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Background/Objectives: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing Escherichia coli -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012—2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. Methods: A total of 436 patients (301 with STEC-HUS and 135 with aHUS) were included. Results: The incidence of STEC-HUS increased during the observation period, with a mean of 3.9 cases per million age-related population (marp). The incidence of aHUS was relatively constant with a mean of 1.8/marp. The majority of patients fully recovered, although kidney sequelae were observed at 5-year follow-ups in 31% of children with STEC-HUS, 57% of aHUS subjects in the pre-eculizumab era, and 37% of aHUS subjects who had received eculizumab. The overall mortality rate was 2% for STEC-HUS and 3.7% for aHUS, with no deaths reported in children on eculizumab and mortality mainly attributed to neurological damage. A decreasing incidence of chronic kidney disease stage 5 (CKD5) due to HUS was observed. Conclusions: Despite an unchanging incidence of aHUS and an increasing incidence of STEC-HUS, the kidney outcomes of both diseases have improved significantly over the last 12 years. Mortality from HUS has dropped due to improved symptomatic treatment and the introduction of anti-C5 therapy. The development of CKD5 in childhood as a consequence of HUS has become exceptional. [ABSTRACT FROM AUTHOR]

The impact of obesity on kidney injury and the development of chronic kidney disease (CKD) is well documented. Unfortunately, the early stages of CKD are asymptomatic, leading to a delayed diagnosis and a worse prognosis. There is a need for more sensitive indicators of kidney damage than those currently used. We aimed to assess the usefulness of serum t-CAF, urinary netrin-1, α-GST, π-GST, calbindin, and calprotectin as biomarkers of early kidney damage in obese children and to investigate the relationship between these indicators and the degree of obesity. A total of 125 simple obese, normoalbuminuric children and 33 non-obese children as controls were selected. Patients were divided into 2 subgroups according to SDS BMI (I: 2 ≤ 4, II: >4). Serum t-CAF was significantly higher in the obese group compared to the controls, as were urinary α-GST, netrin-1, π-GST, and calprotectin. No difference was found between the two obese groups. In normoalbuminuric obese children and adolescents without significant metabolic disorders, serum t-CAF may be a new biomarker for the early detection of renal dysfunction, and urinary netrin-1, α-GST, π-GST, and calprotectin may be better indicators for the detection of early tubular damage, independent of the severity of obesity. [ABSTRACT FROM AUTHOR]

Background: The pathogenesis of idiopathic nephrotic syndrome (INS) has not been fully explained. Among the likely factors, tumor necrosis factor - alpha (TNF-α) is considered. We aimed to evaluate the TNF-α (sTNF-α, uTNF-α) levels in the serum and urine of INS children, with the aim of determining its association with proteinuria, and of determining its usefulness as a marker of the disease severity. Methods: Fifty-one examined patients were divided into subgroups depending on the number of relapses as follows: group IA—first episode; group IB—more than two relapses, and according to treatment modality; group IIA—glucocorticosteroids (GS) alone; and group IIB—GS with immunosuppressants. Healthy age-matched children served as the control group. Results: sTNF-α and uTNF-α levels were significantly increased in active phases in the whole INS group compared to the control group. They decreased in remission, but remained significantly higher when compared to the control group. During remission in the IB group, sTNF-α levels were significantly higher than in IA, whereas, in the relapse phase, these values were similar. In the IA group, a positive correlation between proteinuria and sTNF-α was demonstrated. Conclusions: Our findings suggest that TNF-α plays a role in the development of INS, and may be used as a prognostic marker, as well as an indicator for the continuation of therapy. Additional research is required to verify this statement. [ABSTRACT FROM AUTHOR]

