Diagnosis and treatment of Fabry disease. Expert Opinion of the Polish Cardiac Society and the Polish Forum for Fabry Disease.

Fabry disease (FD) belongs to the group of lysosomal storage diseases (LSD), which are characterised by insufficient activity of enzymes responsible for the intra-lysosomal breakdown of various substrates. The result is an uncontrolled accumulation of by-products of cellular metabolism. Lysosomal storage diseases are inherited diseases, transmitted mainly in an autosomal recessive fashion.

S-ICD Implantation "Tips and Tricks".

An implantable cardioverter-defibrillator (ICD) was developed to provide protection against sudden cardiac death. Despite being effective in terminating ventricular arrhythmias, traditional transvenous ICDs appeared over time to have certain limitations related to the need for vascular access and the presence of foreign material inside the circulatory system (namely lead failure and infections). A subcutaneous implantable cardioverter-defibrillator (S-ICD) was developed to overcome those limitations and to provide prevention against sudden cardiac death from outside the cardiovascular system.

Infections with in Children Undergoing Anticancer Therapy or Hematopoietic Cell Transplantation: A Multicenter Nationwide Study.

is a nosocomial pathogen that causes severe infections in immunocompromised patients. The aim of the study was to conduct a microbiological and clinical analysis of infections in children with malignancies or undergoing hematopoietic cell transplantation in Poland. We conducted a retrospective, multicenter study including children and adolescents under 19 years old treated between 2012 and 2021.

Lung Ultrasound Score in Neonatal RDS: Agreement between Sonography Expert and Neonatologists with Varying Levels of Experience.

This study aimed to assess interrater agreement in lung ultrasound scores (LUS) among neonatologists with varying experience levels and an expert sonographer. A post hoc analysis was conducted on data from a prospective multicenter study involving 155 infants born <34 weeks' gestation, all with respiratory distress syndrome. A total of 629 lung scans were performed and video-recorded by 21 point-of-care sonographers, including both experienced (n = 7) and inexperienced (n = 14) evaluators.

Survival and Treatment Modalities in Primary Vaginal Melanoma-Case Report and a Narrative Review.

Primary vaginal melanoma (PVM) is a rare cancer representing five percent of vaginal cancers and less than one percent of all female vaginal melanomas, with an incidence rate of 0.46 per million women per year. The aim of this study was to present a case of combined therapy and conservative surgical treatment in a young patient with PVM and to perform a systematic review of the same subject.

Polish Cystic Fibrosis Patients' Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study.

Cystic fibrosis (CF) is a disease characterized by long-term and troublesome symptoms that affect the patient's life. This study aimed to assess and compare the health-related quality of life (HRQoL) of Polish CF patients and identify factors influencing it. The study group consisted of 79 patients (6 to 42 years old), who filled in an age-appropriate Cystic Fibrosis Questionnaire-Revised.

The Influence of Gestational Diabetes Mellitus on Maternal and Neonatal Outcomes: A Retrospective Study in Rzeszów, Poland.

BACKGROUND Gestational diabetes mellitus (GDM) affects 5.8-12.9% of pregnant women, while pre-gestational diabetes mellitus (PGDM) affects 0.

Managing Undernutrition in Pediatric Oncology: A Consensus Statement Developed Using the Delphi Method by the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology.

"Managing Undernutrition in Pediatric Oncology" is a collaborative consensus statement of the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology. The early identification and accurate management of malnutrition in children receiving anticancer treatment are crucial components to integrate into comprehensive medical care. Given the scarcity of high-quality literature on this topic, a consensus statement process was chosen over other approaches, such as guidelines, to provide comprehensive recommendations.

Religious meaning system and life satisfaction: the mediating role of meaning in life among Polish people with multiple sclerosis.

Introduction: The complexity of the associations between religiosity and indicators of well-being suggests the presence of a mediating mechanism. Previous studies indicate that religion may influence subjective well-being because it helps to find meaning and purpose. Therefore, the aim of our study was to examine the mediating role of the presence and search dimensions of meaning in life in the relationship between religious meaning system and life satisfaction in patients with multiple sclerosis (MS).

Prognostic Relevance of the Lung Ultrasound Score: A Multioutcome Study in Infants with Respiratory Distress Syndrome.

Objective: There is growing evidence for the usefulness of the lung ultrasound score (LUS) in neonatal intensive care. We evaluated whether the LUS is predictive of outcomes in infants with respiratory distress syndrome (RDS).

