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Disease modeling of neuromuscular disorders, such as amyotrophic lateral sclerosis (ALS), is hindered by limited accessibility of affected cells. This problem can be overcome by generation of human induced pluripotent stem cells (hiPSC), which can be then differentiated into required cells. Here, we describe the detailed protocol of hiPSC establishment from peripheral blood mononuclear cells (PBMC) of two ALS patients with detected expansion of G4C2 (GGGGCC) repeats in the first intron of C9ORF72 gene, known to be linked with the most common form of familial ALS.Successful PBMC reprogramming with non-integrating Sendai vectors was confirmed by expression of pluripotency markers: OCT4, NANOG, SSEA4, and TRA-1-60 in obtained hiPSC and their ability to differentiate into cells of three germ layers.The generated ALS-patient-specific hiPSC create a possibility for deciphering molecular basis of this devastating neuromuscular disease., (© 2024. The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature.)

In this study, the activation energy and ionic conductivity of the Li 6 PS 5 Cl material for all-solid-state batteries were investigated using solid-state nuclear magnetic resonance (NMR) spectroscopy and electrochemical impedance spectroscopy (EIS). The results show that the activation energy values estimated from nuclear relaxation rates are significantly lower than those obtained from impedance measurements. The total ionic conductivities for long-range lithium diffusion in Li 6 PS 5 Cl calculated from EIS studies depend on the crystal size and unit cell parameter. The study also presents a new sample preparation method for measuring activation energy using temperature-dependent EIS and compares the results with the solid-state NMR data. The activation energy for a thin-film sample is equivalent to the long-range lithium dynamics estimated from NMR measurements, indicating the presence of additional limiting processes in thick pellets. Additionally, a theoretical model of Li-ion hopping based on results obtained using density-functional theory methods in comparison with experimental findings was discussed. Overall, the study emphasizes the importance of sample preparation methods in determining accurate activation energy and ionic conductivity values for solid-state lithium batteries and the significance of solid-state electrolyte thickness in new solid-state battery design for faster Li-ion diffusion.

Purpose: to determine management problems of ovarian masses in girls in order to form a baseline for prospective randomized studies of the established topics and quality improvement of our management., Materials and Methods: We performed a national analysis of clinical aspects of ovarian masses in girls operated on in Poland, analyzed retrospectively medical files of all consecutive patients aged 0-18 who underwent surgeries for ovarian lesions between 2012 and 2017 at 17 pediatric surgical departments and complemented the analysis with a scoping review of a recent primary research related to ovarian masses in children., Results: The study group comprised 595 patients. Forty-four (7.39%) girls were diagnosed with malignant tumors. The overall preservation rate was 64.54%. The analysis revealed that positive tumor markers (OR = 10.3), lesions larger than 6 cm (OR = 4.17) and solid mass on ultrasound examination (OR = 5.34) are interdependent variables differentiating malignant tumors from non-malignant lesions (X42 = 79.1; p = 0.00000). Our scoping review revealed 10 major branches of research within the topic of ovarian masses in pediatric population., Conclusions: We have developed an overview of the field with the emphasis on the local environment. Our next step is a multi-institutional prospective study of a quality improvement project implementation based on the obtained knowledge.

Proton tunneling in the hydrogen-bonded imidazole-imidazolium complex ion has been studied theoretically. Ab initio CASSCF/6-311++G(d,p) calculations concerning geometry optimization and vibrational frequencies have been carried out for equilibrium and transition state structures of the system. Two-dimensional double-well model potentials were constructed on the basis of ab initio results and used to analyze the proton dynamics in the hydrogen bond and the influence of the excitation of low-frequency hydrogen-bond vibrations on the proton tunneling splittings. The energy of tunneling-split vibrational sublevels of the high-frequency tunneling mode have been calculated for its ground and first excited vibrational state for the series of excitations of the coupled low-frequency intramolecular hydrogen-bond modes. The promoting and suppressing effect of the low-frequency modes on the proton splittings was shown in the ground and first excited vibrational state of the tunneling mode. The vibrational sublevels form the two separate semicontinuous bands between which the absorption transitions may occur. This mechanism explains the experimentally observed splitting and doublet-component broadening of the high-frequency N-H stretching infrared (IR) absorption band.

