Akutní lymfoblastická leukemie (ALL) tvoří jednu čtvrtinu dětských nádorů a je tak nejčastějším maligním nádorovým onemocněním v dětském věku. Jedná se o heterogenní onemocnění, jehož biologie je odlišná od ALL dospělých. Medián věku při diagnóze dětské ALL je 5 let, nejčastějšími chromozomálními abnormitami jsou fúzní gen TEL-AML a hyperdiploidie nad 50 chromozomů. Pravděpodobnost přežití bez selhání (EFS) je při aplikaci kombinované chemoterapie lišící se dle rizika nepříznivého průběhu více než 75 % a pravděpodobnost vyléčení je 85 %. Relaps postihuje 15–20 % dětí s ALL a v jeho léčbě hraje významnou roli transplantace kostní dřeně. Akutní myeloidní leukemie (AML) tvoří 15 % leukemií v dětství. Nejčastějším genetickým podtypem AML je monocytární leukemie s přestavbou MLL genu na dlouhém raménku 11. chromozomu (20–25 % dětí s AML), která má intermediární prognózu. Prognosticky příznivé podtypy akutní promyelocytární leukemie s t (15; 17), myeloidní leukemie s t (8; 21) a myelomonocytární leukemie s inv (16) se nachází u 30–40 % dětí s AML s šancí na vyléčení 70 %. Celkové výsledky léčby AML se u dětí stále zlepšují s šancí na dosažení remise více než 90 % a pravděpodobností přežití více než 60 %., Acute lymphoblastic leukemia (ALL) represents 25 % of childhood tumors being the most frequent childhood cancer. ALL is a heterogeneous disease, biologically different from the same disease in adults. Median age at diagnosis is 5 years, the most frequent chromosomal abnormalities are fusion gene TEL-AML1 and hyperdiploidy over 50 chromosomes. Event-free-survival is over 75 % and overall survival 85 % in children treated with combined chemotherapy intensified according to the risk group. The incidence of relapse is 15–20 % and bone marrow transplantation is the important treatment modality in such situation. Acute myeloid leukemia (AML) represents 15 % of childhood leukemias. The most frequent genetic subtype is monocytic leukemia with MLL rearrangement at the long arm of chromosome 11 (11q23) (20–25 % of all AML) with intermediate prognosis. Subtypes with good prognosis – acute promyelocytic leukemia with t (15; 17), myeloid leukemia with t (8; 21) and myelomonocytic leukemia with inv (16) represent 30–40 % of AML with overall survival 70 %. Total treatment results are steadily improving with a chance to achieve remission over 90 % and overall survival over 60 %., Jan Starý, and Lit.: 20
Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). It is clinically important to investigate the markers of a poor prognosis. The levels of angiotensinogen (AGT) and interleukin-18 (IL-18) in serum and urine were evaluated. Study was conducted in 29 children with a history of HUS. Serum and urine AGT concentration was significantly higher in children after HUS as compared to the control group. No differences depending on the type of HUS and gender were noted. The serum concentration of IL-18 in children after HUS was significantly lower, whereas in urine did not differ significantly between the sick and healthy children. A negative correlation between the concentration of AGT in serum and albuminuria in patients after HUS was detected. The results indicate that the concentration of AGT in serum and urine in children after HUS increases, which may indicate the activation of the intrarenal renin-angiotensin-aldosterone system. The statement, that AGT may be a good biomarker of CKD after acute kidney injury due to HUS requires prospective studies with follow-up from the acute phase of the disease on a larger group of patients. Reduced IL-18 serum concentration in children after HUS with no difference in its urine concentration may indicate a loss of the protective effects of this cytokine on renal function due to previously occurred HUS., K. Lipiec, P. Adamczyk, E. Świętochowska, K. Ziora, M. Szczepańska., and Obsahuje bibliografii
