Serum fibroblast growth factor 23 and calcium-phosphorus metabolism parameters in children with chronic kidney disease - preliminary report.

INTRODUCTION: In chronic kidney disease (CKD) the function of all factors regulating mineral metabolism is disturbed, leading inevitably to renal osteodystrophy and vascular calcification. The aimof the study is to assess concentrations of fibroblast growth factor 23 (FGF 23), osteoprotegerin (OPG) and other parameters of calcium-phosphate metabolism in children with CKD. MATERIAL AND METHODS: 37 children with CKD 3-5, aged 1.6-17 years were included in the study. In all children serum levels of calcium (sCa), phosphate (sP), creatinine, alkaline phosphatase (ALP), FGF 23, intact parathormone (PTH), OPG and receptor activator nuclear factor κB ligand (RANKL) were measured. RESULTS: Total calcium concentration was within normal limits in all children included in this study. Hyperphosphatemia was found in 2 children from group CKD 3 (12%), 6 from CKD 4 (54%) and 1 from CKD 5 (11%). FGF 23 level increased consecutively in subsequent CKD stages achieving the highest values in CKD 5 group. In all children with CKD, serum levels of OPG were correlated with FGF 23. In children with CKD 3-4 negative correlation between FGF 23 and PTH (r=-0.45; p=0.02) and positive correlation between FGF 23 and RANKL (r=0,59; p=0.006) has been found. Positive correlation between OPG concentration and HCO3 -and BE levels has been observed, as well as negative correlation between RANKL/OPG ratio and HCO3 -and BE levels. CONCLUSION: Despite maintaining serum calcium, phosphorus and PTH levels within recommended limits, elevated levels of FGF 23 and OPG were observed in children with chronic kidney disease, especially in it's end-stage.

Changes in bone matrix mineralization after growth hormone treatment in children and adolescents with chronic kidney failure treated by dialysis: a paired biopsy study.

BACKGROUND: Patients with chronic kidney disease (CKD) develop renal osteodystrophy with alterations in bone turnover, mineralization, and volume (TMV). A specific skeletal complication in children is growth impairment, which currently is treated by recombinant human growth hormone (rhGH). The effects on bone material properties are poorly understood. This study assesses the effects of rhGH treatment on bone matrix mineralization. STUDY DESIGN: Observational study. SETTING & PARTICIPANTS: 18 short children and adolescents (aged 3.6-16 years) with CKD on dialysis therapy. PREDICTOR: rhGH treatment for 1 year. OUTCOMES: Tetracycline-labeled bone biopsy classified according to the TMV system. MEASUREMENTS: Bone mineralization density distribution (BMDD) was evaluated by quantitative backscattered electron imaging in trabecular and cortical compartments. Additional data for patients' height and biochemical bone serum parameters were obtained. RESULTS: Prior to rhGH treatment, our cohort showed low bone turnover and high mineralization densities versus reference data: Ca(mean) (weighted mean calcium content) in cancellous bone, +3.3% (P = 0.04); Ca(mean) in cortical bone, +6.7% (P < 0.001); Ca(peak) (mode of the BMDD) in cancellous bone, +5.0% (P < 0.001); Ca(peak) in cortical bone, +8.2% (P < 0.001); Ca(width) (heterogeneity in mineralization), no significant difference for cancellous (P = 0.2) and cortical (P = 0.1) bone; Ca(high) (portion of fully mineralized bone) in cancellous bone, 5-fold greater (P < 0.001); Ca(high) in cortical bone, 14-fold greater (P < 0.001); Ca(low) (portion of low mineralized bone) in cancellous bone, +23.9% (P = 0.02); Ca(low) in cortical bone, -22.2% (P = 0.05). After rhGH treatment, height increased by 9.1 cm (P < 0.001) and bone turnover indices to normal values or beyond. Matrix mineralization was lesser and more heterogeneous compared to baseline: Ca(width) for cancellous bone, +15.3% (P < 0.001); Ca(width) for cortical bone, +34.1% (P < 0.001). Ca(mean), Ca(peak), and Ca(high) for cancellous bone and Ca(mean) and Ca(peak) for cortical bone were no longer significantly different from reference data. Ca(high) for cortical bone dramatically decreased after treatment but was still substantially greater than reference data. LIMITATIONS: Low case number per TMV subgroup, no measurements of fibroblast growth factor 23. CONCLUSIONS: Children and adolescents with CKD and growth deficiency are at risk of having low bone turnover. rhGH treatment improves height and concomitantly bone modeling/remodeling, which appears beneficial for bone matrix mineralization.

