Kidney volume normative values in Central European children aged 0-19 years: a multicenter study.

BACKGROUND AND OBJECITVES: The currently available kidney volume normative values in children are restricted to small populations from single-centre studies not assessing kidney function and including none or only a small number of adolescents. This study aimed to obtain ultrasound-based kidney volume normative values derived from a large European White/Caucasian paediatric population with normal kidney function. METHODS: After recruitment of 1427 children aged 0-19 years, 1396 individuals with no history of kidney disease and normal estimated glomerular filtration rate were selected for the sonographic evaluation of kidney volume. Kidney volume was correlated with age, height, weight, body surface area and body mass index. Kidney volume curves and tables related to anthropometric parameters were generated using the LMS method. Kidney volume predictors were evaluated using multivariate regression analysis with collinearity checks. RESULTS: No clinically significant differences in kidney volume in relation to height were found between males and females, between supine and prone position and between left and right kidneys. Males had, however, larger age-related kidney volumes than females in most age categories. For the prediction of kidney volume, the highest coefficient correlation was observed for body surface area (r = 0.94), followed by weight (r = 0.92), height (r = 0.91), age (r = 0.91), and body mass index (r = 0.67; p < 0.001 for all). CONCLUSIONS: This study presents LMS-percentile curves and tables for kidney volume which can be used as reference values for children aged 0-19 years.

Are the Classical Symptoms of Hypertension in Children Still Sensitive Enough?

The available literature is scarce on the initial symptoms of arterial hypertension in children. Our study aimed to analyze the initial clinical profile of patients referred to the hospital with suspected hypertension and those diagnosed with hypertension for the first time during a hospitalization for other reasons. This study was a retrospective analysis of medical records in 471 patients. More than half of the patients showed no symptoms. The most common symptom reported was a headache-28% (132) of patients. The diagnosis of elevated blood pressure or hypertension was more frequent in asymptomatic patients (P = 0.001). Headaches were seen more often in healthy patients than in patients with hypertension. Newly diagnosed hypertension is mainly diagnosed in asymptomatic children. Moreover, the symptoms previously described in the literature as the most common did not prove to be predictive of hypertension in our study.

A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants.

While 44-83% of children with steroid-resistant nephrotic syndrome (SRNS) without a proven genetic cause respond to treatment with a calcineurin inhibitor (CNI), current guidelines recommend against the use of immunosuppression in monogenic SRNS. This is despite existing evidence suggesting that remission with CNI treatment is possible and can improve prognosis in some cases of monogenic SRNS. Herein, our retrospective study assessed response frequency, predictors of response and kidney function outcomes among children with monogenic SRNS treated with a CNI for at least three months. Data from 203 cases (age 0-18 years) were collected from 37 pediatric nephrology centers. Variant pathogenicity was reviewed by a geneticist, and 122 patients with a pathogenic and 19 with a possible pathogenic genotype were included in the analysis. After six months of treatment and at last visit, 27.6% and 22.5% of all patients respectively, demonstrated partial or full response. Achievement of at least partial response at six months of treatment conferred a significant reduction in kidney failure risk at last follow-up compared to no response (hazard ratio [95% confidence interval] 0.25, [0.10-0.62]). Moreover, risk of kidney failure was significantly lower when only those with a follow-up longer than two years were considered (hazard ratio 0.35, [0.14-0.91]). Higher serum albumin level at CNI initiation was the only factor related to increased likelihood of significant remission at six months (odds ratio [95% confidence interval] 1.16, [1.08-1.24]). Thus, our findings justify a treatment trial with a CNI also in children with monogenic SRNS.

Kidney length normative values - new percentiles by age and body surface area in Central European children and adolescents.

