Relationship between Gd-IgA1 and TNFR1 in IgA nephropathy and IgA vasculitis nephritis in children - multicenter study.

AIM OF THE STUDY: To evaluate the relationship between serum Gd-IgA1 (sGd-IgA1) and serum and urine TNFR1 (sTNFR1, uTNFR1) levels as possible prognostic factors in IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN). MATERIAL AND METHODS: From 299 patients from the Polish Registry of Pediatric IgAN and IgAVN, 60 children (24 IgAN and 36 IgAVN) were included in the study. The control group consisted of 20 healthy children. Proteinuria, haematuria, serum creatinine as well as IgA and C3 levels were measured and glomerular filtration rate (GFR) was calculated at onset and at the end of the follow-up. Kidney biopsy findings were evaluated using the Oxford classification. Serum Gd-IgA1 and serum and urine TNFR1 levels were measured at the end of follow-up. RESULTS: Serum Gd-IgA1 level was significantly higher in IgAN and IgAVN patients in comparison to the control group. Urine TNFR1 was significantly higher in IgAN than in IgAVN and the control group. We did not observe any differences in sTNFR1 level between IgAN, IgAVN and control groups. We found a positive correlation between Gd-IgA1 and creatinine (r = 0.34), and negative between Gd-IgA1 and GFR (r = -0.35) at the end of follow-up. We observed a negative correlation between uTNFR1/creatinine log and albumin level and protein/creatinine ratio. We did not find any correlations between Gd-IgA1 and TNFR1. CONCLUSIONS: The prognostic value of sGd-IgA1 in children with IgAN and IgAVN has been confirmed. TNFR1 is not associated with Gd-IgA1 and is not a useful prognostic marker in children with IgAN/IgAVN and normal kidney function.

The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy-A National Study in Children.

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.

IgA vasculitis nephritis clinical course and kidney biopsy - national study in children.

The aim of the study was to investigate the relationship between the severity of typical clinical symptoms, severity of histopathological lesions in kidney biopsies in IgA vasculitis nephritis (IgAVN) and to propose indications for kidney biopsy in children. MATERIAL AND METHODS: This retrospective study enrolled 106 patients, included in the IgAVN registry of Polish children, diagnosed by kidney biopsy. Renal and extrarenal symptoms at onset of the disease were analyzed. Biopsy results were assessed using Oxford classifications (MEST-C). The patients were divided into 3 groups depending on the severity of proteinuria: A-nephrotic proteinuria with hematuria; B-non-nephrotic proteinuria with hematuria; C-isolated hematuria. RESULTS: The first symptoms of nephropathy were observed at the 0.7 (1-128.4) months from the onset of extrarenal symptoms. Kidney biopsy was performed on 39 (6-782) days after the onset of nephropathy symptoms. MEST-C score 4 or 5 was significantly more frequent in children from group A than in groups B and C. Significantly higher mean MEST-C score was found in patients with abdominal symptoms than without. In group A: S0 and T0 we found in significantly shorter time to kidney biopsy than in S1, T1-2 p < 0.05) and in group B the significantly shorter time in T0 compare to T1-2 p < 0.05). The ROC analysis shows that S1 changes appear in kidney biopsies in group A with cut off 21 days (AUC 0,702, p = 0.004, sensitivity 0.895 specificity 0.444) T1-2 changes after 35 days (AUC 0.685, p = 0.022, sensitivity 0.750, specificity 0.615), and in goupn B T1-2 cut off is 74 days (AUC 0,738, p = 0.002, sensitivity 0.667, specificity 0.833). CONCLUSIONS: In childhood IgAVN, the severity of changes in the urine is clearly reflected in the result of a kidney biopsy. The biopsy should be performed in patients with nephrotic proteinuria no later than 3 weeks after the onset of this symptom in order to promptly apply appropriate treatment and prevent disease progression. Accompanying abdominal symptoms predispose to higher MESTC score.