KBG syndrome is a neurodevelopmental autosomal dominant disorder characterized by short stature, macrodontia, developmental delay, behavioral problems, speech delay and delayed closing of fontanels. Most patients with KBG syndrome are found to have a mutation in the ANKRD11 gene or a chromosomal rearrangement involving this gene. We hereby present clinical evaluations of 23 patients aged 4 months to 26 years manifesting clinical features of KBG syndrome. Mutation analysis in the patients was performed using panel or exome sequencing and array CGH. Besides possessing dysmorphic features typical of the KBG syndrome, nearly all patients had psychomotor hyperactivity (86%), 81% had delayed speech, 61% had poor weight gain, 56% had delayed closure of fontanel and 56% had a hoarse voice. Macrodontia and a height range of -1 SDs to -2 SDs were noted in about half of the patients; only two patients presented with short stature below -3 SDs. The fact that wide, delayed closing fontanels were observed in more than half of our patients with KBG syndrome confirms the role of the ANKRD11 gene in skull formation and suture fusion. This clinical feature could be key to the diagnosis of KBG syndrome, especially in young children. Hoarse voice is a previously undescribed phenotype of KBG syndrome and could further reinforce clinical diagnosis.

In this work we present the comparison study of Adenine and Thymine crystals based on the hydrogen bond dynamics. The ab initio molecular dynamics have been used as the base for the further studied interactions observed inside crystals. The generated power spectra, as well as the fluctuation of the interaction energies, showed large differences between hydrogen bond networks in the considered crystals. The analysis of intermolecular interactions have been done base on the reactivity descriptors as well frontiers orbitals along trajectories. The main results showed that in adenine crystals the intermolecular interactions have three directions and fluctuate, while in the thymine crystal have only two directions and are weak but stable. These results explain also on the difference between adenine and thymine melting temperature., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

In this work, we present the comparison study of guanine and cytosine crystals based on the hydrogen bond (HB) dynamics. The ab initio molecular dynamics gave us a base for detailed analysis. The analysis of the trajectories by power spectrum generation, as well as the fluctuation of the interaction energies, showed large differences between HB networks in the considered crystals. The charge flow is present in the guanine molecule which forms the flat surfaces in the crystals. In the cytosine zigzag structure, the charge flow is blocked. The interaction energy is significantly less stabilizing in the cytosine structure than in the guanine. Finally, the possible influence of charge transfer on the melting temperature has been discussed.

Monoamine oxidase A (MAO A) is a well-known enzyme responsible for the oxidative deamination of several important monoaminergic neurotransmitters. The rate-limiting step of amine decomposition is hydride anion transfer from the substrate α-CH2 group to the N5 atom of the flavin cofactor moiety. In this work, we focus on MAO A-catalyzed benzylamine decomposition in order to elucidate nuclear quantum effects through the calculation of the hydrogen/deuterium (H/D) kinetic isotope effect. The rate-limiting step of the reaction was simulated using a multiscale approach at the empirical valence bond (EVB) level. We applied path integral quantization using the quantum classical path method (QCP) for the substrate benzylamine as well as the MAO cofactor flavin adenine dinucleotide. The calculated H/D kinetic isotope effect of 6.5 ± 1.4 is in reasonable agreement with the available experimental values.

In the article mentioned above an author's name was misspelled. The correct author name reads as follows: Leila Maria Lopes-Bezerra. We apologize for the inconvenience.

Sporotrichosis is an infection of the skin caused by traumatic inoculation of the fungus Sporothrix schenckii. Definitive diagnosis relies on direct visualization of the fungus or its isolation on culture medium, although both have low sensitivity. Alternatively, the detection of the antibody response offers a more rapid alternative for diagnosis. Although the available immunoassays possess good sensitivity and specificity, cross-reactivity is still a problem. This study aimed to evaluate the effect of sodium metaperiodate and 6 M urea solutions on the serological diagnosis of sporotrichosis using an enzyme-linked immunosorbent assay (ELISA) test. Ninety-six-well plates were sensitized with exoantigens from the yeast phase of S. schenckii. Sera of patients with confirmed sporotrichosis, sera of patients with paracoccidioidomycosis, and sera of individuals with a sporotrichin-negative skin test were tested. Two strategies were used; the first consisted of treating the antigen with sodium metaperiodate solution for different incubation times, and the second consisted of treating the serum with 6 M urea solution for different incubation times. ROC curve analysis revealed that the best discrimination parameters were obtained using 6 M urea solution incubated for 5 min and serum dilution at 1/600. The use of 6 M urea solution improves the performance of the ELISA test in the diagnosis of sporotrichosis.

