MMP-8 causes leftward shift in end-diastolic pressure-volume relationship and may explain the development of diastolic dysfunction in septic cardiomyopathy.

Septic cardiomyopathy (SCM) with diastolic dysfunction carries a poor prognosis, and the mechanisms underlying the development of diastolic dysfunction remain unclear. Matrix metalloproteinase-8 (MMP-8) is released from neutrophils and degrades collagen I. MMP-8 levels correlate with SCM severity.

Implementation of pooled saliva tests for universal screening of cCMV infection.

Congenital cytomegalovirus (cCMV) is the most common intrauterine infection, leading to neurodevelopmental disabilities. Universal newborn infant screening of cCMV has been increasingly advocated. In the absence of a high-throughput screening test, which can identify all infected newborn infants, the development of an accurate and efficient testing strategy has remained an ongoing challenge.

Novel Peripheral Perfusion Dynamics Indices for Detecting and Grading Arterial Stenosis.

Purpose: Peripheral artery disease causes severe morbidity, especially in diabetics and the elderly. There is a need for accurate noninvasive detection of peripheral arterial stenosis. The study has tested the hypothesis that arterial stenosis and the associated adaptation of the downstream circulation yield characteristic changes in the leg perfusion dynamics that enable early diagnosis, utilizing impedance plethysmography.

Screening for Fabry's disease in a high-risk subpopulation of FMF.

Background: Familial Mediterranean fever (FMF) is an autosomal recessive disease associated with mutations in the Mediterranean fever gene (MEFV) that manifests with recurrent episodes of febrile serositis. Fabry's disease (FD) is an X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A gene and presents with a wide range of gastrointestinal, skin, vascular, renal and neurological manifestations. FMF and FD share similar manifestations, which may lead to misdiagnosis of one as the other; mostly FD is misdiagnosed as FMF.

Determining the origin of different variants associated with familial mediterranean fever by machine-learning.

A growing number of familial Mediterranean fever (FMF) patients in Israel do not have a single country of origin for all four grandparents. We aimed to predict the Mediterranean fever gene (MEFV) variant most likely to be found for an individual FMF patient, by a machine learning approach. This study was conducted at the Sheba Medical Center, a referral center for FMF in Israel.

Risk communication on vaccines during the COVID19 pandemic: is there room for small size or private initiatives? An Israeli experience.

Unlabelled: The scientific communication landscape has undergone a significant disruption since the COVID-19 pandemic: a huge number of publications, ample use of preprint publications, a fast-spreading digitalized information. This reflected the unprecedented speed of the research on disease and vaccines. Two kinds of infodemic blew up very soon: a scientific and a tabloid one.

The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease.

Familial Mediterranean fever (FMF), the most frequent monogenic autoinflammatory disease, is manifested with recurrent and chronic inflammation and amyloid A (AA) amyloidosis, driven by overproduction of interleukin 1 (IL-1) through an activated pyrin inflammasome. Consequently, non-responsiveness to colchicine, the cornerstone of FMF treatment, is nowadays addressed by IL-1- blockers. Each of the two IL-1 blockers currently used in FMF, anakinra and canakinumab, has its own merits for FMF care.

Safety of the BNT162b2 mRNA COVID-19 vaccine in patients with familial Mediterranean fever.

Objectives: Evidence suggests a possible association between the COVID-19 vaccine and autoimmune disease flares or new onset of various autoinflammatory manifestations, such as pericarditis and myocarditis. The objective of this study was to assess the safety of an mRNA-based BNT162b2 anti-COVID-19 vaccine in individuals with FMF, a prototypic autoinflammatory disease.

Methods: Patients participating in this study fulfilled the criteria for diagnosis of FMF, were older than 18 years and received at least one dose of the vaccine.

Autonomic Nervous System Indices in Patients with Systemic Sclerosis without Overt Cardiac Disease.

Background: Systemic sclerosis (SSc) is a connective tissue disease that may affect the heart and the autonomic nervous system (ANS). There is little knowledge regarding the degree of ANS involvement in SSc patients with unknown cardiac disease.

Objectives: To evaluate cardiac and pupillary autonomic functions in patients before cardiac involvement has emerged.

Early Neonatal C-Reactive Protein Levels and Periventricular Leukomalacia.

Background: Cystic periventricular leukomalacia (cPVL) is a strong indicator of subsequent motor and developmental impairments in premature infants. There is a paucity of publications on biomarkers of cPVL.

Objectives: To determine C-reactive protein (CRP) levels during the first week of life of preterm infants who later developed cPVL and to identify the association between CRP levels with perinatal factors.

Effect of interleukin-1 inhibition in a cohort of patients with colchicine-resistant familial Mediterranean fever treated consecutively with anakinra and canakinumab.

Objectives: To evaluate the efficacy of IL-1 blockers in a cohort of patients with colchicine-resistant familial Mediterranean fever (crFMF) treated consecutively with anakinra and canakinumab.

Methods: Patients with crFMF treated with anakinra and canakinumab in any order were identified using the computerised database of Sheba Medical Centre. Background characteristics of the patients, reason for switching IL-1 inhibitor, and frequency of attacks under colchicine only, anakinra, and canakinumab were extracted from the computerised patient files.

Anakinra for colchicine refractory familial Mediterranean fever: a cohort of 44 patients.

Objective: FMF is an autoinflammatory disease of genetic origin. Colchicine is the mainstay of treatment for the prevention of attacks and long-term complications but 5-10% of FMF patients are resistant to colchicine therapy. The aim of our study was to investigate the real-life safety and efficacy of anakinra in a cohort of patients with colchicine-resistant FMF.

