Features of liver function in pediatric patients infected with Delta variant versus Omicron variant of severe acute respiratory syndrome coronavirus 2.

Objective To summarize and analyze the features of liver function in pediatric patients infected with Delta variant versus Omicron variant of severe acute respiratory syndrome coronavirus 2 (SARS - CoV - 2). Methods In this study,an analysis was performed for the liver function test results of the locally transmitted or imported pediatric patients with SARS - CoV - 2 infection during isolation who were admitted to Guangzhou Eighth People's Hospital,Guangzhou Medical University,since May 21,2021,and the clinical data and the constituent ratio of liver injury were compared between the pediatric patients infected with Delta variant and those infected with Omicron variant. The independent samples t - test or the Mann - Whitney U test was used for comparison of continuous data between two groups,and the chi - square test or the Fisher's exact test was used for comparison of categorical data between two groups. Results A total of 85 pediatric patients infected with SARS - CoV - 2 were enrolled,among whom there were 32 (37. 6%)pediatric patients infected with Delta variant and 53 (62. 4%)pediatric patients infected with Omicron variant,and there were no significant differences between the two groups in age,sex, body height,body weight,and comorbidities (all P > 0. 05). There were no significant differences between the two groups in alanine aminotransferase (ALT),aspartate aminotransferase (AST),alkaline phosphatase (ALP),gamma - glutamyl transpeptidase,total bilirubin,albumin,and cholinesterase (all P > 0. 05),and the pediatric patients infected with Omicron variant had a significantly higher level of total bile acid (TBA)than those infected with Delta variant (Z = - 2. 336,P = 0. 020). However,the median values of TBA were within the normal range and the ratios of abnormal TBA were no significant difference between the two groups (P > 0. 05). Among the 85 pediatric patients,10 (11. 8%)had a mild increase in liver function parameters,among whom 7 had an increase in TBA,1 had an increase in ALT, 1 had increases in ALT and AST,and 1 had an increase in ALP. The analysis of liver injury in the pediatric patients infected with Delta variant or Omicron variant showed that there was no significant difference in the constituent ratio of liver injury caused by the two variants (6. 3% vs 15. 1%,chi2 = 0. 691,P = 0. 406). Conclusion Mild liver injury is observed in pediatric patients infected with Delta and Omicron variants of SARS - CoV - 2,but further studies are needed to evaluate the long - term influence of such infection on liver function.Copyright © 2022 Editorial Board of Jilin University

A Case of X-Linked Moesin-Associated Immune Deficiency in a Pediatric Patient

Background: X-Linked Moesin-Associated Immune Deficiency (X-MAID) is a rare severe combined immunodeficiency (SCID) subtype that can present at any age due to its variability. Depending on severity, patients demonstrate failure to thrive, recurrent bacterial and viral infections, and increased susceptibility to varicella zoster. It has been characterized by marked lymphopenia with hypogammaglobulinemia and impaired T-cell migration and proliferation. Case Presentation: This is a report of a Cuban 7-year-old male with poor weight gain and facial dysmorphia. He had a history of recurrent bacterial gastrointestinal infections and pneumonia beginning at 4 months of age. He additionally had 4-6 upper respiratory tract and ear infections annually. While still living in Cuba, he was admitted for a profound EBV infection in the setting of significant leukopenia. A bone marrow biopsy confirmed no malignancy. After he moved to the United States, his laboratory work-up revealed marked leukopenia with low absolute neutrophil and lymphocyte count with low T and B cells, very low immunoglobulin levels IgG, IgA, and IgM, and poor vaccination responses to streptococcus pneumonia, varicella zoster, and SARS-CoV-2. Genetic testing revealed a missense pathogenic variant c.511C>T (p.Arg171Trp) in the moesin (MSN) gene associated with X-MAID. He was managed with Bactrim and acyclovir prophylaxis, and immunoglobulin replacement therapy, and considered for hematopoietic stem cell transplantation. Discussion(s): Diagnosis of X-MAID should be considered in patients with recurrent infections and profound lymphopenia. As with SCID, early diagnosis and intervention is of utmost importance to prevent morbidity and mortality. This case demonstrates the importance of genetic testing in identifying this disease as it may prompt an immunologist to consider HSCT if conservative management is suboptimal. In the current literature, HSCT appears promising, but the long-term outcomes have yet to be described.Copyright © 2023 Elsevier Inc.