The Neurofascins (NFASCs) are a family of proteins encoded by alternative transcripts of NFASC that cooperate in the assembly of the node of Ranvier in myelinated nerves. Differential expression of NFASC in neurons and glia presents a remarkable example of cell-type specific expression of protein isoforms with a common overall function. In mice there are three NFASC isoforms: Nfasc186 and Nfasc140, located in the axonal membrane at the node of Ranvier, and Nfasc155, a glial component of the paranodal axoglial junction. Nfasc186 and Nfasc155 are the major isoforms at mature nodes and paranodes, respectively. Conditional deletion of the glial isoform Nfasc155 in mice causes severe motor coordination defects and death at 16-17 days after birth. We describe a proband with severe congenital hypotonia, contractures of fingers and toes, and no reaction to touch or pain. Whole exome sequencing revealed a homozygous NFASC variant chr1:204953187-C>T (rs755160624). The variant creates a premature stop codon in 3 out of four NFASC human transcripts and is predicted to specifically eliminate Nfasc155 leaving neuronal Neurofascin intact. The selective absence of Nfasc155 and disruption of the paranodal junction was confirmed by an immunofluorescent study of skin biopsies from the patient versus control. We propose that the disease in our proband is the first reported example of genetic deficiency of glial Neurofascin isoforms in humans and that the severity of the condition reflects the importance of the Nfasc155 in forming paranodal axoglial junctions and in determining the structure and function of the node of Ranvier.

Lithium primary cells are commonly used in applications where high energy density and low self-discharge are the most important factors. This include small coin cells for electronics, power backup batteries for complementary metal-oxide-semiconductor memory or as a long-term emergency power source. In our study we present a fast, electrochemical method of the positive electrode preparation for lithium primary cells. The influence of the current density and oxygen presence in a solution on the preparation of the electrode and thus its electrochemical behavior is examined. Electrode compositions were characterized by X-ray photoelectron spectroscopy (XPS). The prepared electrodes may be used in Li cells as competition to Zn-MnO₂ primary batteries.

Lithium-ion cells are currently very promising electrochemical power sources. New high-capacity electrodes made from silicon are currently under intensive study. As well as its high capacity, silicon undergoes a significant volume increase (up to 300%) during lithiation. This leads to the generation of internal stresses and fast cell degradation due to active material pulverization and separation from the current collector. Stress formation and its effect on silicon lithiation has been theoretically investigated by many researchers. It has been shown that internal compressive stress can slow down or stop silicon lithiation. In our study we applied external stress to an electrode active layer and measured the cell electrochemical parameters: capacity, cycle life, and charge transfer resistance. In contrast with theoretical estimations we observed an increase in capacity and cycle life when high compressive stress was applied. We believe this behavior is related to stress-induced lithiation front slowdown, which entails a longer stress relaxation period and as a consequence improves the cell parameters., Competing Interests: There are no conflicts to declare., (This journal is © The Royal Society of Chemistry.)

Hydrogen bond networks in uracil, 1-methyluracil and 1-methyl-4-thiouracil were studied by ab initio molecular dynamics as well as analysis of the orbital interactions. The power spectra calculated by ab initio molecular dynamics for atoms involved in hydrogen bonds were analyzed. We calculated spectra by using anharmonic approximation based on the autocorrelation function of the atom positions obtained from the Born-Oppenheimer simulations. Our results show the differences between hydrogen bond networks in uracil and its methylated derivatives. The studied methylated derivatives, 1-methyluracil as well as 1-methyl-4-thiouracil, form dimeric structures in the crystal phase, while uracil does not form that kind of structures. The presence of sulfur atom instead oxygen atom reflects weakness of the hydrogen bonds that build dimers., (Copyright © 2018 Elsevier B.V. All rights reserved.)

In this study we present complementary computational and experimental studies of hydrogen bond interaction in crystalline benzoic acid and its deuterated and partially deuterated derivatives. The experimental part of the presented work includes preparation of partially deuterated samples and measurement of attenuated total reflection (ATR)-FTIR spectra. Analysis of the geometrical parameters and time course of dipole moment of crystalline benzoic acid and its deuterated and partially deuterated derivatives by Born-Oppenheimer molecular dynamics (BOMD) enabled us to deeply analyze the IR spectra. Presented simulations based on BOMD gave us opportunity to investigate individual motion and its contribution to the IR spectra. The band contours calculated using Fourier transform of autocorrelation function are in quantitative agreement with the experimental spectra. Characterization of single bands was carried out by "normal coordinate analysis". The salient point of our study is a comparison of the spectra of the deuterated and partially deuterated crystalline benzoic acid with that of the nondeuterated one. Furthermore, we have applied the principal component analysis for analysis of the number of components in partially deuterated systems. In this study, we reveal that the arrangements of hydrogen and deuterium atoms in partially deuterated samples are random.