(1) Background: this study aimed to assess the physical activity of obese pediatric patients under specialized outpatient care and its potential determinants. (2) Methods: A total of 83 subjects aged 7–18 years with simple obesity and their parents were enrolled. Data were collected with the use of physical activity questionnaires (PAQs) for children and adolescents and additional questions concerning selected socio-demographic characteristics. (3) Results: The mean final PAQ score was 2.09 ± 0.69. The most frequently chosen types of physical activity included walking, gymnastics, and jogging or running. We found a weak correlation inversely proportional between the child's age and mean final PAQ score (r = −0.25; p = 0.02). Younger children were more active during lunchtime at school and after school compared to adolescents (p = 0.03 and p = 0.04). The final PAQ score differed according to the place of residence; the lowest score was obtained by subjects living in cities >100,000 inhabitants (p = 0.025). We found a positive correlation between PAQ-Ch score and the father's physical activity, and between PAQ-A score and the mother's education. (4) Conclusions: The physical activity of obese pediatric patients is low, particularly in adolescents. It seems that age and place of residence have an impact on the physical activity of obese children and adolescents. The PAQs used in this study are useful in physical activity assessment and identification of time segments during the day in which activity might be improved. However, this requires confirmation in a larger group of pediatric patients. [ABSTRACT FROM AUTHOR]

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Eosinophilic cystitis (EC) is a rare inflammatory disease of unclear etiopathology and various clinical presentations. We present a case of a 10-year old boy who was referred to our center because of acute kidney injury. The imaging diagnostic showed hydronephrosis, irregular thickening and infiltration of the posterior bladder wall. During cystoscopy, no ureter orifices were identified. Based on histopathological examination of bladder biopsy specimens, EC in an acute phase was diagnosed. To our knowledge the presented case is the first where EC led to post-renal acute kidney injury requiring hemodialysis. In the article we describe the diagnostic difficulties, therapy, clinical outcomes, and patient's follow-up. [ABSTRACT FROM AUTHOR]

Introduction: Immunoglobulin A nephropathy (IgAN) may lead to end stage renal disease and severely affect patient functioning and wellbeing. The aim of the study was to evaluate health-related quality of life (HRQoL) in children and adolescents with IgAN, and compare HRQoL in relation to the disease course, social status and psychological factors, such as expressing anger and perceived personal competence.Material and Methods: The multicentre cross-sectional study included 51 patients ≥ 8 years from 7 paediatric nephrology centres in Poland. Psychometric analysis was performed using the Kidscreen-52 questionnaire to evaluate HRQoL, the Anger Expression Scale to evaluate the severity of anger and the Personal Competence Scale to measure general perception of personal competence.Results: Mean age of patients was 14.54 ±3.69 years; duration since the diagnosis of IgAN was 4.98 ±3.9 years. Patients with IgAN rated their psychological wellbeing as significantly worse compared to healthy peers (p < 0.05). The presence of proteinuria was associated with significantly worse physical wellbeing (58.72 ±18.45 vs. 74.44 ±22.97; p < 0.05). Current therapy (steroids/immunosuppressive drugs) had no effect on HRQoL in the study group. Perceived personal competence was rated high by 49% of children in the study group. Children with IgAN were characterized by lower intensity of expressed anger (p < 0.001) and significantly higher intensity of suppressed anger (p < 0.01) compared to reference ranges. Severity of expressed anger correlated positively with the parent relations and school environment dimensions of HRQoL.Conclusions: We found lower HRQoL in regard to physical and psychological wellbeing in a group of Polish children with IgAN compared to healthy peers. HRQoL should be monitored in this patient group. [ABSTRACT FROM AUTHOR]

Background. The status of the cardiovascular (CV) system in children with chronic kidney disease (CKD) is significantly influenced by increasing stiffness of the arterial wall. This largely depends on the shortage of local and systemic inhibitors of soft tissue calcification. Objectives. The aim of the study was to evaluate the role of fetuin-A in conjunction with other factors in the progressive hardening of the vascular wall in these children. We examined serum fetuin-A concentrations in relation to renal function, dialysis modality, and other clinical and biochemical markers promoting vascular calcification. Material and methods. Twenty children on peritoneal dialysis (PD), 20 on hemodialysis (HD), 36 treated conservatively, and 26 healthy subjects were enrolled into a cross-sectional study. In all children, fetuin-A and numerous clinical and biochemical parameters were measured. Results. The fetuin-A concentration was significantly lower in children on hemodialysis (HD) vs children on peritoneal dialysis (PD), conservatively treated subjects, and the control group. In sick children, fetuin-A concentration negatively correlated with dialysis vintage, PWV/ht, phosphate concentration, calcium phosphate product (CaxP), cumulative doses of calcium, and vitamin D3. In the whole study population, fetuin-A negatively correlated with blood pressure (BP), pulse wave velocity indexed to height (PWV/ht), intact parathyroid hormone (iPTH), high sensitivity C-reactive protein (hsCRP), and cholesterol concentrations. Conclusions. In children with CKD, the decreased concentration of fetuin-A is related to other vascular calcification risk factors. Serum fetuin-A concentration may play a role in the identification of vascular disease risk factors in this population. [ABSTRACT FROM AUTHOR]