Anxiety in children and adolescents with chronic kidney disease--multicenter national study results.

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.

Psychosocial aspects of children and families of children treated with automated peritoneal dialysis.

BACKGROUND: The aim of this study was to analyze psychosocial aspects of chronic kidney disease (CKD) in children treated with automated peritoneal dialysis (APD). METHODS: The study assessed 41 children > 2 (range 2.1-18) years of age and their parents. Data concerning the illness and sociodemographic parameters were collected. Patients completed the Paediatric Quality of Life Inventory (PedsQL) and their parents the PedsQL-proxy version, General Health Questionnaire (GHQ-12), Berlin Social Support Scales (BSSS), and Caregiver's Burden Scale (CBS). RESULTS: Parents rated their children's overall health-related quality of life (QoL) as well as their physical and emotional functioning lower than the patients themselves. The majority of primary caregivers had a medium level of the total burden index in the CBS and higher values in the scales need for support and perceived available support than in the received support (BSSS). In the GHQ-12, 51.2% of primary caregivers had scores >2 points, which indicated the possible occurrence of abnormal mental functioning. CONCLUSIONS: Financial support for patients' families is necessary. Parents who provide primary care to children on PD require, above all, emotional support and assistance in self-fulfilment. More than half of them may have impaired mental function. There is the strong need to provide continuous psychological care for caregivers. Differences in perception of the children's activity in varied areas by the patients themselves and their caregivers may contribute to further problems within families.

Perception of health-related quality of life in children with chronic kidney disease by the patients and their caregivers: multicentre national study results.

OBJECTIVE: The aim of the study was to analyse the health-related quality of life (HRQoL) in Polish children with chronic kidney disease (CKD) dependant on the CKD stage, treatment modality and selected social life elements in families of the patients. Furthermore, potential differences between self-report and parent/proxy reports and the factors influencing them were assessed. METHODS: A total of 203 CKD children (on haemodialysis (HD), peritoneal dialysis (PD) and conservative treatment (CT)) and their 388 parent/proxies were enrolled into a cross-sectional national study. The demographic and social data were evaluated. We used the Paediatric Quality of Life Inventory 4.0 Generic Core Scales to assess the HRQoL in children. RESULTS: Health-related quality of life scores for all CKD groups were significantly lower in all domains compared with population norms, the lowest one being in the HD group. In CT children, HRQoL did not depend on the CKD stage. Both parents assessed the HRQoL of their children differently depending on their involvement in the care. There are differences between the HRQoL scores of the children and their parents. CONCLUSION: The HRQoL in children with CKD is lower than in healthy children. This is already observed in the early stages of the disease. The disease itself influences the child's mental state. Children on HD require special support on account of the lowest demonstrated overall HRQoL. Children's lower rating of the quality of life observed by their parents may render the patients unmotivated and adversely affect their adjustment to life in later years. It may also create conflicts between the parents and the children.

Anaemia treatment in chronically dialysed children: a multicentre nationwide observational study.

