Księgozbiór Konstancji Sapieżyny w świetle inwentarza z 1757 roku

Celem artykułu jest zaprezentowanie krótkiej charakterystyki księgozbioru Konstancji z Radziwiłłów Sapieżyny (1697–1756), znanego dzięki zachowanemu inwentarzowi. Regestr ten jest jednym z nielicznych zachowanych spisów księgozbiorów kobiecych, gromadzonych na terenie Rzeczypospolitej w 1. poł. XVIII w. Ponadto jest to spis książek zebranych przez przedstawicielkę najpotężniejszego rodu litewskiego, a zarazem żonę jednego z najwyższych dostojników państwowych z okresu panowania Augusta III – Jana Fryderyka Sapiehy (1680-1751). I wreszcie, co nie jest bez znaczenia, właścicielkę pierwszego znanego ekslibrisu z napisem w języku polskim. Spis zawiera informacje o 805 egzemplarzy, podzielonych na dwa główne działy – książki polskie i francuskie – przy czym te pierwsze dominowały. Większą część kolekcji stanowiły dzieła o treści religijnej (godzinki, kazania, psałterze), literatura hagiograficzna oraz różnego rodzaju rozmyślania i ćwiczenia duchowe. Niewielka liczba pozycji o tematyce świeckiej to przede wszystkim poradniki praktyczne, gramatyki francuskie, niemieckie i polskie, konstytucje sejmowe, kalendarze, utworzy literackie i panegiryki. Całość spisu mieści się na pięciu stronach formatu folio.  Inwentarz przechowywany jest obecnie w Archiwum Głównym Akt Dawnych i jest wykazem mobiliów, wśród których znajdują się książki

General characteristics and comorbidities in patients with palmoplantar pustulosis

The aim of this prospective study was to analyze comorbidities in patients with palmoplantar pustulosis (PPP). The current study comprised 63 consecutive patients with palmoplantar pustulosis. The control group consisted of 37 patients with psoriasis vulgaris (PSV). The study included a standardized anamnesis, a clinical examination, blood tests for thyroid hormones, as well as calcium, magnesium, antiendomysial antibody, and patch tests. Hypertension was observed in 28/63 (44.44%) patients with PPP. Eight (12.7%) had ischaemic heart disease, and 7/63 (11.11%) had type 2 diabetes mellitus. There was no statistically significant difference between the patients with PPP and those in the control group. Metabolic syndrome was diagnosed in 19/63 (30.16%) patients with PPP and in 12/37 (32.43%) patients with PSV. Thyroid disease was more prevalent among patients with PPP in comparison to patients with PSV (31.75% vs. 13.51%; p=0.0421). Body mass index was statistically significantly higher in patients with PSV (28.25 vs. 25.86 kg/m², p=0.0144). BMI was higher than 25 kg/m2 in 18.03% patients with PPP and 26.47% patients with PSV (p=0.333). Positive patch tests were observed in 12/39 (30.77%) patients with PPP. The most common allergens were nickel chloride (5/12, 41.67%) and fragrances (5/12, 41.67%). In the control group, patch tests were positive in 2/11 (18.18%) cases (p&lt;0.05). Patients with PPP, like patients with PSV, often presented with hypertension and metabolic syndrome. Given that many studies have focused on cardiovascular risk in PSV, there is a need for further research on the association between PPP and cardiovascular risk. In addition, patients resistant to PPP treatment should be screened for contact allergies.</h2

A peptide from the staphylococcal protein Efb binds P-selectin and inhibits the interaction of platelets with leukocytes

