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Subcutaneous implantable cardioverter-defibrillators are a modern alternative to classic cardioverter-defibrillators. Prior to implantation of the device, qualification based on assessment of the heart's specific rhythm shape during a resting electrocardiogram examination must be performed. In the presented case, a patient with a subcutaneous implantable cardioverter-defibrillator experienced numerous discharges during exercise. An exercise test was performed, which revealed a bundle branch block that appeared during exercise. The wide QRS complex was double- or triple-counted by the device, resulting in an inaccurate heart rate estimation and multiple discharges. Optimising the sensing vectors during exercise solved this problem.

PURPOSE: To perform comprehensive genotyping of TSC1 and TSC2 in a cohort of 94 infants with tuberous sclerosis complex (TSC) and correlate with clinical manifestations. METHODS: Infants were enrolled at age

Backgroud Drug-resistant epilepsy is the main risk factor for future intellectual disability in patients with tuberous sclerosis complex. Clinical epileptic seizures are often preceded by electroencephalographic changes, which provide an opportunity for preventive treatment. We evaluated the neuropsychologic and epilepsy outcomes at school age in children with tuberous sclerosis complex who received preventive antiepileptic treatment in infancy. Methods We performed a prospective, nonrandomized clinical trial with 14 infants diagnosed with tuberous sclerosis complex in whom serial electroencephalographic recordings were performed and preventive treatment with vigabatrin initiated when active epileptic discharges were detected. An age-matched control group consisted of 31 infants with tuberous sclerosis complex in whom treatment with vigabatrin was given only after onset of clinical seizures. Results of clinical assessment of epilepsy and cognitive outcomes were analyzed. Results All patients in the preventive group (n = 14) and 25 of 31 patients in the standard treatment group were followed through minimum age five years, median 8.8 and 8.0 years in the preventive and standard groups, respectively. The median intelligence quotient was 94 for the preventive group when compared with 46 for the standard group (P Conclusions This study provides evidence that preventive antiepileptic treatment in infants with tuberous sclerosis complex improves long-term epilepsy control and cognitive outcome at school age.

Objective To correlate fetal brain magnetic resonance imaging (MRI) findings with epilepsy characteristics and neurodevelopment at 2 years of age in children with tuberous sclerosis complex (TSC) to improve prenatal counseling. Study design This retrospective cohort study was performed in a collaboration between centers of the EPISTOP consortium. We included children with definite TSC, fetal MRIs, and available follow-up data at 2 years of age. A pediatric neuroradiologist masked to the patient's clinical characteristics evaluated all fetal MRIs. MRIs were categorized for each of the 10 brain lobes as score 0: no (sub)cortical lesions or doubt; score 1: a single small lesion; score 2: more than one small lesion or at least one large lesion (>5 mm). Neurologic manifestations were correlated to lesion sum scores. Results Forty-one children were included. Median gestational age at MRI was 33.3 weeks; (sub)cortical lesions were detected in 97.6%. Mean lesion sum score was 4.5. At 2 years, 58.5% of patients had epilepsy and 22% had drug-resistant epilepsy. Cognitive, language, and motor development were delayed in 38%, 81%, and 50% of patients, respectively. Autism spectrum disorder (ASD) was diagnosed in 20.5%. Fetal MRI lesion sum scores were significantly associated with cognitive and motor development, and with ASD diagnosis, but not with epilepsy characteristics. Conclusions Fetal cerebral lesion scores correlate with neurodevelopment and ASD at 2 years in children with TSC.

