Depressive disorders in children with chronic kidney disease treated conservatively.

BACKGROUND: Children with chronic kidney disease (CKD) experience a lot of mental and emotional stress, which can lead to the development of depressive disorders. The prevalence of depressive disorders in CKD children is estimated to be between 7% and 35%. OBJECTIVES: The aim of our study was to analyze the prevalence and characteristics of depression and depressive symptoms in children and adolescents with CKD treated conservatively. MATERIAL AND METHODS: The cross-sectional, multicenter study was conducted in 73 CKD children aged 8-18 and in 92 of their parents. To assess the mental wellbeing of CKD children, Kovacs's Children's Depression Inventory 2 (CDI2) was used as CDI2: Self-Report and CDI2: Parent Form. RESULTS: The majority of CKD children acquired medium scores in CDI2, 11% of participants reported symptoms suggesting depressive disorder, and among them 8.2% met the criteria for depression. A significant relationship was found between age and interpersonal problems, age at CKD diagnosis, and total score and ineffectiveness, CKD duration and total score/emotional problems. Depressive symptoms were associated with the stage of CKD, and they differed significantly between stages III and IV. We noticed the child-parent disagreement on reported depressive symptoms. Parents perceive their children's mental state as worse than the children themselves. CONCLUSIONS: There is a problem of depression in children with CKD treated conservatively. Variables associated with depressive symptoms in CKD children treated conservatively require further study. Key factors predisposing to the development of depression seem to be age at the time of diagnosis, disease duration, and progression of CKD from stage III to IV. Disparities between depressive symptoms self-reported by CKD children and their parents' assessment require further analysis. However, these disparaties indicate that the final diagnosis of the occurrence of depressive disorders should be based on a multidimensional assessment of the patient's situation.

Clinical Phenotype and Management of Severe Neurotoxicity Observed in Patients with Neuroblastoma Treated with Dinutuximab Beta in Clinical Trials.

Neurotoxicity is an off-tumour, on-target side effect of GD2-directed immunotherapy with monoclonal antibodies. Here, we report the frequency, management and outcome of patients enrolled in two prospective clinical trials who experienced severe neurotoxicity during immunotherapy with the anti-GD2 antibody dinutuximab beta (DB) administered as short-term infusion (HR-NBL1/SIOPEN study, randomisation R2, EudraCT 2006-001489-17) or as long-term infusion (HR-NBL1/SIOPEN study, randomisation R4, EudraCT 2006-001489-17 and LTI/SIOPEN study, EudraCT 2009-018077-31), either alone or with subcutaneous interleukin-2 (scIL-2). The total number of patients included in this analysis was 1102. Overall, 44/1102 patients (4.0%) experienced Grade 3/4 neurotoxicities (HR-NBL1 R2, 21/406; HR-NBL1 R4, 8/408; LTI study, 15/288), including 27 patients with severe neurotoxicities (2.5%). Events occurred predominantly in patients receiving combined treatment with DB and scIL-2. Neurotoxicity was treated using dexamethasone, prednisolone, intravenous immunoglobulins and, in two patients, plasmapheresis, which was highly effective. While neurological recovery was observed in 16 of 21 patients with severe neurotoxicities, 5/1102 (0.45%) patients experienced persistent and severe neurological deficits. In conclusion, severe neurotoxicity is most commonly observed in patients receiving DB with scIL-2. Considering the lack of clinical benefit for IL-2 in clinical trials so far, the administration of IL-2 alongside DB is not recommended.

Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD.

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450-1098) ml/m in Null/null, 403 (260-538) ml/m in Null/mis, 230 (169-357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150-267) ml/m in CKD stage 1, 472 (266-880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies.

Genome-Wide Survey for Microdeletions or -Duplications in 155 Patients with Lower Urinary Tract Obstructions (LUTO).

