were more evident in cBSgroup. In patients with cBS, nine different CLCNKB gene mutations were detected, five of them were novel. Novel mutations were:one nonsense (c.66G>A, p.Trp22*), one missense (c.499G>A, p.Gly167Ser) and three splice-site (c.867-2delA; c.499-2insG; c.1930-2A>C) mutations. In patients with GS, six different SLC12A3 gene mutations were found.Conclusions: It may not always be possible to clinically distinguish cBS from GS. We suggest to perform a genotypic classification ifgenetic analysis is possible.

Background Cubilin is one of the receptor proteins responsible for reabsorption of albumin in proximal tubules and is encoded by the CUBN gene. We aimed to evaluate clinical and genetic characterization of six patients with proteinuria who had CUBN mutations. Methods Patients’ characteristics, serum creatinine, albumin, vitamin B12 levels, urine analysis, spot urine protein/creatinine, microalbumin/creatinine, beta-2 microglobulin/creatinine ratios, estimated glomerular fltration rates (eGFR), treatments, kidney biopsies, and genetic analyses were evaluated. Results Six patients (2 female, 4 male) with an incidental finding of proteinuria were evaluated. Mean admission age and follow-up time were 7.3 ± 2.9 and 6.5 ± 5.6 years, respectively. Serum albumin, creatinine, and eGFR were normal; urine analysis revealed no hematuria, and C3, C4, ANA, and anti-DNA were negative; kidney ultrasonography was normal for all patients. Urine protein/creatinine was 0.9± 0.3 mg/mg, and microalbumin was high in all patients. Serum vitamin B12 was low in two patients and normal in four. Kidney biopsy was performed in four patients, three demonstrated normal light microscopy, and there was one focal segmental glomerulosclerosis (FSGS). Genetic tests revealed four homozygous and two compound heterozygous mutations in the C-terminal part of cubilin. All patients had normal eGFR and still had non-nephrotic range proteinuria at last visit. Conclusions CUBN gene mutations should be considered in patients with isolated non-nephrotic range proteinuria and normal kidney function. Diagnosing these patients, who are thought to have a better prognosis, is important in terms of avoiding unnecessary treatment and predicting prognosis. CUBN gene mutations may also present as FSGS which extends the spectrum of renal manifestation of these patients.

© 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.

[No Abstract Available], 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 (no. 01EO0802), the European Renal Association-European Dialysis and Transplant Association, and Roche Organ Transplant Research Foundation (no. 365520785). Several co-authors are members of the European Reference Network for Rare Kidney Diseases.

BACKGROUND AND AIMS Chronic kidney disease (CKD) is a complex medical and social problem worldwide due to high prevalence and mortality rates. According to the ESPN/ERA-EDTA, the prevalence of CKD stages 3–5 in children is about 55–60 pmarp [1]. Moreover, CKD usually causes different severe complications, including pathologic changes in the cardiovascular system, which significantly affect long-term survival. Unlike many complications of CKD, hypertension can be present in the earliest stages of the disease [2]. Nowadays, there has been a scientific and practical interest in Fibroblast growth factor 23 (FGF-23) which is mostly considered as a phosphate-regulating biomarker [3]. There are some speculations that FGF-23 affects blood pressure (BP) in adults due to the impact on the renin-angiotensin-aldosterone system (RAAS) by decreasing calcitriol [4] and the direct effect of FGF-23 on sodium reabsorption, which has been demonstrated in experimental models [5]. Therefore, the aim of our study was to investigate the link between FGF-23 and BP in children with CKD. METHOD There were 73 children with CKD stages 1–5, mean age was 9.79 ± 0.58 years. BP was determined by 3 times measurement and calculating the mean value. Received results were compared with percentile norms according to age and gender in order to divide patients into two groups: normotensive and hypertensive. FGF-23 was determined in serum by multimatrix ELISA kit (Biomedica Medizinprodukte GmbH, Austria). Statistical analysis was performed using SPSS version 26 (IBM, USA). RESULTS In the group with normal BP the median of FGF-23 in serum was 1.8 [0.7–3.4] pmol/L. In comparison, in the group with a hypertensive level of BP median indicator of FGF-23 was 7.6 (1.98–18.5) pmol/L (P CONCLUSION Our findings confirm that FGF-23 is linked to BP in children with CKD what makes us conclude that more careful attention to children with a high level of FGF-23 is needed in relation to hypertension and as a consequence cardiovascular complications. However, more investigations should be done in order to establish a causal relationship.

