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Background: The hyperinsulinism/hyperammonaemia (HI/HA) syndrome is the second most common cause of hyperinsulinaemic hypoglycaemia, caused by activating mutations in GLUD1. In this article, we report a series of three unrelated patients with HI/HA syndrome who demonstrated variable phenotypes, ranging from delayed presentation to spontaneous resolution of hypoglycaemia, thereby expanding the current knowledge and understanding of GLUD1 mutations. Case presentation: This paper is a retrospective analysis of patients with HI/HA syndrome who demonstrated a variable disease course. Patient 1 presented with hypoglycaemic seizures at the age of 7 months and was diagnosed with HI/HA syndrome. Patient 2, a 5-year-old boy, on anti-convulsants since 8 months of age, was diagnosed with HI/HA at the age of 4 years. Patient 3, an 11-year-old girl with a history of transient neonatal hypoglycaemia, was diagnosed with HI/HA at the age of 12 months following evaluation for absence seizures. Patients 1 and 2 had raised ammonia levels, whilst patient 3 had normal ammonia level. The genetic analysis in all three patients confirmed GLUD1 mutation. Good glycaemic control was observed in all following diazoxide treatment. All patients have learning difficulties. Patient 1 demonstrated spontaneous resolution of hypoglycaemia at the age of 8 years, enabling discontinuation of diazoxide. Conclusions: The cases highlight the diagnostic challenges in HI/HA syndrome due to a highly variable presentation. Knowledge of variable phenotypes would enable early diagnosis, thereby decreasing the risk of long-term neurological damage. Spontaneous resolution of hyperinsulinism could occur, and it is important to consider a trial off diazoxide therapy especially if the patients are on a small dose of diazoxide. [ABSTRACT FROM AUTHOR]

Background/Objective: Recent studies suggest that uric acid (UA) is a mediator of diabetic nephropathy. We hypothesized that serum UA would associate with the prevalence of diabetic nephropathy in youth with type 1 diabetes (T1D), and that this relationship would differ by sex. Methods: We examined 120 young boys and the same number of girls with T1D. C‐reactive protein (CRP), interleukin‐6 (IL‐6), interleukin‐10 (IL‐10), tumor necrosis factor α (TNF‐α), UA, cystatin C serum concentrations, albumin excretion rate and blood pressure were also analyzed. Results: T1D boys had higher serum UA and creatinine concentration, as well as albumin excretion rate and estimated glomerular filtration rate than T1D girls. Moreover, newly diagnosed nephropathy was more common in male subjects in comparison to female patients. Only in T1D boys serum UA was positively correlated with concentrations of subclinical inflammatory markers (CRP, IL‐6, TNF‐α), the indicators of renal function (albumin excretion rate, serum cystatin C level), blood pressure and negatively correlated with anti‐inflammatory IL‐10. In addition, only in T1D girls serum UA concentration was negatively correlated with hemoglobin A1c. Conclusions: Serum UA is associated with nephropathy prevalence, albeit only in boys with T1D and may be an important risk factor for predicting diabetes‐related cardiorenal complications in these patients. [ABSTRACT FROM AUTHOR]

Experimental near edge X-ray absorption fine structure (NEXAFS) spectra, X-ray photoelectron (XP) spectra and Auger electron spectra are reported for sulfur in ionic liquids (ILs) with a range of chemical structures. These values provide experimental measures of the atomic charge in each IL and enable the evaluation of the suitability of NEXAFS spectroscopy and XPS for probing the relative atomic charge of sulfur. In addition, we use Auger electron spectroscopy to show that when XPS binding energies differ by less than 0.5 eV, conclusions on atomic charge should be treated with caution. Our experimental data provides a benchmark for calculations of the atomic charge of sulfur obtained using different methods. Atomic charges were computed for lone ions and ion pairs, both in the gas phase (GP) and in a solvation model (SMD), with a wide range of ion pair conformers considered. Three methods were used to compute the atomic charges: charges from the electrostatic potential using a grid based method (ChelpG), natural bond orbital (NBO) population analysis and Bader’s atoms in molecules (AIM) approach. By comparing the experimental and calculated measures of the atomic charge of sulfur, we provide an order for the sulfur atoms, ranging from the most negative to the most positive atomic charge. Furthermore, we show that both ChelpG and NBO are reasonable methods for calculating the atomic charge of sulfur in ILs, based on the agreement with both the XPS and NEXAFS spectroscopy results. However, the atomic charges of sulfur derived from ChelpG are found to display significant, non-physical conformational dependence. Only small differences in individual atomic charge of sulfur were observed between lone ion (GP) and ion pair IL(SMD) model systems, indicating that ion–ion interactions do not strongly influence individual atomic charges. [ABSTRACT FROM AUTHOR]