OBJECTIVE: Erythropoiesis-stimulating agents (ESAs) are applied as a standard therapy in children with anaemia in chronic kidney disease. The aim of this study was to describe the efficacy and details of ESA treatment in a population of dialysed children in Poland. MATERIAL AND METHODS: The study had a prospective observational design and was performed in 12 dialysis centres. The study group comprised 117 dialysed children with a mean age at enrolment of 165.33 (97.18-196.45) months. RESULTS: Dialysed children were treated mostly with epoietin beta and darbepoietin. The mean dose of ESA was 99 (68-147) U/kg/week with a significant difference between patients on peritoneal dialysis [83 (54-115)] and haemodialysis [134 (103-186)] (p < 0.0001). The mean haemoglobin of all the time-point tests during 6 months was 10.91 ± 1.18 g/dl. The efficacy of anaemia treatment was unsatisfactory in 52% of subjects. In multivariate analysis, initial haemoglobin level <10 g/l, any infection, younger age at first dialysis, malnutrition and inadequate ESA dosage remained significant predictors of anaemia. CONCLUSIONS: The study revealed that anaemia treatment in Polish children is unsatisfactory. Late commencement of the treatment, inadequate dosing, malnutrition and infections could constitute risk factors for therapy failure.

[Glomerulopathies as causes of end-stage renal disease in children]. / Klebuszkowe choroby nerek jako przyczyna schylkowej niewydolnosci nerek.

UNLABELLED: The aim of the study was assessment of the frequency of glomerular diseases (GD) as a cause of end-stage renal disease (ESRD) in children. PATIENTS AND METHODS: Retrospectively, causes of ESRD, frequency, duration of the disease until diagnosis of ESRD in 195 children treated in 1973-2008 were analysed. RESULTS: Among children with ESRD, GD were diagnosed in 94 (48.2%)--mean age 10.9 +/- 4.3 years, congenital abnormalities of urinary tract in 61 (31.2%) aged 10.6 +/- 4.3 years and other causes in 40 (20.5%). Among 94 children with GD the 49 (52%) patients had primary glomerulonephritis (GN): 24--crescentic GN, 9--focal segmental glomerulosclerosis, 8--mesangioproliferative GN, 5--IgA nephropathy, 3--membranoproliferative GN. In 37 (39.5%) the causes of ESRD were secondary glomerulopathies: 20--amyloidosis, 10--hemolytic-uremic syndrome, 6--Schönlein-Henoch nephropathy, 1--Wegener granulomatosis. In 8 (8.5%) patients causes of ESRD were: Alport syndrome in 2, congenital nephrotic syndrome in 1 and in 5 type of glomerulopathy was unknown. Time between diagnosis of nephropathy and start of dialysis was 0-13.75 years, median 1.1 in patients with GD and was significantly shorter (p<0.003) than this time in the group with congenital abnormalities of the urinary tract (0-14.9 years; median 3.83). There was no difference between primary GN and secondary glomerulopathies. CONCLUSIONS: Chronic glomerulopathies were the most frequent cause of ESRD in investigated group. Time between diagnosis of chronic renal disease and initiation of renal replacement therapy was significantly shorter in children with glomerulopathies than with congenital abnormalities of urinary tract.

[Severe course of typical hemolytic-uremic syndrome in a 14-year-old boy]. / Ciezki przebieg typowego zespolu hemolityczno-mocznicowego u 14-letniego chlopca.

The authors present a 14-year old boy with acute renal failure in the course of hemolytic-uremic syndrome (HUS), preceded by bloody diarrhea of unknown origin. The course of HUS was complicated with hypertensive crisis, pleural effusion. Pleural puncture was complicated with massive hemorrhage which required thoracotomy. Additional risk factor were subendocardial perfusion disorders found in MRI scan of the heart and peripheral peroneal nerve palsy (in neuro-motorical conduction examination--severe neuropathy). Renal replacement therapy was necessary for 11 days (hemodialyses--3 days, continuous hemodiafiltration--9 days). Transfusions of: 3000 mL of packed erythrocyte mass, 2700 mL of fresh frozen plasma, 1000 mL of packed platelet mass were performed. Full parenteric nutrition was needed for 11 days. Full recovery of renal function, gradual improvement of heart muscle function, regression of lung abnormalities have been obtained.