Background: Nephrotic Syndrome (NS) is one of the most common renal diseases in children. Clinical response to corticosteroids is an important prognostic factor for long-term renal outcome. We aimed to evaluate the clinical parameters, histopathological findings and complications of NS patients and factors predicting steroid resistance in a single tertiary center. Methods: A hundred and sixty two children (57 girls, 105 boys) with NS followed between 1998 and 2018 in Department of Pediatric Nephrology were analyzed in a retrospective cohort. Secondary causes and infantile NS cases were excluded. Results: The median (IQR; range) age of the children at presentation and follow up time were 4.9(5.7; 0.1-16.8) and 5.5(5.4; 0.1-20.3) years, respectively. A hundred and thirty four (82.7%) patients were steroid-sensitive nephrotic syndrome (SSNS) and 28(17.3%) were steroid-resistant nephrotic syndrome (SRNS). Age at the first presentation was lower in SSNS group (p=0.002). Hypertension, macroscopic and microscopic hematuria were higher in SRNS group (pConclusion:The late onset is still an important sign for SRNS. Microscopic and macroscopic hematuria and hypertension may also give a hint for potential steroid resistance. We emphasize that FSGS is a biopsy finding that may reveal to different diagnosis in SRNS.

The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20. A total of 46 confirmed cases of COVID-19 were reported from 12 centers, of which 17 were dialysis patients, and 29 were KTx recipients. Thus, the incidence rate of COVID-19 was 9.3% among dialysis patients and 9.2% among KTx recipients over a 9-month period in Istanbul. Twelve KTx recipients and three dialysis patients were asymptomatic (p = 0.12). Most of the symptomatic patients in both the dialysis and KTx groups had a mild respiratory illness. Only two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. In the entire cohort, one hemodialysis patient with multiple comorbidities died. Conclusion: while most cases are asymptomatic or have a mild disease course, pediatric patients undergoing dialysis and a kidney transplant are at increased risk for COVID-19. What is known: in adult population, both dialysis patients and kidney transplant recipients are at increased risk for severe illness of COVID-19 and have higher mortality rate. Children with kidney transplantation are not at increased risk for COVID-19 and most have mild disease course. Data on children on dialysis are scarce. What is new: pediatric patients undergoing dialysis and kidney transplantation have an increased risk for COVID-19. Most patients undergoing renal replacement therapy either on dialysis or transplanted develop asymptomatic or mild COVID-19 disease with a favorable outcome., NA

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.

Background The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation. We explored the added value of uHSP70 in discriminating UTI from other infections and conditions confused with UTI. Methods A total of 802 children from 37 pediatric centers in seven countries participated in the study. Patients diagnosed with UTI (n = 191), non-UTI infections (n = 178), contaminated urine samples (n = 50), asymptomatic bacteriuria (n = 26), and healthy controls (n = 75) were enrolled. Urine and serum levels of HSP70 were measured at presentation in all patients and after resolution of the infection in patients with confirmed UTI. Results Urinary (u)HSP70 was selectively elevated in children with UTI as compared to all other conditions (p < 0.0001). uHSP70 predicted UTI with 89% sensitivity and 82% specificity (AUC = 0.934). Among the 265 patients with suspected UTI, the uHSP70 > 48 ng/mL criterion identified the 172 children with subsequently confirmed UTI with 90% sensitivity and 82% specificity (AUC = 0.862), exceeding the individual diagnostic accuracy of leukocyturia, nitrite, and leukocyte esterase positivity. uHSP70 had completely normalized by the end of antibiotic therapy in the UTI patients. Serum HSP70 was not predictive. Conclusions Urine HSP70 is a novel non-invasive marker of UTI that improves the diagnostic accuracy of conventional urinalysis. We estimate that rapid urine HSP70 screening could spare empiric antibiotic administration in up to 80% of children with suspected UTI., Scientific Research Projects Coordination Unit of Istanbul University; European Society for Pediatric Nephrology (ESPN); Society for Children's Kidney Health; Istanbul Faculty of Medicine Foundation, UTILISE study was supported by Scientific Research Projects Coordination Unit of Istanbul University, by the European Society for Pediatric Nephrology (ESPN), by the Society for Children's Kidney Health, and by Istanbul Faculty of Medicine Foundation.