In this study, the proton dynamics of hydrogen bonds for two forms of crystalline aspirin was investigated by the Born-Oppenheimer molecular dynamics (BOMD) method. Analysis of the geometrical parameters of hydrogen bonds using BOMD reveals significant differences in hydrogen bonding between the two crystalline forms of aspirin, Form I and Form II. Analysis of the trajectory for Form I shows spontaneous proton transfer in cyclic dimers, which is absent in Form II. Quantization of the O-H stretching modes allows a detailed discussion on the strength of hydrogen-bonding interactions. The focal point of our study is examination of the hydrogen bond characteristics in the crystal structure and clarification of the influence of hydrogen bonding on the presence of the two crystalline forms of aspirin. In the BOMD method, thermal motions were taken into account. Solving the Schrödinger equation for the snapshots of 2D proton potentials, extracted from MD, gives the best agreement with IR spectra. The character of medium-strong hydrogen bonds in Form I of aspirin was compared with that of weaker hydrogen bonds in aspirin Form II. Two proton minima are present in the potential function for the hydrogen bonds in Form I. The band contours, calculated by using one- and two-dimensional O-H quantization, reflect the differences in the hydrogen bond strengths between the two crystalline forms of aspirin, as well as the strong hydrogen bonding in the cyclic dimers of Form I and the medium-strong hydrogen bonding in Form II.

Interaction energies, molecular structure and vibrational frequencies of the binary complex formed between H(D)Cl and dimethyl ether have been obtained using quantum-chemical methods. Equilibrium and vibrationally averaged structures, harmonic and anharmonic wavenumbers of the complex and its deuterated isotopomer were calculated using harmonic and anharmonic second-order perturbation theory procedures with Density Functional Theory B3LYP and B2PLYP-D and ab initio Møller-Plesset second-order methods, and a 6-311++G(3d,3p) basis set. A phenomenological model describing anharmonic-type vibrational couplings within hydrogen bonds was developed to explain the unique broadening and fine structure, as well as the isotope effect of the Cl-H and Cl-D stretching IR absorption bands in the gaseous complexes with dimethyl ether, as an effect of hydrogen bond formation. Simulations of the rovibrational structure of the Cl-H and Cl-D stretching bands were performed and the results were compared with experimental spectra.

We studied proton dynamics of a hydrogen bonds of the crystalline l-ascorbic acid. Our approach was based on the Car-Parrinello molecular dynamics. The focal point of our study was simulation of the infrared spectra of l-ascorbic acid associated with the O-H stretching modes that are very sensitive to the strength of hydrogen bonding. In the l-ascorbic acid there are four kinds of hydrogen bonds. We calculated their spectra by using anharmonic approximation and the time course of the dipole moment function as obtained from the Car-Parrinello simulation. The quantization of the nuclear motion of the protons was made to perform detailed analysis of strength and properties of hydrogen bonds. We presented double minimum proton potentials with small value of barriers for medium-strong hydrogen bonds. We have also shown the difference character of medium-strong hydrogen bonds compared to weaker hydrogen bonds in the l-ascorbic acid.