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Acidic anions of ionic liquids have been demonstrated as efficient catalysts for the cleavage of the β-O-4 ether linkage prevalent in the lignin superstructure. Through the use of lignin model compounds with varying functionality and by monitoring reaction kinetics, a full mechanistic investigation into the hydrolysis of the β-O-4 linkage in acidic ionic liquid solutions is reported. Hammett acidities are reported for different 1-butyl-3-methylimidazolium hydrogen sulfate [C4C1im][HSO4] ionic liquid systems with varying acid and water concentrations and were correlated to substrate reactivity. Results show that the rate of ether cleavage increases with an increase in acidity and the initial dehydration of the model compound is the rate-determining step of the reaction. The Eyring activation parameters of the reaction in hydrogen sulfate ionic liquids with a variety of cations are reported, indicating a consistent E1 dehydration mechanism. Hydrogen bonding in protic ionic liquids was shown to significantly influence anion–cation interactions, consequently altering the solvation of the protonated starting material and therefore the overall rate of reaction. Comparison of reaction rates in these ionic liquids with results within aqueous or aqueous/organic media indicate that the ionic liquids facilitate more rapid cleavage of the β-O-4 ether linkage even under less acidic conditions. All the reported results give a complete overview of both the mechanistic and solvation effects of acidic ionic liquids on lignin model compounds and provide scope for the appropriate selection and design of ionic liquids for lignin processing. [ABSTRACT FROM AUTHOR]

A number of ionic liquids have been shown to be excellent solvents for lignocellulosic biomass processing, and some of these are particularly effective in the production of the versatile chemical building block 5-hydroxymethylfurfural (HMF). In this study, the production of HMF from the simple sugar glucose in ionic liquid media is discussed. Several aspects of the selective catalytic formation of HMF from glucose have been elucidated using metal halide salts in two distinct ionic liquids, 1-butyl-3-methylimidazolium chloride and 1-butyl-3-methylimidazolium hydrogen sulfate as well as mixtures of these, revealing key features for accelerating the desired reaction and suppressing byproduct formation. The choice of ionic liquid anion is revealed to be of particular importance, with low HMF yields in the case of hydrogen sulfate-based salts, which are reported to be effective for HMF production from fructose. The most successful system investigated in this study led to almost quantitative conversion of glucose to HMF (90% in only 30 minutes using 7 mol% catalyst loading at 120°C) in a system which is selective for the desired product, has low energy intensity and is environmentally benign. [ABSTRACT FROM AUTHOR]

