Mild X-linked Alport syndrome due to the COL4A5 G624D variant originating in the Middle Ages is predominant in Central/East Europe and causes kidney failure in midlife.

A study of 269 children enrolled into a National Registry for children with persistent glomerular hematuria identified 131 individuals with genetically confirmed X-linked Alport Syndrome. A single variant c.1871G>A p.Gly624Asp (G624D) in COL4A5 was predominant and accounted for 39% of X-linked Alport Syndrome in unrelated Polish families (44 of 113). To evaluate its origins, the genetic variation in a 2.79 Mb segment encompassing the COL4A5 locus on chromosome X was assessed. All G624D alleles were found on the same rare haplotype background, indicating a founder effect dating back to the 12-13th century. The phenotypic data of 131 children with X-linked Alport Syndrome and their 195 affected adult relatives revealed that the G624D variant was associated with a significantly milder clinical course in comparison to other pathogenic COL4A5 variants. Furthermore the clinical course of this genetically uniform cohort was milder than that observed in individuals with other COL4A5 missense mutations. In spite of the benign clinical manifestation throughout childhood and early adulthood, the G624D variant confers significant risk for both kidney failure and deafness in males, albeit 20-30 years later than that observed in individuals with other COL4A5 pathogenic variants (50% cumulative risk of starting dialysis at 54 years (95% confidence interval: 50-62) v. 26 years (95% confidence interval: 22-30)). Thus, males with G624D are candidates for existing and emerging therapies for Alport Syndrome.

Clinical profile of a Polish cohort of children and young adults with cystinuria.

BACKGROUND: Cystinuria is an inherited disorder that results in increased excretion of cystine in the urine. It accounts for about 1-2% of pediatric kidney stones. In this study, we sought to identify the clinical characteristics of patients with cystinuria in a national cohort. METHODS: This was a retrospective study involving 30 patients from the Polish Registry of Inherited Tubulopathies. Initial data and that from a 6-month follow-up were analyzed. Mutational analysis was performed by targeted Sanger sequencing and, if applicable, MLPA analysis was used to detect large rearrangements. RESULTS: SLC7A9 mutations were detected in 15 children (50%; 10 males, 5 females), SLC3A1 mutations in 14 children (47%; 5 males, 9 females), and bigenic mutations in one male patient. The first clinical symptoms of the disease were detected at a median of 48 months of age (range 3-233 months). When individuals with different mutations were compared, there were no differences identified in gender, age of diagnosis, presence of UTI or urolithiasis, eGFR, calcium, or cystine excretion. The most common initial symptoms were urolithiasis in 26 patients (88%) and urinary tract infections in 4 patients (13%). Urological procedures were performed in 18 out of 30 (60%). CONCLUSIONS: The clinical course of cystinuria is similar among patients, regardless of the type of genetic mutation. Most patients require surgery before diagnosis or soon after it. Patients require combined urological and pharmacological treatment for prevention of stone recurrence and renal function preservation.

Hemodialysis vascular access and subsequent transplantation: a report from the ESPN/ERA-EDTA Registry.

BACKGROUND: Current guidelines advocate use of arteriovenous fistula (AVF) over central venous catheter (CVC) for children starting hemodialysis (HD). European data on current practice, determinants of access choice and switches, patient survival, and access to transplantation are limited. METHODS: We included incident patients from 18 European countries who started HD from 2000 to 2013 for whom vascular access type was reported to the ESPN/ERA-EDTA Registry. Data were evaluated using descriptive statistics, logistic and Cox regression models, and cumulative incidence competing risk analysis. RESULTS: Three hundred ninety-three (55.1%) of 713 children started HD with a CVC and were more often females, younger, had more often an unknown diagnosis, glomerulonephritis, or vasculitis, and lower hemoglobin and height-SDS at HD initiation. AVF patients were 91% less likely to switch to a second access, and two-year patient survival was 99.6% (CVC, 97.2%). Children who started with an AVF were less likely to receive a living donor transplant (adjusted HR, 0.30; 95% CI, 0.16-0.54) and more likely to receive a deceased donor transplant (adjusted HR, 1.50; 95% CI, 1.17-1.93), even after excluding patients who died or were transplanted in the first 6 months. CONCLUSIONS: CVC remains the most frequent type of vascular access in European children commencing HD. Our results suggest that the choice for CVC is influenced by the time of referral, rapid onset of end-stage renal disease, young age, and an expected short time to transplantation. The role of vascular access type on the pattern between living and deceased donation in subsequent transplantation requires further study.

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.

The effect of peritoneal dialysis method on residual renal function in children.