Aim: Evaluation of diagnostic and treatment procedures in children with cystic fibrosis (CF) operated on because of meconium ileus (MI)., Material and Methods: The authors retrospectively reviewed the documentation of 10 CF newborn patients operated on in the years 2000-2014 because of MI. In prenatal ultrasound (US) examinations, suspicion of bowel abnormalities was raised in 2 cases, even though all the 10 mothers had a minimum of 3 US examinations during pregnancy. The mean gestational age of the newborns was 39.2 weeks - 36-41 weeks), their mean birth weight 3472g (2560-4550 g). Family history of CF was positive in two patients. Genetic testing was performed in all the children operated on., Results: In all the children operated on, mutations in both alleles of the CFTR gene were found. Five patients were F508del homozygotic, 4 were heterozygotic for this mutation, one had another mutation. Sweat tests were positive in all the children. Abdominal distention was observed in 9 patients, vomiting and retention of gastric contents in 5. In 8 children meconium was not passed at all. 2 children passed a small amount of viscid meconium. Before the operation, rectal saline washouts were done in 5 newborns. Five patients were operated on during the first day of life, four on the second day and one on the third day of life. Intra-operatively a simple form of MI was diagnosed in 8 cases, a complicated form in 2 cases. In patients with the simple form of MI, a Bishop-Koop stoma was created after the evacuation of meconium. Two of these children needed a resection of some centimetres of dilated terminal ileum with doubtful viability. In newborns with the complicated form of MI, the treatment was individualized, always with stoma formation. The time of postoperative meconium evacuation through enterostomy ranged from 6 to 15 days. Enteral feeding was started on average on the 9th day postoperatively. The mean hospital stay was 22.9 days. In 8 children the stoma was taken out at the mean age of 19.4 months, in one patient the stoma closed spontaneously. No disturbances in electrolyte balance or excessive fluid loss, nor any body weight deficits connected with the stoma were observed. There were no complications during stoma closure. All the patients are alive. The time of observation ranges from 7 to 146 months (average 95 months). All the patients currently present respiratory symptoms, have pancreatic insufficiency and need pancreatic enzyme supplementation. Seven do not, however, have body weight and height deficits. All the children with weight and height deficits have abnormal liver function tests. During observation two patients had MI equivalent symptoms, which was resolved by conservative treatment., Conclusions: 1. In every case of intra-operative diagnosis of MI, it is necessary to perform genetic testing and sweat tests to confirm or exclude CF. 2. Mechanical intra-operative decompression of the bowel from inspissated meconium with a temporary stoma, which makes the continuation of bowel decompression possible in the postoperative period, is an effective treatment in children with MI. 3. The Bishop-Koop stoma, permitting the passage through the whole gastrointestinal tract, is a safe option. In our material, no complications of this stoma, such as stoma care problems or dyselectrolithemia were observed. 4. The decision of stoma closure in children with MI and CF should be delayed until the moment of introducing a broadened diet and should be undertaken together with a pediatrician who is a specialist in CF therapy. .

Aim of the Study: To analyze changes of the cephalic index (CI) values in patients with sagittal synostosis operated on in the Department of Surgery of Children and Adolescents of the Institute of Mother and Child in Warsaw., Material and Methods: The study group consisted of 14 consecutive cases (11 boys and 3 girls). In 8 patients younger than 3 months of age (1st subgroup) the modified reversed-pi technique (craniotomy in the shape of the reversed Greek π letter) was utilized. In 6 older infants and in patients with severe head deformity - the radical cranial vault reconstructions were performed (2nd subgroup). The statistical analysis of the preoperative (preop) and postoperative (postop) cephalic index (CI) values was done., Results: For the entire study group the preop CI values ranged from 61 to 77 (mean 68.14, median 68.5) and postop CIs from 73 to 89 (mean 77.64, median 76). In the 1st subgroup the preop CIs ranged from 63 to 77 (mean 69.625, median 69) whereas the postop CIs from 69 to 89 (mean 78.125, median 76.5). In the 2nd subgroup the preop CI values ranged from 61 to 72 (mean 66.1667, median 66) and postop from 73 to 81 (mean 77, median 77). All the differences were statistically significant., Conclusion: The values of cephalic index increased after both types of procedures. It signifies the correction of dolichocephaly in both subgroups of the patients. However, because of the small number of operated children, further studies are necessary.

The aim of this study was to perform calculations using the method of Car-Parrinello molecular dynamics, leading to the optimized geometry of the molecules of 1,4-benzenedicarboxylic acid (terephthalic acid) in crystals, for the hydrogen form and three variants of substitution of deuterium atoms inside a carboxyl group. Based on the results, trajectories and dipole moments were calculated, what makes possible to simulate vibrations in different systems, and to make calculation of theoretical infrared spectra and atomic power spectra. Theoretical results were compared with the experimental spectra, which verifies the correctness of the method and also was compared with the results obtained by quantum-mechanical calculations using DFT for the isolated dimer. Comparison of the spectra of different forms, allowed for in-depth analysis of the effect of isotopic substitution on the frequency of vibrations and shapes of bands, and confirm the presence of possible coupling effects and intra- and intermolecular interactions. Comparison with the DFT results for the dimer show influence of the crystal structure on the spectra., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Twenty-four children with giant congenital melanocytic nevi underwent brain MRI at 1.5 T scanner. Melanin deposits in the brain were found in seven children (29.2%) located in temporal lobes, thalamus, cerebellum, and pons. One patient showed leptomeningeal involvement. Six patients were asymptomatic, and one had epilepsy. As opposed to previous reports, localization of skin nevi on anterior part of trunk was correlated to central nervous system involvement. In all patients with brain involvement skin nevi showed picture of compound nevus with neurofibromatic component., (© 2013.)