A recently discussed cardiovascular risk factor, asymmetric dimethylarginine (ADMA), is known to act as an endogenous inhibitor of endothelial nitric oxide synthase. The aim of this study was to establish 1) the relationship between ADMA and ultrasonographically or biochemically determined endothelial dysfunction in children, and 2) the effect of folate supplementation on these parameters. The study cohort included 32 children with familial hypercholesterolemia (FH), 30 with diabetes mellitus type 1 (DM1) and 30 age-matched healthy children as the control group. Furthermore, twenty-eight randomly selected FH and DM1 children were re-examined after 3-months supplementation with folic acid. Baseline levels of ADMA and oxidized low density lipoproteins (oxLDL) were significantly higher in FH group than in DM1 and healthy children. Children in DM1 group had significantly lower concentration of homocysteine, but ADMA levels were normal. Folic acid supplementation significantly lowered homocysteine and hsCRP levels in both FH and DM1 group; however, ADMA and oxLDL concentrations remained unaltered. In conclusion, ADMA and oxLDL appear to be associated with endothelial dysfunction in children with FH. Administration of folic acid did not influence these markers in both FH and DM1 children., P. Jehlička ... [et al.]., and Obsahuje seznam literatury
The aims of our study were to evaluate plasma levels of gut hormones in children with Type 1 diabetes mellitus (T1DM) in comparison with healthy contro ls and to corr elate plasma concentrations of gut hormones with blood biochemistry, markers of metabolic control and with anthropometric parameters. We measured postprandial levels of specific gut peptide hormones in T1DM children. Amylin, glucos e-dependent insulinotropic polypeptide (GIP), glucagon-like peptide 1 (GLP-1), ghrelin, leptin, pancreatic polypeptide (PP), and polypeptide YY (PYY) were assessed in 19 T1DM children and 21 healthy reference controls. Multiplex assay kit (LINCOplex®) was used for determination of the defined plasma hormone levels. T1DM subjects had significantly reduce d amylin (p<0.001) and ghrelin (p<0.05) levels, whereas GIP (p<0.05) was elevated when compared with healthy controls. Pl asma levels of other measured hormones did not differ statistically between the studied groups. Further analysis of T1DM patien ts demonstrated an association between body mass index and GL P-1 (r=0.4642; p<0.05), leptin (r=0.5151; p<0.05), and amylin (r=0.5193; p<0.05). Ghrelin levels positively correlated with serum HDL cholesterol (r=0.4760; p<0.05). An inverse co rrelation was demonstrated with triglycerides (TG) (r=-0. 5674; p<0.01), insulin dosage (r= -0.5366; p<0.05), and HbA1c% (r= -0.6864; p<0.01). Leptin was inversely correlated with TG (r= -0.6351; p<0.01). Stepwise regression analysis was performed to enlighten the predictive variables. Our study demonstrated an altered secretion pattern of gut peptide hormones in T1DM children. A close correlation was revealed between these peptides as well as with blood biochemistry, markers of me tabolic control and with anthropometric parameters. Further studies are essential to explore this issue in T1DM children., M. Huml ... [et al.]., and Obsahuje bibliografii a bibliografické odkazy
Obesity in children is accompanied by increased circulating leptin concentrations. Girls have higher leptin concentrations than boys. The aim of our study was to compare serum leptin levels before and after a five-week weight reduction program and to study the relationship of leptin levels, serum total cholesterol, and androgens (testosterone, dehydroepiandrosterone, dehydroepiandrosterone sulphate) in 33 obese boys (age: 12.71.97 years, BMI: 30.46±4.54) and 66 obese girls (age: 12.7±2.51 years, BMI: 29.31±4.62). We found that serum leptin concentrations in obese children were significantly decreased after a weight reduction program (before 20.79±9.61 ng/ml, after 13.50±8.65 ng/ml in girls; before 12.25±10.09 ng/ml and after 5.18±3.56 ng/ml in boys, p<0.0001 in both genders). Leptin levels correlated positively with the body mass index before and after weight reduction. There was a positive association in obese boys and a negative one in obese girls between leptin levels and the WHR (waist to hip circumference ratio). Serum leptin also shows a strong relationship to fat distribution (p = 0.02 in boys, p<0.0001 in girls). No significant correlation was found between leptin concentrations and total cholesterol or androgens. We confirmed that leptin is a sensitive parameter of body composition and weight reduction in obese children., R. Pilcová, J. Šulcová, M. Hill, P. Bláha, L. Lisá., and Obsahuje bibliografii