Lower urinary tract obstruction (LUTO) is, in most cases, caused by anatomical blockage of the bladder outlet. The most common form are posterior urethral valves (PUVs), a male-limited phenotype. Here, we surveyed the genome of 155 LUTO patients to identify disease-causing CNVs. Raw intensity data were collected for CNVs detected in LUTO patients and 4.392 healthy controls using CNVPartition, QuantiSNP and PennCNV. Overlapping CNVs between patients and controls were discarded. Additional filtering implicated CNV frequency in the database of genomic variants, gene content and final visual inspection detecting 37 ultra-rare CNVs. After, prioritization qPCR analysis confirmed 3 microduplications, all detected in PUV patients. One microduplication (5q23.2) occurred de novo in the two remaining microduplications found on chromosome 1p36.21 and 10q23.31. Parental DNA was not available for segregation analysis. All three duplications comprised 11 coding genes: four human specific lncRNA and one microRNA. Three coding genes (FBLIM1, SLC16A12, SNCAIP) and the microRNA MIR107 have previously been shown to be expressed in the developing urinary tract of mouse embryos. We propose that duplications, rare or de novo, contribute to PUV formation, a male-limited phenotype.

Copy Number Variant Analysis and Genome-wide Association Study Identify Loci with Large Effect for Vesicoureteral Reflux.

BACKGROUND: Vesicoureteral reflux (VUR) is a common, familial genitourinary disorder, and a major cause of pediatric urinary tract infection (UTI) and kidney failure. The genetic basis of VUR is not well understood. METHODS: A diagnostic analysis sought rare, pathogenic copy number variant (CNV) disorders among 1737 patients with VUR. A GWAS was performed in 1395 patients and 5366 controls, of European ancestry. RESULTS: Altogether, 3% of VUR patients harbored an undiagnosed rare CNV disorder, such as the 1q21.1, 16p11.2, 22q11.21, and triple X syndromes ((OR, 3.12; 95% CI, 2.10 to 4.54; P=6.35×10-8) The GWAS identified three study-wide significant and five suggestive loci with large effects (ORs, 1.41-6.9), containing canonical developmental genes expressed in the developing urinary tract (WDPCP, OTX1, BMP5, VANGL1, and WNT5A). In particular, 3.3% of VUR patients were homozygous for an intronic variant in WDPCP (rs13013890; OR, 3.65; 95% CI, 2.39 to 5.56; P=1.86×10-9). This locus was associated with multiple genitourinary phenotypes in the UK Biobank and eMERGE studies. Analysis of Wnt5a mutant mice confirmed the role of Wnt5a signaling in bladder and ureteric morphogenesis. CONCLUSIONS: These data demonstrate the genetic heterogeneity of VUR. Altogether, 6% of patients with VUR harbored a rare CNV or a common variant genotype conferring an OR >3. Identification of these genetic risk factors has multiple implications for clinical care and for analysis of outcomes in VUR.

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.

eGFR values and selected renal urine biomarkers in preterm neonates with uncomplicated clinical course.

BACKGROUND: Diagnosing acute kidney injury (AKI) in preterm newborns, who are particularly susceptible to renal damage, is a serious challenge as there is no definite consensus about the diagnostic criteria. OBJECTIVES: The objective of this study was to measure the values for selected urinary biomarkers and estimated glomerular filtration rate (eGFR) among a population of preterm infants with uncomplicated clinical course as well as to determine whether these markers depend on birth weight (BW), gestational age (GA), postnatal age (PNA), or gender. MATERIAL AND METHODS: The prospective study was carried out in neonatal intensive care unit (NICU). The evaluation included 57 children that were divided into 3 categories according to BW: low birth weight (LBW) - 1501-2500 g (22 infants); very low birth weight (VLBW) - 1000-1500 g (25 infants); and extremely low birth weight (ELBW) - 750-999 g (10 infants). Urine samples were collected daily between the 4th and 28th day of life for measurements of creatinine (Cr), neutrophil gelatinase-associated lipocalin (NGAL), osteopontin (OPN), and human kidney injury molecule 1 (hKIM1). RESULTS: The values of the 3 urine tubular biomarkers, serum creatinine and eGFR were taken in substantially healthy preterm infants with normal kidney function at 4 time intervals during the neonatal period. Their correlations were determined and a multivariable regression analysis was carried out with respect to BW, GA, PNA, and gender. Trends of the studied markers in terms of PNA and BW were also assessed with the Jonckheere-Terpstra test. CONCLUSIONS: Glomerular and tubular function in preterm neonates during the 1st month of life is significantly influenced by BW, GA, PNA, and gender.