Background. Antiphospholipid syndrome (APS), particularly the catastrophic antiphospholipid syndrome (CAPS), is one of the rare causes of thrombotic microangiopathy (TMA). CAPS is the most severe form of APS, especially when accompanied by complement dysregulation, causes progressive microvascular thrombosis and failure in multiple organs. In this report, a case of CAPS with TMA accompanied by a genetic defect in the complement system is presented.Case. A 13-year-old girl was admitted to the hospital with oliguric acute kidney injury, nephrotic range proteinuria, Coombs positive hemolysis, refractory thrombocytopenia, a low serum complement C3 level and anti-nuclear antibody (ANA) positivity. The kidney biopsy was consistent with TMA. She was first diagnosed with primary APS with clinical and pathological findings and double antibody positivity. As initial treatments, plasmapheresis (PE) was performed and eculizumab was also administered following pulse -steroid and intravenous immunoglobulin treatments. Her renal functions recovered and she was followed up with mycophenolate mofetil, hydroxychloroquine, low dose prednisolone and low molecular weight heparin treatments. The patient presented with severe chest pain, vomiting and acute deterioration of renal functions a few months after the diagnosis of TMA. A CAPS attack was considered due to radiological findings consistent with multiple organ thrombosis and intravenous cyclophosphamide (CYC) was given subsequent to PE. After pulse CYC and PE treatments, her renal functions recovered, she is still being followed for stage-3 chronic kidney disease. Complement factor H-related protein I gene deletion was detected in the genetic study.Conclusions. The clinical course of complement mediated CAPS tends to be worse. Complement system dysregulation should be investigated in all CAPS patients, and eculizumab treatment should be kept in mind if detected.

