Clinical Predictors of Steroid Resistance in Childhood Nephrotic Syndrome.

We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.9 (5.7; 0.1-16.8) and 5.5 (5.4; 0.1-20.3) years. A total of 82.7% of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 17.3% were steroid-resistant nephrotic syndrome (SRNS). The median age at first presentation was lower in the SSNS group (P = .002). The most common histopathological findings were focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD). Hypertension and macroscopic and microscopic hematuria were higher in the SRNS group (P < .001). The age and microscopic hematuria were independent risk factors for steroid resistance (P = .019 and P = .002, respectively). Complement 3 (C3) was evaluated in 148 patients and found low in 7 patients who were subsequently diagnosed as membranoproliferative glomerulonephritis. There is still no better clinical predictor for steroid response than late age of onset and microscopic hematuria. Hypertension may also give a hint for potential steroid resistance.

Assessment of the oral health status of children with chronic kidney disease.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Humoral and cellular immune response to SARS-CoV-2 mRNA BNT162b2 vaccine in pediatric kidney transplant recipients compared with dialysis patients and healthy children.

BACKGROUND: Compared with the general population, the immune response to COVID-19 mRNA vaccines is lower in adult kidney transplant recipients (KTRs). However, data is limited for pediatric KTRs. In this study, we aimed to assess humoral and cellular immune responses to the COVID-19 mRNA vaccine in pediatric KTRs. METHODS: This multicenter, prospective, case-control study included 63 KTRs (37 male, aged 12-21 years), 19 dialysis patients, and 19 controls. Humoral (anti-SARS-CoV2 IgG, neutralizing Ab (nAb)) and cellular (interferon-gamma release assay (IGRA)) immune responses were assessed at least one month after two doses of BNT162b2 mRNA vaccine. RESULTS: Among COVID-19 naïve KTRs (n = 46), 76.1% tested positive for anti-SARS-CoV-2 IgG, 54.3% for nAb, and 63% for IGRA. Serum levels of anti-SARS-CoV-2 IgG and nAb activity were significantly lower in KTRs compared to dialysis and control groups (p < 0.05 for all). Seropositivity in KTRs was independently associated with shorter transplant duration (p = 0.005), and higher eGFR (p = 0.007). IGRA titer was significantly lower than dialysis patients (p = 0.009). Twenty (43.4%) KTRs were positive for all immune parameters. Only four of 11 seronegative KTRs were IGRA-positive. COVID-19 recovered KTRs had significantly higher anti-SARS-CoV-2 IgG and nAb activity levels than COVID-19 naïve KTRs (p = 0.018 and p = 0.007, respectively). CONCLUSIONS: The humoral and cellular immune responses to SARS-CoV-2 mRNA BNT162b2 vaccine are lower in pediatric KTRs compared to dialysis patients. Further prospective studies are required to demonstrate the clinical efficacy of the mRNA vaccine in KTRs. This prospective study was registered in ClinicalTrials.gov (NCT05465863, registered retrospectively at 20.07.2022). A higher resolution version of the Graphical abstract is available as Supplementary information.

Clinical and genetic characterization of children with cubilin variants.

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 filtration 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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Collapsing Glomerulopathy in a Patient with a TRPC6 Mutation Presenting as Rapidly Progressive Glomerulonephritis: A Case Report and Review of the Literature.

Collapsing glomerulopathy (CG) is a proliferative disease characterized by segmental or global wrinkling of the glomerular basement membrane and the formation of pseudocrescents, whereas focal segmental glomerulosclerosis (FSGS) is characterized by podocytopenia, and focal and segmental sclerosis of the glomeruli. Mutations in NPHS1, NPHS2, WT1, PLCE1, CD2AP, ACTN4, and TRPC6 have been reported in steroid-resistant FSGS patients. The mutations p.R895C and p.R895L in Exon 13 are the only ones in TRPC6 causing CG reported to date. Here, we present the case of a 17-year-old male patient with a collapsing variant of familial FSGS caused by a mutation in TRPC6 (p.R895C) who presented with rapidly progressive (crescentic) and proliferative glomerulonephritis.

Phenotypic and genotypic characteristics of children with Bartter syndrome.

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.