Author Correction: The copy number variation landscape of congenital anomalies of the kidney and urinary tract.

In the version of this article initially published, affiliation 38 incorrectly read "ICNU-Nephrology and Urology Department, Barcelona, Spain"; "Renal Division, Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Spain" is the correct affiliation. The error has been corrected in the HTML and PDF versions of the article.

Factors affecting mortality in children requiring continuous renal replacement therapy in pediatric intensive care unit.

BACKGROUND: Acute kidney injury (AKI) occurs in up to 30% of pediatric intensive care unit (PICU) patients and is associated with a high mortality rate. OBJECTIVES: The objective of the study was to evaluate factors associated with the outcome and to identify the prognostic factors in children receiving continuous renal replacement therapy (CRRT). MATERIAL AND METHODS: This was a retrospective, single-center study, including 46 patients. RESULTS: Logistic regression analysis demonstrated significant effects on patient survival exerted by the percentage of fluid overload (FO%) (odds ratio (OR): 1.030; p = 0.044). In the group of patients with FO% < 25%, the mortality was 33.3%, and in the FO% ≥ 25% group, the mortality was 67.9% (p < 0.001). The probability of death without multi-organ failure (MOF) was 13%, while with MOF it was 74%. There was no difference in the duration of hospitalization between the CRRT patients (mean: 21.9 days) and the general population of children hospitalized in PICU in the same period (n = 3,255; mean: 25.4 days); however, a significant difference was noted in mortality between the 2 groups of patients (54% vs 6.5%; p < 0.001). CONCLUSIONS: The mortality of PICU CRRT patients is more than 8-fold higher than the mortality of the total PICU population. Coexisting MOF increases the mortality almost 6 times. The mortality of children with FO% ≥ 25% was more than 2-fold higher than the mortality of children with FO% < 25%.

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.

The copy number variation landscape of congenital anomalies of the kidney and urinary tract.

Congenital anomalies of the kidney and urinary tract (CAKUT) are a major cause of pediatric kidney failure. We performed a genome-wide analysis of copy number variants (CNVs) in 2,824 cases and 21,498 controls. Affected individuals carried a significant burden of rare exonic (that is, affecting coding regions) CNVs and were enriched for known genomic disorders (GD). Kidney anomaly (KA) cases were most enriched for exonic CNVs, encompassing GD-CNVs and novel deletions; obstructive uropathy (OU) had a lower CNV burden and an intermediate prevalence of GD-CNVs; and vesicoureteral reflux (VUR) had the fewest GD-CNVs but was enriched for novel exonic CNVs, particularly duplications. Six loci (1q21, 4p16.1-p16.3, 16p11.2, 16p13.11, 17q12 and 22q11.2) accounted for 65% of patients with GD-CNVs. Deletions at 17q12, 4p16.1-p16.3 and 22q11.2 were specific for KA; the 16p11.2 locus showed extensive pleiotropy. Using a multidisciplinary approach, we identified TBX6 as a driver for the CAKUT subphenotypes in the 16p11.2 microdeletion syndrome.

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

OBJECTIVE: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. STUDY DESIGN: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. RESULTS: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. CONCLUSIONS: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

[B-type natriuretic peptide as a marker of cardiac dysfunction in children with chronic kidney disease]. / Peptyd natriuretyczny typu B jako marker uszkodzenia serca u dzieci z przewlekla choroba nerek.

