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Objective: This work discusses thyroglobulin as an appropriate indicator of physiological and pathophysiological processes taking place in the thyroids of six child patients with thyroid hypofunction who were monitored at the start of treatment and during the 3-5 month intervals for a period of 2-3 years in both the clinical examination and the entire laboratory diagnosis of thyroid function, ie. thyrotropin (TSH ), free thyroxine (FT4) and triiodothyronine (FT3), total thyroxine (T4) and triiodothyronine (T3), reverse triiodothyronine (rT3), thyroglobulin (Tg), thyroid binding globulin (TBG), autoantibodies to Tg (antiTg), thyroid peroxidase (antiTPO), TSH receptor (antiTSHr) and urinary iodine were determined. Patients: Two out of 6 pediatric patients (4 girls and 2 boys aged 9-14 years) had chronic autoimmune thyroiditis diagnosed leading to hypothyroidism, where, in addition to high autoantibodies to Tg and TPO, TSH receptor autoantibodies were also detected at an early stage. Another 2 patients had a chronic form of autoimmune thyroiditis with early hypothyroidism and with high concentrations of antiTg and antiTPO, but no autoantibodies to the TSH receptor were detected. In the last 2 children, the chronic form of non-autoimmune thyroiditis with initial hypothyroidism was diagnosed. The antiTg, antiTPO and antiTSHr concentrations were within the reference range for these patients. After the diagnosis was determined, the patients were administered L-thyroxine in a daily dose of 0.73 to 2.13 pg L-T4/kg per day. Methods: Thyroid hormones and factors were determined in serum using commercially available kits companies Roche Diagnostics, Zentech S.A. and Brahms. Iodine in urine was measured by our laboratory procedure using alkaline melting of urine specimens, followed by a Sandell-Kolthoff reaction in the mineralizate. Results: It can be confirmed that the Tg level drops during the successful treatment of hypothyroidism. The decrease of Tg can serve as an indicator of the thyroid's improving state. Conclusions: The dynamics of change of the circulating Tg can contribute to an improvement of the laboratory diagnosis of the thyroid and the significance of determining the Tg is broader than its common use as an indicator of the state of the patient with differentiated carcinoma of the thyroid. [ABSTRACT FROM AUTHOR]

Reciprocal interactions between a tumor and its microenvironment control expansion of tumor cells. Here we show a specific type of interaction in which blasts of experimental leukemia destroy the bone marrow (BM) structures and kill stromal cells. The in vitro experiments showed that the cytotoxic agent released by leukemic cells is the fragmented DNA derived from their genome and occurring in nucleosome-like complexes. This DNA entered nuclei of BM or other cells and induced H2A.X phosphorylation at serine 139, similar to double-strand break-inducing agents. There was a correlation between large amounts of acquired DNA and death of recipient cells. Moreover, the DNA integrated into chromosomal DNA of recipient cells. Primary human acute myeloid leukemia cells also released fragmented DNA that penetrated the nuclei of other cells both in vitro and in vivo. We suggest that DNA fragments released from leukemic and also perhaps other types of tumor cells can activate DNA repair mechanisms or death in recipient cells of a tumor microenvironment, depending on the amount of the acquired DNA. This can impair DNA stability and viability of tumor stromal cells, undermine homeostatic capacity of tumor microenvironment and facilitate tumor progression. [ABSTRACT FROM AUTHOR]

Lignocellulosic material possesses a source of fermentable saccharides for biofuel production, however it must be supplemented with other nutrients for microbial growth. We have investigated the use of chicken feather as a source of amino acids and peptides. Alkaline hydrolysis of feather under mild conditions (80 °C, 0.6% NaOH) provided a highly solubilized preparation of assimilable nitrogen. Similar conditions were applicable for pre-treatment of wheat straw. The concentration of lignocellulose-derived inhibitors was low so washing or detoxification steps were unnecessary. Therefore, hydrolysis of both materials was carried out simultaneously, resulting in a cultivation medium that was suitable for direct use in biobutanol production. Thirteen strains of solventogenic clostridia were tested for their ability to produce butanol on this mixed hydrolysate; strains of Clostridium beijerinckii and Clostridium saccharoperbutylacetonicum produced the highest butanol concentrations. The proposed process of simultaneous hydrolysis followed by direct inoculation was then demonstrated by experimental production of butanol in a laboratory bioreactor, using C. beijerinckii strain NCIMB 8052 and producing 4.6 g/L of butanol. Although process optimization is still required, we have shown that feather and wheat straw could be co-processed, forming a convenient mixed substrate for biobutanol production. • Chicken feather was introduced as a source of nitrogen for production of butanol. • Mixed feather and wheat straw hydrolysate was used directly in butanol production. • Mild alkaline treatment was applied for both substrates - feather and straw. • No washing or detoxification step was necessary. [ABSTRACT FROM AUTHOR]

Crohn's disease (CD) is a nonspecific, chronic inflammatory disease of the gastrointestinal tract. It is supposed that in etiopathogenesis oxidative stress (OS) plays a role. However, its precise role in the active and non-active states of disease is not known yet. We conducted a pilot study focusing on the relationship between OS of CD in remission and the possibility to influence clinical parameters and markers of OS by polyphenolic extract, Pycnogenol® (Pyc). Compared to 15 healthy controls 15 pediatric CD patients (all were in remission according to their disease activity index - PCDAI) had reduced the activity of Cu/Zn-superoxide dismutase (SOD) and increased the oxidative damage to proteins. We found negative correlations between markers of inflammation (calprotectin, CRP) as well as between PCDAI and total antioxidant capacity (TAC). Activities of antioxidant enzymes, SOD, and glutathione peroxidase (GPX) negatively correlated with calprotectin and PCDAI. Pyc (2 mg/kg) positively influenced the parameters of OS in CD patients after 10 weeks of administration. [ABSTRACT FROM AUTHOR]