Molecular Aspects of Distal Kidney Tubular Acidosis in Children, Its Long-Term Outcome, and Relationship with Hyperammonemia.

OBJECTIVE: We aimed to present the characteristics, genetic analysis results, long-term progno- sis of our patients with distal kidney tubular acidosis, and the relationship between hyperam- monemia and distal kidney tubular acidosis. MATERIALS AND METHODS: Biochemical, clinical, and imaging findings were collected at presen- tation 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 associ- ated with hyperammonemia. We recommend keeping potassium levels at high-normal levels to reduce ammonia levels, especially in the absence of acidosis.

Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study.

BACKGROUND: Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. METHODS: Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. RESULTS: Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 ± 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. CONCLUSIONS: CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.

COVID-19 in pediatric patients undergoing chronic dialysis and kidney transplantation.

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.

PROGRESS STUDY: Progression of chronic kidney disease in children and heat shock proteins.

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.

Clinician's perspective regarding medication adherence in patients with obstructive lung diseases and the impact of COVID-19.

OBJECTIVE: Failure to achieve high levels of medication adherence in obstructive lung diseases is a major cause of uncontrolled disease. The purpose of this study is to reveal clinicians' opinions on the level of patient adherence and the change in adherence during the COVID-19 pandemic. METHODS: A questionnaire containing multiple-choice questions about treatment adherence in patients with obstructive lung diseases was voluntarily applied to doctors working in a tertiary hospital for chest diseases. RESULTS: Eighty-one doctors (mean age, 37.2 years [standard deviation, 9.7 years]; 57 (70.4%) women) answered the questionnaires. Almost all clinicians participating in the study reported that they always or frequently asked patients if they adhered to treatment. Most clinicians think that in 20-50% of patients with asthma and less than 20% of patients with chronic obstructive pulmonary disease, a decrease in medication adherence appears in the first year of treatment. Most clinicians think the main reason for patients with obstructive lung diseases not adhering is patients' reluctance to be treated regularly. Regarding the impact of the COVID-19 pandemic on patients' drug adherence, 43.2% of clinicians observed that adherence increased after the start of the pandemic. CONCLUSIONS: Adherence to medication is not at the desired levels in patients with obstructive lung diseases. However, when faced with a serious health threat, such as the COVID-19 pandemic, patients realize the severity of their illness and begin using their treatments more regularly.

Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?

BACKGROUND: Several renal and urinary tract complications have been reported in patients with epidermolysis bullosa. OBJECTIVE: This study investigated kidney and urinary tract involvement in patients with epidermolysis bullosa. PATIENTS AND METHODS: Patients with epidermolysis bullosa in treatment at the Dermatology Unit were included in the study. Glomerular and tubular functions were investigated. RESULTS: The study included 16 patients (4 females, 12 males) of mean 11.1 years (SD = 8.1 years). Estimated GFR was normal in all patients except one with end-stage renal disease. Excluding this patient, the urinary albumin/creatinine ratio and the fractional excretion of sodium were normal. The mean beta-2 microglobulin/creatinine ratio was 278.8 µg/g, and it was abnormally high in 2 patients. The mean tubular phosphorus reabsorption was 92.6%; it was abnormally low in 1 patient. Severe kidney or urinary tract involvement was present in 2 patients with recessive dystrophic EB-generalized severe (RDEB-GS): one patient had obstructive bullous lesions in the urethra; the other had end-stage renal disease secondary to focal segmental glomerulosclerosis and was on peritoneal dialysis for 3 years. CONCLUSIONS: Assessment for renal and urinary tract involvement should become a routine part of the evaluation of patients with any type of EB, but especially of patients with RDEB-GS. Patients with mild tubular dysfunction need long-term follow-up to detect early deterioration of renal function.

Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study

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/m2) or high (2-4 doses of 375 mg/m2) 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 1.9­17.3 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.