Left ventricular hypertrophy is the most common organ damage in children with chronic kidney disease (CKD). AIM: The aim of the study was to assess the usefulness of B-type natriuretic peptide (BNP) as a marker of heart injury in children with CKD. MATERIALS AND METHODS: We included 66 children (41 boys and 25 girls) aged 0.7 to 18.6 (median 11.6) years with CKD stage 1-5. The concentrations of urea, creatinine, cystatin C and BNP in blood serum were assessed, and the estimated glomerular filtration rate (eGFR) was calculated from the Schwartz and Filler formulas. Patients were divided into groups depending on the CKD stage [group 1: CKD stages 1 + 2 (GFR> 60 ml/min/1.73 m2), group 2: stage 3 (GFR = 30-59 ml/min/1.73 m2), group 3: CKD stage 4 (GFR 15-29 ml/min/ 1.73 m2), group 4 - stage 5 (dialyzed children)]. On the basis of echocardiography, the left ventricular mass (LVM) was calculated, which was indexed for height (left ventricular mass index, LVMI). Left ventricular hypertrophy (LVH) was diagnosed if the LVMI value was > 95th percentile for sex and age. RESULTS: Depending on the CKD stage the median BNP concentrations for group 1, group 2, group 3, and group 4 were 2.5 pg/ml, 6.0 pg/ml, 9.3 pg/ml and 18.0 pg/ml, and the LVH prevalence 27.3%, 33.3%, 60.0% and 63.6% , respectively. Significant correlations between BNP concentration and LVH expressed by LVMI (R=0.256, p=0.038), creatinine (R=0.453, p<0.001), cystatin (R=0.494, p<0.001) and eGFR (R=-0.473, p<0.001) were found. CONCLUSIONS: In children with chronic kidney disease, BNP is an indicator of heart failure correlating with renal function parameters and left ventricular mass index.

Clinical and molecular genetic characterization of a male patient with Sensenbrenner syndrome (cranioectodermal dysplasia) and biallelic WDR35 mutations.

BACKGROUND: Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy first described by Judith Sensenbrenner in 1975. CED is a complex disorder characterized by craniofacial, skeletal, and ectodermal abnormalities. The clinical symptoms are variable and the CED phenotype may present intrafamilial and interfamilial differences. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disease. Mutations in six genes: IFT122, WDR35, IFT43, WDR19, IFT52, and IFT140 have been associated with this disorder. All known CED genes encode proteins that are part of the intraflagellar transport complex, which plays an important role in the assembly and maintenance of cilia. CASE: We report a on 2-year-old male patient affected by Sensenbrenner syndrome. Dysmorphic features included short stature with rhizomelic shortening of limbs, short fingers, narrow chest, high forehead, epicanthal folds, telecanthus, broad nasal bridge, low-set ears, sparse hair, and widely space teeth. Craniosynostosis was surgically corrected at the age of 4 months. The patient presented chronic renal disease. Nephrologic picture showed early stages of nephronophthisis. Psychomotor development was apparently normal. Molecular analysis of the affected individual revealed compound heterozygosity for a novel nonsense p.(Arg113*) and a missense p.(Asp841Val) variant in the WDR35 gene. CONCLUSIONS: The observations of the CED patient in this study provide additional clinical data and expand the molecular spectrum of Sensenbrenner syndrome. Moreover, the two variants identified in the proband provide further evidence for the WDR35 mutations as the most common cause of this rare syndrome.

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.

Low renal but high extrarenal phenotype variability in Schimke immuno-osseous dysplasia.

