Paragangliomas and syringomyelia in Tetralogy of Fallot-A case report and literature review.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, paragangliomas, and syringomyelia are uncommon diseases. Furthermore, in the absence of any genetic link and with less than five reported adult patients surviving unrepaired rare form of Tetralogy of Fallot, our case shows noteworthiness. The possibility of definitive treatment of these conditions is rendered unsafe due to this persistent defect.

Chemotherapy drives tertiary lymphoid structures that correlate with ICI-responsive TCF1+CD8+ T cells in metastatic ovarian cancer.

Purpose: Patients with high-grade serous ovarian carcinoma (HGSOC) are virtually insensitive to immune checkpoint inhibitors (ICIs) employed as standalone therapeutics, at least in part reflecting microenvironmental immunosuppression. Thus, conventional chemotherapeutics and targeted anticancer agents that not only mediate cytotoxic effects but also promote the recruitment of immune effector cells to the HGSOC microenvironment stand out as promising combinatorial partners for ICIs in this oncological indication.

Experimental Design: We harnessed a variety of transcriptomic, spatial and functional assays to characterize the differential impact of neo-adjuvant paclitaxel-carboplatin on the immunological configuration of paired primary and metastatic HGSOC biopsies as compared to NACT-naïve HGSOC samples from 5 independent patient cohorts.

Tertiary lymphoid structures and B cells determine clinically relevant T cell phenotypes in ovarian cancer.

Intratumoral tertiary lymphoid structures (TLSs) have been associated with improved outcome in various cohorts of patients with cancer, reflecting their contribution to the development of tumor-targeting immunity. Here, we demonstrate that high-grade serous ovarian carcinoma (HGSOC) contains distinct immune aggregates with varying degrees of organization and maturation. Specifically, mature TLSs (mTLS) as forming only in 16% of HGSOCs with relatively elevated tumor mutational burden (TMB) are associated with an increased intratumoral density of CD8 effector T (T) cells and TIM3PD1, hence poorly immune checkpoint inhibitor (ICI)-sensitive, CD8 T cells.

High incidence of occult familial cases amongst Czech patients with head and neck paragangliomas.

Introduction: Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, which are mostly benign in nature. Amongst all genes, Succinate Dehydrogenase Subunit D () is the most commonly mutated in familial HNPGLs. In about 30% of HNPGLs, germline mutations in can also occur in the absence of positive family history, thus giving rise to "occult familial" cases.

Peripheral gene signatures reveal distinct cancer patient immunotypes with therapeutic implications for autologous DC-based vaccines.

Dendritic cells (DCs) have received considerable attention as potential targets for the development of novel cancer immunotherapies. However, the clinical efficacy of DC-based vaccines remains suboptimal, largely reflecting local and systemic immunosuppression at baseline. An autologous DC-based vaccine (DCVAC) has recently been shown to improve progression-free survival and overall survival in randomized clinical trials enrolling patients with lung cancer (SLU01, NCT02470468) or ovarian carcinoma (SOV01, NCT02107937), but not metastatic castration-resistant prostate cancer (SP005, NCT02111577), despite a good safety profile across all cohorts.

Novel Patient-Reported Outcome Measures for the Assessment of Patient Satisfaction and Health-Related Quality of Life Following Postmastectomy Breast Reconstruction.

Background: Patient-reported outcome measures (PROMs) have become an integral part of the evaluation of reconstruction surgery outcomes. However, there are limitations in current PROMs when it comes to the assessment of well-being during inpatient stay, patient perception of health, relationship with partner, and vitality (i.e.

Quality of life in CRSwNP: evaluation of ACCESS and Lund-Mackay computed tomography scores versus the QoL questionnaire.

Purpose: Recently, a new CT scan classification of the extent of previous endoscopic sinus surgery called The Amsterdam Classification of Completness of Endoscopic Sinus Surgery (ACCESS) was published. The aim of this study was to investigate possible associations between traditionally used Lund-Mackay CT score and ACCESS score and their possible relationship to pre- and postoperative quality of life in a narrowly defined group of patients with recurrent CRSwNP.

Methods: Forty-nine patients who underwent a revision ESS for CRSwNP were enrolled in a retrospective study.

Multiscale analysis of craniomaxillofacial bone repair: A preclinical mini-pig study.

Background: The rate of reparative osteogenesis controls when an implant is sufficiently stable as to allow functional loading. Using a mini pig model, the rate of reparative osteogenesis in two types of implant sites for example, an osteotomy versus a fresh extraction socket were compared.

Methods: Eight adult mini pigs were used for the study.

Animal Models Used in the Research of Vascularized Lymph Node Transfer: A Systematic Review.

Background: Lymphedema is a common adverse consequence of breast cancer therapy, while still relatively little is known about its pathophysiology. Several treatment options emerged over the past decades, and among them, vascularized lymph node transfer (VLNT) seems to be particularly promising. Animal models are indispensable to improve our understanding of the underlying processes surrounding the transplantation of a vascularized lymph node.

Oral Ibandronate Therapy in Three Patients with Osteogenesis Imperfecta.

Introduction: Treatment with orally administered ibandronate is an effective way to increase bone mineral density (BMD) and reduce fracture rate in post-menopausal women and in men with osteoporosis. There are only very few reports concerning ibandronate therapy in children and adolescents, and in patients with osteogenesis imperfecta (OI), as bisphosphonates are not registered for therapeutic use in pediatrics.

Case Report: We present three patients with OI, where once-monthly oral ibandronate increased spinal BMD after two and four years, respectively, of therapy without any occurrence of new fractures and no adverse reactions.

Clinical decision support system RHINA in the diagnosis and treatment of acute or chronic rhinosinusitis.

Background: Rhinosinusitis is an inflammation of the sinonasal cavity which affects roughly one in seven people per year. Acute rhinosinusitis (ARS) is mostly, apart from allergic etiology, caused by a viral infection and, in some cases (30-50%), by a bacterial superinfection. Antibiotics, indicated only in rare cases according to EPOS guidelines, are nevertheless prescribed in more than 80% of ARS cases, which increases the resistant bacterial strains in the population.

Otorhinolaryngology in the COVID-19 era: Are there significant differences between hospital-based and private practices?

Objectives: The COVID-19 disease has an incredible impact on both hospital-based and private practices in the field of otorhinolaryngology and head and neck surgery. Practical issues faced by both types of practices have not been well addressed in most studies. A national survey was conducted in April 2020 to identify the challenges faced by otorhinolaryngologists practicing in the Czech Republic.

Extremely wicked, shockingly evil and undoubtedly COVID-19: the silent serial killer.

Background: The SARS-COV-2 virus has more than just an infectious role to play in the society. The rapid spread has also led to significant personal, professional, financial and economic recession globally. Health care professionals are getting seriously compromised due to these issues.

Which medical disciplines diagnose and treat melanoma in Europe in 2019? A survey of experts from melanoma centres in 27 European countries.

Background And Objectives: The incidence of melanoma is increasing. This places significant burden on societies to provide efficient cancer care. The European Cancer Organisation recently published the essential requirements for quality melanoma care.

A novel cryo-embedding method for in-depth analysis of craniofacial mini pig bone specimens.

The disconnect between preclinical and clinical results underscores the imperative for establishing good animal models, then gleaning all available data on efficacy, safety, and potential toxicities associated with a device or drug. Mini pigs are a commonly used animal model for testing orthopedic and dental devices because their skeletons are large enough to accommodate human-sized implants. The challenge comes with the analyses of their hard tissues: current methods are time-consuming, destructive, and largely limited to histological observations made from the analysis of very few tissue sections.

Patient-Reported Outcomes of Three Different Types of Breast Reconstruction with Correlation to the Clinical Data 5 Years Postoperatively.

Background: The aim of this study was to compare long-term clinical and patient-reported outcomes in terms of satisfaction and health-related quality of life (HRQoL) following postmastectomy breast reconstruction using BREAST-Q in patients undergoing implant-based reconstruction, abdominal-based autologous reconstruction, and combined reconstruction (with implant and LD flap or implant and TDAP flap).

Methods: A cross-sectional study was conducted. Patients had undergone delayed postmastectomy breast reconstruction and completed the BREAST-Q reconstruction module.

M2-like macrophages dictate clinically relevant immunosuppression in metastatic ovarian cancer.

Background: The immunological microenvironment of primary high-grade serous carcinomas (HGSCs) has a major impact on disease outcome. Conversely, little is known on the microenvironment of metastatic HGSCs and its potential influence on patient survival. Here, we explore the clinical relevance of the immunological configuration of HGSC metastases.

Face to face with COVID-19: highlights of challenges encountered in various ENT practices across the Czech Republic (a national survey).

