Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients.

Objective: To evaluate the fulfilment of classification criteria for cryoglobulinaemic vasculitis (CV) at diagnosis in a large cohort of patients with primary SS and their correlation with poor outcomes.

Methods: We included 515 consecutive patients tested for serum cryoglobulins who fulfilled the 2002 classification criteria for primary SS. CV classification criteria and serum cryoglobulins at diagnosis were assessed as predictors of death and lymphoma using Cox proportional-hazards regression analysis adjusted for age and gender.

Cardio-thoracic surgical patients' experience on bedside nursing handovers: Findings from a qualitative study.

The purpose of this study was to describe the experiences of postoperative cardio-thoracic surgical patients experiencing nursing bedside handover. A descriptive qualitative approach was undertaken. A purposeful sampling technique was adopted, including 14 patients who went through cardio-thoracic surgery and witnessed at least two bedside handovers.

Patient-reported impact of spondyloarthritis on work disability and working life: the ATLANTIS survey.

Background: The aim was to establish how patients experience the impact of spondyloarthritis (SpA) on work disability and working life.

Methods: The survey was performed in 17/20 regions in Italy (1 January to 31 March 2013). A multiple-choice questionnaire was published on the official website of the sponsor - the National Association of Rheumatic Patients (ANMAR) - and hard-copies were distributed at outpatient clinics for rheumatic patients.

[The Pacemaker and Implantable Cardioverter-Defibrillator Registry of the Italian Association of Arrhythmology and Cardiac Pacing--Annual report 2014].

Background: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) Registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2014 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers.

Methods: The Registry collects prospectively national PM and ICD implantation activity on the basis of European cards.

Efficacy of belimumab and targeting of rheumatoid factor-positive B-cell expansion in Sjögren's syndrome: follow-up after the end of the phase II open-label BELISS study.

Objectives: Belimumab, a monoclonal anti-B lymphocyte Stimulator (BLyS) antibody, appeared effective in Sjögren's syndrome (SS) in the phase II open-label 52-week BELISS study. Herein, the follow-up after the end of the BELISS study and suspension of the drug was reported in order to further verify the efficacy of belimumab in SS.

Methods: 13 SS patients were followed after the end of the belimumab treatment.

Phenotyping asthma in the elderly: allergic sensitization profile and upper airways comorbidity in patients older than 65 years.

Background: Data about allergic rhinitis in elderly patients with asthma are lacking.

Objective: To investigate the presence of rhinitis and the role of sensitization of airborne allergens in elderly patients with asthma.

Methods: This was a multicenter cross-sectional study involving subjects at least 65 years old with asthma.

Specific chemoluminescence and immunoasdorption tests for anti-DFS70 antibodies avoid false positive results by indirect immunofluorescence.

Objective: To evaluate two new diagnostic methods for the identification of anti-DFS70 antibodies in samples showing a DFS70-staining pattern by indirect immunofluorescence (IIF).

Methods: We studied 731 patients: 576 were collected consecutively among those that in the ANA test on HEp-2 cells had produced a DFS70 fluorescence pattern and 155 were a consecutive series of patients sent by referring physicians for routine ANA testing. As controls we studied 50 patients with autoimmune diseases and 120 patients with active infectious disease.

A 16q deletion involving FOXF1 enhancer is associated to pulmonary capillary hemangiomatosis.

Background: Pulmonary capillary hemangiomatosis (PCH) is an uncommon pulmonary disorder, with variable clinical features depending on which lung structure is affected, and it is usually linked to pulmonary arterial hypertension. Congenital PCH has been very rarely described and, so far, the only causative gene identified is EIF2AK4, which encodes for a translation initiation factor. However, not all PCH cases might carry a mutation in this gene.

The Frequencies of Different Inborn Errors of Metabolism in Adult Metabolic Centres: Report from the SSIEM Adult Metabolic Physicians Group.

Background: There are few centres which specialise in the care of adults with inborn errors of metabolism (IEM). To anticipate facilities and staffing needed at these centres, it is of interest to know the distribution of the different disorders.

Methods: A survey was distributed through the list-serve of the SSIEM Adult Metabolic Physicians group asking clinicians for number of patients with confirmed diagnoses, types of diagnoses and age at diagnosis.

Physical assessment techniques performed by Italian registered nurses: a quantitative survey.

Aims And Objectives: The aims of the study were to describe which of the core techniques of the physical assessment are regularly performed by a sample of Italian nurses, and to investigate the potential predictors of a more complete examination.

Background: Physical examination is among the essential tasks of nursing professionals, who are requested to perform a correct and complete physical assessment.

Design: Cross-sectional survey.