Comprehensive Metabolic Signature of Renal Dysplasia in Children. A Multiplatform Metabolomics Concept.

Renal dysplasia is a severe congenital abnormality of the kidney parenchyma, which is an important cause of end-stage renal failure in childhood and early adulthood. The diagnosis of renal dysplasia relies on prenatal or postnatal ultrasounds as children show no specific clinical symptoms before chronic kidney disease develops. Prompt diagnosis is important in terms of early introduction of nephroprotection therapy and improved long-term prognosis. Metabolomics was applied to study children with renal dysplasia to provide insight into the changes in biochemical pathways underlying its pathology and in search of early indicators for facilitated diagnosis. The studied cohort consisted of 72 children, 39 with dysplastic kidneys and 33 healthy controls. All subjects underwent comprehensive urine metabolic profiling with the use of gas chromatography and liquid chromatography coupled to mass spectrometry, with two complementary separation modes of the latter. Univariate and multivariate statistical calculations identified a total of nineteen metabolites, differentiating the compared cohorts, independent of their estimated glomerular filtration rate. Seven acylcarnitines, xanthine, and glutamine were downregulated in the urine of renal dysplasia patients. Conversely, renal dysplasia was associated with higher urinary levels of dimethylguanosine, threonic acid or glyceric acid. This is the first metabolomic study of subjects with renal dysplasia. The authors define a characteristic urine metabolic signature in children with dysplastic kidneys, irrespective of renal function, linking the condition with altered fatty acid oxidation, amino acid and purine metabolisms.

The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results.

BACKGROUND: The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically. RESULTS: Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4.1%). Genetically confirmed diagnoses were reported in 24% of all pediatric and 12% adult patients, whereas glomerulopathies had been confirmed by kidney biopsy in 80.4% adult versus 38.5% pediatric glomerulopathy cases. CONCLUSIONS: ERKReg is a rapidly growing source of epidemiological information and patient cohorts for clinical research, and an innovative tool to monitor management quality and patient outcomes.

Indoxyl sulfate associates with cardiovascular phenotype in children with chronic kidney disease.

BACKGROUND: Cardiovascular disease is the leading cause of death in children with chronic kidney disease (CKD). Serum levels of gut-derived uremic toxins increase with deterioration of kidney function and are associated with cardiac comorbidities in adult CKD patients. METHODS: Indoxyl sulfate (IS) and p-cresyl sulfate (pCS) were measured by high-performance liquid chromatography in serum of children participating in the Cardiovascular Comorbidity in Children with CKD (4C) Study. Results were correlated with measurements of the carotid intima-media thickness (cIMT), central pulse wave velocity (PWV), and left ventricular mass index (LVMI) in children aged 6-17 years with initial eGFR of 10-60 ml/min per 1.73 m2. RESULTS: The median serum levels of total IS and of pCS, measured in 609 patients, were 5.3 µmol/l (8.7) and 17.0 µmol/l (21.6), respectively. In a multivariable regression model, IS and pCS showed significant positive associations with urea and negative associations with eGFR and uric acid. Furthermore, positive associations of pCS with age, serum albumin, and non-Mediterranean residency and a negative association with glomerular disease were observed. By multivariable regression analysis, only IS was significantly associated with a higher cIMT SDS at baseline and progression of PWV SDS within 12 months, independent of other risk factors. CONCLUSIONS: Serum levels of gut-derived uremic toxins IS and pCS correlated inversely with eGFR in children. Only IS was significantly associated with surrogate markers of cardiovascular disease in this large pediatric CKD cohort.

Association of Serum Soluble Urokinase Receptor Levels With Progression of Kidney Disease in Children.

