Expert consensus on the treatment of patients with adult-onset still's disease with the goal of achieving an early and long-term remission.

We performed a comprehensive systematic targeted literature review and used the Delphi method to formulate expert consensus statements to guide the treatment of adult-onset Still's disease (AOSD) to achieve an early and long-term remission. Seven candidate statements were generated and reached consensus in the first round of voting by the panel of experts. We postulate: (i) In patients with AOSD with predominant arthritis at onset who achieved no disease control with glucocorticoids (GCs), the use of methotrexate can be considered, whereas the use of cyclosporin A and low-dose GCs should not (Statements 1-3); (ii) In patients with AOSD with poor prognostic factors at diagnosis, an IL-1 inhibitor (IL-1i) in addition to GCs should be taken into consideration as early as possible (Statement 4); (iii) A switch to an IL-6 inhibitor (IL-6i) may be considered in patients with AOSD with prevalent joint involvement, who are unresponsive or intolerant to IL-1i (Statement 5); (iv) Drug tapering or discontinuation may be considered in patients who achieved a sustained clinical and laboratory remission with IL-1i (Statement 6); (v) In patients with AOSD who failed to attain a good clinical response with an IL-1i, switching to an IL-6i may be considered in alternative to a different IL-1i.

Echocardiography findings in COVID-19 patients admitted to intensive care units: a multi-national observational study (the ECHO-COVID study).

Purpose: Severely ill patients affected by coronavirus disease 2019 (COVID-19) develop circulatory failure. We aimed to report patterns of left and right ventricular dysfunction in the first echocardiography following admission to intensive care unit (ICU).

Methods: Retrospective, descriptive study that collected echocardiographic and clinical information from severely ill COVID-19 patients admitted to 14 ICUs in 8 countries.

The effects of exposure to severe hyperoxemia on neurological outcome and mortality after cardiac arrest.

Introduction: Hyperoxemia during cardiac arrest (CA) may increase chances of successful resuscitation. However, episodes of severe hyperoxemia after intensive care unit admission occurs frequently (up to 60%), and these have been associated with higher mortality in CA patients. The impact of severe hyperoxemia on neurological outcome is more unclear.

Preventive strategies, exercises and rehabilitation of hand neuropathy in cyclists: A systematic review.

Background: Recreation, transportation and sport are the most common uses of bicycles. Unfortunately, repetitive bicycle use can also cause injuries, such as osteoarticular direct and undirect traumatisms and sometimes compression nerve entrapment caused by an extrinsic compressive force.

Purpose: The aim of the study is to define diagnostic process, preventive strategies, and treatment of ulnar and median neuropathies in cyclists.

Tailored first-line biologic and targeted synthetic disease modifying anti-rheumatic drugs therapy in patients with rheumatoid arthritis: 2021 updated ITABIO statements.

Introduction: In 2015, the Italian board for the TAilored BIOlogic therapy (ITABIO) proposed evidence-based decisional statements for first-line tailored biologic therapy in patients with rheumatoid arthritis (RA). Taking into account the new licensed drugs, the aim of the present review was to update the previous statements.

Areas Covered: A narrative review of the most recent evidence on the efficacy and safety of old and newly licensed drugs for the treatment of articular and extra-articular RA was performed.

The role of exercise on peripheral nerve regeneration: from animal model to clinical application.

Peripheral nerve injury is a complex condition with a variety of signs and symptoms depending on the severity and nerves involved. Peripheral nerve damage may lead to sensory and motor functions deficits and even lifelong disability, causing important socioeconomic costs worldwide. Despite the increase in knowledge of the mechanisms of injury and regeneration, a full functional recovery is still unsatisfying in the majority of patients.

F9 missense mutations impairing factor IX activation are associated with pleiotropic plasma phenotypes.

Background: Circulating dysfunctional factor IX (FIX) might modulate distribution of infused FIX in hemophilia B (HB) patients. Recurrent substitutions at FIX activation sites (R191-R226, >300 patients) are associated with variable FIX activity and antigen (FIXag) levels.

Objectives: To investigate the (1) expression of a complete panel of missense mutations at FIX activation sites and (2) contribution of F9 genotypes on the FIX pharmacokinetics (PK).

Fabry-Stroke Italian Registry (FSIR): a nationwide, prospective, observational study about incidence and characteristics of Fabry-related stroke in young-adults. Presentation of the study protocol.

Background: TIA and stroke, both ischemic and hemorrhagic, may complicate Fabry disease at young-adult age and be the first manifestation that comes to the clinician's attention. No definite indications have yet been elaborated to guide neurologists in Fabry disease diagnostics. In current practice, it is usually sought in case of cryptogenic strokes (while Fabry-related strokes can also occur by classical pathogenic mechanisms) or through screening programs in young cerebrovascular populations.