Prognostic clinical factors in pretreated colorectal cancer patients receiving regorafenib: implications for clinical management.

Background: We assessed the impact on survival of angiogenesis and inflammation-related factors, particularly LDH serum levels, platelet, neutrophil and lymphocyte counts, and neutrophil-to-lymphocyte ratio (NLR), in metastatic colorectal cancer patients receiving regorafenib monotherapy.

Methods: LDH serum levels, neutrophil, lymphocyte and platelet counts were collected at the start of regorafenib monotherapy. Cut-off values were calculated by ROC curve analysis.

Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution.

Objective: This study aims to compare clinical and laboratory features of patients who, while satisfying the American European Consensus Group (AECG) criteria for primary Sjögren's syndrome (SS), do not present the positivity for anti-Ro/SSA and/or anti-La/SSB, with patients that meet the AECG criteria and are positive for anti-Ro/SSA and/or anti-La/SSB.

Methods: 548 patients were selected based on the following criteria, and exclusion of patients negative for histopathology but positive for anti-Ro/SSA and anti-La/SSB: 1. Fulfilment of the AECG criteria, 2.

International phase 3 study of azacitidine vs conventional care regimens in older patients with newly diagnosed AML with >30% blasts.

This multicenter, randomized, open-label, phase 3 trial evaluated azacitidine efficacy and safety vs conventional care regimens (CCRs) in 488 patients age ≥65 years with newly diagnosed acute myeloid leukemia (AML) with >30% bone marrow blasts. Before randomization, a CCR (standard induction chemotherapy, low-dose ara-c, or supportive care only) was preselected for each patient. Patients then were assigned 1:1 to azacitidine (n = 241) or CCR (n = 247).

Somatic amplifications and deletions in genome of papillary thyroid carcinomas.

Somatic gene copy number variation contributes to tumor progression. Using comparative genomic hybridization (CGH) array, the presence of genomic imbalances was evaluated in a series of 27 papillary thyroid carcinomas (PTCs). To detect only somatic imbalances, for each sample, the reference DNA was from normal thyroid tissue of the same patient.

Buried in the Middle but Guilty: Intronic Mutations in the TCIRG1 Gene Cause Human Autosomal Recessive Osteopetrosis.

Autosomal recessive osteopetrosis (ARO) is a rare genetic bone disease with genotypic and phenotypic heterogeneity, sometimes translating into delayed diagnosis and treatment. In particular, cases of intermediate severity often constitute a diagnostic challenge and represent good candidates for exome sequencing. Here, we describe the tortuous path to identification of the molecular defect in two siblings, in which osteopetrosis diagnosed in early childhood followed a milder course, allowing them to reach the adult age in relatively good conditions with no specific therapy.

Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus.

Objective: The aim of this study was to develop and validate an algorithm to assist the attribution of neuropsychiatric (NP) events to underlying disease in SLE patients.

Methods: Phase 1 identified and categorized candidate items to be included in the algorithm for the attribution of an NP event to SLE and their relative weights through a literature-informed consensus-driven process. Using a retrospective training cohort of SLE, phase 2 validated items selected in phase 1 and refined weights through a data-driven process, fitting items as independent variables and expert evaluation (clinical judgement) as reference standard in logistic models.

Electroencephalographic Evaluation for Early Diagnosis of Limbic Encephalitis.

Limbic encephalitis (LE) is an inflammation of structures of limbic system. It may be an autoimmune disease or secondary to a neoplasia. Onset is subacute within a few weeks and clinical presentation is characterized by behavioral changes, psychiatric symptoms, short-term memory loss, and epileptic seizures.

Validation of the classification criteria for cryoglobulinaemic vasculitis.

Objective: The aim of this study was to validate the classification criteria for cryoglobulinaemic vasculitis (CV).

Methods: Twenty-three centres were involved. New patients with CV (group A) and controls, i.

Efficacy of long detection interval implantable cardioverter-defibrillator settings in secondary prevention population: data from the Avoid Delivering Therapies for Nonsustained Arrhythmias in ICD Patients III (ADVANCE III) trial.

Background: Three trials demonstrated recently that a long detection window reduces implantable cardioverter-defibrillator (ICD) therapy in primary prevention patients. Avoid Delivering Therapies for Nonsustained Arrhythmias in ICD Patients III (ADVANCE III) was the only trial that enrolled both primary and secondary prevention patients.

Methods And Results: Of the 1902 patients enrolled in the ADVANCE III trial, 477 received a defibrillator for secondary prevention; 248 patients were randomly assigned to a long detection setting (30 of 40 intervals) and 229 to the nominal setting (18 of 24 intervals) for ventricular arrhythmias with cycle length ≤ 320 ms.