BACKGROUND: Kidney size evaluation is an essential examination in pediatric nephrology. While body length/height is the best predictor of kidney length, age-based and body surface area (BSA)-based normative values may be useful in clinical practice or research. This study aimed to establish ultrasound-based kidney length lambda-mu-sigma (LMS) percentiles by age and BSA in healthy children. METHODS: In 1758 Polish and Lithuanian children (868 boys, 49%) aged 0-19 years, kidney length was measured using ultrasonography. In all participants, anthropometric measurements were taken and kidney function was evaluated based on serum creatinine concentration. Participants with chronic or kidney diseases, abnormal kidney function, or pathologies in sonographic examination were excluded from the analysis. RESULTS: Kidney length (median kidney length) increased progressively from infancy to the age of 18 years, from 60.1 to 114.2 mm in males, and from 57.3 to 105.2 mm in females. A gradual increase of kidney length (50th percentile) in relation to BSA (from 46.1 mm in infants with a BSA of 0-1.2 m2 to 118.3 mm in adolescents with a BSA of 2.6-2.8 m2) was also observed. LMS percentiles by age (stratified by sex) and BSA were determined and presented as graphs and tables of percentiles and LMS parameters by 1-year age intervals and 0.2 m2 of BSA, respectively. CONCLUSIONS: We present the first age- and BSA-based kidney length LMS normative values based on the largest pediatric cohort to date, which can be used in both clinical practice and research studies. A higher resolution version of the Graphical abstract is available as Supplementary information.

Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.

Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in genes involved in CoQ10 biosynthesis leading to multidrug-resistant nephrotic syndrome as the hallmark kidney manifestation. Promising early results have been reported anecdotally with oral CoQ10 supplementation. However, the long-term efficacy and optimal prescription remain to be established. In a global effort, we collected and analyzed information from 116 patients who received CoQ10 supplements for primary CoQ10 deficiency due to biallelic pathogenic variants in either the COQ2, COQ6 or COQ8B genes. Median duration of follow up on treatment was two years. The effect of treatment on proteinuria was assessed, and kidney survival was analyzed in 41 patients younger than 18 years with chronic kidney disease stage 1-4 at the start of treatment compared with that of an untreated cohort matched by genotype, age, kidney function, and proteinuria. CoQ10 supplementation was associated with a substantial and significant sustained reduction of proteinuria by 88% at 12 months. Complete remission of proteinuria was more frequently observed in COQ6 disease. CoQ10 supplementation led to significantly better preservation of kidney function (5-year kidney failure-free survival 62% vs. 19%) with an improvement in general condition and neurological manifestations. Side effects of treatment were uncommon and mild. Thus, our findings indicate that all patients diagnosed with primary CoQ10 deficiency should receive early and life-long CoQ10 supplementation to decelerate the progression of kidney disease and prevent further damage to other organs.

Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy.

Primary Coenzyme Q10 deficiency is a rare mitochondriopathy with a wide spectrum of organ involvement, including steroid-resistant nephrotic syndrome mainly associated with disease-causing variants in the genes COQ2, COQ6 or COQ8B. We performed a systematic literature review, PodoNet, mitoNET, and CCGKDD registries queries and an online survey, collecting comprehensive clinical and genetic data of 251 patients spanning 173 published (47 updated) and 78 new cases. Kidney disease was first diagnosed at median age 1.0, 1.2 and 9.8 years in individuals with disease-causing variants in COQ2, COQ6 and COQ8B, respectively. Isolated kidney involvement at diagnosis occurred in 34% of COQ2, 10.8% of COQ6 and 70.7% of COQ8B variant individuals. Classic infantile multiorgan involvement comprised 22% of the COQ2 variant cohort while 47% of them developed neurological symptoms at median age 2.7 years. The association of steroid-resistant nephrotic syndrome and sensorineural hearing loss was confirmed as the distinctive phenotype of COQ6 variants, with hearing impairment manifesting at average age three years. None of the patients with COQ8B variants, but 50% of patients with COQ2 and COQ6 variants progressed to kidney failure by age five. At adult age, kidney survival was equally poor (20-25%) across all disorders. A number of sequence variants, including putative local founder mutations, had divergent clinical presentations, in terms of onset age, kidney and non-kidney manifestations and kidney survival. Milder kidney phenotype was present in those with biallelic truncating variants within the COQ8B variant cohort. Thus, significant intra- and inter-familial phenotype variability was observed, suggesting both genetic and non-genetic modifiers of disease severity.

Burnout Syndrome among Pediatric Nephrologists-Report on Its Prevalence, Severity, and Predisposing Factors.

BACKGROUND AND OBJECTIVES: Burnout is an occupation-related syndrome comprising emotional exhaustion, depersonalization, and reduced feelings of work-related personal accomplishments. There are reports on burnout among adult nephrologists and general pediatricians, but little is known about burnout among pediatric nephrologists. The aim of our study was to assess the prevalence and severity of burnout syndrome among Polish pediatric nephrologists. MATERIALS AND METHODS: A 25-item study survey consisting of abbreviated Maslach Burnout Inventory and additional self-created questions about work-related factors was completed by 97 physicians affiliated with the Polish Society of Pediatric Nephrology. Women comprised 75.3%, with median time of professional experience in the study group was 15 years. RESULTS: A high level of emotional exhaustion, depersonalization, and reduced feeling of personal accomplishments were observed in 39.2%, 38.1%, and 21.6% of the participants, respectively. At least a medium level of burnout in all three dimensions were observed in 26.8% of the participants and 8.2% of them presented high three-dimensional burnout. About 41.2% of the participants stated that they would like to take part in burnout prevention and support programs. According to the study participants, excessive bureaucracy in healthcare systems, rush at work, and overtime work were the main job-related problems that could influence burnout intensity. CONCLUSIONS: Burnout is an important factor in the professional landscape of pediatric nephrology. Actions aimed at reducing the risk of occupational burnout among pediatric nephrologists should be applied, both at the personal and institutional levels.

Kidney length normative values in children aged 0-19 years - a multicenter study.

BACKGROUND: Currently used pediatric kidney length normative values are based on small single-center studies, do not include kidney function assessment, and focus mostly on newborns and infants. We aimed to develop ultrasound-based kidney length normative values derived from a large group of European Caucasian children with normal kidney function. METHODS: Out of 1,782 children aged 0-19 years, 1,758 individuals with no present or past kidney disease and normal estimated glomerular filtration rate had sonographic assessment of kidney length. The results were correlated with anthropometric parameters and estimated glomerular filtration rate. Kidney length was correlated with age, height, body surface area, and body mass index. Height-related kidney length curves and table were generated using the LMS method. Multivariate regression analysis with collinearity checks was used to evaluate kidney length predictors. RESULTS: There was no significant difference in kidney size in relation to height between boys and girls. We found significant (p < 0.001), but clinically unimportant (Cohen's D effect size = 0.04 and 0.06) differences between prone vs. supine position (mean paired difference = 0.64 mm, 95% CI = 0.49-0.77) and left vs. right kidneys (mean paired difference = 1.03 mm, 95% CI = 0.83-1.21), respectively. For kidney length prediction, the highest coefficient correlation was observed with height (adjusted R2 = 0.87, p < 0.0001). CONCLUSIONS: We present height-related LMS-percentile curves and tables of kidney length which may serve as normative values for kidney length in children from birth to 19 years of age. The most significant predictor of kidney length was statural height.

Serum intact fibroblast growth factor 23 in healthy paediatric population.

INTRODUCTION: It is believed that fibroblast growth factor 23 (FGF23) can become an early biomarker of chronic kidney disease progression. Data on FGF23 age dependency are inconsistent. We present the results of the cross-sectional study concerning FGF23 levels in healthy Polish children. MATERIAL AND METHODS: This study was conducted in 121 children aged 0-18 years. Kidney function and intact FGF23 levels in serum were assessed. Differences between age groups and according to gender were analysed. RESULTS: The difference in FGF23 between age groups and according to gender was statistically insignificant. In the youngest and the oldest group, a trend to higher FGF23 levels was observed. FGF23 level in girls tended to be higher than boys, apart from the age group between 1 and 4 years. There was a negative correlation between eGFR and FGF23 (r = -0.26, p < 0.05) - strong in girls (r = -0.38, p < 0.05), but not in boys. In each age group, we found no significant correlation between eGFR and FGF23. CONCLUSIONS: Our study supports the evidence that the FGF23 level in paediatric population is not age or sex dependent. The results can serve as a reference point under clinical conditions and for other studies on the topic.

