Convulsive status epilepticus management in adults and children: Report of the Working Group of the Polish Society of Epileptology

Introduction The Working Group was established at the initiative of the General Board of the Polish Society of Epileptology (PSE) to develop an expert position on the treatment of convulsive status epilepticus (SE) in adults and children in Poland. Generalized convulsive SE is the most common form and also represents the greatest threat to life, highlighting the importance of the choice of appropriate therapeutic treatment. Aim of guideline We present the therapeutic options separately for treatment during the early preclinical (>5–30min), established (30–60min), and refractory (>60min) SE phases. This division is based on time and response to AEDs, and indicates a practical approach based on pathophysiological data. Results Benzodiazepines (BZD) are the first-line drugs. In cases of ineffective first-line treatment and persistence of the seizure, the use of second-line treatment: phenytoin, valproic acid or phenobarbital is required. SE that persists after the administration of benzodiazepines and phenytoin or another second-line AED at appropriate doses is defined as refractory and drug resistant and requires treatment in the intensive care unit (ICU). EEG monitoring is essential during therapy at this stage. Anesthesia is typically continued for an initial period of 24h followed by a slow reversal and is re-established if seizures recur. Anesthesia is usually administered either to the level of the “burst suppression pattern” or to obtain the “EEG suppression” pattern. Conclusions Experts agree that close and early cooperation with a neurologist and anesthetist aiming to reduce the risk of pharmacoresistant cases is an extremely important factor in the treatment of patients with SE. This report has educational, practical and organizational aspects, outlining a standard plan for SE management in Poland that will improve therapeutic efficacy

Clinical features, etiology, and survival in patients with restrictive cardiomyopathy: A single-center experience

Background: Numerous prognostic factors have been proposed for cardiac amyloidosis (CA). The knowledge about other subtypes of restrictive cardiomyopathy (RCM) is scant.Aims: This study aimed to elucidate the etiology and prognostic factors of RCM as well as assess cardiac biomarkers: high-sensitive troponin T (hs-TnT), growth differentiation factor-15 (GDF-15), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and soluble suppression of tumorigenicity 2, as mortality predictors in RCM.Methods: We enrolled 36 RCM patients in our tertiary cardiac department. All patients were screened for CA. Genetic testing was performed in 17 patients without CA.Results: Pathogenic or likely pathogenic gene variants were found in 86% of patients, including 5 novel variants. Twenty patients died, and 4 had a heart transplantation during the study. Median overall survival was 29 months (8–55). The univariate Cox models analysis indicated that systolic and diastolic blood pressure, GDF-15, hs-TnT, NT-proBNP, left ventricular stroke volume, the ratio of the transmitral early peak velocity (E) estimated by pulsed wave Doppler over the early mitral annulus velocity (e’), tricuspid annulus plane systolic excursion, early tricuspid valve annular systolic velocity, the presence of pulmonary hypertension, and pericardial effusion influenced survival (P <0.05). A worse prognosis was observed in patients with GDF-15 >1316 pg/ml, hs-TnT >42 ng/l, NT-proBNP >3383 pg/ml, and pericardial effusion >3.5 mm (Kaplan-Meier analysis, log-rank test, P <0.001).Conclusions: Genetic testing should be considered in every RCM patient where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF-15, hs-TNT, NT-proBNP, and pericardial effusion are associated with worse prognosis. Further studies are warranted

Targeting BTK with Ibrutinib in Relapsed or Refractory Mantle-Cell Lymphoma – Results of an International, Multicenter, Phase 2 Study of Ibrutinib (PCI-32765) – EHA Encore

Bruton's tyrosine kinase (BTK) is a central mediator of B-cell receptor (BCR) signaling essential for normal B-cell development. Ibrutinib is an oral BTK inhibitor that induces apoptosis and inhibits migration and adhesion of malignant B-cells. Updated results of this international, multicenter, phase 2 study of single agent ibrutinib in relapsed or refractory MCL will be presented.Ibrutinib 560mg PO QD was administered continuously until disease progression. Tumor response was assessed every 2 cycles (one cycle=28 days). The study enrolled 115 patients (65 bortezomib-naïve, 50 bortezomib-exposed); 111 patients were treated; 110 were evaluable for response. Baseline characteristics included: median age 68 years, time since diagnosis 42 months, number of prior treatments 3; bulky disease (>10cm) 13%, prior stem cell transplant 10%, high risk MIPI 49%.Median time on treatment was 9.2 months; 53% of patients remain on therapy. Median PFS was 13.9 months and DOR has not yet been reached. Responses increased with longer treatment: comparing to previous data described at ASH 2011, the CR rate increased from 16% to 39%, and the ORR increased from 69% to 75%