Characterization of Demographics and Concomittant Symptoms in Patients with Post COVID-19 Conditions or Long COVID

Objectives: Post COVID-19 conditions or long COVID continues to burden the healthcare system. With the introduction of new code in October 2021 to appropriately capture this condition (U09.9), we have enough data to understand the detailed demographic and clinical characterization of the patients with long COVID. As this new clinical entity continues to evolve, our study will provide insights for care management and planning. Method(s): We conducted a retrospective cohort study from a large deidentified database of US health insurance claims. The study population included all individuals with at least one ICD-10 code for COVID (U07.1) between June 1, 2021, and November 30, 2022. Individuals with at least one ICD-10 code for long COVID (U09.9), at least 7 days after COVID diagnosis were termed "Long COVID" patients. Index date was defined as the first long COVID diagnosis date. We also assessed the most prevalent diagnosis codes within the 30 days pre- and post-index to understand top symptoms. Result(s): A cohort of 253,145 patients (62% female patients;38% male patients) were identified. Among this cohort, 3.2% were pediatric patients aged 0 - 17 years;73.3 % aged 18 - 64 years and 23.5 % aged 65+ years. Most prevalent symptoms that increased in the 30 day pre- and post-index: Nervous system symptoms (6 fold), fatigue (7 fold), Dyspnea (4.3 fold), esophagitis (1.6 fold) chronic kidney disease (1.3 fold) among others. Conclusion(s): Our findings indicate that long COVID is more prevalent in females, with fatigue and dyspnea emerging as top symptoms. These findings are consistent with the published literature. However, we uncovered additional symptoms such as nervous system symptoms, chronic kidney disease among others. Additional analysis is planned to evaluate the association of these symptoms with sociodemographic features to understand the health inequity aspects of long COVID.Copyright © 2023

Mild COVID-19 clinical course among paediatric patients with primary immunodeficiency

Background: The clinical course of coronavirus disease-2019 (COVID-19) varies from those who are asymptomatic, experience mild symptoms such as fever, cough, and dyspnea, to more severe outcomes including acute respiratory distress, pneumonia, renal failure, and death. Early reports suggested severe outcomes in patients with primary immunodeficiency (PID), particularly those with type 1 interferon signalling defects. This prompted a rigid approach to social distancing to protect this patient population, particularly children. To date, real-world data describing the course and outcome of COVID-19 in paediatric PID patients remains scarce. Method(s): In this retrospective case series, we describe the clinical course of 36 paediatric patients with underlying primary immunodeficiency (PID) followed by SickKids Hospital (Toronto, Canada) who were symptomatic and tested positive for SARS-CoV-2 infection between October 2020 to November 2022. Result(s): Our cohort consisted of patients with combined immunodeficiency (66.7%), antibody deficiency (22.2%), neutrophil dysfunction (8.3%), and immune dysregulation (2.8%). The median age was 7.5 years (range: 8 months - 17 years), with 21 male and 15 female patients. Three (8.3%) patients were post-hematopoietic stem cell transplant (HSCT) and 12 (33%) patients were on immunoglobulin replacement. Nine (25%) patients had underlying lung problems including bronchiectasis (1), interstitial lung disease on home oxygen therapy (1), and underlying asthma (7). Most patients had mild clinical course and were managed at home. The most common symptoms were fever (80%), cough (75%) and other upper respiratory tract symptoms (72%). Nineteen (52.7%) patients experienced other symptoms which included headache, lethargy, or gastrointestinal upset. At the time of the infection, 13 patients (36.1%) had received 2 doses of a SARS-CoV-2 vaccine, 5 patients (13.9%) had received 1 dose, and 18 (50%) were not vaccinated. None of the patients received antiviral or monoclonal antibody as prophylaxis or treatment. Only 1 patient required hospital admission out of precaution given the close proximity to HSCT. All patients recovered without complications. Conclusion(s): The paediatric patients with PID followed by our centre experienced mild to moderate COVID-19 symptoms and recovered fully without complications. These findings support the return of much needed social interactions among children, which were impacted severely during the COVID-19 pandemic.Copyright © 2023 Elsevier Inc.