IL-33 is an IL-1 cytokine family member, with ability to induce both Th1 and Th2 immune responses. It binds to ST2 receptor, whose deficiency is associated with enhanced inflammatory response. The most recent studies have shown the immunoregulatory effect of IL-33 on Tregs in animal models. As type 1 diabetes is an autoimmune, inflammatory disease, where Treg defects have been described, we aimed to analyze the in vitro influence of recombinant IL-33 on quantitative properties of regulatory CD4+CD25highFOXP3+ T cells. CD4+CD25highFOXP3+ as well as CD4+CD25highFOXP3+ST2+ Tregs were analyzed by flow cytometry. In a group of patients with type 1 diabetes in vitro IL-33 treatment induced regulatory CD4+CD25highFOXP3+ cell frequencies as well as upregulating the surface expression of ST2 molecule. In addition, the number of CD4+CD25highFOXP3+ cells carrying ST2 receptor increased significantly. Similar effect was observed in case of the FOXP3 expression. We did not observe any significant changes in IL-33 treated cells of healthy controls. The level of ST2 was higher in serum of patients with type 1 diabetes in comparison to their healthy counterparts. We propose that IL-33 becomes an additional immunostimulatory factor used to induce Treg expansion in future clinical trials of adoptive therapy in type 1 diabetes. [ABSTRACT FROM AUTHOR]

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Recently, acidic ionic liquid water mixtures based on the hydrogen sulfate anion have been shown to effectively extract lignin from lignocellulosic biomass. This study analyses Miscanthus giganteus lignin isolated after extraction with the protic ionic liquid 1-butylimidazolium hydrogen sulfate ([HC4im][HSO4]) followed by precipitation with the antisolvent water. Several analytical techniques were employed, such as quantitative 13C-NMR, 1H–13C HSQC NMR, 31P-NMR, Py-GC-MS, GPC and elemental analysis. The analysis shows that the ionic liquid pretreatment breaks lignin-hemicellulose linkages and depolymerizes the lignin through the cleavage of glycosidic, ester and β-O-4 ether bonds. This is accompanied by solubilization of the newly generated lignin fragments. At longer pretreatment times, repolymerization of lignin fragments through condensation reactions occurs. The isolated lignins were carbohydrate-free, had low sulfur contents, low molecular weights. Early stage lignins were structurally similar to ball-milled lignin, while more treated lignins were enriched in p-hydroxyphenyl and guaiacyl units and had a high phenolic hydroxyl group content. We conclude that, depending on the treatment conditions, lignins with a variety of characteristics can be isolated using this type of ionic liquid solution. [ABSTRACT FROM AUTHOR]

The cost of ionic liquids (ILs) is one of the main impediments to IL utilization in the cellulosic biorefinery, especially in the pretreatment step. In this study, a number of ionic liquids were synthesized with the goal of optimizing solvent cost and stability whilst demonstrating promising processing potential. To achieve this, inexpensive feedstocks such as sulfuric acid and simple amines were combined into a range of protic ionic liquids containing the hydrogen sulfate [HSO4]− anion. The performance of these ionic liquids was compared to a benchmark system containing the IL 1-ethyl-3-methylimidazolium acetate [C2C1im][OAc]. The highest saccharification yields were observed for the triethylammonium hydrogen sulfate IL, which was 75% as effective as the benchmark system. Techno-economic modeling revealed that this promising and yet to be optimized yield was achieved at a fraction of the processing cost. This study demonstrates that some ILs can compete with the cheapest pretreatment chemicals, such as ammonia, in terms of effectiveness and process cost, removing IL cost as a barrier to the economic viability of IL-based biorefineries. [ABSTRACT FROM AUTHOR]

The application of the protic ionic liquid 1-butylimidazolium hydrogen sulfate in the deconstruction (aka pretreatment) and fractionation of lignocellulosic biomass has been investigated. A cellulose rich pulp and a lignin fraction were produced. The pulp was subjected to enzymatic saccharification which allowed recovery of up to 90% of the glucan as fermentable glucose. The influence of the solution acidity on the deconstruction of Miscanthus giganteus was examined by varying the 1-butylimidazole to sulfuric acid ratio. Increased acidity led to shorter pretreatment times and resulted in reduced hemicellulose content in the pulp. Addition of water to the ionic liquid resulted in enhanced saccharification yields. The ability to tune acidity through the use of protic ionic liquids offers a significant advantage in flexibility over dialkylimidazolium analogues. [ABSTRACT FROM AUTHOR]