Interfamilial clinical variability in four Polish families with cranioectodermal dysplasia and identical compound heterozygous variants in WDR35.

Cranioectodermal dysplasia (CED) is a rare autosomal recessive disorder primarily characterized by craniofacial, skeletal, and ectodermal abnormalities. CED is a chondrodysplasia, which is part of a spectrum of clinically and genetically heterogeneous diseases that result from disruptions in cilia. Pathogenic variants in genes encoding components of the ciliary transport machinery are known to cause CED. Intra- and interfamilial clinical variability has been reported in a few CED studies and the findings of this study align with these observations. Here, we report on five CED patients from four Polish families with identical compound heterozygous variants [c.1922T>G p.(Leu641Ter) and c.2522A>T; p.(Asp841Val)] in WDR35. The frequent occurrence of both identified changes in Polish CED families suggests that these variants may be founder mutations. Clinical evaluation of the CED patients revealed interfamilial clinical variability among the patients. This includes differences in skeletal and ectodermal features as well as variability in development, progression, and severity of renal and liver insufficiency. This is the first report showing significant interfamilial clinical variability in a series of CED patients from unrelated families with identical compound heterozygous variants in WDR35. Our findings strongly indicate that other genetic and non-genetic factors may modulate the progression and expression of the patients' phenotypes.

A new procedure for the determination of 21 macro- and trace elements in human fetal urine using an inductively coupled plasma mass spectrometry with dynamic reaction cell (ICP-DRC-MS) equipped with a micro-flow nebulizer.

The procedure for determination of 21 macro- and trace elements - Li, Na, Mg, Al, K, Ca, V, Cr, Mn, Fe, Co, Cu, Zn, Se, Sr, As, Cd, Sb, Ba, Pb and U - in human fetal urine by inductively coupled plasma mass spectrometry (ICP-MS) was developed and validated. The application of a micronebulizer and a dynamic reaction cell (DRC) allowed to perform a full analysis of small volumes (200 µL) of urine collected from human fetuses without the need for sample digestion with closed microwave systems. The procedure and ICP-MS instrument was thoroughly optimized in order to reliably determine both macroelements and ultra-trace concentrations of elements. The internal standard method (Ge, Rh and Tb) was applied in order to encompass signal drift and non-spectral interferences. The rules of metrology were used in order to ensure the quality of the results: (1) the procedure was validated, (2) the uncertainty of the measurement results was estimated and (3) the traceability of the measurement result was established by using the certified reference material with matching matrix (Seronorm Trace Elements Urine L-1). Also, the analyte addition method to the artificial urine was employed for additional confirmation of trueness of the procedure. The selected parameters of the procedure were as follows: (a) limits of detection - (0.00023-53 µg L-1) for U and Ca, respectively, (b) recoveries of the reference value - 81%-136% for Mn and Cd, respectively (c) linearity expressed as R - greater than 0.999, and (d) expanded relative uncertainties (k = 2) - 13%-66% for Sr and Cd, respectively. The developed and validated procedure was applied to 58 samples of urine collected from human fetuses. The samples were diluted with nitric acid and analyzed without further treatment. The procedure allowed to reliably determine both macro- and trace elements in very low volume of sample in a single analytical run.

"Apple does not fall far from the tree" - subclinical atherosclerosis in children with familial hypercholesterolemia.