Schimke immuno-osseous dysplasia (SIOD) is a rare multisystem disorder with early mortality and steroid-resistant nephrotic syndrome (SRNS) progressing to end-stage kidney disease. We hypothesized that next-generation gene panel sequencing may unsurface oligosymptomatic cases of SIOD with potentially milder disease courses. We analyzed the renal and extrarenal phenotypic spectrum and genotype-phenotype associations in 34 patients from 28 families, the largest SMARCAL1-associated nephropathy cohort to date. In 11 patients the diagnosis was made unsuspectedly through SRNS gene panel testing. Renal disease first manifested at median age 4.5 yrs, with focal segmental glmerulosclerosis or minimal change nephropathy on biopsy and rapid progression to end-stage kidney disease (ESKD) at median age 8.7 yrs. Whereas patients diagnosed by phenotype more frequently developed severe extrarenal complications (cerebral ischemic events, septicemia) and were more likely to die before age 10 years than patients identified by SRNS-gene panel screening (88 vs. 40%), the subgroups did not differ with respect to age at proteinuria onset and progression to ESKD. Also, 10 of 11 children diagnosed unsuspectedly by Next Generation Sequencing were small at diagnosis and all showed progressive growth failure. Severe phenotypes were usually associated with biallelic truncating mutations and milder phenotypes with biallelic missense mutations. However, no genotype-phenotype correlation was observed for the renal disease course. In conclusion, while short stature is a reliable clue to SIOD in children with SRNS, other systemic features are highly variable. Our findings support routine SMARCAL1 testing also in non-syndromic SRNS.

Filter Size Not the Anticoagulation Method is the Decisive Factor in Continuous Renal Replacement Therapy Circuit Survival.

BACKGROUND/AIM: As continuous renal replacement therapy (CRRT) has emerged as a standard therapy in pediatric intensive care units (PICU), many related issues that may have an impact on circuit survival have gained in importance. Objective of the study was an evaluation of factors associated with circuit survival, including anticoagulation (ACG). METHODS: Retrospective study that included 40 patients, who in total received 7636 hours of CRRT during 150 sessions (84 filters, 4260 hours with heparin anticoagulation (Hep-ACG); 66 filters, 3376 hours with regional citrate anticoagulation (RCA)). RESULTS: The Kaplan-Meier analysis of the total circuit survival time depending on the type of ACG did not demonstrate a significant difference between Hep-ACG and RCA. The percentage of clotted filters was significantly higher in case of smaller filters (HF20: 58.8%; ST60: 29.5%; ST100: 15.8%), and their lifetime was significantly lower regardless of ACG (the mean and median lifetime for HF20: 38.7/27.0 h; for ST60: 54.1/72.0 h., for ST100: 62.1/72.0 h, respectively). CONCLUSIONS: Irrespectively of filter size, filter clotting occurs within the first 24 hours after the initiation of CRRT. Most commonly, clotting affects small filters, and their lifetime is significantly shorter as compared to larger filters regardless of the type of the ACG.

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.

Reference ranges and impact of selected confounders on classic serum and urinary renal markers in neonatal period.

PURPOSE: Renal injury in term and pre-term neonates may be an antecedent to chronic kidney disease in the child's further life. The aim of the paper was to compile a reference range for selected serum and urinary renal markers in the neonatal period for full-term (FT) and pre-term (PT) newborns. MATERIAL AND METHODS: The prospective study included 23 FT infants (birth weight - BW≥2500g and gestational age - GA≥37Hbd) and 32 PT children (BW<2500g and GA<37Hbd) in good general condition, without acute kidney injury (AKI) or sepsis. Between the 4th and 28th DOL, urinary concentrations of the studied renal markers (uCr, uNa, uOsm) were determined on a daily basis, while serum creatinine (SCr) was assessed minimum every 48-72h. RESULTS: The mean GA and BW of the FT and PT infants were respectively as follows: 38.5±1.7Hbd; 3433±495.2g and 32.7±2.6Hbd; 1836.7±419.8g. For serum glomerular (SCr, eGFR) and tubular markers (FENa, RFI), the median values with normal ranges were compiled. For urinary renal markers (uCr, uNa, uOsm) and those values standardized for kg of body weight, percentile tables for 4-28DOL were elaborated. CONCLUSIONS: The study has resulted in determining the normal ranges of serum glomerular and tubular renal markers, as well as percentile tables of selected urinary renal parameters during the neonatal period. The percentile tables may prove to be helpful for further standardization of other urinary parameters per urinary creatinine in neonatal population.