We set out to assess the effect of continuous ambulatory peritoneal dialysis (CAPD) and automated peritoneal dialysis (APD) on residual renal function (RRF) in children with end-stage renal disease (ESRD). In 101 children (age: 8.84 +/- 5.25 years; 44 on CAPD, 57 on APD) over 36 months, we evaluated RRF [as daily diuresis (DD) in mL/kg/24 h and mL/m2/24 h], glomerular filtration rate [GFR (in mL/min/1.73 m2)], ESRD cause, presence of arterial hypertension (HTN), biochemical parameters, peritoneal equilibration test (PET), adequacy [as total weekly Kt/V (twKt/V) and creatinine clearance (twCCr)], and infectious complications of PD. Initially, the CAPD and APD groups did not differ significantly in DD, but mean GFR was significantly higher in the APD group (p < 0.05). In the CAPD group, the volume of high osmolarity PD fluid was significantly lower (p < 0.05), and the rates of peritonitis and exit-site infection and of aminoglycoside use were higher (p < 0.001, p < 0.05, and p < 0.005 respectively). Over 36 months, the mean twKt/V and twCCr were within norms in both groups, but were higher in APD, significantly so (p < 0.05) for twKt/V at 24 and 36 months and for twCCr initially. In both groups, RRF decreased systematically, with a significantly lower (p < 0.05) rate of DD (mL/m2/24 h) and GFR decline in the first year in CAPD, but without a difference in the next 2 years. The longest RRF preservation was in children with tubulointerstitial nephropathies, particularly hypoplasia and dysplasia (p < 0.05). Children with hemolytic uremic syndrome (HUS) and hereditary nephropathy were at the highest anuria risk. Compared with the 22 children (7 CAPD, 15 APD) who became anuric, the 20 children (10 CAPD, 10 APD) with RRF preserved for 36 months had a higher DD and GFR before dialysis onset; higher hemoglobin and albumin; and lower HTN prevalence, cholesterol, triglycerides, and proteinuria (p < 0.05). Risk of anuria during 36 months did not differ significantly between the CAPD and APD groups. In children on CAPD or APD, risk factors for RRF loss include HUS, hereditary nephropathy, low diuresis and GFR before dialysis onset, HTN, anemia, hypoalbuminemia, hyperlipidemia, and proteinuria. Compared with children on APD, those on CAPD show better preservation of RRF during year 1, although the risk of anuria seems to be the same for both methods. In children with risk factors for rapid diuresis loss, CAPD might be considered the preferred initial dialysis method.

Galectin-3-A New Player of Kidney Damage or an Innocent Bystander in Children with a Single Kidney?

The aim of this study was to evaluate the galectin-3 (Gal-3) level in children with a congenital solitary functioning kidney (cSFK) and determine its association with common renal function parameters. The study consisted of 68 children (49 males) with cSFK. We demonstrated that children with cSFK had a lower level of galectin-3 than that of healthy subjects (p < 0.001). No significant differences in serum cystatin C (Cys C) levels between the cSFK children and the reference group were found. The subjects with cSFK and reduced estimated glomerular filtration rate (eGFR) had significantly higher levels of Gal-3 and Cys C compared to those with normal eGFR (p < 0.05). Children with eGFR <60 mL/min/1.73 m2 showed significant statistical differences between the values of area under ROC curve (AUC) for Gal-3 (AUC 0.91) and Cys C (AUC 0.96) compared to that for creatinine level (AUC 0.76). Similar analyses carried out among cSFK children with eGFR <90 mL/min/1.73 m2 revealed an AUC value of 0.69 for Gal-3, 0.74 for Cys C, and 0.64 for creatinine; however, no significant superiority was shown for any of them. The receiver operating characteristic (ROC) analyses for identifying the SFK children among all participants based on the serum levels of Gal-3 and Cys C did not show any diagnostic profile (AUCs for Gal-3 and Cys C were 0.22 and 0.59, respectively). A positive correlation between the Gal-3 and Cys C concentrations was found (r = 0.39, p = 0.001). We demonstrated for the first time that Gal-3 might play an important role in the subtle kidney damage in children with cSFK. However, further prospective studies are required to confirm the potential applicability of Gal-3 as an early biomarker for kidney injury and possible progression to CKD.

[Calcium-phosphate disturbances in dialysed children and adolescents--one centre analysis between 1991-2008]. / Zaburzenia gospodarki wapniowo-fosforanowej u dzieci i mlodziezy dializowanych w latach 1991-2008--doswiadczenia jednego osrodka.