BACKGROUND: Familial hypercholesterolemia (FH) increases the risk of atherosclerosis in children and adults. Atherosclerotic cardiovascular disease in young patients FH is usually subclinical but recognition of children with more pronounced changes is crucial for adjusting effective management. Aim of this research was to use ultrasonography with two-dimensional speckle tracking (2DST) and tonometry to evaluate atherosclerotic changes in patients with FH (parents and their offspring). METHODS: Applanation tonometry and carotid arteries sonography with evaluation of the intima-media complex thickness (IMCT) and application of the 2DST were performed in 20 families with FH (20 parents and 29 children). The same size control group (age and sex matched) was included. Results were compared between peers and between generations together with the correlation analysis. RESULTS: Adults with FH, in comparison with healthy peers, presented significantly more atherosclerotic plaques (9 vs. 2, p = 0.0230), had significantly thicker IMC (0.84 ± 0.19 vs. 0.56 ± 0.06 mm, p < 0.0001) and had stiffer arterial wall (for stain: 6.25 ± 2.3 vs. 8.15 ± 2.46, p = 0.0103). In children from both groups there were no atherosclerotic plaques and IMCT did not differ significantly (0.42 ± 0.07 vs. 0.39 ± 0.04, p = 0.1722). However, children with FH had significantly stiffer arterial wall according to 2DST (for strain: 9.22 ± 3.4 vs. 11.93 ± 3.11, p = 0.0057) and tonometry (for the pulse wave velocity: 4.5 ± 0.64 vs.3.96 ± 0.62, p = 0.0047). These parameters correlated with atherosclerosis surrogates in their parents (p < 0.001) but were not significantly affected by presence of presumed pathogenic gene variant. CONCLUSIONS: Children with FH presented subclinical atherosclerosis manifested as decreased arterial wall elasticity. Degree of stiffening was associated with advancement of atherosclerosis in their parents but did not present significant association with gene variants. Sonography with application of 2DST seems to be a good candidate for comprehensive evaluation of atherosclerosis in families with FH.

Comprehensive Evaluation of the Safety and Efficacy of BAFASAL® Bacteriophage Preparation for the Reduction of Salmonella in the Food Chain.

Bacteriophages are bacterial predators, which are garnering much interest nowadays vis-à-vis the global phenomenon of antimicrobial resistance. Bacteriophage preparations seem to be an alternative to antibiotics, which can be used at all levels of the food production chain. Their safety and efficacy, however, are of public concern. In this study, a detailed evaluation of BAFASAL® preparation was performed. BAFASAL® is a bacteriophage cocktail that reduces Salmonella in poultry farming. In vivo acute and sub-chronic toxicity studies on rats and tolerance study on targeted animals (chicken broiler) conducted according to GLP and OECD guidelines did not reveal any signs of toxicity, which could be associated with BAFASAL® administration. In addition, no evidences of genotoxicity were observed. The tolerance study with 100-times concentrated dose also did not show any statistically significant differences in the assessed parameters. The in vitro crop assay, mimicking normal feed storage and feed application conditions showed that BAFASAL® reduced the number of Salmonella bacteria in experimentally contaminated feed. Moreover, reductions were observed for all examined forms (liquid, powder, spray). Furthermore, the in vivo efficacy study showed that treatment with BAFASAL® significantly decreased Salmonella content in caeca of birds infected with Salmonella Enteritidis. Detailed examination of BAFASAL® in terms of safety and efficacy, adds to the body of evidence that bacteriophages are harmless to animals and effective in the struggle against bacteria.

Anti-Salmonella Potential of New Lactobacillus Strains with the Application in the Poultry Industry.