Functional iron deficiency seco ndary to inflammation and increased serum hepcidin lead to erythropoietin-resistant anemia in children with chronic kidney disease. Vitamin D deficiency, parathyroid hormone, and phosphate can also participate in chronic inflammation and anemia. The aim of this study was to evaluate the association between hepcidin, bone mineral metabolism, and anemia in non-dialysis pediatric patients with chronic kidney disease.Thirty-five patients with stage 2-4 chronic kidney disease and 35 healthy subjects were enrolled in the study. Serum creatinine, blood urea nitrogen, uric acid, C-reactive protein, interleukin-6, hepcidin, complete blood count, ferritin, calcium, phosphorus, parathyroid hormone, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D, and fibroblast growth factor-23 levels were compared between the groups.Ferritin, C-reactive protein, interleukin-6, blood urea nitrogen, creatinine, uric acid levels, and percentages of reticulocytes were significantly higher than in the controls (p0.05). The mean serum hepcidin levels in the chronic kidney disease and control groups were 9.6±5.2 (range, 2.15-25.3) and 9.7±4.3 (range, 3.4-22.2) ng/mL and were not significantly different in either group. There were no differences in terms of serum phosphorus, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D and fibroblast growth factor-23 levels between the groups (p0.05). Serum hepcidin levels were not correlated with anemia parameters, serum fibroblast growth factor-23, phosphorus, uric acid, C-reactive protein, parathyroid hormone, and 25-hydroxyvitamin D levels (p0.05). However, serum hepcidin levels were correlated with 1,25-dihydroxyvitamin D and interleukin-6 levels (p=0.013 and p=0.002, respectively).Serum hepcidin levels may not increase significantly in non-dialysis pediatric patients with chronic kidney disease despite high levels of inflammatory markers such as C-reactive protein and interleukin-6. The increase of serum hepcidin levels may be inhibited by effective treatment of anemia with iron supplementation and erythropoietin, and the treatment of secondary hyperparathyroidism with phosphate binders and the active form of vitamin D, which decrease serum parathyroid hormone and fibroblast growth factor-23 levels, and control inflammation to some extent.İnflamasyona ikincil fonksiyonel demir eksikliği ve artmış hepsidin düzeyleri kronik böbrek hastalığı olan çocuklarda eritropoetin dirençli anemiye neden olmaktadır. Vitamin D eksikliği, parathormon ve fosfor da kronik inflamasyon ve anemiye katkıda bulunabilmektedir. Bu çalışmanın amacı, diyaliz ihtiyacı olmayan kronik böbrek hastalıklı çocuklarda hepsidin ile anemi ve kemik mineral metabolizmasının ilişkisini araştırmaktır.Evre 2–4 kronik böbrek hastalığı olan 35 çocuk ile 35 sağlıklı çocuk çalışmaya alındı. Serum kreatinin, kan üre nitrojeni, ürik asit, C-reaktif protein, interlökin-6, hepsidin, tam kan sayımı, ferritin, kalsiyum, fosfor, parathormon, 25-hidroksivitamin D, 1,25- hidroksivitamin D ve fibroblast büyüme faktörü-23 düzeyleri iki grup arasında karşılaştırıldı.Ferritin, C-reaktif protein, kan üre nitrojeni, kreatinin ve ürik asit düzeyleri ile retikülosit yüzdesi kontrol grubuna göre anlamlı yüksek idi (p0,05). Kronik böbrek hastalığı olan grup ile sağlıklı çocuklardan oluşturulan grupta, ortalama serum hepsidin düzeyleri sırası ile 9,6±5,2 (en düşük-en yüksek, 2,15–25,3) ve 9,7±4,3 (en düşük-en yüksek, 3,4–22,2) ng/mL olup iki grup arasında anlamlı fark saptanmadı. İki grup arasında serum fosfor, 25-hidroksivitamin D, 1,25- hidroksivitamin D ve fibroblast büyüme faktörü-23 düzeyleri bakımından da anlamlı farklılık saptanmadı (p0,05). Hasta grubundaki serum hepsidin düzeyi ile anemi belirteçleri, fibroblast büyüme faktörü-23, fosfor, ürik asit, C-reaktif protein, 25-hidroksivitamin D ve parathormon düzeyleri korele değildi (p0,05). Bununla birlikte serum hepsidin düzeyleri ile 1,25- hidroksivitamin D ve interlökin-6 düzeylerinin korelasyon gösterdiği görüldü (sırasıyla p=0,013 ve p=0,002).Diyaliz ihtiyacı olmayan kronik böbrek hastalıklı çocuklarda, C-reaktif protein ve interlökin-6 gibi inflamasyon belirteçlerindeki artışa rağmen serum hepsidin düzeyleri anlamlı olarak artmayabilir. Demir desteği ve eritropoetin ile etkin anemi tedavisi ile fosfat bağlayıcılar ve aktif vitamin D ile ikincil hiperparatiroidizmin etkin tedavi edilerek serum parathormon ve fibroblast büyüme faktörü-23 düzeylerinin düşürülmesi bu hastalardaki inflamasyonu kontrol altına alarak serum hepsidinin artmasını engelleyebilir.

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.

The aim of the study is to present incidence of COVID-19 in children with renal replacement therapy (RRT) and compare the severity and outcomes of the disease between the groups undergoing dialysis and kidney transplantation (KTx). This multicenter observational study was based on data collected from children receiving RRT, either chronic dialysis or KTx, diagnosed with COVID-19. All members of the Istanbul branch of the Turkish Pediatric Nephrology Association were requested to submit all their confirmed COVID-19 cases between April 1 and December 31, 2020. They were also asked to report the number of their prevalent patients on RRT under the age of 20. A total of 46 RRT patients diagnosed with COVID-19 were reported from 12 centers. Of these, 29 were KTx recipients, and 17 were on dialysis. COVID-19 cases represented 9.3% of all prevalent dialysis patients and 9.2% of all prevalent KTx recipients. Twelve KTx recipients (41.4%) and three dialysis patients (17.6%) were asymptomatic (p = 0.12). The hospitalization rate was higher in dialysis patients than KTx recipients (82.4% vs. 27.6%, p = 0.001). Two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. Acute kidney injury developed in 36% of KTx recipients, but none required dialysis or lost their graft. In the entire cohort, one patient on hemodialysis with multiple comorbidities died.Conclusion: Although most of cases were asymptomatic or had a mild disease, children on RRT seem to have an increased risk for COVID-19.

Renal abscess, the accumulation of infected fluid in the kidney, is a rare condition seen in children as well as adults. It leads to long term hospital admission and antibiotic use. Early diagnosis is an important factor in the outcome of renal abscess because the management may differ. Urinalysis test results and radiologic imaging findings of the patients who are admitted to hospital with complaints of fever, vomiting, abdominal pain or flank pain are important for the early diagnosis. Undertreated cases have high risk for renal scar formation. In this paper, we aim to present three pediatric patients, who showed the complication of renal abscess and were treated with long term antibiotic use without a need for surgical drainage.