Background. Kidney transplantation (Tx) is regarded as the optimal treatment method for renal replacement therapy (RRT) for end-stage renal disease (ESRD) patients. Children qualified for Tx should receive the organ as soon as possible in order to improve their chances for healthy development. In our center, RRT for children with ESRD has been conducted for 36 years: hemodialysis (HD) since 1982, peritoneal dialysis (PD) since 1992 and the first transplant in 1987. Objectives. To analyze the rates of different RRT methods in children with ESRD. Special attention was paid to Tx. Material and methods. We compared the rates of RRT methods over 3 subsequent decades (1987--1996, 1997--2006 and 2007--2017). Results. In the period analyzed, 153 children aged from 2 weeks to 18 years were dialyzed. The mean age of the start of RRT was 9.4 years. In 80 children (52.2%), first method was HD, while in 73 patients (47.7%) it was PD. In 25 children, the type of dialysis was changed. Kidney transplantation was performed in 40%, 60.34% and 73% of patients dialyzed in the periods 1987--1996, 1997--2006 and 2007--2017, respectively. The average waiting time for a transplant in the abovementioned decades was 2.25 years, 2.65 years and 1.97 years, respectively. Three children underwent transplantation with a family donor; 1 boy received a transplanted kidney and liver. Two children underwent a preemptive transplant from a deceased donor. Conclusions. The percentage of children with ESRD treated with Tx continues to increase, but in our assessment, it still remains too low. Among the types of dialysis, PD was much more frequently used, which is consistent with pediatric recommendations. Small number of transplants from a living donor and preemptive transplants indicates the need to promote organ donation in Polish society. [ABSTRACT FROM AUTHOR]

Background. The progression of chronic kidney disease is accompanied by multi-organ disorders, among which cardiovascular diseases have the status of a serious clinical problem. The body surface potential mapping (BSPM) technique is a non-invasive method which enables the detection of pathological changes in the bioelectrical activity of the heart. Objectives. The aim of this study was to identify possible disturbances in the intraventricular conduction system in peritoneally dialyzed children. Material and methods. Cardiac examination consisted of 12-lead electrocardiography, echocardiography and BSPM. The evaluation of disturbances in the cardio-electrical field was performed by comparing the qualitative and quantitative features of the heart potentials on the isopotential map. Results. Data was collected from 10 children treated with automatic peritoneal dialysis (APD) (mean age: 13.6 ±2.3 years) and 26 healthy children. The maps of dialyzed children showed a shift in positive isopotentials toward the left lower part of the thorax, while negative values were observed in its left upper part. A distribution of lines on the isopotential maps revealed disturbances in the stimulation spread within the heart ventricles, especially within the anterior fascicle of the left bundle branch of His. Conclusions. Intraventricular conduction disturbances were observed in the left bundle branch of His in the peritoneally dialyzed children. The body surface potential mapping was a more sensitive method in identifying the early stage of conduction disturbances within the heart ventricles than 12-lead electrocardiography. Further research involving a larger population of dialyzed children is planned. [ABSTRACT FROM AUTHOR]

