Surgical Treatment of Wounds Using Stem Cells in Epidermolysis Bullosa (EB)

Epidermolysis bullosa (EB) is a group of hereditary skin diseases, or genodermatoses, characterized by the formation of severe, chronic blisters with painful and life-threatening complications. Despite the previous and ongoing progress in the field, there are still no effective causative treatments for EB. The treatment is limited to relieving symptoms, which—depending on disease severity—may involve skin (blisters, poorly healing wounds caused by the slightest mechanical stimuli, contractures, scarring, pseudosyndactyly) and internal organ abnormalities (esophageal, pyloric, or duodenal atresia; renal failure; and hematopoietic abnormalities). The last decade saw a series of important discoveries that paved the way for new treatment methods, including gene therapy, bone marrow transplantation, cell therapy (allogenic fibroblasts, mesenchymal stem cells [MSCs], and clinical use of induced pluripotent stem cells. Tissue engineering experts are attempting to develop skin-like structures that can facilitate the process of healing to promote skin reconstruction in injuries that are currently incurable. However, this is incredibly challenging, due to the complex structure and the many functions of the skin. Below, we characterize EB and present its potential treatment methods. Despite the cure for EB being still out of reach, recent data from animal models and initial clinical trials in humans have raised patients’, clinicians’, and researchers’ expectations. Consequently, modifying the course of the disease and improving the quality of life have become possible. Moreover, the conclusions drawn based on EB treatment may considerably improve the treatment of other genetic diseases

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies

Stosować czy nie stosować? Oto jest pytanie. Deskryptory Biblioteki Narodowej jako propozycja zmian w opracowaniu zbiorów w opinii bibliotek państwowych wyższych szkół zawodowych w Polsce / To apply or not to apply? That is the question. Descriptors of the National Library as a proposal of changes in the development of sets in the opinion of the libraries of Higher Vocational Schools in Poland

Wstęp. Celem artykułu jest zaprezentowanie opinii katalogerów pracujących w bibliotekach państwowych wyższych szkół zawodowych w Polsce na temat deskryptorów Biblioteki Narodowej (dBN), przedstawienie danych dotyczących stosowania wybranych standardów Resource Description & Access (RDA) w procesie opracowania zbiorów oraz informacji na temat systemu bibliotecznego. Materiał i metody. Badania przeprowadzono w maju i czerwcu 2018 roku. Dane zostały przygotowane na podstawie ankiety, wysłanej do 33 bibliotek państwowych wyższych szkół zawodowych w Polsce. Zarejestrowano odpowiedzi z 14 instytucji (42%). Wyniki. Większość bibliotek, które stosowały JHP BN stosuje obecnie dBN i ma pozytywną opinię na ich temat. Przeważająca część stosuje wybrane standardy RDA. Wyszukiwanie w katalogach OPAC tylko w niewielu przypadkach opiera się na wyszukiwaniu fasetowym, nie wszystkie systemy dają taką możliwość, a wymiana oprogramowania wiąże się z dużymi nakładami finansowymi. Wnioski. Uzyskane wyniki pokazują, że biblioteki, które odpowiedziały na ankietę, są otwarte na zmiany, chętnie stosują nowe standardy i starają się sprostać potrzebom odwiedzających je użytkowników.Introduction. The aim of this article is to present the opinion of catalogue specialists working in state libraries of Higher Vocational Schools (HVS) in Poland about descriptors of the National Library (dNL), presentation of data relating to the application of selected standards of Resource Description & Access (RDA) in the process of development of sets and information about the library system. Material and methods. The studies were conducted in May and June of 2018. Data were prepared on the basis of a survey sent to 33 libraries of the Higher Vocational Schools in Poland. The answers were registered from 14 institutions (42%). Results. The majority of the libraries which applied JHP BN currently apply dBN and have a positive opinion about them. The majority of them apply selected standards RDA. Search in the OPAC catalogues only in several cases is based on faceted search, whilst not all systems give such opportunity and the exchange of software carries the burden of significant finance means. Conclusions. The results show that libraries, which replied to the survey are open to changes, willingly applying new standards and making efforts to meet the needs of users visiting them

The EVER genes – the genetic etiology of carcinogenesis in epidermodysplasia verruciformis and a possible role in non-epidermodysplasia verruciformis patients

In recent years, the two adjacent novel EVER1 and EVER2 genes have been identified, whose mutations are responsible for the development of epidermodysplasia verruciformis (EV). Epidermodysplasia verruciformis is a rare, autosomal recessive genodermatosis associated with increased risk of skin carcinoma. Up to now 7 mutations in the EVER1 gene and 5 mutations in the EVER2 gene have been identified only in EV. It was also determined that the EVER genes belong to a novel gene family, the transmembrane channel-like (TMC) family, and are responsible for properly functioning zinc homeostasis. These observations have given new insights into EV pathogenesis

Selected novel aspects in pathogenesis of lupus erythematosus – interdisciplinary view

Lupus erythematosus is an autoimmune disease characterized by complex immune disturbances concerning humoral and cell-mediated immune responses. Despite intensive research, many pathological processes regarding this disorder remain unexplained. During interdisciplinary investigations on the etiology of lupus and its complications special interest has recently been referred to heat-shock proteins, antibodies directed against such proteins and galectin-3. These issues are extensively studied not only by dermatologists and rheumatologists, but also by cardiologists (in terms of cardiovascular complications) and nephrologists (in terms of lupus nephritis). This study presents the current state of dermatological, nephrological and cardiological knowledge on this topic

Dynamics of Acute Local Inflammatory Response after Autologous Transplantation of Muscle-Derived Cells into the Skeletal Muscle

The vast majority of myoblasts transplanted into the skeletal muscle die within the first week after injection. Inflammatory response to the intramuscular cell transfer was studied in allogeneic but not in autologous model. The aim of this study was to evaluate immune reaction to autotransplantation of myogenic cells and to assess its dynamics within the first week after injection. Muscle-derived cells or medium alone was injected into the intact skeletal muscles in autologous model. Tissue samples were collected 1, 3, and 7 days after the procedure. Our analysis revealed the peak increase of the gene expression of all evaluated cytokines (Il-1α, Il-1β, Il-6, Tgf-β, and Tnf-α) at day 1. The mRNA level of analyzed cytokines normalized in subsequent time points. The increase of Il-β gene expression was further confirmed at the protein level. Analysis of the tissue sections revealed rapid infiltration of injected cell clusters with neutrophils and macrophages. The inflammatory infiltration was almost completely resolved at day 7. The survived cells were able to participate in the muscle regeneration process. Presented results demonstrate that autotransplanted muscle-derived cells induce classical early immune reaction in the site of injection which may contribute to cellular graft elimination