Clinician's perspective regarding medication adherence in patients with obstructive lung diseases and the impact of COVID-19

SUMMARY OBJECTIVE: Failure to achieve high levels of medication adherence in obstructive lung diseases is a major cause of uncontrolled disease. The purpose of this study is to reveal clinicians' opinions on the level of patient adherence and the change in adherence during the COVID-19 pandemic. METHODS: A questionnaire containing multiple-choice questions about treatment adherence in patients with obstructive lung diseases was voluntarily applied to doctors working in a tertiary hospital for chest diseases. RESULTS: Eighty-one doctors (mean age, 37.2 years [standard deviation, 9.7 years]; 57 (70.4%) women) answered the questionnaires. Almost all clinicians participating in the study reported that they always or frequently asked patients if they adhered to treatment. Most clinicians think that in 20-50% of patients with asthma and less than 20% of patients with chronic obstructive pulmonary disease, a decrease in medication adherence appears in the first year of treatment. Most clinicians think the main reason for patients with obstructive lung diseases not adhering is patients' reluctance to be treated regularly. Regarding the impact of the COVID-19 pandemic on patients' drug adherence, 43.2% of clinicians observed that adherence increased after the start of the pandemic. CONCLUSIONS: Adherence to medication is not at the desired levels in patients with obstructive lung diseases. However, when faced with a serious health threat, such as the COVID-19 pandemic, patients realize the severity of their illness and begin using their treatments more regularly.

Intravesical hyaluronic acid treatment in recurrent urinary tract infections in children with spina bifida and neurogenic bladder.

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 < 0.001) but showed no significant difference in the control group (p = 0.174). When study and control groups were compared, the mean UTIs per patient-month showed no significant difference before treatment (p = 0.77) but significantly decreased in the study group after the treatment (p < 0.001). 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.

Isolated nocturnal and isolated daytime hypertension associate with altered cardiovascular morphology and function in children with chronic kidney disease: findings from the Cardiovascular Comorbidity in Children with Chronic Kidney Disease study.

INTRODUCTION: Prevalence of isolated nocturnal hypertension (INH) and isolated daytime hypertension (IDH) is around 10% in adults. Data in children, especially in chronic kidney disease (CKD), are lacking. The aim of this cross-sectional multicenter cohort study was to define the prevalence of INH and IDH and its association with cardiovascular morphology and function, that is, pulse wave velocity (PWV), carotid intima-media thickness (cIMT), or left ventricular mass index (LVMI) in children with CKD. METHODS: Ambulatory blood pressure (BP) monitoring profiles were analyzed in 456 children with CKD stages III-V participating in the Cardiovascular Comorbidity in Children with Chronic Kidney Disease Study (64.3% males, 71.3% congenital anomaly of the kidney and urinary tract, age 12.5 ±â€Š3.2 years, estimated glomerular filtration rate 29 ±â€Š12 ml/min per 1.73 m). Baseline PWV, cIMT, and LVMI were compared in normotension, INH, IDH, or sustained 24-h hypertension. RESULTS: Prevalence of sustained hypertension was 18.4%, of INH 13.4%, and of IDH 3.7%. PWV SDS (SD score) and cIMT SDS were significantly higher in sustained hypertension and INH, and PWV SDS was significantly higher in IDH, compared with normotension. LVMI was significantly increased in sustained hypertension, but not in INH or IDH. Determinants of INH were smallness for gestational age, older age, higher height SDS and parathyroid hormone, and shorter duration of CKD. In logistic regression analysis, day/night-time hypertension or ambulatory BP monitoring pattern (normal, INH, IDH, sustained hypertension) were independently associated with cardiovascular outcome measures: elevated night-time BP was associated with increased cIMT, PWV, and left ventricular hypertrophy; INH was associated with cIMT. CONCLUSION: INH is present in almost one out of seven children with predialysis CKD; INH and nocturnal hypertension in general are associated with alterations of arterial morphology and function.

Is hepcidin related with anemia and bone mineral metabolism in children with non-dialysis chronic kidney disease?

AIM: 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. MATERIAL AND METHODS: 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. RESULTS: Ferritin, C-reactive protein, interleukin-6, blood urea nitrogen, creatinine, uric acid levels, and percentages of reticulocytes were significantly higher than in the controls (p<0.05). 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 (p>0.05). 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 (p>0.05). However, serum hepcidin levels were correlated with 1,25-dihydroxyvitamin D and interleukin-6 levels (p=0.013 and p=0.002, respectively). CONCLUSION: 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.