Turkey-based COVID-19 publications in pediatrics: A bibliographic analysis

Aim: The aim of this research is to analyze the pediatric COVID-19 literature published in Turkey and to guide future research. Material(s) and Method(s): Between 11.03.2010 and 11.12.2022, the Web of Science (WoS) All Databases collection was searched for publications related to COVID-19 and pediatric patients. The keywords used during this search were coronavirus-19, COVID-19, SARS-CoV-2, novel coronavirus, 2019-nCoV, pandemic, and/or pediatric, pediatric, children, child. After this search, the selected publications were scanned one by one to determine whether they were suitable for the present study. Authors, organizations, journals, document types, distribution of publications by years (months), most used keywords were obtained from the Web of Science (WoS) All Databases collection. Descriptive analyzes were made from all these obtained data. Result(s): The number of COVID-19 publications originating from Turkey in the field of pediatrics was determined as 375. 48.2% of all publications were published in 2022. These 375 publications were published in 167 different journals. In these publications, the most active author, journal and organization were Yasemin Ozsurekci, Turkish Archives of Pediatrics and University of Health Sciences, Turkey, respectively. The most commonly used keywords were ''child, patient, pandemic, SARS-CoV and vaccine. The most active document types were research articles (295 (78.6%)), editorial materials (15 (4.0%)), letters (43 (11.5%)) and review articles (22 (5.9%)). Discussion(s): We analyzed all articles about COVID-19 from Turkey in the field of pediatrics published so far in the WoS Databases collection. It is obvious that a large literature has emerged in our country on pediatric patients, although not as much as in adults. The long-term adverse effects of the pandemic on pediatric practice and especially on children will need to be evaluated in more detail in future research.Copyright © 2023, Derman Medical Publishing. All rights reserved.

Mastocytosis and COVID19 infection and vaccination: A pediatric series

Introduction: Mastocytosis encompasses a heterogeneous group of diseases characterized by an accumulation of clonal mast cells (MC) in the skin and/or internal organs, and symptoms of MC activation. This MC activation can be elucidated by several factors, including infections or vaccination. Objective(s): We present our experience with COVID infection and vaccination in a series of 133 patients with pediatric mastocytosis. Method(s): Between January 1998 and December 2022, 133 pediatric patients have been referred to our hospital owing to clinically suspected MC disorder, mainly with mastocytosis in the skin. The final diagnoses of mastocytosis were established by the presence of typical skin lesions together with an increase of MC numbers in a biopsy from lesional skin or activating KIT mutations in lesional skin tissue. Serum baseline tryptase and total immunoglobulin E levels were measured, and patients underwent a comprehensive allergy workup to confirm atopic status and history of anaphylaxis. Regarding vaccination, REMA's (Spanish Network on Mastocytosis) protocol was followed. Result(s): 13 patients with COVID infection were identified, of which 25 (56,8%) were female and 0% had symptoms of MC activation. All of them had an asymptomatic or mild course of COVID infection. None of the patients experimented MC activation symptoms during viral illness. Regarding COVID vaccination, all patients received premedication with antihistamine 60 minutes prior vaccination. No one experimented immediate reactions and only one patient (0,75%) referred worsening of MC activation symptoms (baseline pruritus, urtication and brain fog) only after the first doses, recovering without changes in his treatment (oral cromoglycate and antihistamine) in two months. Discussion(s): Although MC have been implicated in the pathogenesis of cytokine storm in COVID19, there is no clinical evidence of SARSCoV- 2-induced MC activation, perhaps related to the fact that bone marrow MC lack angiotensin-converting enzyme 2 receptors.

Cardiac manifestations of post-COVID syndrome in children and adolescents.