The objective was to compare the impact of clinical and genetic factors on body mass index (BMI) in children with type 1 diabetes (T1DM) without severe obesity. A total of 1,119 children with T1DM (aged 4-18 years) were qualified to take part in the study. All children were genotyped for variants of FTO, MC4R, INSIG2, FASN, NPC1, PTER, SIRT1, MAF, IRT1, and CD36. Results. Variants of FTO showed significant association with BMI-SDS in the T1DM group. The main factors influencing BMI-SDS in children with T1DM included female gender (P = 0.0003), poor metabolic control (P = 0.0001), and carriage of the A allele of the FTO rs9939609 gene (P = 0.02). Conclusion. Our research indicates, when assessing, the risk of overweight and obesity carriage of the A allele in the rs9939609 site of the FTO gene adds to that of female gender and poor metabolic control. This trial is registered with ClinicalTrials.gov (NCT01279161). [ABSTRACT FROM AUTHOR]

Purpose. The study aimed to investigate the influence of estrogen receptor α (ER-α) genotypes on inflammatory response and development of microvascular complications in girls with type 1 diabetes. Methods. 152 young regularly menstruating girls with diagnosed type 1 diabetes and 84 young, healthy menstruating girls were recruited. ER-α genotypingwas carried out by PCR. Serum concentrations of 17β-estradiol, as well as IL-6, TNF-α, VEGF, and IL-10, weremeasured.CD4+Foxp3+ TH17 cells were isolated and analyzed by flow cytometry. Results. Type 1 diabetic girls carrying TT genotype were characterized by the lowest serum estradiol level and IL-10 and highest IL-6, TNF-α , and VEGF.The association between the level of certain cytokine and the genetic variant of estrogen receptor α polymorphism was analyzed. Frequencies of CD4+Foxp3+ TH17 cells were also enhanced in TT bearing girls with type 1 diabetes and correlated with the level of analyzed cytokines. In addition, the correlation between serum estradiol level and cytokine concentrations was observed. Conclusions. We propose that TT variant of estrogen receptor α polymorphism may be associated with enhanced inflammatory response, which in turn may lead to acceleration of diabetic retino- and nephropathy in girls with type 1 diabetes. This finding may help the physicians to predict the onset and progression of diabetic microvascular complications. [ABSTRACT FROM AUTHOR]

A range of methods for the computational prediction of experimentally derived α and β Kamlet–Taft parameters, indicators of hydrogen bond (H-bond) acidity and basicity for ionic liquids (ILs) have been explored. Most usefully, a good correlation has been established between several simple and easily computed quantities which allow for a “quick bench-top” evaluation. More accurate, but also more sophisticated methods employing TD-DFT calculations involving the Kamlet–Taft dyes have been examined and evaluated. Importantly, these techniques open up the opportunity for pre-screening and a priori prediction of properties for ILs not yet synthesised. A key fundamental insight into IL H-bonds has been the determination of an estimate for the energy associated with replacing both neutral molecules in a H-bond with ionic molecules, thus forming the “doubly ionic” H-bond found in ILs. [ABSTRACT FROM AUTHOR]

This paper reviews the application of ionic liquids to the deconstruction and fractionation of lignocellulosic biomass, in a process step that is commonly called pretreatment. It is divided into four parts: the first gives background information on lignocellulosic biomass and ionic liquids; the second focuses on the solubility of lignocellulosic biomass (and the individual biopolymers within it) in ionic liquids; the third emphasises the deconstruction effects brought about by the use of ionic liquids as a solvent; the fourth part deals with practical considerations regarding the design of ionic liquid based deconstruction processes. [ABSTRACT FROM AUTHOR]

Ionic liquids are of great interest as potential solvents/catalysts for the production of fuels and chemicals from lignocellulosic biomass. Attention has focused particularly on the pretreatment of lignocellulose to make the cellulose more accessible to enzymatic hydrolysis. Any biomass processing requires a reduction in the size of the harvested biomass by chipping and/or grinding to make it more amenable to chemical and biological treatments. This paper demonstrates that significant energy savings can be achieved in the grinding of pine wood chips when the ionic liquid is added before the grinding operation. We show that this is due to the lubricating properties of the ionic liquids and not to physico-chemical modifications of the biomass. A brief impregnation of the chipped biomass results in higher savings than a longer treatment. [ABSTRACT FROM AUTHOR]