Familial Mediterranean fever (FMF)-response to TNF-blockers used for treatment of FMF patients with concurrent inflammatory diseases.

Objective: Familial Mediterranean fever (FMF) is the most common interleukin 1 (IL-1)-driven monogenic autoinflammatory disease. Yet published data also suggest that tumor necrosis factor (TNF) may have a role in the pathogenesis of FMF and may serve as a target for treatment. In the present study we evaluate this hypothesis.

Lessons from applied large-scale pooling of 133,816 SARS-CoV-2 RT-PCR tests.

Pooling multiple swab samples before RNA extraction and real-time reverse transcription polymerase chain reaction (RT-PCR) analysis has been proposed as a strategy to reduce costs and increase throughput of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) tests. However, reports on practical large-scale group testing for SARS-CoV-2 have been scant. Key open questions concern reduced sensitivity due to sample dilution, the rate of false positives, the actual efficiency (number of tests saved by pooling), and the impact of infection rate in the population on assay performance.

Repolarization dispersion in patients with systemic sclerosis.

Objective: Systemic sclerosis (SSc) is associated with increased cardiac morbidity and mortality. Whether some electrocardiographic markers of arrhythmias predispose to early cardiogenic death in SSc remains controversial. This study evaluated the occurrence of previously reported as well as unstudied markers of repolarization in patients with SSc and assessed their prognostic implications.

Amyloid storm: acute kidney injury and massive proteinuria, rapidly progressing to end-stage kidney disease in AA amyloidosis of familial Mediterranean fever.

Objective: Amyloid A nephropathy of FMF usually progresses over many years to end-stage renal disease (ESRD). We aim to describe an acute condition, termed here 'amyloid storm', typically manifesting with a rapid (≤2 weeks) increase in serum creatinine and urine protein, that has never been characterized in FMF amyloidosis.

Methods: This retrospective analysis features amyloid storm by comparing between FMF amyloidosis patients who have experienced an episode of amyloid storm (study group) and matched patients who have not (control group).

Burden of illness in hereditary periodic fevers: a multinational observational patient diary study.

Objectives: This study aimed to characterise the burden of illness of patients with inadequately controlled hereditary periodic fevers (HPFs), during and outside of flares. It was focused on the burden to the patients and also considered the wider impact on their caregivers and families.

Methods: The target population was patients or caregivers of patients with clinically/genetically confirmed colchicine resistant FMF (crFMF), mevalonate kinase deficiency/hyperimmunoglobinaemia D with periodic fever syndrome (MKD/HIDS) or TRAPS, who were expected to flare at least once in a 6-month period based on patient history.

Evaluation of a Proarrhythmic Repolarization Marker (Total Cosine R to T) in Patients With Uncomplicated Familial Mediterranean Fever.

Unlabelled: Familial Mediterranean fever (FMF) is a systemic autoinflammatory disorder characterized by recurrent attacks of fever and serous inflammation. The association between FMF and risk of cardiac arrhythmia is continuously questioned; some studies report abnormal cardiac repolarization, while others do not. Considering this controversy, we aim to perform in a large cohort of FMF patients a total cosine R to T (TCRT) analysis, a previously unexplored repolarization marker in this disorder.

Saliva immunoglobulin free light chain analysis for monitoring disease activity and response to treatment in multiple sclerosis.

Background: Immunoglobulin free light chains (FLC) have recently gained considerable interest as new promising intrathecal biomarkers of multiple sclerosis (MS). However, lumbar puncture is invasive and not practical for monitoring disease course. This study aimed to assess the utility of saliva FLC as a biomarker of disease activity and response to treatment in MS METHODS: Western blotting was used to study saliva FLC monomers and dimers.

Associations between intrauterine exposure to polychlorinated biphenyls on neonatal ano-genital distance.

Polychlorinated Biphenyls (PCBs) are widespread environmental contaminants. PCBs have endocrine disrupting properties which raises concerns regarding their effect on the developing fetus. This study aimed to examine the association between prenatal exposure to PCBs and anogenital distance (AGD) in newborns.

The Epidemiology and Susceptibility of Candidemia in Jerusalem, Israel.

Invasive infections pose a major public health problem worldwide and is a major cause of nosocomial bloodstream infection. Our aim was to assess dynamics in incidence, species distribution and antifungal susceptibility of candidemia episodes in Jerusalem, to better understand the epidemiology of invasive isolates and to better direct therapy. We analyzed the incidence dynamics, species distribution and susceptibility pattern of 899 candidemia episodes during 2005-2016 in Jerusalem.

Gynecology and Oncology Fetal Myofibromatosis: A Challenge for Prenatal Diagnosis Mini Review of the English Literature.

Importance: Infantile myofibromatosis (IM) is a benign neoplasm with a reported incidence of 1:150,000. The "solitary" type is characterized by a single lesion in the skin, muscle, or bone, whereas the "multicentric" type may also involve the viscera.

Objective: This report describes the prenatal diagnosis of IM and recommendations for future pregnancy follow-up.

QT variability index in patients with systemic sclerosis.

Objective: Systemic sclerosis (SSc) may affect the heart with microvascular dysfunction and lead to an early cardiac death, but the association between certain repolarization indexes and SSc heart disease remains controversial. Our goal was to evaluate a previously unstudied marker of repolarization dynamics, i.e.

The Role of an Interventional Program for Improving Pharmacovigilance at a Pediatric Facility.

The reporting rate of adverse drug reactions (ADRs) by healthcare professionals is low. ADR interventional programs may improve the reporting rate by the medical team. Our literature search revealed that only a few interventional studies among the pediatric population have been published.