The concept for the development of a functional area illustrated by the case of the Functional Area of the Oder Communes

The aim of this article is to present the essence of a functional area as a new tool of the EU regional policy and the course of designing an integrated approach to the development of the functional area. The study is a case study and relates to the methodical process of creating a development concept for the Functional Area of Oder Communes (FAOC). This article is based on the analysis of the domestic and foreign literature on regional development and own experiences and reflections of authors resulting from the work in the team for the development of a strategy for this area. The analysis of domestic and foreign literature allowed for presenting a new paradigm of the local development. The current approach is characterized by a focus on the use of endogenous potentials and territorial targeting of the development, which promotes the creation of functional and spatial structures such as functional areas. Their development requires integrated development planning that provides benefits both in the social and economic dimension. The study presents the new paradigm of the local development in the context of the National Spatial Development Concept 2030. The study presents the theoretical and practical basis for creating a concept for integrated development of a functional area

Hydraulic losses generated by modern drainage systems during backwash of rapid filters

Badaniom poddano dwustopniowe filtry pospieszne (antracytowo-piaskowe i węglowe) w Stacji Uzdatniania Wody w Gruszczynie (zaopatrującej w wodę Poznań i Swarzędz), wyposażone w dwa rodzaje nowoczesnych systemów drenażowych TRITON i SE-LOP. Podczas modernizacji stacji filtrów dokonano pomiaru strat hydraulicznych powodowanych przez oba systemy drenażowe przy różnej intensywności płukania złóż filtracyjnych wodą oraz porównawczo - przez same systemy drenażowe (bez złóż filtracyjnych). Wyznaczone doświadczalnie rzeczywiste straty hydrauliczne na badanych drenażach porównano z wartościami obliczeniowymi opartymi na danych podawanych przez ich producentów. Porównanie wysokości strat ciśnienia podawanych przez producenta oraz wartości zmierzonych doświadczalnie na stacji filtrów wykazało, że straty rzeczywiste na samym drenażu SE-LOP były większe o prawie 50%, natomiast obliczeniowe i pomierzone straty ciśnienia w warstwie złoża filtracyjnego były porównywalne.The objects under study were two-stage rapid filters (anthracite-sand and active carbon) operated by the Water Treatment Plant in Gruszczyn, which supplies municipal water to the cities of Poznan and Swarzedz. The filters were fitted with two types of advanced drainage systems, TRITON and SE-LOP. During modernization of the water treatment plant, measurements were conducted to determine the hydraulic losses caused by the two drainage systems at a varying rate of filter backwash water flow, and (for comparison) the hydraulic losses caused by the drainage systems alone. Actual hydraulic losses obtained with the drainage systems tested were compared with those calculated based on the manufacturers' specifications. Analysis of the hydraulic head losses specified by the manufacturers and those measured in the filter plant has produced the following finding: the actual hydraulic head losses caused by the SE-LOP drainage system alone are by approximately 50% higher compared to the calculated values, whereas the hydraulic head losses measured in the filter beds are comparable with the calculated ones

Akwaton decolorization of azo dyes solutions, Schäffer acid and salt R derivatives

Ścieki pobarwnikarskie wymagają skutecznej dekoloryzacji ze względu na ich negatywny wpływ na odbiornik, jak nienaturalne zabarwienie wód i możliwe działanie toksyczne produktów ich rozpadu na organizmy wodne. W celu usunięcia związków barwnych ze ścieków zastosowano koagulację z użyciem poliheksametyleno guanidyny (Akwatonu). W zależności od budowy barwnika uzyskano zmniejszenie zabarwienia nawet o 88%.Color wastewater require effective decolorization due to their negative impact on the receiver, as unnatural color of water and possible decomposition products toxicity in relation to aquatic organisms. In order to remove color compounds from wastewater coagulation with polyhexamethylene guanide (Akwaton) was used. Depending on the dye structure color reduction was achieved up to 88%