Late Development of Pneumatoceles in Necrotizing PneumoniaSila Y. Kocer 1, Nathan C. Hull MD2, D. Dean Potter, Jr. MD 3, Theresa Madigan MD 4, Jennifer M. Boland MD5 and Nadir Demirel MD 61Ondokuz Mayis University School of Medicine, Samsun, Turkey2Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Infectious Diseases, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Pulmonology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] : Sila Y. Kocer, Ondokuz Mayis University School of Medicine, Körfez, 55270 Samsun, Turkey. Email address: [email protected]. Tel.: +905514571963Conflict of interest: The authors declare no conflict of interest.Author contributions : Sila Y. Kocer: Writing – original draft. Nathan C. Hull: Writing – review and editing.D. Dean Potter Jr.: Writing – review and editing.Theresa Madigan: Writing – review and editing.Jennifer M. Boland: Writing – review and editing.Nadir Demirel: Writing – review and editing.Keywords : Lung cysts; complicated pneumonia; lobectomy; imaging; childrenTo the editor,Community-acquired pneumonia (CAP) is one of the most common serious infections in children, although it usually has a good prognosis1. Necrotizing pneumonia (NP), a rare but severe complication of CAP, consists of the destruction of the consolidated lung parenchyma, potentially leading to the formation of thin-walled cavities known as pneumatoceles 2. While they often resolve spontaneously without sequalae, progressively enlarging pneumatoceles have been reported 5. We report a pediatric case with an unusual course of pneumatocele development.A 3-year-old female with history of congenital hypothyroidism and mild asthma presented to her primary care physician for evaluation of intermittent fever for the past 6 days, with a maximum temperature of 38.8°C. She had a worsening wet cough and complained of chest pain while coughing. Lung examination was normal except for mild tachypnea. Based on the patient’s presentation, empiric oral amoxicillin 90 mg/kg/day was started for possible CAP. The patient presented to the clinic on day 6 of her antibiotic treatment with persistent low-grade fever and ongoing cough. She had normal vital signs, and there were no signs of respiratory distress. The lung examination revealed crackles at right lung fields. A chest X-ray (CXR) showed a large consolidation and an airspace with an air-fluid level in the right upper lobe (RUL) (Figure 1A). There was no pleural effusion. The patient was diagnosed with complicated pneumonia and was referred to our hospital.A chest computed tomography (CT) without intravenous (IV) contrast showed a large consolidation in the RUL with scattered internal cystic areas containing air-fluid levels (Figure 1B, C). These findings raised concern for NP, abscess, or congenital pulmonary airway malformation with superimposed pneumonia. Laboratory test results showed mild anemia (hemoglobin concentration of 10 g/dl), leukocytosis (white blood cell count of 14.1 x 109/L), thrombocytosis (platelet count of 752 x 109/L), and high C-reactive protein (CRP) (80 mg/L, normal: <5 mg/L). She was admitted and started on IV ceftriaxone and IV vancomycin. She remained afebrile during admission and was clinically well appearing. A nasal swab culture for Methicillin-resistant Staphylococcus aureus was negative, therefore, on day 2 of admission, IV vancomycin was discontinued. AStreptococcus pneumoniae urine antigen test was positive. Serologic testing for endemic fungi was negative. A QuantiFERON-TB Gold was indeterminate due to inadequate mitogen response. On day 3 of admission, in preparation for discharge, IV ceftriaxone was switched to oral cefdinir 14 mg/kg/day to complete a 4-week course. She remained afebrile and well after an additional 24-hour period of observation and was subsequently discharged.Towards the conclusion of her antibiotic course, a follow-up CXR showed near resolution of the RUL consolidative opacity with a few small residual lucencies in the RUL, presumed to be residual pneumatoceles (Figure 1D). She was asymptomatic without a cough, and her lung examination was normal. Inflammatory markers, including CRP and sedimentation rate, as well as white blood cell count and platelet count, were in the normal range. One month later, while the patient remained asymptomatic, a follow-up CXR revealed an enlarged pneumatocele (Figure 2A, B). A chest CT with IV contrast demonstrated a 7.2 x 5.3 x 7.6 cm air-filled cavity in the RUL (Figure 2C, D). No definite connection to the adjacent airways was seen on the chest CT. The patient then underwent a thoracoscopic right upper lobectomy. The procedure was challenging due to adhesions and bleeding (Figure 2E). The pathology examination of the resected lung tissue showed a simple fibrous-walled cyst devoid of epithelial lining, consistent with pneumatocele (Figure 2F). Gram