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.

Intrauterine deaths - an unsolved problem in Polish perinatology.

Objectives: The Polish criteria for "intrauterine death" include fetal demise after 22 weeks of gestation, weighing > 500 g and body length at least 25 cm, when the gestational age is unknown. The rate of fetal death in Poland in 2015 is 3:10,000. In 2020, 1,231 stillbirths were registered.

Relationships between CD34-, CD105- and bcl-2-Expression Levels and Contrast-Enhanced Ultrasound-Based Differential Diagnosis of Adnexal Tumours.

The relationships between CEUS parameters of adnexal tumours and postoperative immunohistochemical assessments of CD34, CD105 and bcl-2 were analysed. This study aimed to investigate whether contrast-enhanced ultrasonography (CEUS) parameters depend on the microvascular density of the tumour lesion found after surgery. Fifty-one patients with a diagnosis of adnexal tumours were included in this single-centre, prospective study.

Evaluation of Health Pattern and Stress Levels among Patients Undergoing Alcohol Addiction Treatment-A Cross Sectional Study.

Alcohol consumption is linked to over 200 diseases and injuries. It is also classified as a risk factor for several types of neoplasms as well as infectious diseases (i.e.

Basics of prevention and management of iodine-based contrast media-induced thyroid dysfunction - position paper by the Polish Society of Endocrinology.

Medical practice involves a high number of radiological examinations using iodinated contrast media (ICM). Therefore, it is crucial for doctors of different specialties to be aware of possible adverse effects associated with ICM use. The most common and well characterized adverse effect is contrast-induced nephropathy, whereas thyroidal adverse reactions remain a diagnostic and therapeutic dilemma.

Nurses' Readiness to Undertake Controlled Negative Pressure Therapy in the Treatment of Chronic Wounds-Research Report.

Local wound treatment with negative pressure wound therapy (NPWT) shortens the healing process but requires the supervision of trained medical personnel for administering the therapeutic procedures. Professional supervision and control of the effectiveness of NPWT, as well as education conducted by nurses, are of particular importance for therapeutic and caring processes, both in hospital and at home. The aim of the study was the assessment of the perception of NPWT by certified nurses in the topical treatment of chronic wounds.

Risk of venous thromboembolism during the use of oral estrogen-progestogen hormone therapies in light of most recent research findings.

Two important studies evaluating the safety profile of oral estrogen-progestogen hormonal therapies conducted in standard clinical practice with respect to the venous system were recently published. A large prospective controlled cohort study (PRO-E2) based on the non-inferiority design has shown that the relative risk of developing venous thrombosis (VTE) in women using combined oral hormonal contraceptives (COHC) containing 17β-estradiol (1.5 mg) and nomegestrol acetate (2.

Colorectal neuroendocrine neoplasms - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne jelita grubego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynych)].

Colorectal neuroendocrine neoplasm (CRNEN), especially rectal tumours, are diagnosed with increased frequency due to the widespread use of colonoscopy, including screening examinations. It is important to constantly update and promote the principles of optimal diagnostics and treatment of these neoplasms. Based on the latest literature and arrangements made at the working meeting of the Polish Network of Neuroendocrine Tumours (June 2021), this paper includes updated and supplemented data and guidelines for the management of CRNEN originally published in Endokrynologia Polska 2017; 68: 250-260.

Neuroendocrine neoplasms of the small intestine and the appendix - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)].

Updated Polish recommendations for the management of patients with neuroendocrine neoplasms (NENs) of the small intestine (SINENs) and of the appendix (ANENs) are presented here. The small intestine, and especially the ileum, is one of the most common locations for these neoplasms. Most of them are well-differentiated and slow-growing tumours; uncommonly - neuroendocrine carcinomas.

Pancreatic neuroendocrine neoplasms - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polską Sieć Guzów Neuroendokrynych)].

In this paper, we present the current guidelines for the diagnostics and management of pancreatic neuroendocrine neoplasms (PanNENs) developed by Polish experts providing care for these patients in everyday clinical practice. In oncological diagnostics, in addition to biochemical tests, molecular identification with the use of NETest liquid biopsy and circulating microRNAs is gaining importance. Both anatomical and functional examinations (including new radiopharmaceuticals) are used in imaging diagnostics.

Gastroduodenal neuroendocrine neoplasms including gastrinoma - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma - uaktualnione zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)].

After another meeting of experts of the Polish Network of Neuroendocrine Tumours, updated recommendations for the management of patients with gastric and duodenal neuroendocrine neoplasms, including gastrinoma, have been issued. As before, the epidemiology, pathogenesis and clinical symptoms of these neoplasms have been discussed, as well as the principles of diagnostic procedures, including biochemical and histopathological diagnostics and tumour localisation, highlighting the changes introduced in the recommendations. Updated principles of therapeutic management have also been presented, including endoscopic and surgical treatment, and the options of pharmacological and radioisotope treatment.

Update of the diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours) [Aktualizacja zaleceń ogólnych dotyczących postępowania diagnostyczno-terapeutycznego w nowotworach neuroendokrynnych układu pokarmowego (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)].

Continuous progress in the diagnostics and treatment of neuroendocrine neoplasms (NENs), the emerging results of new clinical trials, and the new guidelines issued by medical societies have prompted experts from the Polish Network of Neuroendocrine Tumours to update the 2017 recommendations regarding the management of neuroendocrine neoplasms. This article presents the general recommendations for the management of NENs, resulting from the findings of the experts participating in the Fourth Round Table Conference, entitled "Polish Guidelines for the Diagnostics and Treatment of Neuroendocrine Neoplasms of the gastrointestinal tract, Żelechów, June 2021". Drawing from the extensive experience of centres treating these cancers, we hope that we have managed to formulate the optimal method of treating patients with NENs, applying the latest reports and achievements in the field of medicine, which can be effectively implemented in our country.

Assessment of Knowledge on the Prevention of Central-Line-Associated Bloodstream Infections among Intensive Care Nurses in Poland-A Prospective Multicentre Study.

The presence of a central venous catheter (CVC) leads to a high risk of blood infections, which are considered major causes of morbidity, mortality and high medical costs. The aim of this study was to assess the knowledge of nursing staff working in intensive care units (ICUs) regarding the prevention of central-line-associated bloodstream infections (CLABSIs). A nationwide survey was conducted among ICU nurses from August 2016 to April 2017.

"Vaccinate, Do Not Hesitate!". Vaccination Readiness against COVID-19 among Polish Nursing Undergraduate Students: A National Cross-Sectional Survey.

COVID-19 vaccination raises numerous concerns among the public, and also among medical personnel including nurses. As nurses play a crucial role in the process of vaccination, it is important to recognize the attitudes of students of nursing, nurses in spe, toward COVID-19 vaccination, as well as to define the factors influencing students' pro-vaccine choices. The study was conducted between March and April 2021 at all medical universities in Poland educating nurses in spe.

Three-year (2017-2019) activity report of the Replantation Service for hand amputations in Poland.

This article summarises activity of the Replantation Service for hand amputations in Poland in years 2017-2019. Over this period a total of 551 cases of total and subtotal amputations as well as other complex injuries to the hand were referred to the referenced centres. Of these, 330 referrals were accepted and 221 rejected, for various reasons.

Effects of a nine-strain bacterial synbiotic compared to simethicone in colicky babies - an open-label randomised study.

The aim of the study was to determine effects of administration of simethicone and a multi-strain synbiotic on the crying behaviour of colicky babies. The study design consisted of an open-label, two parallel treatment group study involving 87 infants aged 3-6 weeks with infantile colic (defined as crying episodes lasting 3 or more hours per day and occurring at least 3 days per week within 3 weeks prior to enrolment) randomly, unequally [1:1.5] assigned to receive simethicone (n=33) or a multi-strain synbiotic (n=54) orally for 4 weeks.

Infantile Colic-The Perspective of German and Polish Pediatricians in 2020.

The objective of the study was to characterize how infantile colic is perceived and managed by German and Polish pediatricians. Data in both countries were collected by using a paper questionnaire with seven questions and predefined and free text fields for the answers. Answers from 160 German and 133 Polish pediatricians were collected.

Novel mutation in the first fully-diagnosed patient with Fanconi anemia in Polish population - case report.

Background: Fanconi anemia is a rare genetic disorder caused by mutations in genes which protein products are involved in replication, cell cycle control and DNA repair. It is characterized by congenital malformations, bone marrow failure, and high risk of cancer. The diagnosis is based on morphological and hematological abnormalities such as pancytopenia, macrocytic anaemia and progressive bone marrow failure.

Simultaneous placement of leadless pacemaker and dialysis catheter in patient with exhausted vasculature.

The problem with limited venous access may occur in patients receiving long-term hemodialysis treatment with no possibility of arteriovenous access or in patients with cardiac implantable electronic device-related infection leading to the removal of cardiac implantable electronic device. We present a case report where both situations occur simultaneously. Using recent development in cardiac pacing-leadless cardiac pacemaker-we manage to overcome the vascular access problem.

Diagnostic value of implantable loop recorder in patients undergoing cryoballoon ablation of atrial fibrillation.

Background: Due to limited data, implantable loop recorders (ILR) are not currently recommended by the guidelines to routinely monitor patients after atrial fibrillation (AF) ablation.

Aims: To validate the diagnostic value of ILR after AF ablation, modern generation ILRs (LINQ) were implanted in patients scheduled for cryoballoon ablation of AF (CBA).

Methods: We included 29 patients with frequent and symptomatic episodes of paroxysmal AF.

Influence of Biological Parameters Assessed in [18F]FDG PET/CT on Overall Survival in Cervical Cancer Patients.

Aim: The aim of this study was to estimate the influence of biological parameters assessed in [F]FDG PET/CT on overall survival (OS) in cervical cancer patients.

Methods: Retrospective analysis was performed on a group of 371 patients with newly diagnosed and histologically confirmed cervical cancer. PET biological parameters in primary tumor including SUVmax, SUVmean, total lesion glycolysis (TLG), metabolic tumor volume (MTV), heterogeneity, and parameters referring both to primary tumor and metastatic lesions: SUVtotal, TLGtotal, and MTVtotal, were analyzed.

Transesophageal echocardiography.

Transthoracic and transesophageal examinations should be considered as mutually complementary. Transesophageal echocardiography is performed in cases of a justified need to visualize structures that are poorly visible or invisible on transthoracic echocardiogram. Primary indications for transesophageal echocardiography include an assessment of cardiac source of embolism, suspected endocarditis, suspected prosthetic valve dysfunction, an assessment of thoracic aorta and other vessels, an assessment prior to valvular repairs and closures of septal defects, intraoperative monitoring of cardiac or percutaneous interventions, ablation, non-diagnostic transthoracic examination, especially in patients after cardiac surgeries.

Stability studies of two compounded solutions potentially used in tumor lysis syndrome.

Objective: The aim of the study was to determine the stability of two non-commercially produced solutions: 1.68% sodium bicarbonate in 5% glucose (BIC solution) and 1.6% calcium chloride in 0.