Rare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation.

Objectives: The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).

Methods: A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a pair of authors to perform a literature search and article review.

IL-6 Signaling Link between Inflammatory Tumor Microenvironment and Prostatic Tumorigenesis.

Benign prostatic hyperplasia and prostate cancer are tumoral pathologies characterized by the overexpression of inflammatory processes. The exploration of tumor microenvironment and understanding the sequential events that take place in the stromal area of the prostate could help for an early management of these pathologies. This way, it is feasible the hypothesis that normalizing the stromal environment would help to suppress or even to reverse tumor fenotype.

International eDelphi Study to Reach Consensus on the Methotrexate Dosing Regimen in Patients With Psoriasis.

Importance: A clear dosing regimen for methotrexate in psoriasis is lacking, and this might lead to a suboptimal treatment. Because methotrexate is affordable and globally available, a uniform dosing regimen could potentially optimize the treatment of patients with psoriasis worldwide.

Objective: To reach international consensus among psoriasis experts on a uniform dosing regimen for treatment with methotrexate in adult and pediatric patients with psoriasis and identify potential future research topics.

The use of antenatal corticosteroids for fetal maturation: clinical practice guideline by the WAPM-World Association of Perinatal Medicine and the PMF-Perinatal Medicine foundation.

This practice guideline follows the mission of the World Association of Perinatal Medicine in collaboration with the Perinatal Medicine Foundation, bringing together groups and individuals throughout the world, with the goal of improving the use of antenatal corticosteroids (ACS) for fetal maturation. In fact, this document provides further guidance for healthcare practitioners on the appropriate use of ACS with the aim to increase the timely administration and avoid unnecessary or excessive use. Therefore, it is not intended to establish a legal standard of care.

Outcome of SARS-CoV-2-Infected Polish Patients with Chronic Lymphocytic Leukemia.

Background: The severe acute respiratory syndrome coronavirus (SARS-CoV-2) has become the cause of a worldwide pandemic, and its clinical infection course in patients with hematological malignancies may be severe.

Methods: We performed a retrospective study on 188 chronic lymphocytic leukemia patients (CLL) with COVID-19 infection.

Results: At the time of infection 51 patients (27.

Melflufen or pomalidomide plus dexamethasone for patients with multiple myeloma refractory to lenalidomide (OCEAN): a randomised, head-to-head, open-label, phase 3 study.

Background: Melphalan flufenamide (melflufen), an alkylating peptide-drug conjugate, plus dexamethasone showed clinical activity and manageable safety in the phase 2 HORIZON study. We aimed to determine whether melflufen plus dexamethasone would provide a progression-free survival benefit compared with pomalidomide plus dexamethasone in patients with previously treated multiple myeloma.

Methods: In this randomised, open-label, head-to-head, phase 3 study (OCEAN), adult patients (aged ≥18 years) were recruited from 108 university hospitals, specialist hospitals, and community-based centres in 21 countries across Europe, North America, and Asia.

Diabetic Mastopathy. Review of Diagnostic Methods and Therapeutic Options.

Diabetic mastopathy is a rare breast condition that may occur in insulin-treated men and women of any age. The etiology is still unclear; however, the autoimmunological background of the disease is highly suspected. The changes in diabetic mastopathy may mimic breast cancer; therefore, its diagnostic process is demanding, and treatment options are not clear and limited.

Information impact on synaptic arousal and formation of permanent memory trace.

Memory trace is an effect of temporary arousal (perception, experience, action) that causes a specific change in the nervous system. Memory allows to record and recall various information, thus enabling to learn new things. It is an extremely active and dynamic process.

State of the Art in Hepatic Dysfunction in Pregnancy.

Hepatic dysfunction in pregnant women is always challenging for the obstetrician, as the spectrum of hepatic abnormalities can be very large and have various implications, both for mother and fetus. There is a diagnostic and therapeutic polymorphism of hepatic dysfunction in pregnancy and insufficient knowledge related to the etiopathogenesis and epidemiology of this disease. The clinical forms of hepatic dysfunction encountered in pregnancy can vary from liver diseases related to pregnancy (e.

COVID-19 severity and mortality in patients with CLL: an update of the international ERIC and Campus CLL study.

Patients with chronic lymphocytic leukemia (CLL) may be more susceptible to Coronavirus disease 2019 (COVID-19) due to age, disease, and treatment-related immunosuppression. We aimed to assess risk factors of outcome and elucidate the impact of CLL-directed treatments on the course of COVID-19. We conducted a retrospective, international study, collectively including 941 patients with CLL and confirmed COVID-19.

Why Did All Patients with Atrial Fibrillation and High Risk of Stroke Not Receive Oral Anticoagulants? Results of the Polish Atrial Fibrillation (POL-AF) Registry.

Background: Most atrial fibrillation (AF) patients are at high risk of thromboembolic, and the use of oral anticoagulants (OACs) is advised in such cases. The aim of the study was to evaluate the frequency at which OACs were used in patients with AF and high risk thromboembolic complications, and identify factors that result in OACs not being used in the researched group of patients.