Effects of Relaxed Lockdown on Pediatric ER Visits during SARS-CoV-2 Pandemic in Italy.

Previously, we demonstrated an 81% reduction in pediatric Emergency Room (ER) visits in Italy during the strict lockdown due to the SARS-CoV-2 pandemic. Since May 2020, lockdown measures were relaxed until 6 November 2020, when a strict lockdown was patchily reintroduced. Our aim was to evaluate the impact of the relaxed lockdown on pediatric ER visits in Italy.

Targeted Temperature Management after Cardiac Arrest: A Systematic Review and Meta-Analysis with Trial Sequential Analysis.

Target temperature management (TTM) in cardiac arrest (CA) survivors is recommended after hospital admission for its possible beneficial effects on survival and neurological outcome. Whether a lower target temperature (i.e.

Gastric cancer, inflammatory bowel disease and polyautoimmunity in a 17-year-old boy: CTLA-4 deficiency successfully treated with Abatacept.

Gut involvement is frequent in immunologic disorders, especially with inflammatory manifestations but also with cancer. In the last years, advances in functional and genetic testing have improved the diagnostic and therapeutic approach to immune dysregulation syndromes. CTLA-4 deficiency is a rare disease with variable phenotype, ranging from absence of symptoms to severe multisystem manifestations and complications.

Self-citation policies and journal self-citation rate among Critical Care Medicine journals.

Background: Inappropriate authors' self-citation (A-SC) is a growing mal-practice possibly boosted by the raising importance given to author's metrics. Similarly, also excessive journals' self-citation (J-SC) practice may factitiously influence journal's metrics (impact factor, IF). Evaluating the appropriateness of each self-citation remains challenging.

Dermatome Mapping Test in the analysis of anatomo-clinical correlations after inguinal hernia repair.

Background: Nerve identification is recommended in inguinal hernia repair to reduce or avoid postoperative pain. The aim of this prospective observational study was to identify nerve prevalence and find a correlation between neuroanatomy and chronic neuropathic postoperative inguinal pain (CPIP) after 6 months.

Material: A total of 115 patients, who underwent inguinal hernia mesh repair (Lichtenstein tension-free mesh repair) between July 2018 and January 2019, were included in this prospective observational study.

The PRICES statement: an ESICM expert consensus on methodology for conducting and reporting critical care echocardiography research studies.

Purpose: Echocardiography is a common tool for cardiac and hemodynamic assessments in critical care research. However, interpretation (and applications) of results and between-study comparisons are often difficult due to the lack of certain important details in the studies. PRICES (Preferred Reporting Items for Critical care Echocardiography Studies) is a project endorsed by the European Society of Intensive Care Medicine and conducted by the Echocardiography Working Group, aiming at producing recommendations for standardized reporting of critical care echocardiography (CCE) research studies.

A Systematic Review of Pharmacologic and Rehabilitative Treatment of Small Fiber Neuropathies.

The aim of this systematic review is to guide the physician in defining the pharmacologic and rehabilitative therapeutic approaches for adopting the best strategies described in the current literature. The search was conducted in PubMed, EMBASE, Cochrane Library and Web of Science to identify the treatment of small fiber neuropathies. Two reviewers independently reviewed and came to a consensus on which articles met inclusion/exclusion criteria.

Ketogenic diet for infants with epilepsy: A literature review.

The ketogenic diet (KD) is an established, nonpharmacological treatment for drug-resistant epilepsy (DRE). Actually, KD and its variants have been shown to be elective and resolute for patients with glucose transporter type 1 (GLUT1) deficiency. The aim of this review was to study the use of KD and its variants in infancy, including the neonatal age, and demonstrate the safety and efficacy of this treatment in patients with the age of 0-23 months affected by DRE already subjected to pharmacological approach attempts.

Immune Therapy, or Antiviral Therapy, or Both for COVID-19: A Systematic Review.

Background: Based on current evidence, recent guidelines of the National Institute of Health, USA indicated the use of remdesivir and dexamethasone for the treatment of COVID-19 patients with mild-moderate disease, not requiring high-flow oxygen. No therapeutic agent directed against the immunologic pathogenic mechanisms related to the cytokine release syndrome complicating the disease was indicated.

Objectives: The purpose of this review was to assess the clinical impact of different therapies for COVID-19; thus, helping to identify the optimal management of the disease.

Psoriasis and its management in women of childbearing age: tools to increase awareness in dermatologists and patients.

Psoriasis is a common, chronic inflammatory disease with a multifactorial pathogenesis. Mean age at presentation of psoriasis is 28 years in women, which is almost the height of fertility age. Since women of childbearing potential represent a significant proportion of psoriatic patients, the impact of psoriasis and its treatment on fertility, pregnancy, and breastfeeding should be highlighted for a proper management.