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.
Growth references are useful in monitoring a child's growth, which is an essential part of child care. The aim of this paper was to provide updated growth references for Polish school-aged children ...and adolescents and show the prevalence of overweight and obesity among them. Growth references for height, weight, and body mass index (BMI) were constructed with the lambda, mu, sigma (LMS) method using data from a recent, large, population-representative sample of school-aged children and adolescents in Poland (
n
= 17,573). The prevalence of overweight and obesity according to the International Obesity Taskforce definition was determined with the use of LMSGrowth software. Updated growth references for Polish school-aged children and adolescents were compared with Polish growth references from the 1980s, the Warsaw 1996–1999 reference, German, and 2000 CDC references. A positive secular trend in height was observed in children and adolescents from 7 to 15 years of age. A significant shift of the upper tail of the BMI distribution occurred, especially in Polish boys at younger ages. The prevalence of overweight or obesity was 18.7% and 14.1% in school-aged boys and girls, respectively. The presented height, weight, and BMI references are based on a current, nationally representative sample of Polish children and adolescents without known disorders affecting growth. Changes in the body size of children and adolescents over the last three decades suggest an influence of the changing economical situation on anthropometric indices.
Fuel catalytic additives have been tested for many years. Herein, their influence on the overall efficiency of combustion engines is investigated, and their pro-ecological impact is assessed. The ...majority of this research concerns diesel engines. Despite many advantages, to this day, the use of catalytic additives has not become widespread. Wishing to clarify the situation, a research group from the Wroclaw University of Science and Technology decided to investigate this matter, starting with verification tests. This article presents the methodology and results of testing an actual diesel engine, and evaluates the effects of the use of a fuel catalytic additive. The focus was on the analysis of fuel consumption and exhaust gas emissions from a Doosan MD196TI engine. The tested additive was a commercial fuel performance catalyst (FAMAX) with up to 5% ferric chloride as an organometallic compound. The proportion of the mixture with the fuel was 1:2000. These studies provide an energy and ecological assessment of propulsion in inland vehicles relative to current exhaust emission standards. The tests were carried out in accordance with the ISO 8178 standard, albeit on a much broader scale regarding engine operation than required by the standard. In this way, a set of previously published data was more than doubled in scope. Detailed conclusions indicate the positive effect of the tested fuel additive. The emission values decreased, on average by 16.7% for particulate matter (PM), 10.1% for carbon monoxide (CO), and 7.9% for total hydrocarbons (THC). Unfortunately, the amount of nitrogen oxides (NOx) increased by 1.2%. The average difference in specific fuel consumption (BSFC) between the fuel with additive and pure diesel fuel was 0.5%, i.e. below the level of measurement error. The authors formulated the following scientific relationship between the thermal efficiency of the engine and the operation of the catalyst: the effect of the catalyst on the combustion process decreases with the increase of the thermodynamic efficiency of the engine. This conclusion indicates that despite the proven positive effect of catalysts on the combustion process, they can only be used in markets where engines with low thermal efficiency are used, i.e., older generation engines.
Objective of this work was to realize a test of durability of railway engine EMD645 with power about 1300 kW. Within the framework of this test were investigated a prototyped piston’s rings with ...diamond embankment. Piston rings are made of chromium layer with including of diamond powders technology with a porous chromium coating, where in pores is deposited on said diamond powder with a grain size about 1 micron. The work will be carried out of an analysis of collaboration piston – piston rings – cylinder unit in internal combustion engine and an analysis of the use of hard materials (diamond powder) in friction pairs. During work of this unit we can observe wear of piston rings, precisely - of coating which is deposited on ring to prolong service life. After testing of the locomotive engine EMD645 on the basis of the collected results are developed conclusions of the wearing intensity on piston ring and relating them to the requirements for coatings. The work aims to show the possibilities and benefits of the application of new protective coatings on structural elements of the internal combustion engine in order to reduce their wearing, which is consistent with the observed trend of technology development.
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
R
2
= 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.
Graphic Abstract
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.
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.
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 (
= -0.26,
< 0.05) - strong in girls (
= -0.38,
< 0.05), but not in boys. In each age group, we found no significant correlation between eGFR and FGF23.
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.
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 m
2
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.
Are Ancient Logics Explosive? Tkaczyk, Marcin
History and philosophy of logic,
04/2024, Letnik:
45, Številka:
2
Journal Article
Recenzirano
The twentieth-century logical mainstream, derived from works by Łukasiewicz and Scholz, pictures the history of logic for the most part as the prehistory of Boolean-Fregean mathematical logic. ...Particularly, with respect to classical propositional calculus, the Stoic logic has been pictured as an early stage of it and Aristotle's or the Peripatetics' logic as a theory that assumes it. Although it was not emphasised, it follows that the ancient logics contain the principle of explosion. In the endmost quarter of the twentieth century, a competitive view began to spread to the effect that the ancient systems of logic were paraconsistent or relevantistic. In the twenty-first century, the latter view prevails and has every chance of becoming a new orthodoxy. It is claimed that although in Łukasiewicz's argument for the classicality of ancient logics, there are gaps, it may be demonstrated that the ancient logics contain the principle of explosion.
The growth of children is an indicator of health and society's wellbeing. Growth references are useful in monitoring a child's growth, which is a very important part of child care. Poland's growth ...references are not updated regularly. Although several growth reference ranges have been developed in Poland over recent years, sampling was restricted to urban populations of major cities. The aim of this study was to assess how well Polish children match with, or diverge from, regional charts and to compare them with international growth references.
Four Polish and two international (WHO 2007 and USCDC2000) growth references were used to calculate the height, weight and BMI z-scores in a recent, large, population-representative sample of school-aged children and adolescents in Poland. The distributions of z-scores were analysed with descriptive and inferential statistical methods.
Mean height z-scores calculated with the use of the WHO 2007 and USCDC2000 references were positive and significantly different from zero over the entire age range. The mean height z-score was closest to zero in the Poznan reference for boys (0.05) and Warszawa reference for girls (0.01). Median weight z-scores were positive under all weight references over the entire age range with only the exception of 18-year-old girls' weight z-score calculated relative to USCDC2000. Median BMI z-scores were positive in males in early childhood, decreasing with age. In the case of girls, the median BMI z-score calculated using WHO 2007 and USCDC2000 was close to zero in early childhood, decreased in adolescents and reached minimum values at age 18 years. Median BMI z-scores calculated with the use of the Lodz reference fluctuated between 0.05 and 0.2 over the studied age range.
In this contemporary sample of Polish school-aged children, distributions of height, weight and BMI differed from those of children from the international growth references. These differences should be considered when using the references. There exist certain limitations to the analysis of height, weight, and BMI z-scores when Polish regional references are used.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK