Dysplazja obojczykowo-czaszkowa u 15-miesięcznego chłopca i 14-letniej dziewczynki : prezentacja kliniczna i obraz radiologiczny

Background: Cleidocranial dysplasia (CCD) is a rare, autosomal dominant skeletal dysplasia with a relatively mild course. The most characteristic symptoms are clavicle hypoplasia or aplasia, abnormalities in the skull ossification, and a variety of dental pathologies. Case report: The clinical course and radiological presentation of two cases (a 15-month-old boy and a 14-year-old girl) of CCD are presented. Among the characteristic phenotype features the patients demonstrated were abnormalities in the skull formation, with wide sutures and large, delayed closure of the fontanelles, additional Wormian bones, and significant dental abnormalities, in both cases with delayed eruption of teeth and, in the girl, intensive caries, cross bite, and impacted teeth. The boy presented hypoplasia of the clavicles, while aplastic clavicles were confirmed in the girl. In both cases clavicle defects resulted in descended shoulders with abnormal movement faculty. Metacarpal and phalangeal defects were also found in both children as well as abnormalities in pelvic structure, with wide pubic symphysis and vertical arrangement of the pelvic bones in the girl. Conclusions: CCD is a skeletal dysplasia with characteristic clinical and radiological presentation. Although the course of the disorder is relatively mild, early orthopedic, dental, and orthodontic care, as well as rehabilitation, are essential in limiting complications resulting from the congenital malformation of the skeletal system

Comparing Tumor-Specific CD8+ T Cells in the Bone Marrow with Tumor Infiltrating Lymphocytes: Implications for Adoptive Cell Therapy in Solid Tumors

The variety of diseases encompassed by the term “cancer” result in substantial global disease burden, comprising one of the leading causes of premature mortality. Immunotherapy, comprised largely of immune checkpoint blockade and T cell therapy, has progressed immensely in recent years, becoming an integral strategy in cancer care. Strategies employing adoptive cell therapy (ACT) utilize cells derived from a patient or from a donor to develop a cellular product for infusion. Current ACT approaches have seen the establishment of chimeric antigen receptor-T cells (CAR-T cells) using peripheral blood lymphocytes as well as tumor infiltrating lymphocytes (TILs) with significant clinical results. Alongside marked progress, the limitations of current strategies are also emerging. Notably, current ACT modalities face challenges of persistence following infusion and duration of clinical responses. TIL therapy specifically requires candidate patients to be at an advanced disease stage, decreasing the likelihood of a positive outcome following therapeutic intervention. Additionally, although TILs have seen clinical success in limited settings, they are also a dysfunctional population of T cells. As the quality of an initial T cell population has been linked to improved outcomes following adoptive transfer, this implies that TILs may be a sub-optimal T cell source for broad clinical applications. Due to these limitations, novel approaches are needed. The bone marrow (BM) is an immunological niche that houses T cells with specificity for previously encountered antigens, including tumor-associated antigens from certain solid cancers. BM T cells possess an enhanced reactivity to a variety of hematologic and solid cancers as compared to T cells from the peripheral blood. As TILs are an approved treatment modality for at least two types of solid tumors, t¬¬his project sought to improve our understanding of the biology of tumor-specific BM T cells in the context of solid cancer by comparing them with TILs. In doing so, we intended to further the rationale for using the BM as a source of T cells for ACT against solid malignancies. Herein, we used the B16.OVA murine melanoma model to profile tumor specific CD8+ T cells from the BM and contrast them with TILs. Through immunophenotyping via polychromatic flow cytometry, bulk RNA-sequencing, and a combination of in vitro and in vivo assays, we demonstrate that T cells from the BM exhibit characteristics that could make them a superior source of cells for cellular therapy as compared to TILs; these include a stem-like memory phenotype, improved effector function, enhanced persistence within a tumor-bearing host, and increased tumor infiltration. Additionally, our studies indicate that the BM appears to maintain a stable population of tumor-specific T cells during disease progression. If this holds true in humans, this could make the BM a reservoir of tumor-specific T cells that is accessible in and harvestable from all patients, even at early, pre-metastatic stages of disease. Collectively, the data presented here provide a foundation for further exploring the BM as a source of tumor-specific T cells for ACT in solid malignancies

Selected issues concerning application of the successful attempt and inept attempt in contemporary jurisprudence of the Polish courts

The institution of attempt is used relatively frequently in the practice of justice system.Importantly, it always separates the punishable stage of attempt from punishable preparatory activities, and therefore constitutes the limit of criminalization. Despite its practical importance, using of the indicated legal structure, raises a number of doubts that are related to the proper interpretation of the normative concepts comprising it. A consequence is moving the limit of punishability of the crime by adjudicating authorities. It can raise some objections – from fundamental principle of criminal law inthe form of nullum crimen sine lege point of view. The article discusses the problems of that appear in jurisdiction in connection with the application of the attempt institution; these problems raise interest among doctrine. The author confronts irreconcilable judgments. The Author searches interpretative solutions that seem the most appropriate from theoretical and dogmatic structure of the successful attempt and inept attempt point of view