[Fetuin A in children with nephrotic syndrome]. / Fetuina A u dzieci z zespolem nerczycowym.

AIM OF THE STUDY: To estimate concentration of fetuin A in children with nephrotic syndrome (NS). MATERIAL AND METHODS: 53 children with NS aged from 2.5 to 17.2 years (mean age 7.7 +/- 4.4 years), 27 in remission of NS, 26 in relapse of NS were involved into the study. The control group consisted of 22 healthy children, aged from 6.5 to 17.5 years, mean age 10.8 +/- 3.3 years. In all children serum concentration of fetuin A, calcium, phosphorus, total cholesterol, albumin, triglycerides, and total daily urine protein loss were measured. RESULTS: The concentration of fetuin A in children in relapse was significantly lower compared to children in remission and control group (65.9 +/- 28; 87.2 +/- 23.2; 101.9 +/- 11.6 ng/mL, respectively, p < 0.0001). Significant correlation was found between concentrations of fetuin A and albumin level (r = 0.36, p < 0.05) and calcium concentration (r = 0.30, p < 0.05) and negative correlation between concentration of fetuin A and daily proteinuria (r = -0.36, p < 0.05). CONCLUSION: Low concentration of fetuin A in children in relapse of NS it may depends on proteinuria.

Antihypertensive treatment prescription in pediatric dialysis patients in Poland: A comparison between two nationwide studies 2003/2004-2013.

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.

Minimizing bone abnormalities in children with renal failure.

Renal osteodystrophy (ROD), a metabolic bone disease accompanying chronic renal failure (CRF), is a major clinical problem in pediatric nephrology. Growing and rapidly remodeling skeletal systems are particularly susceptible to the metabolic and endocrine disturbances in CRF. The pathogenesis of ROD is complex and multifactorial. Hypocalcemia, phosphate retention, and low levels of 1,25 dihydroxyvitamin D(3) related to CRF result in disturbances of bone metabolism and ROD. Delayed diagnosis and treatment of bone lesions might result in severe disability. Based on microscopic findings, renal bone disease is classified into two main categories: high- and low-turnover bone disease. High-turnover bone disease is associated with moderate and severe hyperparathyroidism. Low-turnover bone disease includes osteomalacia and adynamic bone disease. The treatment of ROD involves controlling serum calcium and phosphate levels, and preventing parathyroid gland hyperplasia and extraskeletal calcifications. Serum calcium and phosphorus levels should be kept within the normal range. The calcium-phosphorus product has to be <5 mmol(2)/L(2) (60 mg(2)/dL(2)). Parathyroid hormone (PTH) levels in children with CRF should be within the normal range, but in children with end-stage renal disease PTH levels should be two to three times the upper limit of the normal range. Drug treatment includes intestinal phosphate binding agents and active vitamin D metabolites. Phosphate binders should be administered with each meal. Calcium carbonate is the most widely used intestinal phosphate binder. In children with hypercalcemic episodes, sevelamer, a synthetic phosphate binder, should be introduced. In children with CRF, ergocalciferol (vitamin D(2)), colecalciferol (vitamin D(3)), and calcifediol (25-hydroxyvitamin D(3)) should be used as vitamin D analogs. In children undergoing dialysis, active vitamin D metabolites alfacalcidol (1alpha-hydroxy-vitamin D(3)) and calcitriol (1,25 dihydroxyvitamin D(3)) are applied. In recent years, a number of new drugs have emerged that hold promise for a more effective treatment of bone lesions in CRF. This review describes the current approach to the diagnosis and treament of ROD.

[Osteoprotegerin and calcium-phosphorus metabolism parameters in children with chronic renal failure]. / Osteoprotegeryna a parametry przemiany wapniowo-fosforanowej u dzieci z przewlekla niewydoinoscia nerek.