Importance: Conventional methods to diagnose and monitor chronic kidney disease (CKD) in children, such as creatinine level and cystatin C-derived estimated glomerular filtration rate (eGFR) and assessment of proteinuria in spot or timed urine samples, are of limited value in identifying patients at risk of progressive kidney function loss. Serum soluble urokinase receptor (suPAR) levels strongly predict incident CKD stage 3 in adults. Objective: To determine whether elevated suPAR levels are associated with renal disease progression in children with CKD. Design, Setting, and Participants: Post hoc analysis of 2 prospectively followed up pediatric CKD cohorts, ie, the ESCAPE Trial (1999-2007) and the 4C Study (2010-2016), with serum suPAR level measured at enrollment and longitudinal eGFR measured prospectively. In the 2 trials, a total of 898 children were observed at 30 (ESCAPE Trial; n = 256) and 55 (4C Study; n = 642) tertiary care hospitals in 13 European countries. Renal diagnoses included congenital anomalies of the kidneys and urinary tract (n = 637 [70.9%]), tubulointerstitial nephropathies (n = 92 [10.2%]), glomerulopathies (n = 69 [7.7%]), postischemic CKD (n = 42 [4.7%]), and other CKD (n = 58 [6.5%]). Total follow-up duration was up to 7.9 years, and median follow-up was 3.1 years. Analyses were conducted from October 2016 to December 2016. Exposures: Serum suPAR level was measured at enrollment, and eGFR was measured every 2 months in the ESCAPE Trial and every 6 months in the 4C Study. The primary end point of CKD progression was a composite of 50% eGFR loss, eGFR less than 10 mL/min/1.73 m2, or initiation of renal replacement therapy. Main Outcomes and Measures: The primary end point in this study was renal survival, defined as a composite of 50% loss of GFR that persisted for at least 1 month, the start of renal replacement therapy, or an eGFR less than 10 mL/min/1.73 m2. Results: Of the 898 included children, 560 (62.4%) were male, and the mean (SD) patient age at enrollment was 11.9 (3.5) years. The mean (SD) eGFR was 34 (16) mL/min/1.73 m2. The 5-year end point-free renal survival was 64.5% (95% CI, 57.4-71.7) in children with suPAR levels in the lowest quartile compared with 35.9% (95% CI, 28.7-43.0) in those in the highest quartile (P < .001). By multivariable analysis, the risk of attaining the end point was higher in children with glomerulopathies and increased with age, blood pressure, proteinuria, and lower eGFR at baseline. In patients with baseline eGFR greater than 40 mL/min/1.73 m2, higher log-transformed suPAR levels were associated with a higher risk of CKD progression after adjustment for traditional risk factors (hazard ratio, 5.12; 95% CI, 1.56-16.7; P = .007). Conclusions and Relevance: Patients with high suPAR levels were more likely to have progression of their kidney disease. Further studies should determine whether suPAR levels can identify children at risk for future CKD.

Infants Requiring Maintenance Dialysis: Outcomes of Hemodialysis and Peritoneal Dialysis.

BACKGROUND: The impact of different dialysis modalities on clinical outcomes has not been explored in young infants with chronic kidney failure. STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: Data were extracted from the ESPN/ERA-EDTA Registry. This analysis included 1,063 infants 12 months or younger who initiated dialysis therapy in 1991 to 2013. FACTOR: Type of dialysis modality. OUTCOMES & MEASUREMENTS: Differences between infants treated with peritoneal dialysis (PD) or hemodialysis (HD) in patient survival, technique survival, and access to kidney transplantation were examined using Cox regression analysis while adjusting for age at dialysis therapy initiation, sex, underlying kidney disease, and country of residence. RESULTS: 917 infants initiated dialysis therapy on PD, and 146, on HD. Median age at dialysis therapy initiation was 4.5 (IQR, 0.7-7.9) months, and median body weight was 5.7 (IQR, 3.7-7.5) kg. Although the groups were homogeneous regarding age and sex, infants treated with PD more often had congenital anomalies of the kidney and urinary tract (CAKUT; 48% vs 27%), whereas those on HD therapy more frequently had metabolic disorders (12% vs 4%). Risk factors for death were younger age at dialysis therapy initiation (HR per each 1-month later initiation, 0.95; 95% CI, 0.90-0.97) and non-CAKUT cause of chronic kidney failure (HR, 1.49; 95% CI, 1.08-2.04). Mortality risk and likelihood of transplantation were equal in PD and HD patients, whereas HD patients had a higher risk for changing dialysis treatment (adjusted HR, 1.64; 95% CI, 1.17-2.31). LIMITATIONS: Inability to control for unmeasured confounders not included in the Registry database and missing data (ie, comorbid conditions). Low statistical power because of relatively small number of participants. CONCLUSIONS: Despite a widespread preconception that HD should be reserved for cases in which PD is not feasible, in Europe, we found 1 in 8 infants in need of maintenance dialysis to be initiated on HD therapy. Patient characteristics at dialysis therapy initiation, prospective survival, and time to transplantation were very similar for infants initiated on PD or HD therapy.