Ground lignocellulosic biomass (Miscanthus giganteus, pine (Pinus sylvestris) and willow (Salix viminalis)) was pretreated with ionic liquid–water mixtures of 1-butyl-3-methylimidazolium methyl sulfate and 1-butyl-3-methylimidazolium hydrogen sulfate. A solid fraction enriched in cellulose was recovered, which was subjected to enzymatic hydrolysis. Up to 90% of the glucose and 25% of the hemicellulose contained in the original biomass were released by the combined ionic liquid pretreatment and the enzymatic hydrolysis. After the pretreatment, the ionic liquid liquor contained the majority of the lignin and the hemicellulose. The lignin portion was partially precipitated from the liquor upon dilution with water. The amount of hemicellulose monomers in the ionic liquid liquor and their conversion into furfurals was also examined. The performance of ionic liquid–water mixtures containing 1,3-dialkylimidazolium ionic liquids with acetate, methanesulfonate, trifluoromethanesulfonate and chloride anions was investigated. The applicability of the ionic liquid 1-butylimidazolium hydrogensulfate for lignocellulose pretreatment was also examined. It was found that ionic liquid liquors containing methyl sulfate, hydrogen sulfate and methanesulfonate anions were most effective in terms of lignin/cellulose fractionation and enhancement of cellulose digestibility. [ABSTRACT FROM AUTHOR]

The present study provides insight into the dissolution behavior of renewable materials in ionic liquids. Beech, spruce and rye straw were dissolved in 1-ethyl-3-methylimidazolium acetate as the ionic liquid of choice, which is currently one of the most frequently used cation-anion combinations among ionic liquids for biomaterial processing. The dissolution was followed by selective precipitation of cellulose, hemicelluloses, and lignin. The obtained lignin was analyzed with Curie-point pyrolysis gas chromatography/mass spectrometry (Cu-Py-GC/MS) and the separated cellulose/hemicellulose fractions with gel permeation chromatography (GPC). Time dependence of the dissolution process was studied on rye straw, eucalyptus kraft pulp and beech sulfite pulp. The results show a changing dissolution profile over time, which is due to progressing degradation of the cellulose in the ionic liquid. [ABSTRACT FROM AUTHOR]

Abstract: Carbanions of 3-substituted-3-chloropropyl phenyl sulfones add to carbonyl groups of aldehydes to produce aldol type adducts that undergo 1,5-intramolecular substitution giving 2,3,5-trisubstituted tetrahydrofurans. The effect of substituents in the 3-position of these sulfones on the relative rates of 1,3-intramolecular substitution of the corresponding γ-chlorocarbanions and their intramolecular addition are disclosed. [Copyright &y& Elsevier]

Abstract: New isolates of solventogenic bacteria exhibited high hemicellulolytic activity. They produced butanol and acetone with high selectivity for butanol (about 80% of butanol from the total solvent yield). Their 16S rDNA sequence was 99% identical to that of Clostridium saccharobutylicum. The genes responsible for the last steps of solventogenesis and encoding crotonase, butyryl-CoA dehydrogenase, electron-transport protein subunits A and B, 3-hydroxybutyryl-CoA dehydrogenase, alcohol dehydrogenase, CoA-transferase (subunits A and B), acetoacetate decarboxylase, and aldehyde dehydrogenase were identified in the new C. saccharobutylicum strain Ox29 and cloned into Escherichia coli. The genes for crotonase, butyryl-CoA dehydrogenase, electron-transport protein subunits A and B, and 3-hydroxybutyryl-CoA dehydrogenase composed the bcs-operon. A monocistronic operon containing the alcohol dehydrogenase gene was located downstream of the bcs-operon. Genes for aldehyde dehydrogenase, CoA-transferase (subunits A and B), and acetoacetate decarboxylase composed the sol-operon. The gene sequences and the gene order within the sol- and bcs-operons of C. saccharobutylicum Ox29 were most similar to those of Clostridium beijerinckii. The activity of some of the bcs-operon genes, expressed in heterologous E. coli, was determined. [Copyright &y& Elsevier]