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Copyright of Paediatrics & Family Medicine / Pediatria i Medycyna Rodzinna is the property of Medical Communications Sp. z o.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background. Blood pressure in pediatric dialyzed patients is under poor control. Objectives. The aim of the study was to assess the strategy and efficacy of antihypertensive drugs used for the treatment of hypertension in pediatric dialyzed patients in 2013 in comparison with the data collected in 2003/2004. The results have been viewed against present strategies of antihypertensive treatment in children. There is still limited data concerning the treatment of hypertension in dialyzed pediatric patients. Material and methods. The study embraced 10 of 12 pediatric dialysis units in Poland treating 59 pediatric patients (mean age - 132 months). Collected information included present antihypertensive treatment with regard to drug classes and the dose of antihypertensive agent. The treatment was regarded as effective if both systolic and diastolic values of blood pressure were below 1.64 SDS. The results from 2013 were juxtaposed with previously analyzed data from a similar study on hypertension in dialyzed children conducted in 2003/2004. Results. Forty subjects have been provided with antihypertensive treatment. In monotherapy and polytherapy 50% of the subjects were treated with ACEI (enalapril and ramipril), 67.5% with amlodipine, 50% with beta-blockers. Only 10% of the subjects were treated with angiotensin II receptor blocker (losartan). Thirty percent of the subjects received furosemide, whereas 5% were given doxazosin. Antihypertensive drugs regarded as the 2nd and 3rd choice in treating high blood pressure (doxazosin, beta-blockers and furosemide) were applied as monotherapy in 46% of the patients. Satisfactory control of treated blood pressure was reached in 45% of them. Conclusions. Antihypertensive treatment in dialyzed children did not change significantly during the last decade with regard to the groups of drugs being used. Despite a wider feasibility of antihypertensive substances, the effectiveness of this therapy was still unsatisfactory. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study was to analyze the selected psychosocial aspects of chronic kidney disease in children treated with hemodialysis (HD). Methods: The study included 25 children treated with HD aged 2 to 18 years and their parents. Data concerning the illness and socio-demographic parameters was collected. We used the Paediatric Quality of Life Inventory (PedsQL) for patients and for their parents the PedsQL-proxy version, General Health Questionnaire (GHQ-12), Berlin Social Support Scales (BSSS), and the Caregivers Burden Scale (CBS) to evaluate health-related quality of life (QoL) of HD children and their primary caregivers. Findings: In the PedsQL test, the QoL of HD children was lower than in healthy children. Children treated with HD assessed their QoL on the PedsQL questionnaire higher than the primary caregivers, on all subscales as well as an overall health-related QoL. Scoring below 2 on the GHQ-12 test was reported in 56% of mothers, which may indicate that psychological symptoms have intensified. There was no correlation between BSSS, CBS, and GHQ-12. Discussion: The assessment of QoL in pediatric patients would allow for the earliest possible identification of their nonsomatic problems and irregularities. This could, consequently, contribute to improving QoL in both children with chronic kidney disease and their families. [ABSTRACT FROM AUTHOR]

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Background. INS pathogenesis has been still unclear. Among many factors th2. Derived IL-13 cytokine is taken into account. Objectives. Assessment of serum and urine level of IL-13 in children with ins as a pathogenic factor and a marker of the course of the disease. Material and methods. 51 children with INS and 18 healthy children were examined. Patients were divided into groups depending on the number of relapses of the disease, medical treatment and morphological types. The serum and urinary concentration of IL-1 3 were determined by ELISA. Results. The significant increase in IL-13in serum and urine of patients in the acute phase of the disease, compared to healthy subjects, was shown. in remission its level was significantly reduced, but still remained higher than in the control group. regardless of the stage of the disease, the concentration of IL-13 in the urine of patients with the first relapse of ins was significantly lower than in children with recurrent INS or requiring immunosuppressive therapy. Serum level of IL-13 was not different between these groups. In all histopathological types of ins, IL-13 in serum and urine were similar. Conclusions. Increased concentration of IL-13 in serum and urine of children with INS in the acute phase of the disease, and significant decrease in remission, indicate its role in the pathogenesis. Higher levels of IL-13 in the urine of patients with recurrent INS and requiring immunosuppressive therapy suggest the usefulness of this parameter as a prognostic marker. IL-13 in serum and urine does not allow one to distinguish between the morphological types of INS. [ABSTRACT FROM AUTHOR]