UNLABELLED: Disturbances of calcium-phosphate metabolism are one of the most prominent complications of chronic kidney disease with high impact on cardiovascular complications and outcome. The aim of the study was to assess the clinical efficacy of CKD-related calcium-phosphate homeostasis treatment in children and adolescents on chronic dialysis in a tertiary reference centre between 1991 to 2008. MATERIAL AND METHODS: Study group consisted of 81 subjects (29F, 52M): 40 hemodialysis and 41 peritoneal dialysis patients (aged at start of dialysis: 1m.-17 y.). Treatment period ranged from 7 to 150 months. 9 subjects died on dialysis. A retrospective analysis of medical records was performed for serum calcium, phosphate, parthormon, alkaline phosphatase, CaxP product and applied treatment (conservative or surgery). The analysis was conducted in 4-5 y time intervals. RESULTS: We did not observed significant differences in the most of analyzed parameters apart a tendency to increase in PTH levels in last 9 years. Conservative treatment comprised a diet, calcium phosphate binders, vitamin D metabolites and non-calcium phosphate binders (since 2003). Parathyroidectomy was performer as surgery. CONCLUSIONS: The efficacy of treatment of CKD-related calcium-phosphate disturbances in children and adolescents on chronic dialysis has not changed in last several years despite introduction of new methods of treatment.

[Difficulties in renal osteodystrophy treatment in patient undergoing long-term renal replacement therapy--a case study]. / Trudnosci w leczeniu osteodystrofii mocznicowej u chorego w przebiegu wieloletniego leczenia nerkozastepczego--opis przypadku.

Abnormal mineral metabolism and altered bone structure and composition occur early in the course of chronic kidney disease. We present difficulties in renal osteodystrophy treatment in patient undergoing renal replacement therapy for twenty two years (dialysis, transplant, dialysis), which is not in the waiting list for kidney transplant (patient disagreement). Due to failure of conventional therapy of hyperparathyroidism (calcium, phosphate binders, vitamin D) he was needed parathyroidectomy twice. Now he presents a very low PTH level but hyperphosphatemia, hypercalcemia and calcium/phosphate product over upper limit. This disturbances led to extra skeletal calcification (skin, vessels, eyes - "red eyes syndrome", central nervous system). Even now having new phosphate binders we cannot keep plasma phosphate, calcium in normal range, probably due to inadequate diet and non-compliance. Effective therapy is still difficult in this patient.

Twenty years of growth hormone treatment in dialyzed children in Poland-Results of national multicenter study.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

[Reflux and obstructive nephropathy as a cause of renal failure in chronic dialysis children]. / Nefropatia refluksowa i zaporowa jako przyczyna niewydolnosci nerek u dzieci leczonych nerkozastepczo.

We carried out a retrospective analysis of medical files to evaluate causes of chronic renal failure in 80 children (M--49, F--31), age 1 month to 20 years) who started renal replacement therapy in the Department of Nephrology and Dialysis of the Polish Mothers Memorial Hospital in the years 1990-2007. In 28 children (35%) reflux and obstructive nephropathy was a cause of renal failure. In 5 children the disease was secondary to the neurogenic bladder. The incidence of these nephropathies in our population was constant in the analyzed years. In our group there were 2 neonates and 7 adolescent who were diagnosed with nephropathy as late as in the endstage phase. Boys with posterior urethral valve required renal replacement therapy earlier (146 +/- 55 months). We conclude that obstructive and reflux nephropathy are still the essential cause of end stage renal disease in children.

Glomerular filtration decrease after diagnostic cardiac catheterisation in children with congenital cardiac malformation - the role of serum creatinine, cystatin C, neutrophil gelatinase and urine output monitoring.

INTRODUCTION: Diagnosis of contrast induced-nephropathy (CIN) by a classic renal biomarker such as creatinine concentration can be delayed because of various factors that can influence this marker. Changes in new biomarkers such as neutrophil-gelatinase associated lipocalin (NGAL) and cystatin C are postulated to be more sensitive for recognizing patients prone to CIN-acute kidney injury (AKI). AIM: To investigate the role of NGAL and cystatin C as early biomarkers in the diagnosis of kidney injury after cardiac catheterisation. MATERIAL AND METHODS: The study group consisted of 50 patients with congenital heart malformation admitted for scheduled cardiac catheterisation. The biomarkers serum creatinine, serum NGAL and serum cystatin C were tested at 5 time-points sequentially from start to 48 h after the procedure. RESULTS: Significant changes were noted during the research in the serum creatinine concentration (p < 0.001) and serum NGAL concentration (p < 0.001). CIN-AKI, diagnosed by the modified Schwartz formula, occurred in 16 (32%) patients after 24 h and in 8 (16%) after 48 h. Subsequent analysis showed that serum creatinine significantly rose in the first 2 h of the study with simultaneous reduction in the eGFR. Maximum growth in serum NGAL occurred at 6 h after contrast administration and then returned to the baseline values at 24 h. Serum cystatin C level did not significantly change during the study. CONCLUSIONS: We observed a transient decrease in eGFR and a rise of serum NGAL after 2 h but NGAL was most pronounced at 6 h after the procedure. The potential role of cystatin C as a biomarker of CIN-AKI was not proved.