Infantile hemangioma (IH) occurs in 5 to 10% of children under one year of age. This benign vascular tumor is characterized by three phases of growth – proliferation, plateau and involution. Modern non-selective β-blocker systemic therapy (propranolol) offers children an effective, safe and non-invasive solution. Starting the therapy in the proliferative phase is crucial for effectiveness. [ABSTRACT FROM AUTHOR]

Background: Since 2014, the Czech national registry REPAR has been collecting longitudinal data on patients treated with recombinant growth hormone (rhGH). It contains converted data from terminated international databases KIGS, Nordinet® IOS, GeNeSIS, ECOS and new subsequently added patients. Objective: The aim of this study is to present the basic demographic data of patients included in the REPAR database and to analyze selected longitudinal data. Methods: Data from all ten pediatric clinical centers for the rhGH treatment in the Czech Republic are entered into web-based database via web case report forms. The database contains data before the start of the treatment and data from follow-up visits until treatment termination. Patients belong to the one of six pediatric indication groups – growth hormone deficiency (GHD), Turner syndrome (TS), chronic renal failure (CRI), postnatal growth failure following intrauterine growth restriction (SGA), Prader-Willi syndrome (PWS) and SHOX deficiency. Results: By January 1st, 2020, the REPAR register contained data on 4330 pediatric patients, of which 1896 were currently treated (1130 boys, 766 girls). The dose of rhGH varied from 0.020 to 0.046 mg/kg/day (5th and 95th percentiles; median 0.033 mg/kg/day). In currently treated patients, treatment was initiated at age 5.8 years (median; 0.2–13.4 years, 5th and 95th percentiles), the earliest in PWS (girls 1.5 years, boys 1.3 years) and at the latest in boys with CRI (8.0 years) and also in girls with SHOX deficiency (8.3 years). The age at treatment onset was 7.2 years (girls) and 7.5 years (boys) in GHD, 6.6 years in TS, and 6.4 years (boys) and 6.2 years (girls) in children born SGA on average. In GHD, TS and PWS, the age at treatment initiation decreases significantly over time (p<0.001). At the end of treatment, the median height reaches -1.8 SDS in SGA patients and -1.5 SDS in girls with TS. Conclusion: Analyses of data from the registry contribute to an overview of numbers of treated patients, but also to the evaluation of the clinical recognition of growth disorders in pediatric practice. They can serve to better understanding of physiological effects of growth hormone, to analyzing predictors of therapy success, and also to pharmacoeconomic assessments. [ABSTRACT FROM AUTHOR]

Pendred syndrome (PS, OMIM #274600) is an autosomal recessive genetic disease, which becomes manifest in a combination of sensoric-neural deafness and dyshormonogenetic goitre. In the PS we frequently encounter abnormal formation of inner ear structures - enlarged vestibular aqueduct (EVA) and/or Mondini dysplasia (MD). PS is caused by mutations in the SLC26A4 gene (called also PDS gene, OMIM *605646), which encodes the anion transporter pendrin, expressed especially in thyroid gland and the inner ear. Pendrin in the thyroid gland is localized on the apical pole of thyreocytes. Its main function is the transport of iodine into colloids in follicle lumen, where it is subsequently bound to organic molecules. The affection of thyroid gland in the Pendred syndrome becomes manifest mostly in the second decade of life under the picture of euthyroid and hypothyroid goitre, rarely there is dyshormogenesis at birth and the disease is then diagnosed in the newborn screening for congenital hypothyreosis. The mutation in the SLC26A4 gene causes also a non-syndrome hearing loss associated with EVA (formerly called DFNB4, OMIM #600791). Only recently the digenetic heredity has been established -- mutation in the SLC26A4 gene in combination with a mutation in the FOXI1 gene (OMIM *601093) or KCNJ10 gene (OMIM *602208). PS represents the most frequent syndrome deafness and the SLC26A4 gene is apparently the second most frequent gene responsible for AR non-syndrome hearing loss (after GJB2 gene for Connexin 26, which is responsible for 40% of AR non-syndrome hearing loss).A recently undertake study in the Czech Republic confirmed mutation in the SLC26A4 gene in 8.5% of predominantly child patients with inborn hearing loss; however only less than 27% of these children displayed a completely expressed PS (after puberty in all of them). Thanks to molecular genetic examination it is therefore possible to predict with high probability the development of thyroid gland disorder and to prevent the development of goitre by an early intervention. The examination of the SLC26A4 gene in presently available in the Czech Republic. [ABSTRACT FROM AUTHOR]

Photoactive semiconducting crystalline films of non-stoichiometric tungsten oxides (WO 3- x , 0 ≤ x ≤ 3) were deposited by DC hollow cathode discharge from a tungsten nozzle in a reactive Ar + O 2 atmosphere. The ratio of tungsten to oxygen in the layer was driven by controlling the O 2 flow. All layers were prepared on soda-lime glass substrate (SLG) and on glass substrates coated with an FTO thin film and after deposition were annealed in air or argon atmosphere. The properties of WO 3-x layers were analyzed with respect to their potential applications. Surface morphology was analyzed by SEM, qualitative phase analysis by XRD and Raman spectroscopy, chemical composition by EDX and photoactivity of individual samples by linear voltammetry. The layers stoichiometrically closest to WO 3 and annealed in an argon atmosphere showed the highest photoactivity response. • Non-stoichiometric WO 3-x thin films were deposited by DC hollow cathode discharge. • Higher O 2 flow-rate resulted in higher deposition rate. • Highest photoactivity was noticed for WO 3 samples annealed in Ar atmosphere. [ABSTRACT FROM AUTHOR]