Patients with end-stage renal disease are affected by cardiovascular complications, including disturbances of the heart intraventricular conduction. Body surface potential mapping is a non-invasive electrocardiographic detection method of initial disturbances in heart activation propagation. A goal of the study was to analyze the effects of single hemodialysis ( HD) session on ventricular activation time ( VAT) maps obtained from hemodialyzed children. The study group consisted of 13 hemodialyzed children (age: 6-18 years). The control group is composed of 26 healthy subjects. In each HD patient, 12-lead electrocardiogram and echocardiography examinations were performed. Isochrone heart maps, reflecting body surface distribution of VAT isolines, were recorded from an 87-electrode HPM-7100 system for body surface potential mapping, before (group B) and after HD session (group A). The distribution of isochrones and VAT values, as recorded in the HD patients, differed significantly from the reference VAT map for controls. The highest VAT maximal value was noted in group B (Me: 110 vs. 62 ms in the control group; P < 0.001), becoming significantly lower after HD session ( Me: 98 ms for group A vs. 110 ms for group B; P < 0.001). Ventricular activation time maps, recorded before HD session, showed significant VAT delays with isochrone arrangement specific for the left bundle branch block. After HD session, VAT maps presented significant changes, suggesting a normalization process. Ventricular activation time maps in children with end-stage renal disease exhibited disturbances of intraventricular conduction within the left bundle branch block, undetectable on standard electrocardiogram. A single HD session resulted in VAT map improvement related to overall HD treatment duration. [ABSTRACT FROM AUTHOR]

Background/Aims: Chronic medical illness is a significant risk factor for the development of psychiatric disorders. The aims of the study were: to investigate the level of anxiety in children with chronic kidney disease (CKD) and to identify factors associated with the presence of that emotional problem. Methods: CKD children on hemodialysis (HD, n=22), peritoneal dialysis (PD, n=20,) and on conservative treatment (CT, n=95) were enrolled in the study. We used State-Trait Anxiety Inventory (STAI) for adolescents and STAI-C for children. Socio-demographic and physical factors were assessed. Results: There was a significantly higher level of anxiety-state among HD children (8-12 years) compared with other groups of participants of the same age and Polish population norms. The level of anxiety among adolescents (13-18 years), both anxiety-state and anxiety-trait, was significantly higher in the HD group compared with other groups, which did not differ among themselves. In the HD adolescents, there was a correlation between the anxiety-state and the duration of the disease as well as with the number of hospitalizations. PD adolescents in the mainstream education had higher levels of anxiety-state and anxiety-trait compared with home schooled patients. Conclusions: Even though children and adolescents with CKD are at risk of developing a variety of emotional disorders, the level of anxiety among the researched group, with the exception of HD patients, was not significantly different than the level of anxiety among healthy subjects. Adolescents on HD who present a high level of anxiety should undergo long-term psychological treatment. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Copyright of Advances in Hygiene & Experimental Medicine / Postepy Higieny i Medycyny Doswiadczalnej is the property of Sciendo and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Congenital anomalies of the kidney and urinary tract (CAKUT) occur at a frequency of 1 in 500 live births and are a common cause of renal insufficiency in childhood. CAKUT encompass a wide spectrum of malformations including anomalies of the kidney, collecting system, bladder and urethra. Most cases of CAKUT are sporadic and limited to the urinary tract, but some of them are syndromic or associated with positive family history. To understand the basis of human renal anomalies, knowledge of kidney and urinary tract development is necessary. This process is very complicated, requires precise integration of a variety of progenitor cell populations of diverse embryonic origins and is controlled by many factors at every stage of development. This review focuses on the genetic factors leading to developmental errors of important morphogenetic processes, particularly in metanephric kidney induction and ureteric bud branching. The essential results of genetic studies in regard to CAKUT, performed on experimental models and in humans, are presented. However, further investigations are required to complete understanding of the complex molecular network, which will help us to determine novel preventive and therapeutic strategies for CAKUT [ABSTRACT FROM AUTHOR]