The aim of the research. To study the features of cardiovascular system disorders in post-covid syndrome (PCS) in children and adolescents after a mild form of coronavirus infection (COVID-19). Material and methods. From 260 children and adolescents after a mild form of COVID-19, a total of 30 patients aged 7-17 years with cardiac manifestations of PCS were selected. Therewith, 32 patients with an uncomplicated form of the disease were selected to form a comparison group. In 3 and 6 months after disease onset, a comprehensive examination of patients was performed with a questionnaire on the subjective scale for MFI-20 assessment asthenia (Multidimensional Fatigue Inventory-20), electrocardiography (ECG), echocardiography;daily monitoring of ECG and blood pressure. The biochemical blood test included assay of creatine phosphokinase-MB (CPK-MB), troponin I and lactate dehydrogenase (LDH). Results. The incidence of PCS with cardiac manifestations amounted to 11.5 %. After 3 months from the disease onset, complaints of pain and discomfort in the chest, palpitations, fatigue, and poor exercise tolerance persisted. Asthenic syndrome was diagnosed in 70 % of patients. The "general asthenia" indicator totalled14 [12;16] points (p<0.001) and was associated with the age of patients (r=+0.5;p<0.05). Arrhythmic syndrome and conduction disorders were detected in 67% of children. Labile arterial hypertension and hypotension occurred in 23 % of the adolescents. The increase in CPK-MB remained in 17% of the children, LDH - in 10%. In the sixth month after the onset of the disease, there were no significant differences in the results of the examination in the observation groups. However, a decrease in the level of resistance within 6 months was recorded in 43.3% of the schoolchildren with PCS (p<0.001). Conclusion. The data obtained indicate the need for early verification of cardiopathies in children with COVID-19, determination of a set of therapeutic and rehabilitation measures as well as ECG monitoring.Copyright © 2022, Krasnoyarsk State Medical University. All rights reserved.

PPP1R13L-Related Cardiomyopathy Stability Following IL1 Inhibition

Introduction: Variants in PPP1R13L are associated with severe childhood-onset cardiomyopathy resulting in rapid progression to death or cardiac transplantation. PPP1R13L is proposed to encode a protein that limits the transcriptional activity of the NFkappaB pathway leading to elevated IL-1, IL-6, and TNF-alpha production in murine models. Optimal medical management for PPP1R13L-related cardiomyopathy is unknown. Here we report usage of a targeted anti-IL-1 immuno-modulatory therapy resulting in cardiac stabilization in a pediatric patient with congenital cardiomyopathy secondary to PPP1R13L variants. Case Report: A 4-year-old boy presented acutely with fever in the setting of persistent abdominal pain, vomiting, fatigue, and decreased appetite for two months following a mild COVID-19 related illness. Echocardiogram revealed severely depressed biventricular systolic function with an ejection fraction of 30%. Due to acute decompensated heart failure symptoms with hemodynamic instability, he was intubated and placed on continuous inotropic infusions with aggressive diuresis. Cardiac MRI demonstrated extensive subepicardial to near transmural fibrosis by late gadolinium enhancement in right and left ventricles. An implantable cardioverter-defibrillator (ICD) was placed due to frequent runs of polymorphic non-sustained ventricular tachycardia. Testing for viral pathogens was positive for rhino/enterovirus. Initial genetic testing was non-diagnostic (82-gene cardiomyopathy panel) but given the patient's significant presentation whole genome sequencing was pursued that showed two separate PPP1R13L variants in trans (c.2167A>C,p.T723P and c.2179_2183del,p. G727Hfs*25, NM_006663.4). Patient serum cytokine testing revealed elevations in IL-10 (4.7 pg/mL) and IL-1beta (20.9 pg/mL). Given the patient's tenuous circumstances and concern for continued progression of his cardiac disease, a trial of IL-1 inhibition via anakinra dosed at 3 mg/kg or 45 mg daily was initiated following hospital discharge. With approximately 6 months of therapy, the patient's cardiac function is stable with normalization of IL-10 and IL-1beta serum levels. Notably, the ventricular arrhythmia decreased after initiation of anakinra with no ICD shocks given. Therapy overall has been well tolerated without infectious concerns. Conclusion(s): In patients with PPP1R13L-related cardiomyopathy, immuno-modulatory therapies should be considered in an attempt to slow cardiac disease progression.Copyright © 2023 Elsevier Inc.

