A high prevalence of neutrophil‐specific antibodies in ELANE‐mutated severe congenital neutropenia

alexandrossfakianakis shared this article with you from Inoreader A high prevalence of neutrophil‐specific antibodies in ELANE‐mutated severe congenital neutropenia via Pediatric Blood & Cancer Abstract An assay for neutrophil-specific antibodies is frequently used in the workup of chronic severe neutropenia and is suggestive of autoimmune, or sporadically alloimmune neutropenia, rather than severe congenital neutropenia (SCN). We analyzed a neutropenia consortium database for the outcomes of antibody testing initiated before receiving genetic diagnosis in Polish SCN cohort. Test results, performed in a single reference laboratory, were available for 14 patients with ELANE-mutated SCN or cyclic neutropenia, and were frequently positive (36%). We note that the trigger for genetic studies in severe neutropenia should not be affected by antibody-positivity and should be clinically driven. View on Web

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Differentiation of Bolus Texture During Deglutition via High‐Density Surface Electromyography: A Pilot Study

alexandrossfakianakis shared this article with you from Inoreader Differentiation of Bolus Texture During Deglutition via High‐Density Surface Electromyography: A Pilot Study via The Laryngoscope This pilot study evaluated high-density surface electromyography (HDsEMG) during pharyngeal swallows of five different bolus textures from eight healthy human subjects. Results indicate HDsEMG can differentiate swallows of varying consistencies through analysis of EMG signal features such as power and peak counts while maintaining a spatial orientation. This may prove useful in both future diagnostic and behavioral swallow applications. Objective Swallowing is a complex neuromuscular task. There is limited spatiotemporal data on normative surface electromyographic signal during swallow, particularly across standard textures. We hypothesize the pattern of electromyographic signal of the anterior neck varies cranio-caudally, that laterality can be evaluated, and categorization of bolus texture can be differentiated by high-density surface electromyography (HDsEMG) through signal analysis. Methods An HDsEMG grid of 20 electrodes captured electromyographic activity in eight healthy adult subjects across 240 total swallows. Participants swallowed five standard textures: saliva, thin liquid, puree, mixed consistency, and dry solid. Data were bandpass filtered, underwent functional alignment of signal, and then placed into binary classifier receiver operating characteristic (ROC) curves. Muscular activity was visualized by creating two-dimensional EMG heat maps. Results Signal analysis results demonstrated a positive correlation between signal amplitude and bolus texture. Greater differences of amplitude in the cranial most region of the array when compared to the caudal most region were noted in all subjects. Lateral comparison of the array revealed symmetric power levels across all subjects and textures. ROC curves demonstrated the ability to correctly classify textures within subjects in 6 of 10 texture comparisons. Conclusion This pilot study suggests that utilizing HDsEMG during deglutition can noninvasively differentiate swallows of varying texture noninvasively. This may prove useful in future diagnostic and behavioral swallow applications. Level of Evidence Level 4 Laryngoscope, 2023 View on Web

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Real‐world effectiveness of preemptive therapy (PET) for cytomegalovirus (CMV) disease prevention in CMV high‐risk donor seropositive/recipient seronegative (D+R‐) liver transplant recipients (LTxR)

alexandrossfakianakis shared this article with you from Inoreader Real‐world effectiveness of preemptive therapy (PET) for cytomegalovirus (CMV) disease prevention in CMV high‐risk donor seropositive/recipient seronegative (D+R‐) liver transplant recipients (LTxR) via Transplant Infectious Disease Abstract Background Despite superiority of preemptive therapy (PET) compared to universal prophylaxis for prevention of cytomegalovirus (CMV) disease in the CAPSIL randomized trial among CMV D+R- liver transplant recipients (LTxRs), real-world effectiveness may be lower because of logistical concerns about feasibility of PET. Methods We retrospectively assessed PET as standard clinical care at a single transplant center among 50 consecutive adult CMV D+R- LTxRs undergoing a first liver transplant between 4/4/2019 and 5/18/2021 and compared outcomes and adherence to those randomized to PET in the CAPSIL study (N = 100). The primary outcome was CMV disease and secondary outcomes were biopsy-confirmed acute allograft rejection, retransplant, invasive fungal infections, and death, all assessed by 1-year post-transplant. Exploratory outcomes included virologic parameters and measures of adherence to protocol-specified CMV qPCR monitoring. Results Baseline characteristics were similar between groups. The cumulative incidence of CMV disease at 1-year post-transplant was 4/50 (8%) versus 9/100 (9%) in the real-world and CAPSIL cohorts, respectively, p = 1.0. The rate of breakthrough CMV disease during the 100-day PET period was low (2/50 [4%]) and similar to the PET cohort from the CAPSIL study (3/100 [3%]).  All secondary and exploratory outcomes were not significantly different between the real-world and CAPSIL PET cohorts. Conclusions In this first reported study of real-world PET, the feasibility and effectiveness for CMV disease prevention and for other clinical outcomes in CMV D+R- LTxRs were similar to those reported with PET in a clinical trial. Additional studies to confirm feasibility and generalizability in other settings are warranted. View on Web

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Differences in morphology of temporomandibular joint ankylosis of traumatic and infective origin

alexandrossfakianakis shared this article with you from Inoreader Differences in morphology of temporomandibular joint ankylosis of traumatic and infective origin via International Journal of Oral and Maxillofacial Surgery via MedWorm.com The aim of this study was to determine whether there are any differences in morphology between temporomandibular joint ankylosis (TMJA) of traumatic and infective origin. Cone beam computed tomography (CBCT) scans of 25 patients (28 joints) with TMJA of traumatic origin (trauma group) and 15 patients (15 joints) with TMJA of infectious origin (infection group) were included. The following morphological parameters were evaluated on multiple sections of the CBCT scans: lateral juxta-articular bone growth, residual condyle, residual glenoid fossa, ramus thickening, ankylotic mass fusion line, sclerosis of the ankylosed condyle and spongiosa of the glenoid fossa, and mastoid and glenoid fossa air cell obliteration. (Source: International Journal of Oral and Maxillofacial Surgery) View on Web