The SARS-CoV-2 virus causing the COVID-19 disease created considerable complications and disruptions due to it's pandemic spread. The nature of the disease left certain clinical specialties more vulnerable than others, causing serious problems with organization of care of patients. Most studies concentrated on protocols and strategies to control the situation and reduce further spread amongst medical fields.

Calreticulin exposure correlates with robust adaptive antitumor immunity and favorable prognosis in ovarian carcinoma patients.

Background: Adjuvanticity, which is the ability of neoplastic cells to deliver danger signals, is critical for the host immune system to mount spontaneous and therapy-driven anticancer immune responses. One of such signals, i.e.

TIM-3 Dictates Functional Orientation of the Immune Infiltrate in Ovarian Cancer.

Purpose: In multiple oncological settings, expression of the coinhibitory ligand PD-L1 by malignant cells and tumor infiltration by immune cells expressing coinhibitory receptors such as PD-1, CTLA4, LAG-3, or TIM-3 conveys prognostic or predictive information. Conversely, the impact of these features of the tumor microenvironment on disease outcome among high-grade serous carcinoma (HGSC) patients remains controversial.

Experimental Design: We harnessed a retrospective cohort of 80 chemotherapy-naïve HGSC patients to investigate PD-L1 expression and tumor infiltration by CD8 T cells, CD20 B cells, DC-LAMP dendritic cells as well as by PD-1, CTLA4, LAG-3, and TIM-3 cells in relation with prognosis and function orientation of the tumor microenvironment.

Syndromes that predispose to epistaxis.

Purpose: Our aim was to evaluate if epistaxis is directly associated with the etiology or pathophysiological mechanism which results in the syndrome itself or arises as a secondary effect.

Methods: We performed an extensive literature review of the web-based PubMed database from the National Library of Medicine to ascertain syndromes related to this condition. Etiology, pathophysiological mechanisms, occurrence, clinical features and management were noted for each of these syndromes.

Mature dendritic cells correlate with favorable immune infiltrate and improved prognosis in ovarian carcinoma patients.

A high density of tumor-infiltrating CD8 T cells and CD20 B cells correlates with prolonged survival in patients with a wide variety of human cancers, including high-grade serous ovarian carcinoma (HGSC). However, the potential impact of mature dendritic cells (DCs) in shaping the immune contexture of HGSC, their role in the establishment of T cell-dependent antitumor immunity, and their potential prognostic value for HGSC patients remain unclear. We harnessed immunohistochemical tests and biomolecular analyses to demonstrate that a high density of tumor-infiltrating DC-LAMP DCs is robustly associated with an immune contexture characterized by T1 polarization and cytotoxic activity.

Access to innovative medicines for metastatic melanoma worldwide: Melanoma World Society and European Association of Dermato-oncology survey in 34 countries.

According to data from recent studies from Europe, a large percentage of patients have restricted access to innovative medicines for metastatic melanoma. Melanoma World Society and European Association of Dermato-oncology conducted a Web-based survey on access to first-line recommended treatments for metastatic melanoma by current guidelines (National Comprehensive Center Network, European Society for Medical Oncology [ESMO] and European Organization for Research and Treatment of Cancer/European Association of Dermato-oncology/European dermatology Forum) among melanoma experts from 27 European countries, USA, China, Australia, Argentina, Brazil, Chile and Mexico from September 1st, 2017 to July 1st, 2018. Data on licencing and reimbursement of medicines and the number of patient treated were correlated with the data on health expenditure per capita (HEPC), Mackenbach score of health policy performance, health technology assessment (HTA), ASCO and ESMO Magnitude of clinical benefit scale (ESMO MCBS) scores of clinical benefit and market price of medicines.

Partial maxillectomy for ameloblastoma of the maxilla with infratemporal fossa involvement: A combined endoscopic endonasal and transoral approach.

Ameloblastoma represents the most common epithelial odontogenic tumor. Because of the proximity of the maxillary tumors to the orbit and skull base, it should be managed as radically as possible. Maxillectomy, mainly via the transfacial or transoral approach, represents the most common type of surgical procedure.

Frameshift mutations in the insulin gene leading to prolonged molecule of insulin in two families with Maturity-Onset Diabetes of the Young.

Mutations in the insulin (INS) gene rarely occur in patients with Maturity-Onset Diabetes of the Young (MODY). We aimed to describe in detail two MODY families with INS mutations. The INS gene was screened by direct sequencing.