UNLABELLED: Osteoprotegerin (OPG), a member of the TNF receptor superfamily is a natural inhibitor of osteoclastogenesis and important inhibitor of vascular calcification. The aim of the study was to estimate a correlation of serum OPG level and the other parameters calcium-phosphorus metabolism in children with chronic renal failure. Seventy four children with chronic renal insufficiency, 48 on conservative treatment (CRF) aged 13 +/- 3 years with creatinine clearance 45 +/- 21 ml/min/1.73m2 and 26 with end-stage renal disease (ESRD) aged 14 +/- 5 years were examined. The control group (K) consisted of 23 healthy children aged 10.8 +/- 3 years. In all children serum concentration of OPG, calcium (sCa), phosphorus (sP), PTH, CAP (cyclase activating PTH), CIP (cyclase inactive PTH) and osteocalcin (OC) were measured. OPG was determined by ELISA method (Biomedica), PTH and OC by IRMA (Duo-PTH, Scantibodies, USA and Osteo-Riact firm CIS, F). RESULTS: The concentration of OPG was higher in ESRD group (3 +/-1.6 pmol/l) than in K (1.95 +/- 0.56 pmol/l), p<0.005. The concentration of OPG in CRF group (2.42 +/- 1.4 pmol/l) was not different from this in K and ESRD. The concentration of OPG did not correlate with serum creatinine level. In CRF group no correlation was found between OPG level and the other parameters, in group ESRD the significant correlation between OPG and OC was found (R=0.55, p=0.006). The level of OPG in children CRF + ESRD correlated with PTH concentration (R=0.27, p<0.03), CAP (R=0.29, p<0.02), CIP (R=0.23, p<0.05) and OC (R=0.25, P<0.04). In patients with higher PTH level (> 200 pg/ml) the higher correlations between OPG and PTH, CAP and CIP were found. No significant correlation between PTH and OPG in patients with lower PTH level (< 200 pg/ ml) was found. No significant correlation between OPG and OC in patients with lower and higher PTH was found. CONCLUSION: The elevated levels of OPG in children with ESRD may reflect the higher bone turnover in these patients.

[Diagnostic value of PTH total kit for estimation of bone turnover in children with chronic renal failure]. / Przydatnosc oznaczania calej czasteczki parathormonu dla oceny obrotu kostnego u dzieci z przewlekla niewydolnoscia nerek.

UNLABELLED: The aim of the study was to estimate the usefulness of total PTH and 1-84 molecule (CAP) assay in clinical practice. 118 children with chronic renal failure aged 13.8 +/- 4.5 years were examined: 69 on conservative treatment with creatinine clearance 40.7 +/- 20 ml/min/1.73 m2, 31 on peritoneal dialysis and 16 on hemodialysis. In all patients the following parameters were assessed: serum levels of calcium (sCa), phosphorus (sP), PTH intact (PTHs) by chemiluminescence method (Immulite analyzer), total PTH (PTHc) and CAP with Duo PTH Assay (Scantibodies Laboratories, USA). The value of CIP as the difference between total PTH and CAP was calculated. For the evaluation of bone turnover the serum level of osteocalcin (OC) with IRMA, (OsteoRiact, CIS, F) and activity of serum alkaline phosphatase (AP) were determined. The correlations between parameters were calculated by Pearson's correlation coefficient (r). RESULTS: Significant correlation were found between PTHs and PTHc (r=0.84, p=0.0000), CAP (r=0.79, p=0.0000) and CIP. The increase of PTHs, PTHc, CAP, CIP, sP, OC and percentage of CIP were noticed, when parallel increase of creatinine level was found. The negative correlation between creatinine level and CAP/CIP was observed. The similar correlations between level of PTHs, PTHc, CAP and OC level were observed (r=0.55, 0.49 and 0.50 respectively). CONCLUSION: The assay of total PTH and CAP fragment is not usefull in clinical practice for estimation of bone turnover in children with chronic renal failure.

