Methodology and clinical utility of longitudinal UBA1 tracking in VEXAS syndrome.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) is a haemato-inflammatory syndrome genetically defined by somatic mutations in the X-linked UBA1 gene, typically Val/Thr/Leu substitutions at the Met41 hotspot. Clinical manifestations are heterogeneous and refractory to most haemato-rheumatological treatments. To date, no guidelines exist for the management of VEXAS, and scarce is the evidence on methodology and clinical significance of longitudinal UBA1 clonal burden evaluation upon therapy.

Multidisciplinary and personalized approach to the management of mycosis fungoides with chlormethine gel: a collection of clinical experiences.

Topical chlormethine (CL) gel formulation was approved by the EMA in 2017 for the treatment of adult patients with mycosis fungoides (MF). To expand the knowledge on the management of MF, this paper provides an overview of clinical practice evidence about the MF diagnostic phase and a collection of clinical experiences to better characterize the use of CL gel in daily practice. Collected cases underline the importance of the concomitant biopsy and clinical evaluation in the diagnostic phase, with the contribution of a multidisciplinary team, and support the use of CL gel as a first-line or adjuvant treatment in selected patients.

Ponatinib as a Prophylactic or Pre-Emptive Strategy to Prevent Cytological Relapse after Allogeneic Stem Cell Transplantation in Patients with Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia Transplanted in Complete Cytological Remission.

The administration of TKIs after Allo-SCT in Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph + ALL) remains controversial, and the TKI approach (prophylactic, pre-emptive or salvage) is still heterogeneous in transplant centers. In this context, very little is known about the feasibility and safety of third-generation TKIs. In this paper, we analyze the efficacy and safety of ponatinib (PONA) administered after Allo-SCT to prevent cytologic relapse of Ph + ALL.

Presurgical Virtual Planning and Intraoperative Navigation with 3D-Preformed Mesh: A New Protocol for Primary Orbital Fracture Reconstruction.

Purpose: This pilot study aims to evaluate the feasibility and effectiveness of computer-assisted surgery protocol with 3D-preformed orbital titanium mesh (3D-POTM), using presurgical virtual planning and intraoperative navigation in primary inferomedial orbital fracture reconstruction.

Methods: Between March 2021 and March 2023, perioperative data of patients undergoing surgery for unilateral inferomedial orbital fracture treated with 3D-POTM were analyzed. Presurgical virtual planning with a Standard Triangle Language file of preformed mesh was conducted using the mirrored unaffected contralateral side as a reference, and intraoperative navigation was used.

Orbital Fractures: A New CT-Based Protocol to Guide the Surgical Approach and Reconstruction Material Decision-Making.

Preoperative computer-assisted planning and intraoperative navigation are becoming popular for orbital fracture treatment. However, not all institutions currently have access to these computer-aided applications. The authors present a simple and intuitive operative algorithm to guide orbital fracture reconstructions.

Eltrombopag for Low-Risk Myelodysplastic Syndromes With Thrombocytopenia: Interim Results of a Phase II, Randomized, Placebo-Controlled Clinical Trial (EQOL-MDS).

Purpose: In myelodysplastic syndromes (MDS), severe thrombocytopenia is associated with poor prognosis. This multicenter trial presents the second-part long-term efficacy and safety results of eltrombopag in patients with low-risk MDS and severe thrombocytopenia.

Methods: In this single-blind, randomized, placebo-controlled, phase-II trial of adult patients with International Prognostic Scoring System low- or intermediate-1-risk MDS, patients with a stable platelet (PLT) count (<30 × 10/mm) received eltrombopag or placebo until disease progression.

Nonsurgical Rhinoplasty: Diamond Injection Technique for Nasal Tip Contouring.