Pleuro-peritoneal or pericardio-peritoneal leak in children on chronic peritoneal dialysis-A survey from the European Paediatric Dialysis Working Group.

BACKGROUND: Pleural or pericardial effusions secondary to pleuro-peritoneal fistula (PPF) and pericardio-peritoneal fistula (PcPF) are rare but serious complications of peritoneal dialysis (PD). METHODS: We conducted a 10-year survey across all participating centres in the European Paediatric Dialysis Working Group to review the incidence, diagnostic techniques, therapeutic options and outcome of children on chronic PD with PPF and/or PcPF. RESULTS: Of 1506 children on PD there were ten cases (8 of PPF, 1 each of PcPF and PPF + PcPF), with a prevalence of 0.66%. The median age at presentation was 1.5 [inter-quartile range (IQR) 0.4-2.4] years, and nine children were <3 years. The time on PD before onset of symptoms was 4.3 (IQR 1.3-19.8) months. Eight children had herniae and seven had abdominal surgery in the preceding 4 weeks. Symptoms at presentation were respiratory distress, reduced ultrafiltration and tachycardia. PD was stopped in all children; three were managed conservatively and thoracocentesis was performed in seven (with pleurodesis in 3). PD was restarted in only three children, in two of them with success. CONCLUSION: In conclusion, PPF and PcPF are rare in children on chronic PD, but are associated with significant morbidity, requiring a change of dialysis modality in all cases. Risk factors for PPF development include age of <3 years, herniae and recent abdominal surgery.

Survival and clinical outcomes of children starting renal replacement therapy in the neonatal period.

End-stage renal disease requiring renal replacement therapy (RRT) during the neonatal period is a very rare condition, and little information is available regarding long-term RRT and outcomes. To gain more information, we performed a collaborative study on patient characteristics and treatment outcomes in children who started RRT as neonates during their first month of life between 2000 and 2011 who were prospectively registered in the ESPN/ERA-EDTA, the IPPN (since 2007), the Japanese registry, or the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. During the first month of life, 264 patients from 32 countries started RRT and were followed for a median of 29 months (interquartile range 11-60 months). Most neonates (242) started on peritoneal dialysis, 21 started on hemodialysis, and 1 patient with a transplant. The most important causes of renal failure were congenital anomalies of the kidney and urinary tract in 141, cystic kidneys in 35, and cortical necrosis in 30. Within 2 years after the start of RRT, 69 children changed dialysis modality and 53 received a renal transplant. After a median of 7 months, 45 children had died, mainly because of infection, resulting in an estimated 2-year survival of 81%, and 5-year survival of 76%. Growth retardation (63%), anemia (55%), and hypertension (57%) were still major problems after 2 years. Thus, relatively good medium-term patient survival may be achieved with RRT started during the neonatal period, but specific therapeutic challenges continue to exist in this age group.

Genotype-phenotype associations in WT1 glomerulopathy.