The most common etiology of non- syndromic monogenic obesity are mutations in gene for the Melanocortin -4 receptor ( MC485 ) with variable prevalence in different countries (1.2 -6.3 % of obese children). The aim of our study was 1 ) to search for MC4R mutations in obese children in Slovakia and compare their prevalence with other European countries, and 2 ) to describe the phenotype of the mutation carriers. DNA analysis by direct Sanger sequencing of the coding exons and intron/exon boundaries of the MC4R gene was performed in 268 unrelated Slovak children and adolescents with body mass index above the 97 th percentile for age and sex and obesity onset up to 11 years (mean 4.3±2.8 years). Two different previously described heterozygous loss of function MC4R variants (i.e. p.Ser19Alafs*34, p.Ser127Leu) were identified in two obese probands, and one obese (p.Ser19Alafs*34), and one lean (p.Ser127Leu) adult family relatives. No loss of function variants were found in lean controls. The prevalence of loss -of-function MC4R variants in obese Slovak children was 0.7 %, what is one of the lowest frequencies in Europe., D. Stanikova, M. Surova, L. Ticha, M. Petrasova, D. Virgova, M. Huckova, M. Skopkova, D. Lobotkova, L. Valentinova, M. Mokan, J. Stanik, I. Klimes, D. Gasperikova., and Obsahuje bibliografii
Midazolam is a short acting sedative with small number of adverse effects. Administered orally, it is currently the most common form of conscious sedation in children. The objective of this paper is to describe effect of midazolam administered to children during dental treatment on their vital signs, and to monitor changes in children’s behavior. We described values of vital signs and behavior in 418 sedations conducted in 272 children between 1-12 years of age. To achieve the following results, we used data from 272 all first-time sedations. After administration of midazolam arterial blood pressure and blood oxygen saturation decreased by values which were not clinically significant. The heart rate increased, with values staying within the limits of physiological range. The speed of onset of midazolam’s clinical effects depends on age and dose. The lower age and dose correlated with the higher behavior score. The effectiveness of midazolam treatment is 97.8 %. Unwillingness of child to receive midazolam is predictor for disruptive behavior during sedation. 1.8 % of all sedation cases showed paradoxical reactions. The administration of midazolam in dose of 0.5 mg per 1 kg of child’s body weight is safe and could be recommended for dental treatment in pediatric dentistry., Jana Vasakova, Jana Duskova, Jitka Lunackova, Klara Drapalova, Lucie Zuzankova, Luboslav Starka, Michaela Duskova, Zdenek Broukal., and Obsahuje bibliografii
Článek představuje projekt UNICEF Škola přátelská dětem; seznamuje ve stručnosti s manuálem projektu. Projekt byl motivován skutečností, že denně navštěvuje základní a střední školy více než dvě miliardy dětí a školní docházka není pro všechny pozitivní zkušeností (např. pitná voda, školní nábytek, trestání, ponižování dětí atd.). Cílem projektu je vytvořit přátelské prostředí pro všechny děti. Klíčové principy projektu jsou: inkluzivní vzdělávání, demokratická participace dětí, podporující prostředí s cílem rozvoje osobnosti dětí a zajištění kvalitního vzdělávání. Jádrem projektu jsou práva dětí. Model Škola přátelská dětem zahrnuje následující oblasti: 1. umístění, exteriér a dispozice interiéru, 2. škola a komunita, 3. škola jako ochraňující prostředí a 4. vyučování. Záměrem projektu je, aby všechny školy mohly být identifikované jako školy přátelské dětem. To předpokládá, že si každá škola vypracuje svůj vlastní projekt a jeho realizaci ověří metodou dotazníkového šetření. Škola spolupracuje při realizaci projektu s orgány veřejného zdraví., The article presents the UNICEF project Child Friendly Schools; it acquaints us with the manual of projects in brief. The project was motivated by the reality that basic and secondary schools are visited by more then two billions children daily, and schooling is not for every child a positive experience (for example: drinking water, school furniture, punishment and humiliation and others). The aim of this project is the creation of a friendly school