A more pleasing nasal tip appearance is becoming the most frequent reason for nonsurgical aesthetic rhinoplasty procedures. To present the "Diamond Injection Technique," an innovative 4-point hyaluronic acid (HA) filler injection procedure for nasal tip refinement and to assess its aesthetic outcomes and efficacy. Data were collected from a prospective single-center cohort of patients undergoing nonsurgical rhinoplasty with injectable fillers.

Multicentric Observational Study on Safety and Tolerability of COVID-19 Vaccines in Patients with Angioedema with C1 Inhibitor Deficiency: Data from Italian Network on Hereditary and Acquired Angioedema (ITACA).

Angioedema due to C1 inhibitor deficiency (AE-C1-INH) is a rare disease characterized by recurrent and unpredictable attacks of angioedema. Multiple trigger factors, including trauma, emotional stress, infectious diseases, and drugs, could elicit angioedema attacks. The aim of this study was to collect data on the safety and tolerability of COVID-19 vaccines in a population of patients affected by AE-C1-INH.

Understanding the barriers and facilitators of COVID-19 vaccine hesitancy amongst healthcare workers and healthcare students worldwide: An umbrella review protocol.

Healthcare workers (HCWs) and healthcare students are at increased risk of becoming infected with and being a vector of transmission of COVID-19. Vaccination efforts amongst this group of persons have been hampered in some countries by hesitancy to uptake the COVID-19 vaccine. The factors related to vaccine hesitancy have been reported in several systematic reviews.

Pregnancy in women with Hereditary Angioedema due to C1-inhibitor deficiency: Results from the ITACA cohort study on outcome of mothers and children with exposure to plasma-derived C1-inhibitor.

Background: In women with Hereditary Angioedema (HAE) due to C1-inhibitor (C1INH) deficiency (C1INH-HAE), pregnancy counseling and treatment can be challenging. Despite the evidence of the immediate favorable outcome and safety of plasma-derived (pd)C1INH concentrate, there are no data regarding any difference among women who underwent or not pdC1INH during pregnancy or on children with exposure to pdC1INH. The present interview study aimed at analyzing outcome of C1INH-HAE mothers and children according to pdC1INH-exposure during pregnancies.

Pre-registration nursing students' anxiety and academic concerns after the second wave of COVID-19 pandemic in Italy: A cross-sectional study.

Background: The pandemic and its related social restrictions have led to many uncertainties in nurse education, including the fear of infection in clinical learning settings and the challenge of remote learning. The modification of clinical and academic environments generated anxiety and academic concerns among nursing students.

Objectives: To explore the main determinants of anxiety related to the clinical and classroom environments in nurse education after the second wave of the COVID-19 Pandemic.

Overcoming Resistance: FLT3 Inhibitors Past, Present, Future and the Challenge of Cure.

FLT3 ITD and TKD mutations occur in 20% and 10% of Acute Myeloid Leukemia (AML), respectively, and they represent the target of the first approved anti-leukemic therapies in the 2000s. Type I and type II FLT3 inhibitors (FLT3i) are active against FLT3 TKD/ITD and FLT3 ITD mutations alone respectively, but they still fail remissions in 30-40% of patients due to primary and secondary mechanisms of resistance, with variable relapse rate of 30-50%, influenced by NPM status and FLT3 allelic ratio. Mechanisms of resistance to FLT3i have recently been analyzed through NGS and single cell assays that have identified and elucidated the polyclonal nature of relapse in clinical and preclinical studies, summarized here.

Time With Glucose Level in Target Range Among Children and Adolescents With Type 1 Diabetes After a Software Update to a Closed-Loop Glucose Control System.

This cohort study analyzes changes to the time with glucose level in target range among children and adolescents with type 1 diabetes after a software update to a closed-loop glucose control system.

Buccal Fat Pad Flap in Orthognathic Surgery: Facial Soft Tissue Volume's Redistribution Strategy to Improve the Esthetic Outcomes.