BackgroundEpilepsy develops in 70–90% of children with Tuberous Sclerosis Complex (TSC) and is often resistant to medication. Treatment with mTOR pathway inhibitors is an important therapeutic option in drug-resistant epilepsy associated with TSC. Our study evaluated the antiepileptic effect of rapamycin in the pediatric population of patients diagnosed with TSC.MethodsThis single center, open-label study evaluated safety and anti-epileptic efficacy of 12 months of rapamycin treatment in 32 patients aged from 11 months to 14 years with drug-resistant TSC- associated epilepsy.ResultsAfter the first 6 months of treatment, the improvement in seizure frequency, defined as at least a 50% reduction in the number of seizures per week compared to baseline, was seen in 18 individuals (56.25%). We observed no change in 12 individuals (37.5%) and worsening, defined as increase in the number of seizures—in 2 patients (6.25%). The overall improvement defined as at least a 50% reduction in seizure frequency was found in 65.6% of all patients after 12 months with 28% of patients obtaining complete remission. Another five patients experienced at least an 80% reduction in the frequency of seizures. Concomitant treatment with vigabatrin, and to a much lesser extent topiramate and levetiracetam, was an additional favorable prognostic factor for the success of the therapy. A linear relationship between the cumulative dose of rapamycin and its therapeutic effect was observed. The safety profile of the drug was satisfactory. In none of the observed cases did the adverse events reach the level that required withdrawal of the rapamycin treatment. The reason for dropouts was insufficient drug efficacy in 3 cases.ConclusionsLong-term use of rapamycin, especially in combination with vigabatrin, might be a beneficial therapeutic option in the treatment of drug-resistant epilepsy in children with TSC.

OBJECTIVE: To evaluate the relationship between age at seizure onset and neurodevelopmental outcome at age 24 months in infants with TSC, as well as the effect on neurodevelopmental outcome of early versus conventional treatment of epileptic seizures with vigabatrin (80-150 mg/kg/day). METHODS: Infants with TSC, aged ≤4 months and without previous seizures were enrolled in a prospective study and closely followed with monthly video EEG and serial standardized neurodevelopmental testing (Bayley Scales of Infant Development and Autism Diagnostic Observation Schedule). RESULTS: Eighty infants were enrolled. At the age of 24 months testing identified risk of Autism Spectrum Disorder (ASD) in 24/80 children (30.0%), and developmental delay (DD) in 26/80 (32.5%). Children with epilepsy (51/80; 63.8%) had a higher risk of ASD (P = 0.02) and DD (P = 0.001). Overall, no child presented with moderate or severe DD at 24 months (developmental quotient

Tuberous sclerosis complex (TSC) represents a genetic condition, in which the clinical manifestations are caused by the disinhibition of the mammalian target of rapamycin (mTOR) pathway due to mutations in the TSC1 (hamartin) or TSC2 (tuberin) genes. The deregulated mTOR activity leads to multi-site tumors, including subependymal giant cell astrocytoma (SEGA). SEGA is a brain tumor that affects around 15% of TSC patients. The aim of the study was to evaluate miR-21 expression in the serum of two groups of TSC patients: with or without SEGA tumors. We found no differences in the level of miR-21 depending on the presence of SEGA. Next, we studied the influence of prolonged rapamycin administration on miR-21 level in the blood serum of TSC patients (6-12 months of rapamycin) and in primary cultures of SEGA-derived cells treated with rapamycin in vitro . Here we show that rapamycin treatment leads to the upregulation of miR-21 in both patients’ serum and in primary SEGA tumor cells in the culture indicating the regulatory relationship between rapamycin treatment and miR-21 expression.

Tuberous sclerosis complex (TSC) is a genetic multisystem disorder associated with constitutive overactivation of the mammalian target of rapamycin (mTOR) pathway and characterized by development of benign tumours in various organs. mTOR inhibitors have proven to be effective in the targeted therapy of certain TSC-associated pathologies such as subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas (AMLs). Accumulating experimental and clinical data suggest that mTOR inhibitors might have a systemic, disease-modifying influence on affected individuals. This systematic review provides an analysis of available clinical data concerning systemic effect of mTOR inhibitors and the influence of mTOR inhibition on different manifestations of TSC in individual patients.

