On performance of Topical Opinion Retrieval

We investigate the effectiveness of both the standard evalua- tion measures and the opinion component for topical opinion retrieval. We analyze how relevance is affected by opinions by perturbing relevance ranking by the outcomes of opinion- only classifiers built by Monte Carlo sampling. Topical opin- ion rankings are obtained by either re-ranking or filtering the documents of a first-pass retrieval of topic relevance. The proposed approach establishes the correlation between the accuracy and the precision of the classifier and the perfor- mance of the topical opinion retrieval. Among other results, it is possible to assess the effectiveness of the opinion compo- nent by comparing the effectiveness of the relevance baseline with the topical opinion ranking

Cold-induced urticaria in children

The exposure to cold can induce the development of wheals and angioedema in a group of susceptible individuals: this phenomenon is largely known as cold-induced urticaria. The pathogenesis of cold-induced urticaria is not yet understood, although both autoallergens and immunoglobulin (Ig)E-mediated autoimmunity are presumed to be involved. Flares of cold-induced urticaria might depend on the release of histamine and other pro-inflammatory mediators, such as Interleukin (IL)-1, which is the predominating stakeholder of cryopyrin-associated periodic syndrome, a genetic disease characterized by cold-induced skin manifestations, including urticaria-like rashes. The majority of occurrence of cold-induced urticaria in children is idiopathic, but forms secondary to systemic conditions have been also reported. Primarily, the diagnosis remains a clinical process based on the history of patient, cold stimulation tests, and a few laboratory results, which could be useful for excluding any underlying disorders. The general rules to manage cold-induced urticaria in children can be summarized with cold avoidance, treatment with nonsedating antihistamines, and the anti-IgE monoclonal antibody omalizumab in selected patients. Familiar forms of cryopyrin-associated periodic syndrome could be prevented even in pediatric patients from the selective IL-1 blockade. Injectable epinephrine must be immediately used to manage the potential life-threatening manifestations occurring in a minority of children with cold-induced urticaria

Safety of systemic treatments for Beh\ue7et\u2019s syndrome

Introduction: Treatment of Beh\ue7et\u2019s syndrome (BS) is aimed at controlling all symptoms of such a complex disorder, ensuring a good quality of life and preventing life-threatening complications. A better understanding of the pathogenic role of different chemokines has improved our knowledge of BS and elicited a more specific use of therapies currently available, minimizing the burden of potential side-effects related to treatment. Areas covered: This work aims to provide a detailed overview of the safety profile for current therapies available in the treatment of BS, focusing on the main side-effects, toxicity and contraindications. Expert opinion: The greatest experience in the management of BS has been achieved with the employment of monoclonal anti-tumor necrosis factor antibodies which have been advocated for BS refractory manifestations. Moreover, interleukin-1 inhibitors have proven to be effective as well as safe, despite escalation of their dosage, especially to manage the most severe and difficult-to-treat ocular manifestations. However, general treatment of BS patients remains awkward as protean clinical features may respond differently to the same treatment or even worsen. Therefore, patients\u2019 safety for therapies used in BS promotes the implementation of precision medicine, which could help targeting accurately the pathogenetic mechanisms concealed behind specific clinical phenotype

Assessment of congenital neutropenia in children: common clinical sceneries and clues for management

A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is "cyclic neutropenia," an ultra-rare disorder revealed by sinusoidal variations in the neutrophil count and recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. The aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of patients

Hints for genetic and clinical differentiation of adult-onset monogenic autoinflammatory diseases

Monogenic autoinflammatory diseases (mAIDs) are inherited errors of innate immunity characterized by systemic inflammation recurring with variable frequency and involving the skin, serosal membranes, synovial membranes, joints, the gastrointestinal tube, and/or the central nervous system, with reactive amyloidosis as a potential severe long-term consequence. Although individually uncommon, all mAIDs set up an emerging chapter of internal medicine: recent findings have modified our knowledge regarding mAID pathophysiology and clarified that protean inflammatory symptoms can be variably associated with periodic fevers, depicting multiple specific conditions which usually start in childhood, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome, and mevalonate kinase deficiency. There are no evidence-based studies to establish which potential genotype analysis is the most appropriate in adult patients with clinical phenotypes suggestive of mAIDs. This review discusses genetic and clinical hints for an ideal diagnostic approach to mAIDs in adult patients, as their early identification is essential to prompt effective treatment and improve quality of life, and also highlights the most recent developments in the diagnostic work-up for the most frequent hereditary periodic febrile syndromes worldwide

Children and adults with PFAPA syndrome: similarities and divergences in a real-life clinical setting

Introduction: Analogies or differences of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children and adults are barely known. The aim of our study was to compare the overall characteristics of a large cohort of patients, both children and adults, diagnosed with PFAPA syndrome. Methods: In the last decade, we identified 120 children and 63 adults with periodically recurring fevers, who fulfilled the criteria for PFAPA diagnosis. The two subcohorts were analyzed according to demographic features, clinical manifestations, laboratory data, and responses to therapies. Results: The mean age of onset was 2.4 \ub1 1.5 and 19.7 \ub1 10.3 years, respectively, in children and adults, while attacks occurred every 3.8 \ub1 0.8 and every 4.3 \ub1 2.3 weeks, respectively, in children and adults. A higher prevalence of exudative pharyngitis was observed in children (58.8%), and the majority of children had only two cardinal signs during flares. In adults, there was a higher interpersonal variability of the intercritical periods. Inflammatory markers measured during non-febrile periods were normal in children but altered in the totality of adults during febrile periods. A strong efficacy of corticosteroids in controlling the pediatric syndrome was observed, but response rates to steroids were less brilliant in adults. Colchicine and interleukin-1 inhibitors were used in the management of the steroid-resistant adult syndrome. Conversely, tonsillectomy was performed in a very low number of children, but was effective in 60.7% of adults when treated after 16 years. The mean age of disappearance of PFAPA symptoms has been 6.4 \ub1 2.4 years in children, while only 27% of adults have shown a complete drug-free symptom regression. Conclusions: A linear conformity of the PFAPA syndrome has been observed between pediatric and adult patients. PFAPA symptoms tended to disappear with no sequelae in 94.1% of children, while the disease was still active in almost 3/4 of adults at the time of our assessment