Aim The aim of the study was to assess the relationship between CCR5 -Δ32 polymorphism and the coincidence of celiac and autoimmune thyroid diseases with type 1 diabetes mellitus (T1D) in children. Methods 420 children with T1D aged 15.5 ± 3.0 years and 350 healthy controls were studied. Characterization of CCR5 -Δ32 genotypes (rs333) was analyzed by polymerase chain reaction (PCR). Results The allele frequency was significantly different in diabetic children as compared to the healthy controls (p < 0.0001). We found negative association between T1D and Δ32 allele (OR = 0.383; 95% CI = 0.268–0.549). Besides, we observed alterations in the frequencies of CCR5 -Δ32 genotypes due to celiac and autoimmune thyroid diseases. The risk of celiac disease for patient carriers of the 32-bp deletion was more than threefold higher than for noncarriers (OR = 3.490; 95% CI = 1.357–8.859; p = 0.009). Similar results were obtained in the case of autoimmune thyroiditis. The risk of autoimmune thyroiditis for patient carriers of the 32-bp deletion was also more than threefold higher than for noncarriers (OR = 3.466; 95% CI = 1.754–6.849; p = 0.0004). Conclusions The findings of our studies suggest that the CCR5 -Δ32 polymorphism is associated with type 1 diabetes mellitus and the Δ32 allele increases the risk of celiac disease and autoimmune thyroid disorders in patients with T1D. [ABSTRACT FROM AUTHOR]

Aim The aim of the study was to assess the relationship between the CCR5-Δ32 polymorphism and the risk of diabetic retinopathy (DR) in patients with DM1. Methods We examined 420 patients and 350 healthy controls. The analysis concerned CCR5-Δ32 polymorphism as well as levels of serum inflammatory markers (CRP, TNF-α), adhesion molecules (VCAM, ICAM-1, ICAM-3) and CCR5 ligand (MCP-1). Results We found a negative association between DM1 and Δ32 allele. Moreover, the frequency of Δ32 allele was higher in a group with DR in comparison to control subjects without this complication. We also found that Δ32 carriers had the highest levels of: HbA1c, inflammatory markers, adhesion molecules and CCR5 ligand. Conclusions The findings of our studies suggest that the CCR5-Δ32 polymorphism is associated with DM1 such that the Δ32 allele protects against the development of DM1 and increases the risk of DR in patients who have already developed the disease. [ABSTRACT FROM AUTHOR]

Aim: Estimation of the ocular status in adolescents with diabetes mellitus type 1 (DM1) treated with continuous subcutaneous insulin infusion (CSII), assessment of the development of the diabetic retinopathy (DR) and nephropathy (DN) within 10 years., Methods: 37 patients (74 eyes) aged 16-33 years, treated with CSII were enrolled to the study. Baseline, and a 10- year follow-up evaluation included: best corrected visual acuity (BCVA), tonometry, slit lamp exam and fluorescein angiography (FLA). Additionally, spectral-domain optical coherence tomography (SD-OCT) was done in the 7th year of observation to assess the thickness of the retinal nerve fiber (RNFL) and the ganglion cellinner plexiform layers (GCL-IPL) complex thickness. Glycated haemoglobin (HbA1) and albuminuria were also analysed., Results: During the 10-year observation period DR (non-proliferative - NPDR, proliferative - PDR, diabetic macular edema - DME) was diagnosed in 3 (8%) patients. In the DR group: BCVA was significantly lower, intraocular pressure (IOP) levels and albuminuria were higher. There were no differences in HbA1 in both groups. The thinning of RNFL was observed in both groups. Macular RNFL, GCL-IPL complex thickness assessment showed a significantly higher number of borderline results in the group with DR., Conclusions: Diabetic patients treated with CSII are at a lower risk of developing vascular complications even with poor metabolic control. Increased albuminuria may be a predictive sign for early ocular complications, and requires intense observation. Diagnosis of RNFL and GCL-IPL decreased values is crucial prior to diabetic retinopathy development. SD-OCT is a non-invasive, easy-to-perform, relatively inexpensive procedure, and can be a useful tool to monitor neuropathy progression., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.)