Continuous veno-venous hemodiafiltration in children after cardiac surgery.

OBJECTIVE: Acute renal failure (ARF) is still a frequent complication following extensive cardiac surgery. Renal replacement therapy (RRT) modality preferences to treat critically ill children have shifted from peritoneal dialysis to continuous renal replacement therapy (CRRT), although the experience with the latter is still highly limited in the infants. METHODS: We describe our results with continuous veno-venous hemodiafiltration (CVVHDF) in 25 children (15 males, 10 females) who underwent CRRT from 2001 to 2006 and were retrospectively reviewed. RESULTS: We performed continuous veno-venous hemodiafiltration (CVHDF) using PRISMA (Hospal). The mean age at the onset of CRRT was 26 months (ranging from 7 days to 11.2 years) and the mean body weight was 14 kg. The mean duration of RRT was 67 h (8-243 h) with ultrafiltration rate 4.9 ml/(h kg); the mean filter "lifetime" was 31.5h. Anticoagulation was achieved with non-fractioned heparin infusion (21/25 cases) and enoxaparin (2/16). The mean creatinine concentrations at the beginning, 24, 48 and 72 h were as follows: 171, 100, 65 and 88 micromol/l. Of these 25 treated children, 19 died in the postoperative period (8 during CVVHDF). The mortality rate for the entire group was 76%. The main cause of death was cardiac failure and sepsis with multiorgan dysfunction (MODS). The main complication during CRRT was bleeding, transient hypothermia, thrombocytopenia and filter clotting which occurred in about one-third of the patients. CONCLUSIONS: We conclude that CVVHDF may be an alternative method of renal support for critically ill children after cardiac surgery in experienced centers, but a significant number of specific complications should be taken into account.

Evaluation of the effect of 3-month bladder basic advice in children with monosymptomatic nocturnal enuresis.

INTRODUCTION: Advice (BBA) into the standards of patients' care in both monosymptomatic and non-monosymptomatic nocturnal enuresis. Although the idea of this recommendation was clear and reflects clinical experience, duration and efficacy have not been definitely established. Recent data have demonstrated the lack of efficacy of BBA and a fierce discussion has ensued. The present study was aimed to assess the efficacy of BBA in a group of previously untreated children with primary monosymptomatic nocturnal enuresis (MNE). STUDY DESIGN: The study was a prospective interventional multicenter trial in a cohort of previously untreated MNE patients. Forty-nine children (36 males, 13 females, mean age 7.2 years) were included in the analysis. The treatment efficacy was assessed at the 30th, 60th, and 90th days of BBA. RESULTS: We discovered that the mean number of wet nights decreased significantly (p < 0.001) only after 3 months of BBA from 8.9 to 5.9 episodes every 2 weeks. BBA was fully successful in 2% o the children after 30 day, 12% after 60 days, and 18% after 90 days (Figure). Partial response (by ICCS) was assessed for 8%, 20%, and 34% of the patients. We noted a relatively high rate of non-responders that decreased from 90% to 47% after 90 days. We detected no differences in BBA efficacy between children with night-time polyuria or decreased maximal voided volume. A lower number of wet nights initially predicted the response to the BBA. DISCUSSION: Our study confirmed rather limited efficacy of BBA, similarly to previous observations, but provided more information on isolated MNE, because of a more specific study group and longer period of observation. The limitation of the study was lack of randomization. CONCLUSION: Our study revealed that in treatment-naïve children with monosymptomatic enuresis basic bladder training had a low (18%) and late effect, mostly pronounced after the third month of therapy. It seems that only if the patient presents with a favorable profile of bedwetting, occasionally and with a high maximum voided volume, it is worth maintaining BBA for a longer period of up to 3 months before initiating second-line therapy. In an unfavorable initial profile desmopressin or an alarm may be introduced much earlier.

[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.

[Chronic peritoneal dialysis in infants--preliminary results of the multicenter survey]. / Przewlekla dializa otrzewnowa u niemowlat--wstepna analiza ankiety wieloosrodkowej.