WT1 mutations cause a wide spectrum of renal and extrarenal manifestations. Here we evaluated disease prevalence, phenotype spectrum, and genotype-phenotype correlations of 61 patients with WT1-related steroid-resistant nephrotic syndrome relative to 700 WT1-negative patients, all with steroid-resistant nephrotic syndrome. WT1 patients more frequently presented with chronic kidney disease and hypertension at diagnosis and exhibited more rapid disease progression. Focal segmental glomerulosclerosis was equally prevalent in both cohorts, but diffuse mesangial sclerosis was largely specific for WT1 disease and was present in 34% of cases. Sex reversal and/or urogenital abnormalities (52%), Wilms tumor (38%), and gonadoblastoma (5%) were almost exclusive to WT1 disease. Missense substitutions affecting DNA-binding residues were associated with diffuse mesangial sclerosis (74%), early steroid-resistant nephrotic syndrome onset, and rapid progression to ESRD. Truncating mutations conferred the highest Wilms tumor risk (78%) but typically late-onset steroid-resistant nephrotic syndrome. Intronic (KTS) mutations were most likely to present as isolated steroid-resistant nephrotic syndrome (37%) with a median onset at an age of 4.5 years, focal segmental glomerulosclerosis on biopsy, and slow progression (median ESRD age 13.6 years). Thus, there is a wide range of expressivity, solid genotype-phenotype associations, and a high risk and significance of extrarenal complications in WT1-associated nephropathy. We suggest that all children with steroid-resistant nephrotic syndrome undergo WT1 gene screening.

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.

[Program of early nephro-urologic management of children with congenital hydronephrosis]. / Zalozenia programu wczesnej opieki nefro-urologicznej nad dziecmi z wrodzonym wodonerczem.

Congenital anomalies of kidney and urinary tract (CAKUT) are the main cause of end stage renal disease in childhood. Early prenatal detection with planned postnatal diagnosis and therapy are the mainstay of management of neonates with CAKUT which is aimed at the conservation of renal tissue. The above assumptions led to the establishment of the Pommeranian Program for Management of Children with CAKUT. The strategy of the program is to coordinate prenatal diagnosis performed by obstetricians, postnatal care by neonatologists and early management by pediatric nephrologists and urologists. It will involve approximately 200 neonates annually. The basic concept of the program includes the following: 1. Delivery of a child with congenital hydronephrosis detected prenatal should take place in a center with specialist neonatal care. 2. Child with a congenital hydronephrosis should remain under specialist nephro-urologic care immediately after delivery. 3. Child with a congenital hydronephrosis should be qualified to scheduled urologic surgery after results of diagnostic tests and according to general status. 4. Model of integrated care on a child with congenital hydronephrosis should consist in close cooperation between obstetricians, neonatologists, pediatric nephrologists, and urologists.

[Preliminary results of a program for the early management of children with congenital hydronephrosis]. / Wstepne wyniki programu wczesnej opieki nefro-urologicznej nad dziecmi z wrodzonym wodonerczem.

UNLABELLED: Antenatal hydronephrosis is one of the most frequently diagnosed congenital abnormalities in the fetus. The aim of the study is to present the preliminary results of a newly launched Pommeranian Program for Early Management of CAKUT in Children. MATERIAL AND METHODS: 105 neonates and infants with hydronephrosis were assessed between Jan and Dec 2007 (27 girls and 78 boys). All patients had postnatal ultrasound performed. Static and dynamic scyntigraphy and cystography were performed according to set indications. Therapeutic decisions were made following a team meeting between pediatric nephrologists and urologists. RESULTS: 56.2% of children with hydronephrosis had documented prenatal findings: 61%--pyelectasis, 15.3% renal cysts, 5.1% unspecified renal pathology, in 18.6% no renal pathology. 82.6% of newborns had term births but perinatal complications were observed in 30.5%. Initial postnatal diagnosis was performed by neonatal wards in 22.8% newborns and by the children's pediatrician in 31.4% of infants. Following referral to nephro-urology centre cystography was performed in 22.9% of infants and scintigraphy in 36.2%. 21.9% of infants were qualified for surgical intervention at an average age of 24 + 13 weeks. CONCLUSIONS: 1. Only half of the children with congenital hydronephrosis are to nephro-urology care following prenatal diagnosis. 2. A large number of perinatal complications are observed in newborns with prenatally diagnosed hydronephrosis. 3. The management of newborn hydronephrosis requires better cooperation between obstetricians, neonatologists, pediatric nephrologists and urologists.