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background/Aims: Interleukin (IL)-18, a member of the IL-1 cytokine superfamily, is recognized as an important regulator of immune responses. The aim of our study was to investigate the IL-18 levels in serum and urine from children with idiopathic nephrotic syndrome (INS) during relapse and remission, and to evaluate the role of IL-18 in this disease. Methods: 67 children with INS, aged 3–16 years, and 15 normal controls were included in the study. The patients were divided into two groups according to activity of the disease: I (n = 37) – INS in relapse, II (n = 30) – INS in remission. Serum and urinary IL-18 were determined by ELISA and in urine related to the urinary creatinine (Cr) concentration. Serum creatinine, protein, albumin and 24-hour proteinuria were measured in children with INS. Results: Urinary IL-18 concentration was significantly higher in group I (213.51 ± 162.15 pg/mg Cr) compared to group II (64.74 ± 10.95 pg/mg Cr) and to normal controls (37.03 ± 4.1 pg/mg Cr, p < 0.001). Serum IL-18 concentration was significantly higher in group I than in the controls (146.4 ± 30.2 and 113 ± 10 pg/ml, respectively; p < 0.05); the differences between either groups I and II or group II and controls were not significant. Urinary IL-18 correlated positively with serum IL-18 and with urinary protein excretion, but no correlations were found with other laboratory data. Conclusion: Increased serum and urine IL-18 levels were observed during relapse of INS. These findings indicate the association between the active phase of INS and the levels of IL-18 and can suggest the role of this cytokine in the INS development. The changes in urinary IL-18 excretion in the course of INS are connected with the disease activity. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Extracellular calcium concentrations in humans are thousands times higher than within cells. Maintenance of such gradient requires specific regulation including intracellular stores, Ca binding proteins and transmembrane protein systems. The aim of the study was to estimate PMCA (plasma membrane Ca-transporting adenosine triphosphatase; ATPase 3.6.1.38) activity and calcium homeostasis in erythrocytes of children with chronic kidney disease (CKD). Twenty-one children wth CKD stages 1–3 (group I) and 18 healthy children (group II) were examined. Group I was divided into two subgroups: Ia (8 patients with normal intact parathyroid hormone, iPTH, serum levels) and Ib (13 patients with increased iPTH). iPTH, urea, creatinine, inorganic phosphorus, cytosolic Ca2+ in red blood cells (R-Ca), and PMCA were determined. Significantly elevated R-Ca levels were observed in children from subgroup Ib in comparison with group II and subgroup Ia. The lowest activity of PMCA was found in subgroup Ia and Ib in comparison with group II. There was a negative correlation between PMCA and R-Ca in group Ia and Ib ( r = −0.8, r = −0.9, respectively). In children with CKD treated conservatively, activity of PMCA in erythrocytes is disturbed. An increase in R-Ca and decrease in PMCA activity are also observed. [ABSTRACT FROM AUTHOR]

Diabetic kidney disease belongs to the major complications of diabetes mellitus. Here, hyperglycaemia is a key metabolic factor that causes endothelial dysfunction and vascular changes within the renal glomerulus. The aim of the present study was to assess the function of the vascular endothelium in children with type 1 diabetes mellitus (type 1 diabetes) by measuring selected endothelial lesion markers in blood serum. The selected markers of endothelial lesions (sVCAM-1, sICAM-1, sE-SELECTIN, PAI-1, ADMA and RAGE) were assayed by the immunoenzymatic ELISA method. The study involved 66 patients (age: 5–18 years) with type 1 diabetes and 21 healthy controls (age: 5–16 years). In the type 1 diabetes patients, significantly higher concentrations of all of the assayed markers were observed compared to the healthy controls (p < 0.001). All of the evaluated markers positively correlated with the disease duration, the age, and BMI of the patients, while only PAI-1 and sE-SELECTIN were characteristic of linear correlations with the estimated glomerular filtration rate (eGFR). It can be concluded that endothelial inflammatory disease occurs in the early stages of type 1 diabetes mellitus in children. The correlations between PAI-1, sE-SELECTIN, and eGFR suggest an advantage of these markers over other markers of endothelial dysfunction as prognostic factors for kidney dysfunction in children with type 1 diabetes. [ABSTRACT FROM AUTHOR]