[The causes of the end of growth hormone treatment in children with chronic renal failure]. / Przyczyny zakonczenia leczenia hormonem wzrostu dzieci z przewlekla niewydolnoscia nerek.

UNLABELLED: Growth deficiency is one of complications in chronic renal failure (CRF). In Poland recombinant human growth hormone (rhGH) was introduce to treatment in children with CRF in 1994. The aim of study was to evaluate causes of the end of rhGH treatment in children with CRF. PATIENTS AND METHODS: Among 90 children with different stages of CRF treated with rhGH between 2000-2005 we analyzed 85 patients (33 in CRF and 52 in ESRD), in mean age 9.8 years (from 3 to 17.8 years) which finished therapy. RhGH was used subcutaneously, everyday in doses 1.1 j/kg/week. The anthropometric measurements were performed at the beginning and after one year of the study. RESULTS: The mean time of treatment in 85 children was 18.4 months (1-60 months). The causes of the treatment termination were: renal transplantation in 40 pts (47%), increase of PTH serum level in 13 pts (15.3%), lack of treatment effect in 10pts (11.8%), height achievement at the level of 10c in 10 pts (11.8%), non compliance in 3 pts (3.5%), patient's decision of finishing therapy in 6 pts (7%) and the diagnosis of diabetes during rhGH treatment in 1 pts (1.2%), acute pancreatitis in 1pts (1.2%), epilepsy in 1 pts (1.2%). 25 (29.5%) of out 85 analyzed children had finished the study in the first year. In other 60 patients mean growth velocity was 8.1 cm/year (from 3.3 to 12.4 cm/year). CONCLUSIONS: The main causes of the termination of rhGH treatment in children with CRF were renal transplantation and decompensated secondary hyperparathyroidism.

[Fetuin A in children with renal diseases]. / Fetuina A u dzieci z chorobami nerek.

UNLABELLED: Fetuin A, 59 kDa protein, produced in the liver is a circulating inhibitor of vascular calcification. The aim of the study was to estimate a concentration of fetuin A in children with renal diseases. Fifty three children were examined: 18 with idiopathic nephrotic syndrome (NS) aged 2.5 to 14.5 years, mean 6.6 +/- 3.6 and 35 with chronic renal failure (CRF) aged 4.5 to 20 years, mean 13.3 +/- 3.9. The control group (K) consisted of 22 healthy children, aged from 6.5 to 17.5 years, mean 10.8 +/- 3.3. In all children serum concentration of fetuin A (ELISA method, BioVendor), calcium (sCa), phosphorus (sP), protein (P), albumin (Alb), total cholesterol (TC) and triglycerides (TG) were measured. The daily protein excretion in children with proteinuria (mg/kg/day) and the serum parathormone (PTH) level in children with CRF were analysed. RESULTS: The concentration of fetuin A was the lowest in children with NS (78.1 +/- 24.5 ng/ml) and was statistically different (p=0.0003) than in group K (101.4 +/- 11 ng/ml) and group CRF (106.7 +/- 13.7 ng/ml). In group NS significant correlations were found between concentrations of fetuin A and sCa (r=0.62, p<0.01), P (r=0.59, p= 0.01) and Alb (r=0.64, p<0.01) and negative correlations with concentration of TC (r=-0.68, p<0.01) and daily proteinuria (r=-0.79, p<0.001). In groups K and CRF the correlations of fetuin A level and analysed parameters were not found. CONCLUSION: Low concentration of fetuin A in children with nephrotic syndrome may be the additional agent to promote the atherogenic lesions.

[Peritoneal transport properties for glucose and creatinine in Polish children and adolescents on chronic peritoneal dialysis]. / Ocena przepuszczalnosci otrzewnej dla kreatyniny i glukozy w polskiej populacji dzieci i mlodziezy dializowanych otrzewnowo.