The nature of medium strong intra- and intermolecular hydrogen bonding in 2-hydroxy-5-nitrobenzamide in the crystal phase was examined by infrared spectroscopy and Car-Parrinello molecular dynamics simulation. The focal point of our study was the part of the infrared spectra associated with the O-H and N-H stretching modes that are very sensitive to the strength of hydrogen bonding. For spectra calculations we used an isolated dimer and the fully periodic crystal environment. We calculated the spectra by using harmonic approximation, the time course of the dipole moment function as obtained from the Car-Parrinello simulation, and the quantization of the nuclear motion of the proton for an instantaneous snapshot of the structures in one and two dimensions. Although quantitative assessment of the agreement between the computed and experimental band contour is difficult due to the fact that the experimental band is very broad, we feel that the most reasonable qualitative agreement with the experiment is obtained from snapshot structures and two-dimensional quantization of the proton motion. We have also critically examined the methods of constructing the one-dimensional proton potential. Perspectives are given for the treatment of nuclear quantum effects in biocatalysis.

Unlabelled: Progress in perinatology and neonatal intensive care led to surgical treatment of premature infants born with low (<1500 g) and extremely low (<1000 g) birth weight., Aim: Evaluation of surgical treatment in the group of neonates with very low birth weight (<1500 g) and extremely low birth weight (<1000 g)., Material and Methods: In the years 2000-2009 in the Department of Paediatric Surgery in the Institute of Mother and Child, 617 neonates underwent surgical treatment, 101 of them (16%) were born with very low or extremely low birth weight. In the analyzed group the birth weight ranged from 450 g to 1500 g (mean 952 g), gestational age ranged from 23 weeks to 32 weeks (mean 27 weeks). Fifty four patients (53%) were operated with the weight <1000 g. Indications for surgery were of two categories: pathologies related to prematurity and congenital defects. The extend of surgical intervention is presented. Additional pathologies influencing prognosis such as respiratory distress syndrome, haemodynamic ductus arteriosus, intraventricular haemorrhagia, multiple congenital defects and genetic disorders were also taken into consideration. Mortality in the entire group of patients was evaluated in relation to the birth weight, gestational age, reasons for surgical treatment and additional risk factors., Results: Pathologies related to prematurity were indications for surgical treatment in 70 patients: perforation of the bowel in the course of necrotizing enterocolitis - NEC (28 patients), spontaneous intestinal perforation - SIP (32), gastric perforation - GP (4), meconium obstruction - MO (3) posthemorrhagic hydrocephalus - PH (3). In the remaining 31 neonates the following congenital defects were operated: inguinal hernia (10 patients), oesophageal atresia (8), anal atresia (2), torsion of the bowel (2), bowel atresia (3), hernia of the umbilical cord (3), ruptured omphalocele (1), myelomeningocele (2). In the entire group of 101 premature infants, 99 patients (98%) had respiratory distress syndrome, 56 (56%) required the closure of ductus arteriosus , in 55 patients (55%) intraventricular haemorrhage from II to IV degree was confirmed. In total thirty patients died. Twenty one of them were ELBW neonates. None of the patients died during the operation or within the first postoperative day. Mortality rate in the group with the weight <1000 g was 38%, in the group with the weight 1000 g-1500 g it was 19%. Highest mortality was observed in the patients with oesophageal atresia (62%) In this group the biggest number of additional anomalies or other genetic disorders was found. Twenty infants died (31%) in the group of 64 neonates with perforations. Mortality rate in the groups with the weight <1000 g and 1000 g-1500 g it was 34% and 26% respectively. Our results confirmed the significant difference between mortality in NEC - 65% and in SIP - 19.5% . The remaining 5 deaths were related both to extreme multiorgan prematurity and severe congenital defects., Conclusion: The most frequent indications for surgery in premature neonates (VLBW and ELBW) are acquired pathologies which are related to premature multiorgan insufficiency: perforations in the course of ischaemic or inflamatory changes in the bowel (NEC, SIP), intestinal obstruction related to functional insufficiency of alimentary tract (MO) and posthaemorrhagic hydrocephalus. Congenital anomalies constitute 30% of indications for surgical treatment in this group of patients. Neonates born with low or extremely low birth weight are in the group of patients with the highest intraoperative risk. There is herefore a need to create well equipped, interdisciplinary centres employing paediatric surgeons, anaesthesiologists and neonatologists experienced in treatment of extremely premature neonates.