Chronic kidney disease (CKD) is associated with multifaceted pathophysiological lesions including metabolic pathways in red blood cells (RBC). The aim of the study was to determine the concentration of adenine nucleotide metabolites, i.e., nicotinamide adenine dinucleotide (NAD)-oxidized form, nicotinamide adenine dinucleotide hydrate (NADH)-reduced form, nicotinic acid mononucleotide (NAMN), β-nicotinamide mononucleotide (NMN), nicotinic acid adenine dinucleotide (NAAD), nicotinic acid (NA) and nicotinamide (NAM) in RBC and to determine a relationship between NAD metabolites and CKD progression. Forty-eight CKD children and 33 age-matched controls were examined. Patients were divided into groups depending on the CKD stages (Group II-stage II, Group III- stage III, Group IV- stage IV and Group RRT children on dialysis). To determine the above-mentioned metabolites concentrations in RBC liquid chromatography-mass spectrometry was used. Results: the only difference between the groups was shown concerning NAD in RBC, although the values did not differ significantly from controls. The lowest NAD values were found in Group II (188.6 ± 124.49 nmol/mL, the highest in group IV (324.94 ± 63.06 nmol/mL. Between Groups II and IV, as well as III and IV, the differences were statistically significant (p < 0.032, p < 0.046 respectively). Conclusions. CKD children do not have evident abnormalities of RBC metabolism with respect to adenine nucleotide metabolites. The significant differences in erythrocyte NAD concentrations between CKD stages may suggest the activation of adaptive defense mechanisms aimed at erythrocyte metabolic stabilization. It seems that the implementation of RRT has a positive impact on RBC NAD metabolism, but further research performed on a larger population is needed to confirm it. [ABSTRACT FROM AUTHOR]

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children. [ABSTRACT FROM AUTHOR]

Hemopexin (Hpx) is considered a factor in the pathogenesis of idiopathic nephrotic syndrome (INS). The aim of the study was to evaluate the serum and urine values of Hpx (sHpx and uHpx) in children with INS, analyze the role of Hpx, and assess its usefulness as a marker of the disease course. 51 children with INS and 18 age-matched controls were examined. Patients were divided into subgroups depending on the number of relapses (group IA—the first episode of INS, group IB—with relapses) and according to method of treatment (group IIA treated with gluco-corticosteroids (GCS), group IIB treated with GCS and other immunosuppressants). Hpx concentrations were determined by enzyme-linked immunosorbent assay (ELISA). sHpx and uHpx values in relapse were elevated in the whole INS group versus controls (p < 0.000). In remission their levels decreased, but still remained higher than in the control group (p < 0.000). In group IB uHpx levels were increased during remission as compared to group IA (p < 0.006). No significant impact of immuno-suppressants on sHpx was observed, but uHpx excretion in group IIA was higher in relapse (p < 0.026) and lower in remission (p < 0.0017) as compared to group IIB. The results suggest the role of Hpx in the pathogenesis of INS. Hpx may be a useful indicator for continuation of treatment, but it requires confirmation by further controlled studies. [ABSTRACT FROM AUTHOR]

Introduction: The data of renal catabolism of albumin suggests that albuminuria is associated with tubular reabsorption and not with a damage of glomerular filtration barrier. Aim of the study: The evaluation of albuminuria in children with idiopathic nephrotic syndrome (INS) in relapse and remission regarding chosen markers of proximal tubular cell dysfunction and immunoglobulin G (IgG) concentration in urine. Materials and methods: 28 children with INS aged 4-12 years and 16 children with nocturnal enuresis (control group) were enrolled into the study. Albumin (Al), α1-microglobulin (α1-M) and IgG concentrations and activity of N-acetyl-β-D-glucosaminidase (NAG) were measured in the urine after night. Results were expressed per urine creatinine (cr). Measurements in children with INS were made in relapse and partial remission of the disease. Results: Median concentration of Al was significantly higher in relapse ns (1.49 mg/mg cr) than in remission (0.18 mg/mg cr) and in control group (0.11 mg/mg cr; p <0.001). Statistically significant difference was found between albuminuria in ns children in remission and controls (p <0.001). Median values of α1-M, IgG and NAG were significantly higher in relapsed children (0.019 mg/mg cr, 0.021 mg/mg cr, 6.95 U/mg cr, respectively) than in remission (0.011 mg/mg cr, 0.004 mg/mg cr, 4.25 U/mg cr; p <0.001). a1-M level remained higher in children with remission of ns compared to controls (0.005 mg/mg cr; p <0.05). No differences were found in NAG activity and IgG level between patients in remission and healthy children. Al correlated with IgG and α1-M in relapse of ns. A similar correlation was found between IgG and α1-M. Conclusion: Urine excretion of albumin in INS children is caused not only by a damage of the glomerular filtration barrier but also by proximal tubular cells. This is confirmed by a positive correlation between albuminuria and a1-microglobulinuria. In INS, disorder of protein reabsorption in proximal tubular cells is observed, even in partial remission of the disease. [ABSTRACT FROM AUTHOR]