COVID-19 associated severe mucocutaneous blistering eruptions: A case series

Introduction: Mucocutaneous eruptions are associated with many viral processes and present as erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens Johnson syndrome (SJS) or toxic epidermal necrosis (TEN). Limited reports have described the association of COVID-19 and mucocutaneous eruptions in children and adults to date. Method(s): This was a multicenter descriptive case series performed at six tertiary medical centers. Inclusion required a clinical diagnosis of EM, RIME, SJS or TEN and a positive COVID-19 test (rapid antigen or PCR) less than 4 weeks prior to onset of dermatologic manifestation. Data was collected at time of each patient encounter. Result(s): A total of 7 patients met criteria and had a median age of 15 years for pediatric patients (<18 years of age) and 36 years for adult patients (>18 years of age). Patients were found to have a diagnosis of RIME in 85.7% of cases. Oral mucosal involvement was the most common clinical finding (100%), followed by ocular (57.1%), urogenital (57.1%) and skin (42.9%) involvement. 71.4% of cases required hospitalization for their cutaneous eruption. No patients died from their inflammatory condition. Discussion(s): This case series highlights the development of mucocutaneous eruptions in association with COVID-19 infection. Within our cohort, RIME was the most commonly identified COVID-associated eruption. These findings provide additional evidence that abnormalities in host immune response to viral pathogens play a role in severe mucocutaneous blistering conditions. Further investigation will aid our understanding of this disease to improve diagnostics and advance targeted treatments for patients in the future.

The influence of the Covid-19 pandemic on the shunt revision rate

Introduction: During the first year of the Covid-19 pandemic we observed a decrease of our shunt revision rate. In order to investigate a possible correlation with an assumingly lower general infection rate in children in times of lock down and homeschooling, we performed a detailed analysis of our shunt and general pediatric patient population. Method(s): Electronic patient charts retrieval for children admitted for shunt revision or infectious diseases was performed for four time periods (study period April 2020 - March 2021, control periods from three previous years). A detailed analysis of all shunt revision and infectious cases including age and season specific evaluation followed. Possible correlations were investigated. Result(s): A total of 318 shunt revision and 13,919 pediatric cases have been evaluated. The shunt revision rate during the study period was 29% less compared to the average rate of three previous years (p 0.061), the number of pediatric cases with main diagnosis infection dropped significantly (p < 0.05), whereas other pediatric admissions remained stable. Significant age or seasonal influences did not exist. The number of shunt revisions in association with a documented systemic infection or a primary shunt infection dropped significantly during the study period (p<0.05 each). This was not the case for underdrainage, overdrainage (p>0.05 each) or other indications. In general, infections of upper and lower airways, the gastrointestinal and nervous system decreased during the pandemic, urinary infection rates remained stable. Conclusion(s): The decreased shunt revision rate during the first year of the pandemic seems correlate with a decrease of the general infection rate in children and adolescents at the same time. Infectionassociated shunt failures showed a significant decrease during this period compared to previous years.

The Evolution of the Covid-19 Pandemic in Pediatric Patients with Sickle Cell Disease: From Alpha to Omicron