Probiotics are considered an alternative to antibiotics in the prevention and treatment of Salmonella diseases in poultry. However, to use probiotics as proposed above, it is necessary to evaluate their properties in detail and to select the most effective bacterial strains in the application targeted. In this study, probiotic properties of new Lactobacillus sp. strains were investigated and their antimicrobial activity against 125 environmental strains of Salmonella sp. was determined using the agar slab method. Furthermore, their survival in the presence of bile salts and at low pH, antibiotics susceptibility, aggregation and coaggregation ability, adherence to polystyrene and Caco-2 cells, and cytotoxicity were investigated. Each strain tested showed antagonistic activity against at least 96% of the environmental Salmonella sp. strains and thus representing a highly epidemiologically differentiated collection of poultry isolates. In addition, the probiotic properties of new Lactobacillus strains are promising. Therefore, all strains examined showed a high potential for use in poultry against salmonellosis.Probiotics are considered an alternative to antibiotics in the prevention and treatment of Salmonella diseases in poultry. However, to use probiotics as proposed above, it is necessary to evaluate their properties in detail and to select the most effective bacterial strains in the application targeted. In this study, probiotic properties of new Lactobacillus sp. strains were investigated and their antimicrobial activity against 125 environmental strains of Salmonella sp. was determined using the agar slab method. Furthermore, their survival in the presence of bile salts and at low pH, antibiotics susceptibility, aggregation and coaggregation ability, adherence to polystyrene and Caco-2 cells, and cytotoxicity were investigated. Each strain tested showed antagonistic activity against at least 96% of the environmental Salmonella sp. strains and thus representing a highly epidemiologically differentiated collection of poultry isolates. In addition, the probiotic properties of new Lactobacillus strains are promising. Therefore, all strains examined showed a high potential for use in poultry against salmonellosis.

A single-center study to evaluate the efficacy of a fetal urine peptide signature predicting postnatal renal outcome in fetuses with posterior urethral valves.

BACKGROUND: Posterior urethral valves (PUVs) account for 17% of pediatric renal failure. The management of pregnancies involving fetuses with PUV is hampered by the fact that current clinical parameters obtained from fetal ultrasound and/or fetal urine biochemistry are insufficient to predict postnatal renal function. We previously have developed a fetal urine peptide signature (12PUV) that predicted with high precision postnatal renal failure at 2 years of age in fetuses with PUV. Here, we evaluated the accuracy of this signature to predict postnatal renal outcome in fetuses with PUV in an independent single-center study. METHODS: Thirty-three women carrying fetuses with suspected PUV were included. Twenty-five fetuses received vesicoamniotic shunts during pregnancy. PUV was confirmed postnatally in 23 patients. Of those 23 fetuses, 2 were lost in follow-up. Four and 3 patients died in the pre- and perinatal periods, respectively. Follow-up renal function at 6 months of age was obtained for the remaining 14 patients. The primary outcome was early renal failure, defined by an eGFR < 60 mL/min/1.73 m2 before 6 months of age or pre- or perinatal death. RESULTS: The peptide signature predicted postnatal renal outcome in postnatally confirmed PUV fetuses with an AUC of 0.94 (95%CI 0.74-1.0) and an accuracy of 90% (95%CI 78-100). The signature predicted postnatal renal outcome for the suspected PUV cases with an AUC of 0.89 (95%CI 0.72-0.97) and an accuracy of 84% (95%CI 71-97). CONCLUSIONS: This single-center study confirms the predictive power of the previously identified 12PUV fetal urinary peptide signature.

Two-Dimensional Speckle Tracking Versus Applanation Tonometry in Evaluation of Subclinical Atherosclerosis in Children with Type 1 Diabetes Mellitus.