Traditional osteotomic procedures can fail to restore the natural appearance of the face and can sometimes highlight the intrinsic decrease in the volume of soft tissues; in these cases, soft-tissue-improving procedures should be considered. The authors describe a new strategy that can help redistribute facial soft tissues and enhance facial esthetics in orthognathic surgery using a pedicled buccal fat flap to increase the volume of the malar soft tissue envelope. Between January 2017 and January 2021, 47 patients with dentoskeletal deformity who needed to increase the projection of the cheekbone contour underwent orthognathic surgery with simultaneous pedicled buccal fat pad flap for malar augmentation.

A large cross-sectional survey of COVID-19 vaccination willingness amongst healthcare students and professionals: Reveals generational patterns.

Aims: To determine coronavirus disease 2019 (COVID-19) vaccination hesitancy in healthcare professionals and healthcare students in Italy across four generations (baby boomers, generations X, Y and Z).

Design: A cross-sectional descriptive study was performed through an online survey conducted from May to June 2021. The STROBE guidelines were adopted for reporting.

New Tunneled Buccal Fat Pad Flap for Palatal Reconstruction.

In the palatal defects due to surgical resection, flap selection is very important for a correct reconstruction. Different methods have been suggested over the time, however the pedicled buccal fat pad is a simple, effective, reliable flap for reconstruction after palate tumor resection. The aim of the present study is to introduce a new surgical technique for palate reconstruction with pedicled buccal fat pad flap exposing the advantages.

Customized and Navigated Primary Orbital Fracture Reconstruction: Computerized Operation Neuronavigated Surgery Orbital Recent Trauma (CONSORT) Protocol.

Combined orbital medial wall and floor fractures and large isolated orbital floor fractures commonly require surgical treatment due to the high probability of diplopia and enophthal-mos. Primary reconstruction of these orbital fractures requires a high-level surgeon with a great amount of technical surgical skill. The use of novel technology can greatly improve the accuracy of reconstruction and achieve satisfactory clinical outcomes.

Overview of the signaling pathways involved in metastasis: An intriguing story-tale of the metastatic journey of ovarian cancer cells.

Wealth of information has revolutionized our understanding related to the genetics and functional genomics of this heterogeneous disease. Keeping in view the heterogeneity of ovarian cancer, long-term survival might be achieved by translation of recently emerging mechanistic insights at the cellular and molecular levels to personalize individual strategies for treatment and to identify biomarkers for early detection. Importantly, the motility and invasive properties of ovarian cancer cells are driven by a repertoire of signaling cascades, many components of which have been experimentally verified as therapeutic targets in preclinical models as well as in clinical trials.

Effectiveness of virtual reality in the management of paediatric anxiety during the peri‑operative period: A systematic review and meta-analysis.

Background: Children undergoing surgery generally experience anxiety during the perioperative period, which could impact the surgical outcome, cause long-term psychological consequences and result in later healthcare avoidance. Preoperative anxiety in children is managed using both pharmacological and non-pharmacological therapies. The latter include distraction, a tour of the operating room and parental presence until the induction of anaesthesia.

Retrospective Comparative Analysis of KRAS G12C . Other KRAS Mutations in mCRC Patients Treated With First-Line Chemotherapy Doublet + Bevacizumab.

Background: KRAS mutations in metastatic colorectal cancer (mCRC) define a subset of tumors that have primary resistance to anti-EGFR-based therapy. Data concerning whether different KRAS mutations may also have a prognostic value are lacking. Furthermore, novel KRAS G12C inhibitors are currently in development.

Effectiveness of a family nurse-led programme on accuracy of blood pressure self-measurement: A randomised controlled trial.

Aims And Objectives: To evaluate the effectiveness of a Family Nurse Practitioner (FNP)-led programme on the degree of adherence of current recommendations on home blood pressure self-measurement (HBPM) as compared to routine care and management.

Background: HBPM plays an important role for monitoring hypertensive patients; however, patients' adherence to current guidelines is unsatisfactory. A nurse-led training programme in the community setting could be an effective strategy to achieve high level of patients' adherence to recommendations.