Background: Approximately two years ago, COVID-19 was declared a global pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), and through genomic surveillance, we have seen the emergence of variants of SARS-CoV-2. In the United States, over 78 million cases and >900,000 deaths attributable to COVID-19 have been reported. SCD was identified as a risk factor for severe COVID-19 disease in adults and pediatric patients. The emergence of novel SARs- CoV-2 variants has led to challenges in diagnosis, treatment, and prediction of long-term sequelae in individuals with SCD and COVID-19. Aim(s): We compare the overall seasonal variation of COVID-19 variants and patterns of healthcare utilization and clinical presentation over time in pediatric patients with SCD and COVID-19 at Children's National Hospital (CNH). Method(s): Our single-center, observational cohort study included 193 pediatric patients with SCD (0-21 years) with PCR-confirmed SARSCoV- 2 infection between March 31, 2020, and January 31, 2022. Per the SECURE SCD Registry definitions, clinical severity was classified as asymptomatic, mild, moderate, and severe. Result(s): A total of 193 unique patients with SCD and positive SARS-CoV-2 PCRs between March 2020-January 2022 were included in our registry. Most patients were female (51.8%), and the mean age was 11.2 years (SD 6.5 years). Most of the cohort resides in Maryland (N=135), and HbSS was the dominant genotype (69.4%). During the alpha dominant variant of the COVID-19 pandemic (March 2020- June 2021) there were 70 cases, followed by 40 cases during the Delta variant (July 2021- December 19, 2021), and 83 cases during the Omicron variant dominance (from December 20, 2021-January 31,2022). There were 149 patients (77%) that presented to the emergency department (ED) or were hospitalized. There were a total of 80 hospitalizations (41.5%), and a relative comparison showed that the percentage of hospitalizations was highest during the delta wave (47.5%) and lowest during the omicron wave (36.1%) (p= 0.407). ED-only utilization was highest in the era of omicron (43.4%, N=36), followed by delta (32.5%, N=13), and then alpha (30%, N=21)(p=0.197). The most common SCD-related complication was vaso-occlusive (VOC) pain (33%, N=64) which accounted for half of all hospital admissions (51%, N=41 of 80). Acute chest syndrome (ACS) was reported in 40% (N=32) of admitted patients and was highest in the alpha era (54.8%, N=17). The use of blood transfusion therapy was highest in the alpha (N=17) and delta (N=14) variants, while Remdesivir use was highest in omicron (N=15). A total of 6 patients received monoclonal antibodies (Delta, N=4;omicron, N=2). Throughout all the variants, there was a significant difference in COVID-19 clinical severity (p>0.005). Of the patients classified as asymptomatic (13%, N=25), seventy-two percent (n=18) were diagnosed during the alpha variant. Mild severity was the most prevalent (69%, N=134), with the omicron variant having the highest cases (51.5%, N=69). Severe cases were observed in all variants (6.7%, N=13) but were most prevalent during the alpha variant (46.2%, N=6). Summary - Conclusion(s): Interestingly, while the relative percentage of hospitalizations was lowest during the omicron wave, it saw the highest percentages of ER utilization. Overall, COVID-19 remains mild in pediatric patients with SCD, and notably, there was higher health care utilization in the omicron era.

Pediatric impact of COVID-19.

Children infected with SARS-CoV-2 are underrepresented during the current COVID-19 outbreak. Unlike other respiratory viruses, SARS-CoV-2 rather infects adults who subsequently infect their children. From recent Chinese and Italian data, children commonly present mild to moderate disease, a large proportion of them being asymptomatic. In particular, children present significantly less fever, cough and pneumonia compared to adults. However, more cases of pneumonia were reported from children infected with SARS-CoV-2 compared to those infected with H1N1. No vertical transmission of SARS-CoV-2 has been described so far.Copyright © 2020 Editions Medecine et Hygiene. All rights reserved.

Status of 18 pediatric cases of COVID-19 post-liver transplantation.

We describe our experience with COVID-19 after pediatric liver transplantation (LT). In this study, we analyzed 18 of 196 children who contracted COVID-19 after LT during outpatient follow-up at our department. The severity of COVID-19 was mild in all cases, and all cases were cured without sequelae. COVID-19 after LT in children may have a high risk of severe disease. However, the disease is relatively mild and may be cured.Copyright © 2022 The Japan Society of Hepatology.

ECMO for the immunocompromised pediatric patient: Our experience

Objectives: To present a series of immunosuppressed patients (oncohematological disease, congenital immunosuppression, hematopoietic stem cell (HSCT), and solid organ transplant) assisted on ECMO. Method(s): Descriptive, retrospective study (2011-2020) of a cohort of 9 immunosuppressed patients, supported on ECMO. Medical records were reviewed and demographic, clinical, and analytical variables were collected. Result(s): In our series of 9 patients, 5 were male, the median age was 8 years [RIC 3-11 years]. Considering the underlying disease, 6 were oncologic, 1 liver transplant and 2 with congenital immunodeficiency after HSCT. 4 were under active chemotherapy (median 6 days after the last cycle [RIC 5-188]). 6 were admitted due to acute respiratory failure, 3 due to hemodynamic instability (3/9), (one septic shock). The median PEEP was 12 [RIC 9-15] and FiO2 100% (81-100%). 78% (6) required vasoactive drugs (median inotropic score 35 [RIC 0-75]. 40%. 5 had severe neutropenia and/or plateletopenia in the 24 hours prior to ECMO, and alterations in acid-base balance (median pH 7. 1 [RIC 6.9-7.15]. 5 were on multiorgan failure. TPrimary ECMO transport was performed in 4 patients (44%). Cannulation was peripheral in 80% (57% cervical, 43% femoral) and central in 20%;70% VA-ECMO. Median time of assistance was 15 days [RIC 3.5-31.5] in cardiac ECMO (4), and 29 days [RIC 13.5-42] and in pulmonary ECMO (n=5). The median total time of admission was 45 days [RIC 27-59]. 9 had an infection, 2 COVID after HSCT, and 8 bleeding complications, but only one required surgical revision. Renal replacement therapy was used in 5 (median 9 days [RIC 5-34.5]). Other therapies used were polymyxin hemadsorption(2), intratracheal surfactant(2), plasma exchange(1), infusion of mesenchymal cells(1) and specific memory T lymphocytes(2). 4 patients died, 5 survived decannulation, 2 died later, with an overall survival rate to hospital discharge of 33% (3/9). Conclusion(s): Despite having a worse prognosis, ECMO can increase survival in immunosuppressed patients, in situations that are challenging and require a multidisciplinary approach.