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 and objectives: Chronic kidney disease (CKD) in children is a complex medical and social issue around the world. One of the serious complications is mineral-bone disorder (CKD-MBD) which might determine the prognosis of patients and their quality of life. Fibroblast growth factor 23 (FGF-23) is a phosphaturic hormone which is involved in the pathogenesis of CKD-MBD. The purpose of the study was to determine what comes first in children with CKD: FGF-23 or phosphate. Materials and Methods: This cross-sectional study included 73 children aged 2&ndash, 18 years with CKD stages 1&ndash, 5. We measured FGF-23 and other bone markers in blood samples and studied their associations. Results: Early elevations of FGF-23 were identified in children with CKD stage 2 compared with stage 1 (1.6 (1.5&ndash, 1.8) pmol/L versus 0.65 (0.22&ndash, 1.08), p = 0.029). There were significant differences between the advanced stages of the disease. FGF-23 correlated with PTH (r = 0.807, p = 0.000) and phosphate (r = 0.473, p = 0.000). Our study revealed that the elevated level of FGF-23 went ahead hyperphosphatemia and elevated PTH. Thus, more than 50% of children with CKD stage 2 had the elevating level of serum FGF-23, and that index became increasing with the disease progression and it achieved 100% at the dialysis stage. The serum phosphate increased more slowly and only 70.6% of children with CKD stage 5 had the increased values. The PTH increase was more dynamic. Conclusions: FGF-23 is an essential biomarker, elevates long before other markers of bone metabolism (phosphate), and might represent a clinical course of disease.

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.

We aimed to present five patients presenting with hypertension and diagnosed as renal artery stenosis. Five patients with renal artery stenosis were studied retrospectively. Two of our cases were diagnosed as fibromuscular dysplasia, other cases were diagnosed as Takayasu arteritis and Moyamoya disease, and one of them had congenital vascular abnormality. Renovascular hypertension is one of the most important causes of secondary hypertension in children, and renal artery stenosis constitutes a significant proportion of renovascular disease. We must consider renal artery stenosis in children presenting with resistant hypertension.

Background and Aims Alport syndrome (AS) is an inherited glomerular basement membrane disease caused by mutations in COL4A3, COL4A4 or COL4A5 genes. Recently, it has been reported that focal segmental glomerulosclerosis (FSGS) can be seen in AS and COL4A mutations can be detected in FSGS. In this study, we aimed to define the clinical characteristics of patients with genetically confirmed AS, in order to establish genotype-phenotype correlation and investigate the effects of different treatment regimes. Method A total of 87 pediatric AS patients included in this multicenter study. We retrospectively collected data from medical records and requested other centers to fill out a questionnaire, which included data regarding patient demographic features, family history, clinical and laboratory characteristics at first presentation, histopathological (if available) and genetic tests results, treatments and yearly follow-up results. Results A total of 87 (41 female, 46 male) genetically confirmed AS patients (COL4A5, n=43; COL4A3, n=25; COL4A4, n=19) were studied. Mean age at first presentation was 7.6±4.1 years and the median follow-up duration was 4.3 years (IQR 1.9–7.3). 14 (16.1%) of 87 patients presented with nephrotic syndrome (NS); renal biopsy findings showed FSGS in 11 (79%) of 14 patients with NS, and COL4A3 mutations were the most common (n=7, 50%) in this group. Of 14 NS patients, 12 received steroid, 11 received cyclosporine (CsA) and 4 received other immunosuppressives prior to genetic diagnosis. The inheritance pattern of the patients with NS was consistent with ARAS in 10 patients (71.4%), XLAS in 3 patients (21.4%), and ADAS in 1 patient (7.2%). During follow up, glomerular filtration rate (GFR) decreased below 90 ml/min/1.73 m2 in 24 of 87 patients (27.5%). COL4A3 mutations (n=14, 58.3%) were the leading genetic abnormality in patients who progressed to chronic kidney disease (CKD). At the last visit, GFR loss was significantly higher in patients with COL4A3 mutations when compared to patients with COL4A4 and COL4A5 mutations (p=0.04). Among patients with NS, 9 of 14 (64.2%) progressed to CKD. Genetic results of patients with NS who progressed to CKD were COL4A3 in 6 (66.7%), COL4A4 in 2 (22.2%) and COL4A5 in 1 (11.1%) patients. In survival analysis, renal survival rate without CKD was 12.1 years (95% CI: 6.7-17.5). After the first presentation, the 5-year cumulative risk of CKD was 51.8%, 12.6%, and 12.9% in patients with COL4A3, COL4A4 and COL4A5 mutations, respectively (p=0.001). We observed that patients with COL4A3 mutations, ARAS inheritance pattern, histopathology of FSGS or NS presentation progressed to CKD earlier (p Conclusion Detailed analyses of data from genetically confirmed Turkish patients with AS provided important clues regarding to the presentation, course and outcomes of the disease. COL4A3 mutations, ARAS inheritance pattern, NS presentation and FSGS finding in renal biopsy are major risk factors for progression to CKD. We recommend genetic testing for patients suspected of having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid resistant NS. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications.