Experimental near edge X-ray absorption fine structure (NEXAFS) spectra, X-ray photoelectron (XP) spectra and Auger electron spectra are reported for sulfur in ionic liquids (ILs) with a range of chemical structures. These values provide experimental measures of the atomic charge in each IL and enable the evaluation of the suitability of NEXAFS spectroscopy and XPS for probing the relative atomic charge of sulfur. In addition, we use Auger electron spectroscopy to show that when XPS binding energies differ by less than 0.5 eV, conclusions on atomic charge should be treated with caution. Our experimental data provides a benchmark for calculations of the atomic charge of sulfur obtained using different methods. Atomic charges were computed for lone ions and ion pairs, both in the gas phase (GP) and in a solvation model (SMD), with a wide range of ion pair conformers considered. Three methods were used to compute the atomic charges: charges from the electrostatic potential using a grid based method (ChelpG), natural bond orbital (NBO) population analysis and Bader's atoms in molecules (AIM) approach. By comparing the experimental and calculated measures of the atomic charge of sulfur, we provide an order for the sulfur atoms, ranging from the most negative to the most positive atomic charge. Furthermore, we show that both ChelpG and NBO are reasonable methods for calculating the atomic charge of sulfur in ILs, based on the agreement with both the XPS and NEXAFS spectroscopy results. However, the atomic charges of sulfur derived from ChelpG are found to display significant, non-physical conformational dependence. Only small differences in individual atomic charge of sulfur were observed between lone ion (GP) and ion pair IL(SMD) model systems, indicating that ion-ion interactions do not strongly influence individual atomic charges.

Introduction: Two forms of diabetes can be distinguished during pregnancy: gestational diabetes and pregestational diabetes, which exists prior to pregnancy. In young women, the most common form of pregestational diabetes is type 1 diabetes (T1D). Regarding the decreasing age of sexual initiation and health risks for the mother and child related to hyperglycemia, it is essential that adolescents with T1D possess proper knowledge of pregnancy planning and diabetes management in case of pregnancy. Preconception counseling in adolescent patients with T1D remains a challenge for the whole therapeutic team., Aim of the Study: Assessing the awareness of consequences of uncontrolled diabetes on the course of pregnancy and fetal development among patients with T1D., Material and Methods: The study was carried out in the group of 70 patients with T1D, aaged 15-18 years. The survey was consisted of 25 questions regarding health status, lifestyle, the knowledge of self-management of diabetes and the impact of diabetes on pregnancy and fetal development. Respondents were asked to indicate the sources of information from which they had gianed knowledge about the aforesaid issues. The data obtained were statistically analyzed., Results: 20% (n=14) of respondents declared sexual activity. In the group of sexually active patients, in 50% (n=7) last HbA1c level, reported by subjects, was between 7.5-9%, and in 21.4% (n=3) >9%. The patients were aware of the consequences of uncontrolled diabetes on fetal development, however their knowledge was unsatisfactory. Surveyed adolescents indicated metabolic disorders (61.4 %, n=43), central nervous system malformations (55.7%, n=39) and heart defects (47.1%, n=33) as the most frequent complications. The respondents gathered knowledge mainly from a diabetologist (40%, n=28) and the Internet (40%, n=28). The majority of patients stated that preconception care should be provided by a diabetologist (88.6%, n=62) or a gynecologist (70%, n=49)., Conclusion: In spite of continuous diabetes care, adolescents with T1D do not possess sufficient knowledge regarding the consequences of hyperglycemia during pregnancy. This study has emphasized the need for including reproductive health issues in diabetes education addressed to adolescent patients., (© Polish Society for Pediatric Endocrinology and Diabetology.)