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A new glutamine synthetase index to evaluate hepatic lobular restoration in advanced fibrosis during anti‐HBV therapy

alexandrossfakianakis shared this article with you from Inoreader A new glutamine synthetase index to evaluate hepatic lobular restoration in advanced fibrosis during anti‐HBV therapy via Journal of Medical Virology Abstract Hepatic lobular architecture distortion is a deleterious turning point and a crucial histological feature of advanced liver fibrosis in chronic liver diseases. Regression of fibrosis has been documented in chronic hepatitis B (CHB) patients. However, whether lobular architecture could be restored following fibrosis regression after antiviral therapy is still unclear. Glutamine synthetase (GS) is generally expressed by perivenular hepatocytes around hepatic veins (HV). In this study, we defined abnormal lobular architecture (GSPT) as GS expressing in the vicinity of portal tracts (PT), which denotes parenchymal extinction and lobular collapse. We defined normal lobular architecture (GSHV) as GS positivity area not approximating PTs. Therefore, we propose a new GS-index, defined as the percentage of GSHV/(GSHV+GSPT), to evaluate the extent of architectural disruption and restoration. We evaluated 43 CHB patients with advanced fi brosis (Ishak stage ≥ 4). Post-treatment liver biopsy was performed after 78 weeks of anti-HBV therapy. The median GS-index improved from 7% (IQR: 0%-23%) at baseline to 36% (IQR: 20%-57%) at week 78 (P < 0.001). Totals of 22 patients (51%) had significant GS-index improvement from 0% (IQR: 0%-13%) to 55% (IQR: 44%-81%), while the other half had almost no change between 17% (IQR: 0%-33%) to 20% (IQR: 12%-31%). When GS-index78w ≥ 50% was used to define hepatic lobular restoration, 37% of patients (16/43) achieved lobular restoration, with much improvement in ALT and AST levels (median value of ∆/Baseline in ALT: restored vs. non-restored was 79.1% vs. 48.8%, P = 0.018; median value of ∆/Baseline in AST: restored vs. non-restored was 69.1% vs. 32.5%, P = 0.005). More importantly, lobular restoration correlated with fibrosis regression (median value of ∆/Baseline in Ishak stage: restored vs. non-restored was 25.0% vs. 0%, P = 0.008). Therefore, in the era of antiviral therapy for CHB, restoration of hepatic lobular architecture is achievable in patients with advanced fibrosis. GS-index provides additional insight into fibrosis regression that goes beyond collagen degradation. This article is protected by copyright. All rights reserved. View on Web

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Guidance on the use of convalescent plasma to treat immunocompromised patients with COVID-19

alexandrossfakianakis shared this article with you from Inoreader Guidance on the use of convalescent plasma to treat immunocompromised patients with COVID-19 via Clinical Infectious Diseases Advance Access Abstract COVID-19 convalescent plasma (CCP) is a safe and effective treatment for COVID-19 in immune compromised (IC) patients. IC patients have a higher risk of persistent infection, severe disease and death from COVID-19. Despite the continued clinical use of CCP to treat IC patients, the optimal dose, frequency/schedule, and duration of CCP treatment has yet to be determined, and related best practices guidelines are lacking. A group of individuals with expertise spanning infectious diseases, virology and transfusion medicine was assembled to render an expert opinion statement pertaining to the use of CCP for IC patients. For optimal effect, CCP should be recently and locally collected to match circulating variant. CCP should be considered for the treatment of IC patients with acute and protracted COVID-19; dosage depends on clinical setting (acute vs protracted COVID-19). CCP containing high-titer SARS-CoV-2 antibodies, retains activity against circul ating SARS-CoV-2 variants, which have otherwise rendered monoclonal antibodies ineffective. View on Web

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The relationship between childhood leukaemia and childhood asthma: A pharmacoepidemiological study from the Netherlands

alexandrossfakianakis shared this article with you from Inoreader The relationship between childhood leukaemia and childhood asthma: A pharmacoepidemiological study from the Netherlands via Pediatric Blood & Cancer Abstract Background It has been suggested that childhood asthma lowers the risk of childhood leukaemia. Studies have found an inverse association between these conditions. However, most studies on this relationship are based on questionnaires and telephone interviews, introducing recall bias. Therefore, we conducted a matched case–control study based on drug prescription data to assess the relationship between both conditions. Methods In a large database, covering more than one million individuals, we identified cases of children who had been prescribed 6-mercaptopurine (6-MP). This drug is used in the outpatient maintenance therapy of childhood leukaemia. We matched every child with leukaemia on sex and age (±6 months) to children without leukaemia (controls). The variable of having had asthma was defined as receiving at least two prescriptions for an inhaled corticosteroid within 12 months. Results We identified 59 children aged 2–18 who had been prescribed 6-MP (cases), and they were matched to 21,918 controls. Of the children with childhood leukaemia, three (5%) had childhood asthma, whereas in the control group 4889 (22%) had childhood asthma (odds ratio [OR] 0.19; 95% confidence interval 0.06–0.60). Conclusion In this study on the relationship between childhood asthma and childhood leukaemia, we found a strong inverse association. View on Web

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