Methods: The prospective, multicenter and non-interventional POL-AF registry is a study that includes AF patients from ten Polish cardiology centers.

'First week' is the crucial period for deciding living donor liver transplantation in patients with acute-on-chronic liver failure.

Background And Aims: Acute-on-chronic liver failure (ACLF) is a rapidly progressive illness with high short-term mortality. Timely liver transplant (LT) may improve survival. We evaluated various indices for assessment of the severity of liver failure and their application for eligibility and timing of living donor LT (LDLT).

Practical management of epidermolysis bullosa: consensus clinical position statement from the European Reference Network for Rare Skin Diseases.

Inherited epidermolysis bullosa (EB) comprises rare disorders that manifest with fragility and blistering of the skin and mucous membranes, with variable clinical severity. Management of EB is challenging due to disease rarity and complexity, the wide range of extracutaneous manifestations and a profound impact on daily life for the patient and family members. Although reference centres providing multidisciplinary care for EB exist in each European country, it is common for healthcare professionals that are not specialized in this rare disorder to treat EB patients.

Eosinophilic vacuolated tumor (EVT) of kidney demonstrates sporadic TSC/MTOR mutations: next-generation sequencing multi-institutional study of 19 cases.

A distinct renal tumor has recently been described as "high-grade oncocytic renal tumor" and "sporadic renal cell carcinoma with eosinophilic and vacuolated cytoplasm". The Genitourinary Pathology Society (GUPS) consensus proposed a unifying name "eosinophilic vacuolated tumor" (EVT) for this emerging entity. In this multi-institutional study, we evaluated 19 EVTs, particularly their molecular features and mutation profile, using next-generation sequencing.

Biologicals in childhood severe asthma: the European PERMEABLE survey on the .

Introduction: Severe asthma is a rare disease in children, for which three biologicals, anti-immunoglobulin E, anti-interleukin-5 and anti-IL4RA antibodies, are available in European countries. While global guidelines exist on who should receive biologicals, knowledge is lacking on how those guidelines are implemented in real life and which unmet needs exist in the field. In this survey, we aimed to investigate the and identify open questions in biological therapy of childhood asthma across Europe.

Radical surgical treatment of neuroendocrine tumors of the appendix in children - a Polish multicenter study.

Introduction: The aim of the study was to examine management of pediatric appendiceal neuroendocrine tumors (ANETs) in Poland.

Methods: Records of 27 patients with ANET diagnosed incidentally after appendectomy in the last decade.

Results: Well-differentiated NET G1/G2 was diagnosed in 25 and well-differentiated neuroendocrine carcinoma G3 in 2 patients.

Dynamic assessments of hepatic encephalopathy and ammonia levels predict mortality in acute-on-chronic liver failure.

Background: We evaluated the dynamics of hepatic encephalopathy (HE) and ammonia estimation in acute-on-chronic liver failure (ACLF) patients due to a paucity of evidence.

Methods: ACLF patients recruited from the APASL-ACLF Research Consortium (AARC) were followed up till 30 days, death or transplantation, whichever earlier. Clinical details, including dynamic grades of HE and laboratory data, including ammonia levels, were serially noted.

Comparative accuracy of prognostic models for short-term mortality in acute-on-chronic liver failure patients: CAP-ACLF.

Background: Multiple predictive models of mortality exist for acute-on-chronic liver failure (ACLF) patients that often create confusion during decision-making. We studied the natural history and evaluated the performance of prognostic models in ACLF patients.

Methods: Prospectively collected data of ACLF patients from APASL-ACLF Research Consortium (AARC) was analyzed for 30-day outcomes.

Hyperuricemia as a Marker of Reduced Left Ventricular Ejection Fraction in Patients with Atrial Fibrillation: Results of the POL-AF Registry Study.

: Hyperuricemia is an established risk factor for cardiovascular disease, including atrial fibrillation (AF). The prevalence of hyperuricemia and its clinical significance in patients with already diagnosed AF remain unexplored. : The Polish Atrial Fibrillation (POL-AF) registry includes consecutive patients with AF hospitalized in 10 Polish cardiology centers from January to December 2019.

Is Misoprostol Vaginal Insert Safe for the Induction of Labor in High-Risk Pregnancy Obese Women?

Induction of labor (IOL) is an event that occurs in up to 25% of pregnancies. In Europe, the misoprostol vaginal insert (MVI-Misodel) was approved for labor induction in 2013. Studies on the outcomes and safety of IOL in obese pregnant women are scarce; no data are available on MVI IOL in high-risk pregnancy obese women (HRPO-late-term, hypertension, diabetes).

Unexpected location of endometrial adenocarcinoma - a case report.

Endometrial adenocarcinoma (ADK) is one of the most common uterine cancer and the fourth neoplasia mortality cause in women according to the literature data. ADK is encountered in the sixth decade of life, the mean age being 63 years. Only 2-5% of cases are found in women less than 40 years old.