environment for all children. The key principles of the project are: inclusive education, democratic participation of children, a supporting environment with the aim of developing the child's personality and ensuzing an excellent education. The core of the project are the rights of children. The model of Child Friendly Schools consists of four areas: 1) location, design and construction, 2) the school and the community, 3) the school as a protective environment, and 4) learners, teachers and school management. The purpose of the project is for all schools to be identified us child friendly schools. It anticipates that each school elaborates its own project and its realization is verified by a questionnaire survey. The school cooperates in the materialisation of the project with public health authorities., Eva Vaníčková, and Literatura 5
Autoři ve svém příspěvku uvádějí první výsledky šetření, které bylo zaměřeno na zjištění hmotností školních tašek chlapců a dívek 1. a 2. tříd základních škol v olomouckém regionu. Celkem byla zjištěna hmotnost u 502 školních tašek, změřeno 502 dětí (263 dívek a 239 chlapců). Z výsledků měření je zřejmé, že hmotnost tašky se zvyšuje s postupem žáků do vyšší třídy. Největší rozdíl mezi minimální a maximální hodnotou hmotnosti tašky byl zjištěn u 8letých dívek, činil 8,9 kg. Výsledky ukázaly, že procentuální podíl hmotnosti tašky doporučeným 10 % tělesné hmotnosti dítěte odpovídal pouze u 17,5 % dětí. 82,5 % dětí pak nosí aktovky těžké (10–20 %), přičemž 18,3 % dětí nosí aktovky o hmotnosti nad 20 % své tělesné hmotnosti, což už lze považovat za extrémně těžké. Nepřiměřená hmotnost školní tašky pak může ovlivňovat držení těla a výskyt bolesti zad., Effects of the weights of school bags carried by pupils from the first two classes of primary schools in the Olomouc region (502 schoolbags belonging to 263 girls and 239 boys) on the pupils’ posture were examined. The weight of the schoolbags of pupils from the 2nd year class was found to be higher than of pupils in the first year. The greatest difference between minimum and maximum weights (8.9 kilograms) was detected in the schoolbags of 8 year old girls. Only 17.5% of children carried bags that were within the recommended 10% of the child’s body weight. Thus, 82.5% of the children carry heavier bags and 18.3% of these pupils carry extremely heavy bags (over 20% of their body weight).We conclude that the excessive weight of school bags contributes to poor posture and backache amongst these children., Hana Kabátová, Miroslav Kopecký, Dana Strnisková, Jitka Tomanová, and Literatura 5
Fatty acid (FA) profiles as potentially relevant components of Crohn’s disease (CD) have been insufficiently analysed. We sought to explore the plasma profiles of n-3 and n-6 polyunsaturated fatty acids (PUFAs) in newly diagnosed untreated active CD. We included 26 consecutive CD pediatric patients (<19 years) and 14 healthy controls (HCs). Disease characteristics, including inflammatory markers, dietary histories, and the Pediatric Crohn’s Disease Activity Index (PCDAI), were obtained. The profiles of plasma FAs in plasma lipid classes were analysed by gas chromatography with FID detection of methyl esters. The erythrocyte sedimentation rate, C-reactive protein level and fecal calprotectin level (all p<0.001) were significantly higher in CD patients than in HCs. Most changes were observed in plasma phospholipids (PLs), such as a higher content of n-3 and changes in n-6 long-chain PUFAs in the CD group. The CD group had a lower ratio of n-6/n-3 PUFAs in PLs (p<0.001) and triacylglycerols (TAGs) (p<0.01). Correlations of the FA content in plasma PLs with disease activity scores of CD were also observed, which were positive for the sum of monounsaturated fatty acids (MUFAs) as well as oleic acid (18:1n-9) (both p<0.05). The metabolism of PUFAs is significantly altered even in treatment-naïve newly diagnosed active pediatric CD, and the content of major FAs in PLs correlates with disease activity and inflammatory markers, thus probably contributing to the still unclear early disease pathogenesis., Jan Schwarz, Marek Vecka, František Stožický, Renáta Pomahačová, Barbora Staňková, Eva Tvrzická, Marcela Kreslová, Romana Zahálková, Josef Sýkora., and Obsahuje bibliografii