INTRODUCTION: The mode of PD treatment is dependent on the individual transport properties of the peritoneal membrane. Two multicentre trials performed in the U.S. (PPDSC) and Europe (MEPPS) have established reference curves for solute equilibration in children performed with the use of 1100 ml/m2 fill volume in the former and 1000 ml/m2 in the latter study. AIM OF THE STUDY: Assessment of basal peritoneal membrane equilibration based on PET tests in polish children and adolescents treated with chronic peritoneal dialysis. MATERIAL AND METHODS: 58 PET tests from patients treated at 8 Polish PD centres were analysed. The mean time of performing PET test was 6,5 months after the start of PD therapy. All of the patients had been peritonitis free from onset. The mean fill volume was 1021 (906-1170) ml/m2. RESULTS: Based on the results of creatinine and glucose equilibration we established basal peritoneal solute transport curves for polish PD children using an average fill volume of 1020 ml/m2. The following values were obtained at 4hrs of dwell time for 2.27% glucose solution: D/P for creatinine = 0.68 +/- 0.15 and D/Do for glucose = 0.39 +/- 0.12. CONCLUSIONS: The DIP creatinine equilibration curves were similar to the previously published reference curves for children, whereas those for glucose was significantly lower. Using a fill volume scaled to body surface area of 1020 ml/m2 equilibration curves for glucose and creatinine are similar in children over 1 year of age and adults.

[Congenital and genetic related causes of end-stage renal disease--data from Polish Registry of Renal Rreplacement Therapy in Children 2000-2004]. / Genetycznie uwarunkowane i wrodzone choroby nerek prowadzace do schylkowej niewydolnosci nerek--dane z polskiego rejestru dzieci leczonych nerkozastepczo (2000-2004).

UNLABELLED: One of the objectives of Polish Registry of Renal Replacement Therapy in Children established on 31st Dec. 2000 was to collect complete data on etiology of end stage renal disease (ESRD) in polish children. MATERIAL AND METHODS: Data on 469 patients (251 boys, 218 girls) aged 0-22 years treated with renal replacement therapy (RRT) at 13 pediatric dialysis units in Poland from 2000 to 2004 were analyzed. The mean age at start of dialysis was 10 years and 3 months. Renal diseases were defined according to EDTA coding system. Data is presented for the whole group, in 5-year age groups and separately for both sexes. RESULTS: Congenital and genetic renal diseases were the cause of ESRF in 56% of the polish population of children and adolescents on RRT. 39% of causes were acquired diseases, 5% remained unidentified. Congenital and genetic causes dominated in children < 5 years of age (71%). They accounted for 49%, 61% and 45% of causes in the consecutive 5-year age groups. The most numerous group of congenital diseases leading to ESRF were uropathies 37% and 25% of causes in the consecutive age groups. In boys the most frequent uropathy was obstructive uropathy (25%), the majority caused by posterior urethral valves. In girls the most frequent uropathies were reflux nephropathy (10%) and nephropathy secondary to neurogenic bladder (9%). Uropathies were followed by renal hypo-dysplasia without urinary tract anomalies (11%) and cystic diseases (10%). CONCLUSIONS: Congenital kidney anomalies and genetic diseases are the leading cause of end-stage renal disease in children up to 15 years of age.

Growth and nutritional status in children with chronic kidney disease on maintenance dialysis in Poland.