In the present study we have analyzed hydrogen bonding in dimer and trimer of oxalic acid, based on a recently proposed charge and energy decomposition scheme (ETS-NOCV). In the case of a dimer, two conformations, α and β, were considered. The deformation density contributions originating from NOCV's revealed that the formation of hydrogen bonding is associated with the electronic charge deformation in both the σ-(Δρ(σ)) and π-networks (Δρ(π)). It was demonstrated that σ-donation is realized by electron transfer from the lone pair of oxygen on one monomer into the empty P*(H-O) orbital of the second oxalic acid fragment. In addition, a covalent contribution is observed by the density transfer from hydrogen of H-O group in one oxalic acid monomer to the oxygen atom of the second fragment. The resonance assisted component (Δρ(π)), is based on the transfer of electron density from the π-orbital localized on the oxygen of OH on one oxalic acid monomer to the oxygen atom of the other fragment. ETS-NOCV allowed to conclude that the σ(O---HO) component is roughly eight times as important as π (RAHB) contribution in terms of energetic estimation. The electrostatic factor (ΔE(elstat)) is equally as important as orbital interaction term (ΔE(orb)). Finally, comparing β-dimer of oxalic acid with trimer we found practically no difference concerning each of the O---HO bonds, neither qualitative nor quantitative.

Unlabelled: Myelomeningocoele is a serious congenital deformity requiring multidisciplinary medical care, and parent' s aware cooperation. The accessibility and popularity of foetal ultrasonography enables early prenatal diagnosis which makes possible optimal management during pregnancy and treatment after birth., Aim: Evaluation of the treatment of newborns with myelomeningocoele in the last 20 years in relation to the changes in diagnostics and operative procedures., Material and Methods: The evaluation was performed in 114 newborns, operated on because of myelomeningocoele between 1986-1992 (group I) and 1999-2005 (group II). Prenatal diagnosis, the term and kind of labour, anatomical description of deformity, the level of spinal cord damage were analyzed. Evaluation also included: the term and kind of operation, the presence of active hydrocephalus and its association with the anatomical picture of deformity, the strategy of shunt implantation and the number of deaths during the first hospitalization. Orthopaedic and urological problems were not evaluated., Results: During twenty years, reduction in the number of patients with myelomeningocoele was not observed, despite the introduction of the programme of women' s folic acid prophylaxis. In two analyzed periods there were 8 new cases treated each year. The number of prenatal diagnoses increased: in group I prenatal diagnosis was made only in 8 cases (14%), in group II in 25 cases (44%), but in most cases diagnosis was established late (in the last 3 months of pregnancy). Analysis of different anatomical forms of the deformity showed, that the number of thoracic myelomeningocoeles decreased from 32 cases in group I to 18 cases in group II. In both groups operation was performed during 24 hours following admission of the newborn to the Institute. The surgical technique has not change: large skin defects were closed with the use of rhomboidal muscle and skin flap (Limberg' s plasty). The number of complications in wound healing decreased from 26.4% to 7%. Active hydrocephalus was confirmed in 49 of 57 patients in group I (85%) and in 46 of 57 patients in group II (80%). Patients without active hydrocephalus (group I - 8 children, group II - 11 children) presented lumbo-sacral or sacral localization of defect. In the years 1999-2005 (group II), contemporary with the operation of myelomeningocoele, Rickham reservoir was implanted in 11 newborns with massive hydrocephalus. The shunt was added after the confirmation of active hydrocephalus and after a negative result of cerebrospinal fluid culture. Decrease of deaths during the first hospitalization was observed (8 in group I, 2 in group II). The principal cause of death in both groups was inborn infection., Conclusions: 1. On the basis of own investigations the authors did not find positive results of introducing of women' s folic acid prophylaxis programme in Poland in the decrease of number of newborns, treated surgically on spina bifida in the Institute of Mother and Child. In the last years more often sacral and lumbo-sacral type of defects and, in consequence, more patients without active hydrocephalus are observed, which may be the result of parent' s decision about terminating of pregnancy in cases of high (thoracic) deformity. 2. In the years 1999-2005 the increasing number of prenatal ultrasonography is observed. However, the prenatal diagnosis of myelomeningocele in most cases is established late (in the third trimester). 3. Significant fall in the number of local and general complications of treatment is responsible for the decrease in mortality of the newborns with myelomeningoloele. The method of closing large skin defects using cutaneo-muscular rhomboidal flap, introduced in 1986, showed to be very effective in the prophylaxis of complications of wound healing. 4. Congenital intrauterine infection was the main cause of death of neonates with this deformity, what points to the necessity of regular bacteriological and serological diagnostics during pregnancy and proper perinatal treatment.