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.

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerininaldosterona duyarsızlığı ile karakterize, hiperkalemi,hiponatremi ve metabolik asidoz ile kendini gösteren nadirbir durumdur. Obstrüktif üropatisi olan bebeklerde vepiyelonefrit sırasında görülebilir. Gelişimi devam eden erkenbebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisive enfeksiyonu sekonder PHA hastalarında tübüluslardaaldosteron direncine neden olan üç önemli faktördür. Genelliklehiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır,tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteronseviyelerinin saptanması ile tanı konur. Süt çoçuklarındabiyokimyasal incelemede hiperpotasemi, hiponatremi vemetabolik asidoz saptandığında konjenital adrenal hiperplaziayırıcı tanısında PHA göz önünde bulundurulmalı ve buhastalarda üriner ultrasonografisi (USG) ve idrar analizi rutinolarak yapılmalıdır. Bu yazımızda sekonder PHA gelişenposterior üretral valv (PUV)‘lı iki hasta sunuldu., Secondary pseudohypoaldosteronism (PHA) is a rare conditionthat presents with hyperkalemia, hyponatremia, and metabolicacidosis, which develops due to renal tubular unresponsivenessto aldosterone. It may be seen in infants with obstructiveuropathy and acute pyelonephritis. Three important reasons ofthe aldosterone unresponsiveness of the tubules are early infancykidney, obstructive uropathy, and urinary tract infection. Besidesother findings, diagnosis is established with highly increasedplasma aldosterone and renin levels. In infants who present withhyperkalemia, hyponatremia, and metabolic acidosis, PHA shouldbe considered in the differential diagnosis of congenital adrenalhyperplasia. Urinary ultrasonography and urine analysis should beperformed in these patients. Herein, we present two patients withsecondary PHA caused by posterior urethral valves.

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.

Objective: Renal cystic diseases consist of a broad spectrum of hereditary or acquired conditions that may lead to end stage renal disease. We aimed to evaluate our patients diagnosed as renal cystic disease in terms of their diagnosis, demographic findings and clinical follow-up. Materials and Methods: The patients followed between 1993-2015 in our pediatric nephrology outpatient department with renal cystic diseases were evaluated retrospectively. Results: In 237 patients, 110 (46.41%) were female, 127 (53.59%) were male. One hundred-eight (45.56%) patients were diagnosed antenatally, the mean age at diagnosis was 7.23 +/- 4.72 (0-17) years in 129 patients. The diagnosis were simple-cyst in 36 (15.18%), multicystic displastic kidney disease in 112 (47.25%), autosomal dominant polycystic kidney disease in 56 (23.62%), autosomal recessive polycystic kidney disease in 22 (9.28%), cyst hydatic in three (1.26%), Joubert sydrome in two, nephronophthisis in one, tuberosclerosis in two, Bardet-Biedl syndrome in three patients. Five patients (2.1%) died and ten (4.21%) patients progressed to chronic kidney injury. Proteinuria was found in 15 (6.32 %) and hypertension in 10 (4.21%) patients. Conclusion: Renal cystic disease is an important group that can lead to proteinuria, hypertension and end stage kidney failure. Periodic follow-up is important in these patients to avoid and treat the complications early and properly.

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.