[Program of early diagnosis of chronic renal disease in children--SopKard 15 nephrological project]. / Zalozenia programu wczesnego wykrywania przewleklej choroby nerek u mlodziezy--SopKard 15 modul nefrologiczny.

The Sopot Program of Cardiac Infarct and Stroke Prevention--SopKard 1999-2009 was established for health promotion to decrease cardio-vascular diseases mortality. To serve these purposes a new project SopKard 15 for adolescents was created. The main aim of SopKard 15 is evaluation of health status with particular attention to risk factors of civilization diseases, with chronic kidney disease (CKD) among them. In population of 14-year-old students a complex nephrological examination including medical history, blood pressure measurements, ultrasound examination, and laboratory tests with e.g. albuminuria, urinalysis, and serum creatinine and cystatin C levels was performed. Nephrology part of Program SopKard 15 aspired to early detection of CKD and verifies normal value for this age group.

[Pre-emptive renal transplantation in a boy with obstructive uropathy following reconstruction procedures]. / Przeszczep nerki u pacjenta z zastawka cewki tylnej (ZCT) po zabiegach plastyki pecherza moczowego.

Patients with bladder dysfunction comprise over 30% of pediatric patients on renal replacement therapy. We report on a successful cadaveric pre-emptive renal transplantation performed in a boy born with posterior urethral valve. Following bilateral ureterocutaneostomies, left nephrectomy and valve resection, at 6 years of age a continent ileocolonocystoplasty was performed. The boy started intermittent daytime catheterization, passing urine both via urethra and fistula. At the age of 18 he received a renal transplant. Continuing the previous regime, at 1.5 years follow up his graft is well functioning (GFR >75 ml/min/1.73 m2) with sporadic episodes of urinary tract infection.

[Kidney transplantation into a neurogenic bladder in myelomeningocele patient (MMC)]. / Przeszczepienie nerki do pecherza neurogennego u pacjenta z przepuklina oponowo-rdzeniowa.

UNLABELLED: We report a 24-year-old patient with neurogenic bladder due to myelomeningocele (MMC) who received a kidney transplant without prior bladder reconstruction. Following transplantation recurrent episodes of febrile pyelonephritis were observed with elevations of creatinine. A year after Tx a bladder augmentation was performed with appendicostomy to enable intermittent catheterization. Following surgery only sporadic episodes of UTI have been observed and his renal function is stable. CONCLUSION: bladder reconstruction surgery in patients with neurogenic bladder is feasible post transplantation though the optimal timing is prior to a kidney Tx.

[Estimation of GFR from serum creatinine and cystatin C-A comparison of 2 methods in patients with neurogenic bladder due to myelomeningocle (MMC)]. / Szacowanie GFR na podstawie wartosci kreatyniny i cystatyny C--porównanie dwóch metod w grupie pacjentów z pecherzem neurogennym w przebiegu przepukliny oponowo-rdzeniowej (MMC).

UNLABELLED: Glomerular filtration rate (eGFR) calculated from serum creatinine can be overestimated in patients with muscle mass deficits. AIM: The aim of this study was to compare eGFR calculated from serum levels of creatinine and cystatin C in a group of patients with neurogenic bladder due to MMC. MATERIAL AND METHODS: GFR calculations were performed for 67 patients using Schwartz formula for creatinine measured by colorimetric method and Filler formula for cystatin C measured by immunonephelometric method. RESULTS: Statistically significant lower eGFR values were obtained with calculations based on cystatin C levels. CONCLUSION: Cystatin C is a useful marker for GFR estimations in patients with reduced muscle mass.