PURPOSE: Despite vast availability of modern methods of treatment of chronic kidney disease and its complications, the short stature still is a major point of concern in adolescents with chronic kidney disease. The aim of the study was to assess changes in growth and nutritional status of Polish children on renal replacement therapy in the decade, 2004-2013. MATERIAL AND METHODS: The study was designed as a cross-sectional analysis of anthropometric values and selected indices of growth status amongst children receiving dialysis in Poland between the years 2004 and 2013. Data were acquired during two different multicentre studies on hypertension in dialyzed children in Poland. Basic anthropometric parameters (body weight, body height/length, body mass index - BMI), dialysis adequacy and duration of RRT were assessed. RESULTS: The study showed that anthropometric parameters of children undergoing renal replacement therapy had not significantly changed in the last 10 years of observation. Children on RRT were still of short stature despite availability of modern methods of hormonal therapy and nutrition. Median of height z-score was -2.10 in 2004 and -2.19 in 2013. Expected clinical improvement in these measures was not proven. CONCLUSIONS: The cause of chronic kidney disease, method of dialysis, time on dialysis or dialysis adequacy did not influence the anthropometric parameters significantly in dialyzed children in Poland.

Diagnostic Value of Serological Tests Against Verotoxigenic Escherichia coli in Hemolytic Uremic Syndrome in Children.

BACKGROUND: Diarrhea-associated hemolytic uremic syndrome (HUS D+) caused by verotoxigenic E. coli strains (VTEC) is a major cause of acute kidney injury in children between 1 and 5 years of age. Because of the short presence of VTEC in the gastrointestinal tract as well as difficulties with the detection of the verotoxigenic strain, identification of HUS etiology might be challenging. OBJECTIVES: The aim of the study was to assess the clinical and diagnostic value of serological tests for specific antibodies against verotoxigenic strains of E. coli in patients with HUS. MATERIAL AND METHODS: Eight children aged 8 months - 7.1 years (mean 40 ± 29 months) with symptoms of acute kidney injury, hemolytic anemia and thrombocytopenia observed after hemorrhagic diarrhea were included to the study. VTEC presence was detected in a stool culture with subsequent analysis of the ability to produce verotoxin and the presence of VT1 and VT2 as well as intimin and enterohemolysin genes. In addition, the presence of specific IgA, IgM and IgG antibodies against E. coli serogroups O26, O103, O104, O111, O121, O145 and O157 was measured using ELISA. RESULTS: In 3 subjects, VTEC O26, O157 and O104 serogroups were cultured in the stool and the specific IgA, IgM and IgG antibodies were detected. In 4 subjects, no VTEC strains were cultured, however, high titers of IgA, IgM and IgG antibodies against E. coli O26, O157 and O111 were detected. In a single patient, the negative results of bacteriological and serological analyses excluded VTEC etiology of HUS. CONCLUSIONS: A serological analysis of VTEC can confirm the result of stool culture for verotoxigenic E. coli strains and help to find the cause of HUS in case of negative results of a stool culture.

Do children with end-stage renal disease live shorter? Analysis of mortality on the basis of data from the Polish Registry of Renal Replacement Therapy in Children.

PURPOSE: The mortality of patients with end-stage renal disease (ESRD) is much higher than that of the general population. To date no data has been published on the mortality of children with ESRD in Poland. The aim of this study was to compare the risk of death for pediatric patients on renal replacement therapy (RRT) with that of the general pediatric population and to identify the risk factors of death. MATERIAL/METHODS: Data of 779 children with ESRD registered in the Polish Registry of Children on RRT was analyzed. The relative risk of death was calculated as the ratio of the mortality rate in ESRD patients to the mortality rate in age-adjusted general population. RESULTS: The mortality rate of children with ESRD was 74-fold higher than that of the age- and gender-adjusted general pediatric population (4.05 vs. 0.05/100 person-years). The highest mortality rate (4.53/100 patient-years) was found in the youngest age group. Younger age and duration of dialysis therapy were identified as mortality risk factors. The major causes of death in ESRD patients were infections and cardiovascular complications, whereas deaths in general child population were mainly due to accidents or congenital defects. CONCLUSIONS: The mortality in Polish children with ESRD is 74-fold higher than that of the general pediatric population. Infections, followed by cardiovascular complications, constitute the main causes of mortality in children subjected to RRT. The risk of death is the highest among children who started RRT at a younger age and in those subjected to long-term dialysis treatment.