Comparing Tumor-Specific CD8+ T Cells in the Bone Marrow with Tumor Infiltrating Lymphocytes: Implications for Adoptive Cell Therapy in Solid Tumors

The variety of diseases encompassed by the term “cancer” result in substantial global disease burden, comprising one of the leading causes of premature mortality. Immunotherapy, comprised largely of immune checkpoint blockade and T cell therapy, has progressed immensely in recent years, becoming an integral strategy in cancer care. Strategies employing adoptive cell therapy (ACT) utilize cells derived from a patient or from a donor to develop a cellular product for infusion. Current ACT approaches have seen the establishment of chimeric antigen receptor-T cells (CAR-T cells) using peripheral blood lymphocytes as well as tumor infiltrating lymphocytes (TILs) with significant clinical results. Alongside marked progress, the limitations of current strategies are also emerging. Notably, current ACT modalities face challenges of persistence following infusion and duration of clinical responses. TIL therapy specifically requires candidate patients to be at an advanced disease stage, decreasing the likelihood of a positive outcome following therapeutic intervention. Additionally, although TILs have seen clinical success in limited settings, they are also a dysfunctional population of T cells. As the quality of an initial T cell population has been linked to improved outcomes following adoptive transfer, this implies that TILs may be a sub-optimal T cell source for broad clinical applications. Due to these limitations, novel approaches are needed. The bone marrow (BM) is an immunological niche that houses T cells with specificity for previously encountered antigens, including tumor-associated antigens from certain solid cancers. BM T cells possess an enhanced reactivity to a variety of hematologic and solid cancers as compared to T cells from the peripheral blood. As TILs are an approved treatment modality for at least two types of solid tumors, t¬¬his project sought to improve our understanding of the biology of tumor-specific BM T cells in the context of solid cancer by comparing them with TILs. In doing so, we intended to further the rationale for using the BM as a source of T cells for ACT against solid malignancies. Herein, we used the B16.OVA murine melanoma model to profile tumor specific CD8+ T cells from the BM and contrast them with TILs. Through immunophenotyping via polychromatic flow cytometry, bulk RNA-sequencing, and a combination of in vitro and in vivo assays, we demonstrate that T cells from the BM exhibit characteristics that could make them a superior source of cells for cellular therapy as compared to TILs; these include a stem-like memory phenotype, improved effector function, enhanced persistence within a tumor-bearing host, and increased tumor infiltration. Additionally, our studies indicate that the BM appears to maintain a stable population of tumor-specific T cells during disease progression. If this holds true in humans, this could make the BM a reservoir of tumor-specific T cells that is accessible in and harvestable from all patients, even at early, pre-metastatic stages of disease. Collectively, the data presented here provide a foundation for further exploring the BM as a source of tumor-specific T cells for ACT in solid malignancies

The ICNP® terms cross-mapping on the example of nursing care of patient with rheumatoid arthritis

Wstęp. Reumatoidalne zapalenie stawów (RZS) to choroba autoimmunologiczna, wywołująca głównie stany zapalne tkanki łącznej. Jej przebieg związany jest z symetrycznym zajęciem stawów oraz pozastawowymi zmianami, które prowadzą do dysfunkcji wielu narządów. Cel. Celem niniejszej pracy było mapowanie najczęściej występujących u pacjentów z RZS problemów pielęgnacyjnych, działań pielęgniarskich i ich ewaluacji z frazami opisującymi diagnozy, wyniki i interwencje wg ICNP® Materiały i metody. W pracy zastosowano metodę analizy piśmiennictwa poświęconego zagadnieniom opieki nad chorym z RZS oraz mapowanie diagnoz i interwencji przy użyciu słownika ICNP®. Wyniki i wnioski. Zastosowanie klasyfikacji ICNP® pozwala na stosowanie ujednoliconej, referencyjnej terminologii w praktyce pielęgniarskiej. Usprawnia to komunikację oraz umożliwia harmonizację oraz porównywanie danych. Korzystanie z ujednoliconej terminologii zawodowej jest jednym z wyznaczników autonomii zawodu.Introduction. Rheumatoid arthritis is an autoimmune disease which mainly causes connective tissue inflammation and affects joints.. Its course is related to the symmetrical involvement of joints and non-articular changes that lead to dysfunction of many organs. Aim. The aim of this study was to map the most frequent nursing problems, nursing activities and their evaluation with the phrases describing diagnoses, results and interventions according to ICNP® in RA patients. Material and methods. The study uses the method of analysis of existing literature devoted to the issues of caring for a patient with RA and mapping diagnoses and interventions using the ICNP® vocabulary. Results and conclusion. The use of ICNP® classification allows the use of unified, reference terminology in nursing practice. It facilitates communication and enables harmonization and comparison of data. The use of unified vocational terminology is one of the determinants of the autonomy of the profession