The purpose of this study was to evaluate the epidemiology and clinical significance of hepatic angiomyolipomas in patients with tuberous sclerosis complex.We performed a retrospective analysis of clinical and imaging data from 187 patients with tuberous sclerosis complex. The prevalence, progression, and potential relationship between liver lesions and other clinical findings, including genetic associations, were assessed.Twenty-eight of 187 patients (14.9%) had hepatic lesions. There was a predominance of female over male patients in individuals with liver lesions (17 versus 11), with statistical significance in patients under five years of age (P 0.05). All individuals having hepatic lesions who also had available genetic testing data (n = 20) were diagnosed with a TSC2 gene mutation. All patients with liver lesions had coexisting renal angiomyolipomas (AMLs) (P 0.05). The age of onset of renal lesions was lower and their prevalence was significantly higher in patients with liver involvement (P 0.05). In most instances, hepatic lesions measured several millimeters in diameter and were clinically asymptomatic. Progressive lesion growth was documented in six individuals but with no clinical consequences to date.This study confirms the association of hepatic lesions with TSC2 mutations, a common origin of liver and renal AMLs, as well as the predominance of female patients in this group. Hepatic AMLs are relatively common but mostly benign lesions.

The Centennial Hall, a reinforced concrete structure with an auditorium for 10000 people, was opened in Wroclaw, Poland (then Breslau, Germany), in 1913 after two years of construction. Its dome, covering the whole building, has a diameter of 65.0 m and in the year it was completed was the largest reinforced concrete dome in the world. This broke the previous record, held by the dome of the Pantheon in Rome (43.3 m), which had lasted for 1787 years. This paper describes the structure of the building, its condition after 100 years of use and the renovation works carried out in 2009–2011. An important part of the renovation was strengthening the lower (tension) ring of the ribbed dome by way of external prestressing. Details concerning the assessment of the technical condition of the hall, numerical calculations and the proposed system of strengthening are presented. In 2006 the monumental Centennial Hall was listed as a UNESCO World Heritage Site for its pioneering reinforced concrete structure, designed in the style of modernism.

A fragment of a 13th–14th century painted mosque lamp was identified in the collection of archaeological glass from Ostrów Tumski in Wrocław, held by the Institute of Archaeology and Ethnology of the Polish Academy of Sciences (Research Centre on Late Antique Culture and the Early Middle Ages). An analysis of glass chemical composition as well as the decoration of this fragment showed that it was made of sodic-calcic-silica glass, and that part of the ornament covering it was finished with gold. A comparative study indicated that the artifact had been made in the Middle East, most likely in a glass workshop in Syria or Egypt. Another example of this type of Islamic glass was discovered in Poland during the excavation in the 1930s of Ostrówek in Opole. Large fragments of a painted glass bowl were found there and identified as a mosque lamp. Since then the object has been lost. The fragments of glass mosque lamps from Wrocław–Ostrów Tumski and Opole–Ostrówek will be discussed here in the context of basic raw material studies and chemical composition analyses

The aim of the present study was to assess the origin of the substantia nigra hyperechogenicity in Parkinson disease patients. The cause of hyperechogenicity was tested on an animal model. Fresh porcine brains were injected consecutively with ferritin, apoferritin and water. Then, glioma samples were inserted into animal model. The echogenicity of the region of interest was assessed before and after experimental procedures. We observed the same echogenicity of porcine brain before and after injections of iron-loaded ferritin, apoferritin and water. Increased echogenicity of glioma samples compared to surrounding porcine brain tissue could be clearly seen. We postulate that the relative gliosis might be, at least partially, responsible for the increased echogenicity of the substantia nigra in Parkinson disease patients. Keeping in mind all limitations and inaccuracies of animal model used, it seems that hyperechogenicity of substantia nigra is caused rather by structural changes within the brain tissue than by increased iron concentration.