BACKGROUND Patients with type 1 diabetes mellitus (T1DM) often develop atherosclerosis at an early age. In the subclinical stage of the process, minimal/non-morphological changes can be noticed, but the arterial wall function can be impaired. Applanation tonometry allows to assess the arterial tree stiffness; however, the Two-Dimensional Speckle Tracking (2DST) is an increasingly accepted alternative. This study evaluated arterial wall stiffness using these 2 techniques in children with T1DM. MATERIAL AND METHODS We performed applanation tonometry and carotid arteries sonography with evaluation of the carotid intima-media thickness (cIMT) and use of the 2DST in 50 children with T1DM and in 50 healthy sex- and age-matched controls. We also assessed the reliability of 2DST in 10 random subjects. RESULTS Children with T1DM had increased arterial wall stiffness, which was confirmed by tonometry (PWV: p=0.0386) and 2DST (Strain: p=0.0004; Strain rate: p=0.0081). There was no significant difference in cIMT between groups (0.45±0.06 vs. 0.43±0.05, p=0.073 in children with T1DM and controls, respectively). 2DST presented good intraclass correlation coefficient between researchers and within a single researcher. CONCLUSIONS Children with T1DM presenting with subclinical stage of atherosclerosis were found to have arterial wall stiffening. The 2DST, the same as applanation tonometry, allows to recognize this condition but in a more accessible and reproducible manner.

Neonatal survival and kidney function after prenatal interventions for obstructive uropathies.

OBJECTIVES: Prenatal interventions in LUTO (lower urinary tract obstruction) usually are still question of a debate between gynaecologist and paediatric nephrologist. We aimed the study to assess the early survival rate and renal outcome in LUTO foetuses. MATERIAL AND METHODS: The study was a prospective data analysis of 39 foetuses from singleton pregnancies. All pregnant women with LUTO in the foetus were qualified for VAS based on a local practice. The mean time of first urine analysis ranged between 13-30 weeks of pregnancy. Primary end-point analysis included live birth, 28d-survival, pulmonary and renal function assessment in neonatal period. RESULTS: From initial number of 39, six patients miscarried before the procedure was performed. Overall, 33 VAS were performer at the mean 21 week of pregnancy (range 14-30 weeks). 25/39 foetuses survived until delivery. Three neonates died in first 3 days of life. In the first month 3 children required peritoneal dialysis, but at 28 day all children were dialysis-free. Overall survival rate at 28 day was 56%. Renal function preservation of the initial group (39) turned out to be low - 18% (7/39). CONCLUSIONS: Our study showed average survival curves and complications. LUTO in the foetus had mostly unfavourable outcome in the neonatal period. The prenatal intervention did not increase it significantly and did not guarantee the preservation of normal kidney function.

Complete genome sequences of Aeromonas and Pseudomonas phages as a supportive tool for development of antibacterial treatment in aquaculture.

BACKGROUND: Aquaculture is the fastest growing sector of food production worldwide. However, one of the major reasons limiting its effectiveness are infectious diseases among aquatic organisms resulting in vast economic losses. Fighting such infections with chemotherapy is normally used as a rapid and effective treatment. The rise of antibiotic resistance, however, is limiting the efficacy of antibiotics and creates environmental and human safety concerns due to their massive application in the aquatic environment. Bacteriophages are an alternative solution that could be considered in order to protect fish against pathogens while minimizing the side-effects for the environment and humans. Bacteriophages kill bacteria via different mechanisms than antibiotics, and so fit nicely into the 'novel mode of action' concept desired for all new antibacterial agents. METHODS: The bacteriophages were isolated from sewage water and characterized by RFLP, spectrum of specificity, transmission electron microscopy (TEM) and sequencing (WGS). Bioinformatics analysis of genomic data enables an in-depth characterization of phages and the choice of phages. This allows an optimised choice of phage for therapy, excluding those with toxin genes, virulence factor genes, and genes responsible for lysogeny. RESULTS: In this study, we isolated eleven new bacteriophages: seven infecting Aeromonas and four infecting Pseudomonas, which significantly increases the genomic information of Aeromonas and Pseudomonas phages. Bioinformatics analysis of genomic data, assessing the likelihood of these phages to enter the lysogenic cycle with experimental data on their specificity towards large number of bacterial field isolates representing different locations. CONCLUSIONS: From 11 newly isolated bacteriophages only 6 (25AhydR2PP, 50AhydR13PP, 60AhydR15PP, 22PfluR64PP, 67PfluR64PP, 71PfluR64PP) have a potential to be used in phage therapy due to confirmed lytic lifestyle and absence of virulence or resistance genes.