[Acute renal failure in paediatric oncological disease]. / Ostra niewydolnosc nerek w chorobie nowotworowej u dzieci.

INTRODUCTION: Acute renal failure (ARF) in children with malignancies is a rare clinical situation, but nonetheless it is a serious life threatening condition. It may arise from different clinical situations and may be caused by various factors. The aim of the study was to determine the frequency, aetiology and the course of ARF in children treated for malignancies in the Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk. MATERIAL AND METHODS: A group of 586 pediatric oncology patients treated between 1992 and 2004 were enrolled in a retrospective study. RESULTS: ARF was diagnosed in 29 cases including: 12 patients with prerenal course of ARF (11 due to septic shock and 1 due to dehydration), 16 patients with intrinsic renal aetiology of ARF (as a complication after cisplatinum and carboplatinum therapy in 2 children, in 2 cases after methotrexate, as a consequence of bilateral nephrectomy due to nephroblastoma in 1 patient and in 11 children with tumour lysis syndrome, including 5 patients with neoplasmatic infiltration of kidneys) and postrenal ARF in 1 patient as a first symptom of a tumour located in the small pelvis (Rhabdomyosarcoma). Renal replacement therapy (dialysis) was necessary in 11 children. Among 29 analysed children, in 20 cases renal failure was reversible. Due to appropriate treatment, ARF in course of tumour lysis syndrome is nowadays reversible. ARF due to septic shock or cytostatics nephrotoxicity is a significant therapeutic problem. In most of the cases it is irreversible. CONCLUSIONS: 1. ARF in these studies occurred in 29 out of 586 children with malignancies (4.9%). 2. Prerenal and renal ARF were the most frequent forms. 3. Implementation of tumour lysis prophylaxis in the treatment of children with blood system proliferative diseases reduces the incidence of ARF. 4. In cases of ARF in children's malignancies close cooperation between paediatric oncologist and nephrologist is necessary.

Prevalence of mutations in renal developmental genes in children with renal hypodysplasia: results of the ESCAPE study.

Renal hypodysplasia (RHD) is characterized by a reduced nephron number, small kidney size, and disorganized renal tissue. A hereditary basis has been established for a subset of affected patients, suggesting a major role of developmental genes that are involved in early kidney organogenesis. Gene mutations that have dominant inheritance and cause RHD, urinary tract anomalies, and defined extrarenal symptoms have been identified in TCF2 (renal cysts and diabetes syndrome), PAX2 (renal-coloboma syndrome), EYA1 and SIX1 (branchio-oto-renal syndrome), and SALL1 (Townes-Brocks syndrome). For estimation of the prevalence of these events, an unselected cohort of 99 unrelated patients with RHD that was associated with chronic renal insufficiency were screened for mutations in TCF2, PAX2, EYA1, SIX1, and SALL1. Mutations or variants in the genes of interest were detected in 17 (17%) unrelated families: One mutation, two variants, and four deletions of TCF2 in eight unrelated patients; four different PAX2 mutations in six families; one EYA1 mutation and one deletion in two patients with branchio-oto-renal syndrome; and one SALL1 mutation in a patient with isolated RHD. Of a total of 27 patients with renal cysts, six (22%) carried a mutation in TCF2. It is interesting that a SIX1 sequence variant was identified in two siblings with renal-coloboma syndrome as a result of a PAX2 mutation, suggesting an oligogenic inheritance. Careful clinical reevaluation that focused on discrete extrarenal symptoms and thorough family analysis revealed syndrome-specific features in nine of the 17 patients. In conclusion, 15% of patients with RHD show mutations in TCF2 or PAX2, whereas abnormalities in EYA1, SALL1, and SIX1 are less frequent.