The present paper describes a mathematical formalism for blending to obtain formed fuels from industrial waste. The offered methodology assures obtaining fuels of required technological properties, which result in, for example, a limited gram fraction of chlorine in dry mass of fuel and an appropriate calorific value in the working state. The procedure is illustrated by a simple example of calculations connected with the forming fuels from the waste obtained in the production and processing of cellulose pulp, paper and cardboard. The application of the mathematical formalism to additional limitations resulting in the extension of the technological condition and its linking to the model of generator processes will allow us to estimate the quality of synthetic gas produced from the formed fuels.

Background Tuberous sclerosis complex is a genetically determined multisystem disorder that may affect almost any human organ. The discovery of the mammalian target of rapamycin (mTOR) pathway and its involvement in tuberous sclerosis complex–related pathology has led to the introduction of mTOR inhibitors into clinical practice. Topical administration of mTOR inhibitors for skin lesions related to tuberous sclerosis complex may represent a reasonable alternative for more invasive procedures. A growing number of patients have been described exhibiting positive therapeutic effects from the topical administration of these agents. The aim of this review was to systematically analyze available literature on the use of topical mTOR inhibitors to treat dermatologic lesions related to tuberous sclerosis complex. Results A comprehensive review of PubMed, Medscape, and Cochrane databases between 1995 and 2015 was performed to identify available studies describing topical use of mTOR inhibitors in individuals with tuberous sclerosis complex. In most studies, topical mTOR inhibitor application proved to be effective in the treatment of skin lesions related to tuberous sclerosis complex. Facial angiofibromas were the target lesions in most instances. Few studies reported clinical improvement of hypomelanotic macules. These drugs directly address the molecular defect related to tuberous sclerosis complex manifestations. Conclusions Currently available clinical data suggest that topical application of mTOR inhibitors may be effective in the treatment of facial angiofibromas associated with tuberous sclerosis complex. Ongoing randomized clinical trials of topical mTOR inhibitors for TSC-related cutaneous lesions should add clarity to the role of these agents.

mTOR inhibitors represent a relatively new therapeutic option in the management of patients affected by tuberous sclerosis complex (TSC). Randomized clinical trials support the use of everolimus in the treatment of subependymal giant cell astrocytomas (SEGA) and renal angiomyolipomas (AML) related to TSC. Accumulating data suggest also systemic disease-modifying potential of mTOR inhibitors. Given that increasing number of patients with TSC receive mTOR inhibitors, the issue of adverse events associated with this therapy becomes practically important. In the present study we provide the overview of clinical manifestations and therapeutic options for the most common adverse events related to mTOR inhibitors in TSC patients.

SUMMARYIntroduction.Epilepsy that is associated with neurocutaneous disorders seriously deteriorates quality of life and cognitive outcome of affected children. Recent advances in epilepsy pathophysiology raise hopes for better treatment results in this difficult group of patients.Aim.The aim of this review is to present recent treatment recommendations as well as current research progress in the most frequent neurocutaneous disorders.Material and methods.We analyzed PubMed database to select the most prominent and recent (up to 2014 year) publications on the treatment and mechanisms of epilepsy in selected neurocutaneous disorders. We aimed to emphasize evidence-based medicine recommendations as well as basic experimental studies dealing with molecular mechanisms of epileptogenesis.Discussion and conclusions.Recent advances in disease-modifying treatment options such as mTOR inhibitors in patients with tuberous sclerosis complex open up new perspectives for neurologists. Traditional resective surgery has still a major role as a treatment of choice in carefully selected cases.

In spite of the fact, that subsequent new antiepileptic drugs (AEDs) are being introduced into clinical practice, the percentage of drug-resistant epilepsy cases remains stable. Although a substantial progress has been made in safety profile of antiepileptic drugs, currently available substances have not been unambiguously proven to display disease-modifying effect in epilepsy and their mechanisms of action influence mainly on the end-stage phase of epileptogenesis, namely seizures. Prevention of epileptogenesis requires new generation of drugs modulating molecular pathways engaged in epileptogenesis processes. The mammalian target of rapamycin (mTOR) pathway is involved in highly epileptogenic conditions, such as tuberous sclerosis complex (TSC) and represents a reasonable target for antiepileptogenic interventions. In animal models of TSC mTOR inhibitors turned out to prevent the development of epilepsy and reduce underlying brain abnormalities. Accumulating evidence from animal studies suggest the role of mTOR pathway in acquired forms of epilepsy. Preliminary clinical studies with patients affected by TSC demonstrated seizure reduction and potential disease-modifying effect of mTOR inhibitors. Further studies will determine the place for mTOR inhibitors in the treatment of patients with TSC as well as its potential antiepileptogenic effect in other types of genetic and acquired epilepsies. This review presents current knowledge of mTOR pathway physiology and pathology in the brain, as well as potential clinical use of its inhibitors.