A number of ionic liquids (ILs) with economically attractive production costs have recently received growing interest as media for the delignification of a variety of lignocellulosic feedstocks. Here we demonstrate the use of these low-cost protic ILs in the deconstruction of lignocellulosic biomass (Ionosolv pretreatment), yielding cellulose and a purified lignin. In the most generic process, the protic ionic liquid is synthesized by accurate combination of aqueous acid and amine base. The water content is adjusted subsequently. For the delignification, the biomass is placed into a vessel with IL solution at elevated temperatures to dissolve the lignin and hemicellulose, leaving a cellulose-rich pulp ready for saccharification (hydrolysis to fermentable sugars). The lignin is later precipitated from the IL by the addition of water and recovered as a solid. The removal of the added water regenerates the ionic liquid, which can be reused multiple times. This protocol is useful to investigate the significant potential of protic ILs for use in commercial biomass pretreatment/lignin fractionation for producing biofuels or renewable chemicals and materials.

IL-33 is an IL-1 cytokine family member, with ability to induce both Th1 and Th2 immune responses. It binds to ST2 receptor, whose deficiency is associated with enhanced inflammatory response. The most recent studies have shown the immunoregulatory effect of IL-33 on Tregs in animal models. As type 1 diabetes is an autoimmune, inflammatory disease, where Treg defects have been described, we aimed to analyze the in vitro influence of recombinant IL-33 on quantitative properties of regulatory CD4 + CD25 high FOXP3 + T cells. CD4 + CD25 high FOXP3 + as well as CD4 + CD25 high FOXP3 + ST2 + Tregs were analyzed by flow cytometry. In a group of patients with type 1 diabetes in vitro IL-33 treatment induced regulatory CD4 + CD25 high FOXP3 + cell frequencies as well as upregulating the surface expression of ST2 molecule. In addition, the number of CD4 + CD25 high FOXP3 + cells carrying ST2 receptor increased significantly. Similar effect was observed in case of the FOXP3 expression. We did not observe any significant changes in IL-33 treated cells of healthy controls. The level of ST2 was higher in serum of patients with type 1 diabetes in comparison to their healthy counterparts. We propose that IL-33 becomes an additional immunostimulatory factor used to induce Treg expansion in future clinical trials of adoptive therapy in type 1 diabetes.

Introduction: Congenital hyperinsulinism of Infancy (CHI) comprises heterogenic defects of insulin secretion with diverse molecular aetiology, histological features, severity of symptoms, and response to pharmacotherapy. The study aimed to establish the first clinical characteristics of Polish patients with CHI and to propose a novel clinical algorithm allowing the prioritisation of genetic and radiology studies, based on patient's characteristics and response to pharmacotherapy., Material and Methods: Thirty-one patients with CHI were recruited from five reference centres in Poland. Clinical and biochemical parameters were statistically evaluated and compared to those of a control group (n = 30)., Results: CHI predisposes to increased birth weight (p = 0.004), lower Apgar score (p = 0.004), perinatal complications (74%), and neurological implications (48%). Diagnostic process and therapy were inconsistent. A trial of pharmacotherapy was applied in 21 patients (68%), and diagnostic imaging with 18F-L-DOPA PET was performed in only 3. Eighteen patients (58%) were surgically treated, including 8 infants (44%) aged less than 2 months. Depending on the type of resection, further hypoglycaemia was observed postoperatively in 50% (n = 9) and hyperglycaemia in 39% (n = 7) of cases. Based on foregoing results, a clinical algorithm was proposed., Conclusions: Standardisation of clinical management with the use of pharmacotherapy, genetic screening, and diagnostic imaging will allow the optimisation of therapy and minimisation of treatment complications.