Study Design: Neonates less than 34 weeks of gestational age were eligible for this prospective, multicenter cohort study.

In Silico Analysis, Anticonvulsant Activity, and Toxicity Evaluation of Schisandrin B in Zebrafish Larvae and Mice.

The aim of this study is to evaluate the anticonvulsant potential of schisandrin B, a main ingredient of extracts. Schisandrin B showed anticonvulsant activity in the zebrafish larva pentylenetetrazole acute seizure assay but did not alter seizure thresholds in the intravenous pentylenetetrazole test in mice. Schisandrin B crosses the blood-brain barrier, which we confirmed in our in silico and in vivo analyses; however, the low level of its unbound fraction in the mouse brain tissue may explain the observed lack of anticonvulsant activity.

Prevention of Cardiac Implantable Electronic Device Infections: A Review.

The importance of cardiac implantable electronic devices (CIEDs) in the treatment of cardiac rhythm disturbances, heart failure, and the prevention of sudden cardiac death is indisputable. However, CIED therapy is associated with complications, among which infections are particularly unfavourable in terms of prognosis. The diagnosis and management of CIED infections remain complex, with a significant impact on mortality and healthcare costs.

High in-hospital mortality and prevalence of cardiogenic shock in patients with ST-segment elevation myocardial infarction and concomitant COVID-19.

Introduction: Published data suggest worse outcomes in acute coronary syndrome (ACS) patients with concomitant coronavirus disease (COVID-19) due to delays in standard management caused by burdened healthcare.

Aim: To report the demographics, angiographic findings, and in-hospital outcomes of COVID-19 ST-elevation myocardial infarction (STEMI) patients and to compare these with the non-COVID-19 cohort hospitalized during the same period with the same access to medical care.

Material And Methods: From October 23, 2020 to April 23, 2021 (exactly 6 months) data were collected into a prospective ACS Registry.

Defensins of Larvae and Their Influence on Wound Repair Processes in Practical Assessment-A Study of Three Cases.

Bacteria inhabiting chronic wounds form a biofilm that prolongs and slows down the healing process. Increasingly common antibiotic resistance requires clinicians to search for effective and alternative treatment methods. Defensins are the most common antimicrobial peptides capable of eradicating pathogens.

Subcutaneous implantable cardioverter-defibrillator therapy in Poland: Results of the Polish S-ICD Registry.

Background: The use of subcutaneous implantable cardioverter-defibrillators (S-ICD) has been growing in Poland since 2014. The Polish Registry of S-ICD Implantations was run by the Heart Rhythm Section of the Polish Cardiac Society between May 2020 and September 2022 to monitor the implementation of that therapy in Poland.

Aims: To investigate and present the state-of-the-art of S-ICD implantation in Poland.

Mental Health of Cystic Fibrosis Patients and the COVID-19 Pandemic in Poland: A Single-Center Comparative Study.

Research shows that people with cystic fibrosis are more prone to suffer from psychological problems than healthy people; thus, the outbreak of the COVID-19 pandemic in Poland could have had an impact on their mental health. To assess this impact, we examined the mental health of patients before and during the pandemic. Survey participants were asked to fill in questionnaires that consisted of Beck Depression Inventory (BDI), 12-Item General Health Questionnaire (GHQ-12) and Cystic Fibrosis Questionnaire-Revised (CFQ-R; for the purpose of the study, an emotional functioning domain was used) during their hospital visits.

Selected Factors Influencing the Intensity of Postoperative Pain in Patients after Orthopedic and Gynecological Surgeries.

Currently, pain is considered the fifth vital sign, and its effective relief is a priority in many surgical departments. The aim of this study was to determine the influence of selected factors on the intensity of postoperative pain after orthopedic and gynecological procedures. The study was conducted in a group of 200 patients undergoing orthopedic and gynecological procedures at the County Hospital in Nisko between August 2017 and January 2018.

The GRACE risk score in patients with ST-segment elevation myocardial infarction and concomitant COVID-19.

Introduction: Acute coronary syndrome represents a major cause of mortality throughout the world. To date, there are only a few reports of ST-segment elevation type 1 myocardial infarction in patients with COVID-19. The aim of this study was to describe the clinical and angiographic characteristics alongside the prediction of in-hospital mortality using the GRACE risk score in this group.