The aim of the study was to analyze the effect of recombinant human growth hormone (rhGH) therapy and to establish factors influencing growth rate in dialyzed children in Poland. We retrospectively analyzed medical records of 81 children with end-stage renal disease (ESRD) on chronic dialysis treated with rhGH for ≥12 months between 1994 and 2014. The following data were recorded: cause of ESRD, dialysis modality, age at the dialysis and rhGH initiation [years]. In addition, growth [cm], [standard deviation score - SDS], body mass index [SDS], skeletal age [years], bone mineral density [SDS], hemoglobin, total protein, albumin, urea, creatinine, calcium, phosphorus, calcium phosphorus product, PTH, and alkaline phosphatase were measured at the baseline and after 12 months. Growth velocity in 81 children during one-year rhGH treatment was 7.33 ± 2.63 cm (ΔSDS 0.36 ± 0.43). Height SDS increased significantly (-3.31 ± 1.12 vs. -2.94 ± 1.15, p < 0.001). Children on peritoneal dialysis (PD) (n = 51) were younger than children on hemodialysis (HD) (n = 30) (9.92 ± 3.72 vs. 12.32 ± 3.11 years, p = 0.003). ΔSDS did not differ between PD and HD children (0.40 ± 0.33 vs. 0.30 ± 0.47, p = 0.311). Growth velocity (ΔSDS) correlated with age at dialysis initiation (r=-0.30, p = 0.009), age at rhGH treatment initiation (r=-0.35, p = 0.002), skeletal age (r=-0.36, p = 0.002), BMI SDS (r=-0.27, p = 0.019), and PTH (r=-0.27, p = 0.017). No correlation between growth velocity and other parameters was observed. Treatment with rhGH in children with ESRD is effective and safe irrespective of dialysis modality. Early initiation of rhGH therapy is a crucial factor determining response to the treatment in children with ESRD. [ABSTRACT FROM AUTHOR]

Introduction: Chronic kidney disease (CKD) is characterized by a state of generalized vasculopathy caused mainly by atherosclerosis. It has recently been established that in addition to classical mechanisms, new biochemical markers of endothelial inflammation and dysfunction play a role. Aim of the study was to assess the biochemical markers of endothelial inflammation/dysfunction: sE-selectin, MMP-9 (matrix metalloproteinase-9), TIMP-1 (tissue-1 inhibitor metalloproteinases), ADMA (asymmetric dimethylarginine), SDMA (symmetric dimethylarginine), PAI-1 (plasminogen activator inhibitor type1) and intima-media thickness (IMT) of the carotid artery in 3 groups children. Materials and methods: 20 children (12 boys, 8 girls) mean age 14.3±2.3 years on PD (peritoneal dialysis), 20 children (10 boys, 10 girls) mean age 15.0±3.3 on HD (hemodialysis) were included in the study and compared to 26 healthy age-matched subjects. Concentrations of serum sE-selectin, MMP-9, TIMP-1, ADMA, SDMA, PAI-1, iPTH, creatinine, albumin, hemoglobin, Ca, P, total cholesterol, HDL, LDL, CRP were measured. Intima-media thickness (IMT) of the carotid artery was assessed in all children. Results: cIMT, sE-selectin, MMP-9, TIMP-1, ADMA, SDMA, PAI-1 values were significantly higher in dialysed patients vs. control group. A significant positive correlation was observed between cIMT and age, sE-selectin, ADMA, SDMA, PAI-1 and negative with MMP-9 and TIMP-1. Conclusions: In dialysed children we have observed increased inflammation and endothelial destruction which may be caused by the examined markers. The positive correlation between all endothelial markers and cIMT confirm this observation. [ABSTRACT FROM AUTHOR]