A case of a 44-year-old woman with left ventricular non-compaction which manifested in adulthood as dyspnea, fatique and NYHA class III heart failure, is described. She received treatment typical for heart failure and was offered heart transplantation and cardioverter-defibrillator implantation which she refused. Unfortunately, she died suddenly three months after the diagnosis was established.

The Warta River is a medium size river in central Poland and the project of the Warta river crossing was connected with the project of the town ring road of Konin. The crossing was divided into four independent structures: three flyovers E5, E7, E8 and the main bridge. All structures were designed for two roads. The division of the crossing into particular engineering objects with their lengths and spans are presented in this paper.

Designing of curved cable-stayed bridges allows to increase safety of its users (drivers). They also allow to adjust bridge to the existing or planned road communication configuration. Some unexpected behaviour of such structures may appear during its exploitation. Therefore it is necessary to gather information of ways of designing and non-typical behaviour of such bridges.Two curved cable-stayed bridges located in Poland are presented in the paper, including description of the structures, the way of their construction and their behaviour during exploitation and filed tests. The first one is a cable-stayed bridge in Skorogoszcz (Poland), which has two spans of 59.75 m each. Pylon is made of steel and the deck has composite structure: three beams are made of steel and plate is made of concrete. The second bridge is a viaduct along National Road No. 94 over the A4 highway in Wykroty. It is a continuous double span (45.47 + 45.28 m) cable-stayed structure with a prestressed concrete deck and the V-shaped pylons are made of steel.

Highway ring road of Wrocław is a very important part of an urban communication network which is now under modernization work. It will connect the existing A4 highway with the S8 expressway being under construction. The ring road is 26.765 km long. Forty bridge structures were designed along or over it. Total length of highway bridges and viaducts is more than 6 km, that is equivalent to 22% of the whole route. The longest bridge is a concrete cable-stayed structure over the Odra River. The river flows in its main bed, while inland navigation uses additional channel with two locks. There is an island located between the river and the channel. The planned bridge is 1742 m long and consists of: south flyover 610 m long with continuous eleven span prestressed concrete box structure; main bridge 612 m long with two separate decks connected to a single concrete H-shaped pylon; north flyover 520 m long with continuous girders made of prestressed concrete.

Accumulating data confirm the usefulness of transcranial sonography (TCS) in the diagnosis of Parkinson's disease. The relevance of basal ganglia abnormalities depicted by TCS in atypical parkinsonian syndromes still needs further assessment. In the present study, 20 patients with progressive supranuclear palsy (PSP) and 13 patients with corticobasal syndrome (CBS) were studied with the use of transcranial sonography. Echogenicity of the substantia nigra (SN) and lenticular nucleus (LN) were assessed. 0/20 patients with PSP and 8/12 (66.6 %) patients with CBS were characterized with SN hyperechogenicity. LN hyperechogenicity was observed in 9/20 patients diagnosed with PSP and 0/11 of CBS patients. The combination of SN isoechogenicity and LN hyperechogenicity reached 100 % sensitivity and positive predictive value for the diagnosis of PSP. The results of this study point out that CBS has to be taken into consideration when SN hyperechogenicity is depicted in a patient with parkinsonian syndrome. Normal echogenicity of the SN coexisting with LN hyperechogenicity practically excludes CBS.