Real-World effectiveness of voxelotor for the treatment of sickle cell disease

Introduction: Voxelotor is a first-in- class sickle haemoglobin polymerisation inhibitor that targets the pathophysiology of sickle cell disease (SCD). Studies have shown that voxelotor increases haemoglobin and reduces markers of haemolysis. Emerging evidence suggests that voxelotor may improve the clinical symptoms of SCD, lower vaso-occlusive crisis (VOC) rates, and reduce transfusion needs. Objective(s): To examine the real-world impact of voxelotor on transfusion, VOC, and hospitalisation rates among patients with SCD. Method(s): Medical and pharmacy claims data for patients >=4 years with SCD who started voxelotor between November 2019 and March 2022 were obtained from the Symphony Health database. Patients with >=1 year of data before the index date (date of first voxelotor claim) were included. Annualised study outcomes were calculated for patients with >=1 occurrence of the corresponding event in the 3-month preindex period. Outcomes from a 90-day lookback were reported for the total and paediatric (aged 4 to <18 years) populations. Result(s): Of 4023 eligible patients from the Symphony Health database included in the analysis, 596 were <18 years. Compared with the 3-month preindex period, significantly lower annualised rates of transfusions, VOCs, and hospitalizations, and lower annualised mean number of inpatient days, were observed in the total population and paediatric subgroup over the 3-month postindex period. For the total population, the annualised event rates declined by 50.6% for transfusions (n = 248), 23.1% for VOCs (n = 1368), 35.5% for VOC-related hospitalizations (n = 757), and 39.4% for all-cause hospitalizations (n = 928). The annualised mean number of inpatient days declined by 29.6% for VOC-related hospitalizations (n = 757) and by 22.9% for all-cause hospitalizations (n = 928). For paediatric patients, the annualised event rates declined by 79.6% for transfusions (n = 18), 42.4% for VOCs (n = 157), 56.8% for VOC-related hospitalizations (n = 81), and 51.5% for all-cause hospitalizations (n = 106). The annualised mean number of inpatient days declined by 54.1% for VOC-related hospitalizations (n = 81) and by 45.8% for all-cause hospitalizations (n = 106). Conclusion(s): Treatment with voxelotor may provide a clinical benefit to patients with SCD by reducing the frequencies of transfusions, VOCs, and hospitalizations and decreasing inpatient days. Greater reductions were observed in the paediatric subgroup, potentially due to the smaller sample size, historically greater treatment compliance in paediatric patients, or younger patients having accumulated fewer SCD-related complications, enabling a greater clinical response. Limitations include the study's non-randomized design, reliance on claims data, and changes in healthcare use during the COVID-19 pandemic confounding the data.