Ab initio CIS/6-311++G(d,p) calculations of geometry and vibrational frequencies have been carried out in the A state of tropolone. The grids of potential energy surfaces along the coordinates of high frequency tunneling vibration and the low-frequency coupled vibration have been calculated. Two-dimensional model potentials, formed from symmetric mode coupling potential and squeezed double well potential, have been fitted to the calculated potential energy surfaces and used to analyze proton dynamics. The tunneling splittings for different vibrationally excited states have been calculated and compared with the available experimental data. The model potential energy surfaces, based on the CIS/6-311++G(d,p) calculations, give good estimation of the tunneling energy splittings in the vibrationally ground and excited states of tropolone and explain monotonic decrease in tunneling splittings with the excitation of low-frequency out-of-plane modes and increase in the tunneling splittings with the excitation of low-frequency planar modes.

Theoretical simulation of the band shape and fine structure of the N-H(D) stretching band is presented for 1-methyluracil and its deuterated derivative taking into account anharmonic coupling between the high-frequency N-H(D) stretching and the low-frequency N...O stretching vibrations, resonance interaction between two equivalent hydrogen bonds in the dimer, anharmonicity of the potentials for the low-frequency vibrations in the ground and excited state of the N-H(D) stretching mode, Fermi resonance between the N-H(D) stretching and the first overtone of the N-H(D) bending vibrations, and electrical anharmonicity. The effect of deuteration has been successfully reproduced by our model calculations. Infrared, far-infrared, Raman, and low-frequency Raman spectra of the polycrystalline 1-methyluracil have been recorded. The geometry and experimental frequencies are compared with the results of harmonic and anharmonic B3LYP6-311++G(**) calculations.

Unlabelled: Inguinal hernia in preterm infants is a difficult therapeutic problem because of the risk of anaesthesia and the immaturity of the baby. Despite of high risk of perioperative complications surgical repair of the hernia should be considered before discharge., Aim: evaluation of the perioperative problems in the preterm infants with inguinal hernia., Material and Methods: evaluation was performed in 21 preterm infants (gestational age 23-36 weeks, birth weight 450-1370 g) operated because of inguinal hernia between 2002-2007. Various problems related to prematurity were confirmed in all patients. Incarcerated hernia were operated on in 5 patients, in 16 neonates hernia repair was performed as elective surgery. In two of them, few weeks after unilateral hernia repair, operation on the other side was performed because of incarceration. Nine infants were qualified for general anaesthesia, fourteen for spinal anaesthesia. Indications for surgical treatment, method of anaesthesia and early postoperative results were analysed with reference to various problems connected with prematurity such as intraventricular haemorrhage and chronic lung disease., Results: inclusively 23 operations were performed in 21 infants (gestational age 36-47 weeks, weight 1130 -2750 g). Four of fourteen infants preliminary qualified for spinal anaesthesia turned into general anaesthesia. There were not any problems during the operations. Two patients required short ventilatory support (both with general anaesthesia because of incarcerated hernia). During a follow up examination recurrence of the hernia was not observed in any of the patients., Conclusion: despite of high risk of perioperative complications in preterm infants, surgical repair of the inguinal hernia should be considered before discharge. Spinal anaesthesia is a safe alternative for general anaesthesia in preterm infants especially for infants with chronic lung disease.

Theoretical model for vibrational interactions in the hydrogen-bonded dimer of benzoic acid is presented. The model takes into account anharmonic-type couplings between the high-frequency O-H and the low-frequency O[cdots, three dots, centered]O stretching vibrations in two hydrogen bonds, resonance interactions (Davydov coupling) between two hydrogen bonds in the dimer, and Fermi resonance between the O-H stretching fundamental and the first overtone of the O-H in-plane bending vibrations. The vibrational Hamiltonians and selection rules for the C(2h) geometry in the S(0) state and for the C(s) in-plane bent geometry in the S(1) state of the dimer are derived. The model is used for theoretical simulation of the O-H stretching IR absorption bands of benzoic acid dimers in the gas phase in the electronic ground and first excited singlet states. Ab initio CIS and CIS(D)6-311++G(d,p) calculations have been performed to determine geometry, frequencies, and excited state energies of benzoic acid dimer in the S(1) state.