We retrospectively analysed peritoneal dialysis treatment in 29 infants dialysed in 9 paediatric centres in Poland in the years 1993-2004. The mean age at the start of dialysis was 4.9 +/- 3.5 months (range 2 days to 11 months), mean body mass 5.6 +/- 2.5 kg (range 2.5 to 11 kg). The mean duration of PD was 6.8 +/- 3.9 in the first year of life and total duration of the therapy 34 +/- 27 months. Of the 29 infants 4 died (2 in infancy), 11 underwent renal transplantation, in 2 children PD was stopped (they received a conventional treatment) and 12 were still dialysed at the date of data collection. The peritonitis rate was 1/9.5 patient-month and exit site infection rate 1/16 patient-month up to 1 year of life. 9 children (31%) required hernia repairs and in 9 catheters were replaced. Chronic peritoneal dialysis in infants is associated with high risk of infections and surgical complications and remains a challenge for paediatric nephrologists.

Effect of hypertension and antihypertensive medications on residual renal function in children treated with chronic peritoneal dialysis.

PURPOSE: To evaluate the effect of hypertension (HTN) and antihypertensive medications (AHM) on residual renal function (RRF) in children on CAPD and APD. MATERIAL/METHODS: We retrospectively evaluated underlying kidney disease, systolic and diastolic blood pressure (SBP/DBP), presence and control of HTN (SBP/DBP≥95th percentile), AHM, RRF (daily diuresis, residual glomerular filtration rate [rGFR]), biochemical parameters, BMI Z-score, and dialysis parameters during 12-month follow-up in 87 children (38 CAPD, 49 APD) aged 10.22±4.31 years. The rate of RRF loss was expressed as absolute and relative [%] reduction. RESULTS: At baseline, HTN was found in 74.7% patients (CAPD/APD: 84.2%/67.3%, P=0.06), most commonly in HUS and least frequently in CAKUT. The proportion of CAPD/APD patients with poorly controlled HTN was 70.0%/63.3% (P=0.50). Relative daily diuresis loss in children with uncontrolled HTN was higher (P=0.017) compared to children with SBP/DBP <95th percentile. No effect of AHM on the rate of RRF loss was found. In multivariate analysis, absolute daily diuresis loss was related to baseline diuresis (ß=-0.30, P<0.001) and proteinuria (ß=-0.31, P=0.004); absolute rGFR loss to baseline rGFR (ß=-0.73, P<0.001) and glucose load after 12 months (ß=-0.36, P=0.02); relative daily diuresis loss to mean BMI Z-score (ß=-0.44, P=0.04); and relative rGFR to baseline rGFR (ß=-0.37, P<0.001) and SBP percentile (ß=-0.21, P=0.045).

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.

[Dialysis-related complications in peritoneal dialysis patients]. / Powiklania dializy otrzewnowej u chorych na ostra i przewlekla niewydolnosc nerek.

The hospital records of 56 patients (25M, 31F) with acute or chronic renal failure treated by peritoneal dialysis were retrospectively reviewed. Mean dialysis time was 13 +/- 15 days in acute renal failure and 32 +/- 23 months in chronic renal failure. The incidence of infectious (exit site infections and peritonitis) and non-infectious dialysis-related complication was assessed. Exit site infections were significantly more frequent in children aged 5 or less than in older patients (1/9.6 patient-month and 1/26.5 patient-month, respectively, p < 0.001). Such relationship was not found with regard to the incidence of peritonitis. There was a tendency of peritonitis rate to decrease in consecutive years was noted. This can be probably related to an increase in the number of patients, introduction of automated peritoneal dialysis, increasing experience of medical staff and patients themselves.

[Pneumoperitoneum and peritonitis in a child treated by continuous ambulatory peritoneal dialysis]. / Zapalenie otrzewnej i odma otrzewnowa u chorej leczonej ciagla ambulatoryjna dializa otrzewnowa.

Pneumoperitoneum is a rare complication of peritoneal dialysis, which in most cases occurs soon after the implantation of the peritoneal catheter and does not need any specific treatment. In contrast, pneumoperitoneum due to visceral perforation represents a serious clinical problem and usually needs an urgent surgical intervention. We present a case of a 10-year old girl treated by peritoneal dialysis for 7 months who was admitted to hospital with symptoms of peritonitis and pneumoperitoneum. On admission her condition was severe and visceral perforation was strongly suspected. As her parents did not give consent to surgical treatment, only conservative management was introduced. Peritoneal dialysis was continued and the girl responded well to antibacterial therapy. The final outcome was favourable.