Appropriateness of musculoskeletal soft tissue swelling ultrasound scans

This audit's purpose was to assess the appropriateness of referrals from primary and secondary care for soft tissue lumps and bumps ultrasounds (US) that are being performed in a university teaching hospital imaging department and compare against the British Sarcoma Group (BSG) national guidelines. This is on the background of increasing referrals for ultrasound scans of soft tissue lumps with limited clinical information in the face of increasing workload in the imaging department. This was a retrospective study, analysing data for all US performed for soft tissue swelling - over a one-month period. We focused on all US undertaken in the imaging department for June 2019 (pre-COVID) allowing for a 24 month follow-up period. Indications and findings were recorded. The requests were assessed regarding appropriateness by comparing to NICE and BSG guidelines. 200 ultrasound scans were undertaken during the one month period;14 paediatric patients were excluded from the data. The majority of referrals were from primary care (92%). Of the 186 cases analysed, 102 cases (54%) did not contain any appropriate clinical information as per the guidelines and no descriptive features were mentioned. Of the 81 cases (44%) that did contain relevant clinical information, 14 cases (17%) mentioned clinical features that, according to the guidelines, did not require ultrasound imaging and hence were also inappropriate. This audit shows the significant proportion of inappropriate requests from primary care. There was a lack of relevant clinical information on requests for US soft tissue, making it difficult to ascertain which patients need to be offered a direct access US in the required twoweek time frame. Considerations for improving the quality of referrals include presenting the audit within primary care teams to enhance referrer education and ratifying an US request system with a flowsheet proforma to ameliorate the referral process.

NEI 2022 Abstracts

The proceedings contain 96 papers. The topics discussed include: practical pharmacotherapy for opioid use disorder in the age of fentanyl;can COVID-19 cause acute psychosis in pediatric patients? a case report;a survey of bullying experiences in a child and adolescent psychiatric clinic population;acute emergence of suicidal thoughts following Lemborexant initiation: an adverse reaction case report;assessing the unmet clinical need and opportunity for digital therapeutic intervention in schizophrenia: perspective from people with schizophrenia;rapid antidepressant effects and MADRS item improvements with AXS-05 (DEXTROMETHORPHAN-BUPROPION), an oral NMDA receptor antagonist in major depressive disorder: results from two randomized double-blind, controlled trials;targeting lncRNA NEAT1 impedes Alzheimers disease progression via MicroRNA-193a mediated CREB/BDNF and NRF2/NQO1 pathways;and impact of AXS-05 (DEXTROMETHORPHAN-BUPROPION), an Oral NMDA receptor antagonist, on Anhedonic symptoms in major depressive disorder.

The Kinetics of Anti-SARS-CoV-2 Antibodies in Pediatric Patients and the Characterization of Post-COVID-19 Condition at 6 Months After Infection: Protocol for a Longitudinal Observational Study.

BACKGROUND: Data regarding the kinetics of anti-SARS-CoV-2 antibodies and information about post-COVID-19 condition (colloquially known as "long COVID") in children are scarce, especially in low-income countries. Even though cases of COVID-19 in children are less prevalent than adults, post-COVID-19 condition cases in children are high and have a burden that may impact their growth and development. There are other features of antibody kinetics in connection with SARS-CoV-2 infection that are yet unknown as of this writing, especially in children following infection. Furthermore, the long-term results, risk factors, and underlying pathophysiology are still uncertain. To better understand post-COVID-19 condition in children, it is necessary to further investigate the impact of clinically significant factors such multisystem inflammatory syndrome and disease severity among hospitalized survivors through their SARS-CoV-2 antibody response. OBJECTIVE: We aim to analyze anti-receptor-binding domain SARS-CoV-2 immunoglobulin G antibodies over time and characterize the signs and symptoms of post-COVID-19 condition in pediatric patients at the time of diagnosis and at 2 weeks and 1, 3, and 6 months following infection. METHODS: This is a longitudinal observational study in Indonesia. Pediatric patients diagnosed with COVID-19 by positive molecular assay using nasopharyngeal swab will be tested for anti-SARS-CoV-2 antibodies using the Roche Elecsys Anti-SARS-CoV-2 S assay at the time of diagnosis and at 2 weeks and 1, 3, and 6 months following infection. Antibody titer data will be reported as means and SDs. The respondents' signs and symptoms will be observed up to 6 months after the onset of infection, including the vaccination event, reinfection, rehospitalization, and mortality. The clinical features will be reported as frequencies and percentages. RESULTS: Participant enrollment began in February 2022. As of September 30, 2022, a total of 58 patients were enrolled. After data collection, results are expected to be analyzed in August 2023. CONCLUSIONS: This study will allow us to know the kinetics of anti-receptor-binding domain SARS-CoV-2 immunoglobulin G antibodies and data regarding post-COVID-19 condition up to 6 months following infection in the Indonesian pediatric population. Furthermore, this study has the potential to serve as a foundation for government decisions about vaccination programs and prevention measures. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/43344.