Atypical onset as predictor of poor outcome in Pediatric Systemic Lupus Erythematosus (pSLE)

Poster Presentation

Euclid: The reduced shear approximation and magnification bias for Stage IV cosmic shear experiments

Context: Stage IV weak lensing experiments will offer more than an order of magnitude leap in precision. We must therefore ensure that our analyses remain accurate in this new era. Accordingly, previously ignored systematic effects must be addressed. / Aims: In this work, we evaluate the impact of the reduced shear approximation and magnification bias on information obtained from the angular power spectrum. To first-order, the statistics of reduced shear, a combination of shear and convergence, are taken to be equal to those of shear. However, this approximation can induce a bias in the cosmological parameters that can no longer be neglected. A separate bias arises from the statistics of shear being altered by the preferential selection of galaxies and the dilution of their surface densities in high-magnification regions. / Methods: The corrections for these systematic effects take similar forms, allowing them to be treated together. We calculated the impact of neglecting these effects on the cosmological parameters that would be determined from Euclid, using cosmic shear tomography. To do so, we employed the Fisher matrix formalism, and included the impact of the super-sample covariance. We also demonstrate how the reduced shear correction can be calculated using a lognormal field forward modelling approach. / Results: These effects cause significant biases in Ωm, σ8, ns, ΩDE, w0, and wa of −0.53σ, 0.43σ, −0.34σ, 1.36σ, −0.68σ, and 1.21σ, respectively. We then show that these lensing biases interact with another systematic effect: the intrinsic alignment of galaxies. Accordingly, we have developed the formalism for an intrinsic alignment-enhanced lensing bias correction. Applying this to Euclid, we find that the additional terms introduced by this correction are sub-dominant

International Consensus Statement on Rhinology and Allergy: Rhinosinusitis

Background: The 5 years since the publication of the first International Consensus Statement on Allergy and Rhinology: Rhinosinusitis (ICAR‐RS) has witnessed foundational progress in our understanding and treatment of rhinologic disease. These advances are reflected within the more than 40 new topics covered within the ICAR‐RS‐2021 as well as updates to the original 140 topics. This executive summary consolidates the evidence‐based findings of the document. Methods: ICAR‐RS presents over 180 topics in the forms of evidence‐based reviews with recommendations (EBRRs), evidence‐based reviews, and literature reviews. The highest grade structured recommendations of the EBRR sections are summarized in this executive summary. Results: ICAR‐RS‐2021 covers 22 topics regarding the medical management of RS, which are grade A/B and are presented in the executive summary. Additionally, 4 topics regarding the surgical management of RS are grade A/B and are presented in the executive summary. Finally, a comprehensive evidence‐based management algorithm is provided. Conclusion: This ICAR‐RS‐2021 executive summary provides a compilation of the evidence‐based recommendations for medical and surgical treatment of the most common forms of RS

Preoperative sinonasal computed tomography score in chronic rhinosinusitis with nasal polyps.

This study investigated the relationship between sinonasal inflammatory involvement accord-ing to the computed tomography (CT) staging system (Lund–Mackay score) with clinical, labor-atory, histopathological and prognostic features of chronic rhinosinusitis with nasal polyps (CRSwNP). Seventy-eight patients with CRSwNP who had undergone surgery were enrolled. Total (p = 0.0062), ethmoid (p = 0.0496), sphenoid (p = 0.0335), ostiomeatal complex (OMC) (p = 0.0235) and frontal (p = 0.0164) CT scores were predictive of non-steroidal anti-inflammatory drugs-exacerbated respiratory disease (NERD) in the univariate analysis. Total (p = 0.0022), eth-moid (p = 0.0290), sphenoid (p = 0.0370), frontal (p = 0.0116), maxillary (p = 0.0357) and OMC (p = 0.0058) CT scores were predictve of asthma at the univariate analysis. No significant differences were found between patients with vs. without allergy in terms of total and partial CT scores. High blood eosinophil counts (>0.24 vs. ≤0.24 cells × 109/L) resulted in being associated with total (p = 0.0213), maxillary (p = 0.0227) and ethmoid (p = 0.0491) CT scores in the univariate analysis. Higher ethmoid (p = 0.0006) and total sinonasal (p = 0.0027) CT scores were found to predict his-topathologically eosinophil CRSwNPs in the univariate analysis. CT scores did not result as predictive of NSAID-exacerbated respiratory disease, asthma, or blood eosinophil count at the multivariate analysis. Risk of relapse was related to the presence of NERD (p = 0.0207, HR [95% CI] 3.914 [1.232–12.435]), higher preoperative total (HR = 1.098 95%CI: 1.001–1.204, p = 0.0486) and frontal sinus CT scores (HR = 1.555 95%CI: 1.006–1.886, p = 0.0218), but these results were not confirmed by the multivariable analysis. Sinonasal CT scores showed significant differences in this heterogeneous inflammatory condition. Identifying CRSwNP characteristics is necessary to avoid generic treatments with poor outcomes

Steady improvement of prothrombin levels after cyclophosphamide therapy in pediatric lupus anticoagulant hypoprothrombinemia syndrome (LAHPS).

Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare acquired disorder associated with several different conditions but mostly with systemic lupus erythematosus (SLE). LAHPS probably results from the presence of anti-Factor II antibodies, which usually counterbalance the prothrombotic effect of the lupus anticoagulant (LAC). In fact, Factor II deficiency in SLE is invariably associated with the presence of LAC. No consensus exists for the treatment of LAHPS. Corticosteroids, with or without the addition of vitamin K or blood products, have been a successful first-line treatment. Immunoglobulin (IVIG) treatment has been shown to be effective in the setting of acute bleeding. However, in some patients, conservative treatment is not enough to control bleeding, and the addition of immunosuppressive therapy, usually azathioprine, is needed. In our patients, Factor II deficiency reappeared after tapering steroids. Both children achieved normal Factor II levels with cyclophosphamide. This effect was long-lasting, a phenomenon that has not been documented in children prior to this report

Structured histopathology and laboratory evidence in nasal polyposis with different pathogenesis.

Purpose: Non-steroidal anti-inflammatory drugs-exacerbated respiratory disease (NERD), intrinsic asthma, eosinophilic granulomatosis with polyangiitis (EGPA) and odontogenic sinusitis may be associated with nasal polyps. The aim of the study was to compare circulating inflammatory cells and structural histopathology of these groups of nasal polyposis. Methods: We retrospectively evaluated 71 patients with nasal polyps stratified according to the above-mentioned pathogenesis. All patients underwent preoperative laboratory investigations and primary endoscopic sinus surgery. Surgical specimens were submitted to structured histopathological evaluation. Results: The median tissue eosinophil count (cells/HPF) was significantly different between the considered groups of nasal polyposis (p=0.0004). The median of NERD sub-cohort was significantly higher than intrinsic asthma (p=0.0030), odontogenic CRS (p=0.0001) and EGPA ones (p=0.0094). Eosinophilic aggregates positive rate was significantly higher in NERD sub-cohort than in odontogenic CRS (p=0.0072), EGPA (p=0.0497) and asthma (p=0.0188) ones. EGPA sub-cohort had a higher neutrophil infiltrate positive rate than NERD (p=0.0105) and intrinsic asthma ones (p=0.0040). Odontogenic CRS sub-cohort had a higher neutrophil infiltrate positive rate than NERD (p=0.0140) and asthma ones (p=0.0096). EGPA sub-cohort had a higher presence of fibrosis than NERD (p=0.0237) and odontogenic CRS sub-cohort (p=0.0107). Odontogenic sub-cohort had a lower subepithelial edema positive rate than NERD (p=0.0028) and asthma (p=0.0149) ones. Conclusions: Structural histopathology may identify nasal polyps histotypes with different morphological patterns. The identified histopathological features can facilitate the recognition of rational therapeutic and followup approaches that consider the tissue modifications associated with the response to drugs and surgery

Blood eosinophilic and basophilic trends in recurring and non-recurring eosinophilic rhinosinusitis with nasal polyps

Background: Very few studies have compared blood eosinophil and basophil levels before and after surgery in patients with eosinophilic CRSwNP (eCRSwNP). No investigations seem to have repeatedly measured them pre- and postoperatively in eCRSwNP patients with recurring versus non-recurring disease to examine how their levels evolved. Objective: Hence this study to analyze blood eosinophil and basophil levels in patients with eCRSwNP immediately before surgery and afterwards, at 4months, 1 year, and then yearly up to 5 years. Methods: Fifty-one eCRSwNP patients were enrolled, who all had preoperative laboratory data and the results of at least 4 of the 6 scheduled postoperative follow-up tests. Results: Seventeen patients had recurrent disease. Blood eosinophil counts (p\ubc0.005) and percentages (p\ubc0.002) were both higher in these patients than in those whose eCRSwNPs did not recur after surgery. Blood basophil counts (p\ubc0.04) and percentages (p<0.05) were also significantly higher in patients whose eCRSwNPs relapsed. The time by relapse interaction was not significant for either counts or percentages, though an effect of time was detected for basophil counts (p\ubc0.01). Conclusions: In eCRSwNP, the statistical analysis of repeated quantitative laboratory data can shed light on the evolution of a patient\u2019s systemic inflammatory picture in response to previous treatments, and above all to long-term therapies. Longterm monitoring of blood eosinophil and basophil levels could be of significant value when monoclonal antibodies that inhibit IL signaling will widely enter in clinical practice for eCRSwNP treatment to follow the effectiveness of therapy over time

Conventional septoplasty complications. A Systematic review and meta-analysis.

Purpose: Septoplasty is a commonly performed procedure in otolaryngology. The usual indication is a septal deviation resulting in significant nasal airway obstruction. Although septoplasty is considered a safe technique, some complications can occur. The aim of this investigation was to review and analyze the literature on complications of conventional septoplasty. Methods: A search was run in the PubMed, Scopus, Web of Science databases. To be eligible for this review, a study had to: (i) investigate septoplasty performed on adult patients, (ii) report complications in the short/long period, (iii) be written in English, (iv) be published between January 1, 2017, and May 23, 2022. Results: Ten studies reported quantitative data on post-operative hemorrhage (pooled overall rate [POR] 0.04 [95 % CI, 0.02–0.07]). Twelve investigations were considered for the meta-analysis on septal perforation (POR 0.02 [95 % CI, 0.01–0.04]). Regarding persistent deviation, six studies were eligible (POR 0.13 [95 % CI, 0.07–0.19]). Six papers reported data on post-operative septal hematoma (POR 0.02 [95 % CI, 0.01–0.03]). Finally, six investigations on post-operative synechiae were considered (POR 0.04 [95 % CI, 0.00–0.11]). The funnel plots were substantially symmetrical, ruling out any publication bias for all considered complications, except for synechiae. Conclusions: Calculation of the occurrence rate of the single complication is useful in clinical practice. Proper preand post-operative planning, meticulous attention to anatomy and good visualization during surgery are the keys to a safe and effective septoplasty with a very low complication rate