Aims: P-selectin is a key surface adhesion molecule for the interaction of platelets with leukocytes. We have shown previously that the N-terminal domain of Staphylococcus aureus extracellular fibrinogen-binding protein (Efb) binds to P-selectin and interferes with platelet-leukocyte aggregate formation. Here, we aimed to identify the minimal Efb motif required for binding platelets and to characterize its ability to interfering with the formation of platelet-leukocyte aggregates. Methods and Results: Using a library of synthetic peptides, we mapped the platelet-binding site to a continuous 20 amino acid stretch. The peptide Efb68-87 was able to bind to resting and, to a greater extent, thrombin-stimulated platelets in the absence of fibrinogen. Dot blots, pull-down assays and P-selectin glycoprotein ligand-1 (PSGL-1) competitive binding experiments identified P-selectin as the cellular docking site mediating Efb68-87 platelet binding. Accordingly, Efb68-87 did not bind to other blood cells and captured platelets from human whole blood under low shear stress conditions. Efb68-87 did not affect platelet activation as tested by aggregometry, flow cytometry and immunoblotting, but inhibited the formation of platelet-leukocyte aggregates (PLAs). Efb68-87 also interfered with the platelet-dependent stimulation of neutrophil extracellular traps (NETs) formation in vitro. Conclusions: We have identified Efb68-87 as a novel selective platelet-binding peptide. Efb68-87 binds directly to P-selectin and inhibits interactions of platelets with leukocytes that lead to PLA and NET formation. As PLAs and NETs play a key role in thromboinflammation, Efb68-87 is an exciting candidate for the development of novel selective inhibitors of the proinflammatory activity of platelets

Progressive bronchiectasis and CMC in a patient with STAT1 GOF — a rare case of primary immunodeficiency

Bronchiestasis is a common complication developing in patients with primary immunodeficiency disorders. AD GOF STAT1 defi-ciency is characterized by CMC, repeated infections, and autoimmunity. It is the most frequently diagnosed entity in a group of PIDs with CMC. Here, we present the first Polish case of a female patient with early-onset bronchiestasis accompanied by CMC and a severe course of infections who was genetically diagnosed with AD GOF1 STAT1 mutation at the age of 15

Spinal tuberculosis - diagnostic and therapeutic difficulties

Presented are diagnostic and therapeutic difficulties in two patients with spinal tuberculosis. In both cases pain magement was a necessary supplementation to specific treatment. The need of palliative care in patients with chronic spinal tuberculosis was unerlined.W niniejszej pracy przedstawiono trudności diagnostyczne i lecznicze u 2 chorych na gruźlicze zapalenie kręgosłupa. W obu przypadkach poza leczeniem przyczynowym konieczne było zastosowanie terapii przeciwbólowej. Opracowanie wskazuje na potrzebę opieki paliatywnej u chorych z przewlekłymi swoistymi zmianami kręgosłupa

Targeting NETs using dual-active DNase1 variants

Background: Neutrophil Extracellular Traps (NETs) are key mediators of immunothrombotic mechanisms and defective clearance of NETs from the circulation underlies an array of thrombotic, inflammatory, infectious, and autoimmune diseases. Efficient NET degradation depends on the combined activity of two distinct DNases, DNase1 and DNase1-like 3 (DNase1L3) that preferentially digest double-stranded DNA (dsDNA) and chromatin, respectively. Methods: Here, we engineered a dual-active DNase with combined DNase1 and DNase1L3 activities and characterized the enzyme for its NET degrading potential in vitro. Furthermore, we produced a mouse model with transgenic expression of the dual-active DNase and analyzed body fluids of these animals for DNase1 and DNase 1L3 activities. We systematically substituted 20 amino acid stretches in DNase1 that were not conserved among DNase1 and DNase1L3 with homologous DNase1L3 sequences. Results: We found that the ability of DNase1L3 to degrade chromatin is embedded into three discrete areas of the enzyme's core body, not the C-terminal domain as suggested by the state-of-the-art. Further, combined transfer of the aforementioned areas of DNase1L3 to DNase1 generated a dual-active DNase1 enzyme with additional chromatin degrading activity. The dual-active DNase1 mutant was superior to native DNase1 and DNase1L3 in degrading dsDNA and chromatin, respectively. Transgenic expression of the dual-active DNase1 mutant in hepatocytes of mice lacking endogenous DNases revealed that the engineered enzyme was stable in the circulation, released into serum and filtered to the bile but not into the urine. Conclusion: Therefore, the dual-active DNase1 mutant is a promising tool for neutralization of DNA and NETs with potential therapeutic applications for interference with thromboinflammatory disease states