stain, fungal smear, bacterial culture and fungal culture of the explanted lung tissue were negative. The patient made a full recovery, both radiologically and clinically.Patients with NP usually present with symptoms of CAP, unresponsiveness to initial outpatient treatment, such as high fever, cough, tachypnea, and general unwell appearance 1. The initial treatment of NP consists of IV antibiotics covering the most common etiologic agents of NP, which are known to be S. pneumoniae , Group AStreptococci and S. aureus . The optimal duration of antimicrobial therapy is not clearly defined; however, usually prolonged with a median duration of 4 weeks reported in the literature2, which aligns with guideline suggestions for therapy of empyema and parapneumonic effusion 3. Improvement in clinical and laboratory parameters usually allows for IV to oral antibiotic transition, which was accomplished relatively early for our patient, due to her less severe initial presentation and rapid clinical improvement. Pneumatoceles, air-filled cysts that can arise as a complication of NP, typically regress over weeks to months when NP is treated, but might require segmental or lobar resection if they become tense (exceeding more than 50% of the involved lobe), infected, or rupture 1. In our patient, many of the small pneumatoceles decreased in size after antibiotic treatment, with subsequent delayed and marked enlargement of one of them. A report on giant lung cysts emerging after NP suggested that, when patients remain clinically stable, treatment of pneumatoceles should be conservative with antibiotics alone regardless of the size of the cysts, as interventional procedures carry a risk of complications such as bronchopleural fistula 4. However, a study that proposed a treatment algorithm for pneumatoceles, recommended surgical resection for those that remained unresolved despite a conservative approach and gradually grew in size and wall thickness5. Our patient had no symptoms related to the pneumatocele. Nevertheless, it can be challenging to anticipate the progression of pneumatoceles, as they can enlarge enough to compromise respiration. The unusual expansion within a span of 1 month in our case, led to the decision of surgical resection.To summarize, we present a 3-year-old otherwise healthy girl with NP. After 4 weeks of antibiotic therapy, the right lung consolidations resolved and pneumatoceles decreased in size. However, one month later, while she remained clinically asymptomatic, a follow-up CXR revealed the progressive enlargement of a pneumatocele which eventually required surgical resection. Based on this experience, we suggest a close radiological follow-up of patients with post-infectious pneumatoceles, regardless of symptoms, until complete radiologic resolution is demonstrated.References1. de Benedictis FM, Kerem E, Chang AB, Colin AA, Zar HJ, Bush A. Complicated pneumonia in children. Lancet. 2020;396(10253):786–798. doi:https://doi.org/10.1016/s0140-6736(20)31550-62. Masters IB, Isles AF, Grimwood K. Necrotizing pneumonia: an emerging problem in children? Pneumonia (Nathan). 2017;9(1). doi:https://doi.org/10.1186/s41479-017-0035-03. Bradley JS, Byington CL, Shah SS, Alverson B, Carter ER, Harrison C, Kaplan SL, Mace SE, McCracken GH, Moore MR, et al. The Management of Community-Acquired Pneumonia in Infants and Children Older Than 3 Months of Age: Clinical Practice Guidelines by the Pediatric Infectious Diseases Society and the Infectious Diseases Society of America. Clin Infect Dis. 2011;53(7):e25–e76. doi:https://doi.org/10.1093/cid/cir5314. Gross I, Gordon O, Cohen‐Cymberknoh M, Reiter J, Tsabari R, Gileles‐Hillel A, Erlichman I, Hevroni A, Shoseyov D, Kerem E. Giant lung cysts following necrotizing pneumonia: Resolution with conservative treatment. Pediatr Pulmonol. 2019;54(6):901–906. doi:https://doi.org/10.1002/ppul.243215. Imamoğlu M, Cay A, Koşucu P, Ozdemir O, Cobanoğlu U, Orhan F, Akyol A, Sarihan H. Pneumatoceles in postpneumonic empyema: an algorithmic approach. J Pediatr Surg. 2005;40(7):1111–1117. doi:https://doi.org/10.1016/j.jpedsurg.2005.03.048Authors and affiliations : Sila Y. Kocer 1, Nathan C. Hull MD 2, D. Dean Potter, Jr. MD3, Theresa Madigan MD 4, Jennifer M. Boland MD 5 and Nadir Demirel MD 61Ondokuz Mayis University School of Medicine, Samsun, Turkey2Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Infectious Diseases, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Pulmonology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of interest: The authors declare no conflict of interest.

Pediatric sleep-related breathing disorders, or sleep-disordered breathing (SDB), cover a range of conditions including obstructive sleep apnea (OSA), central sleep apnea (CSA), sleep-related hypoventilation disorders, and sleep-related hypoxemia disorder. Pediatric SDB is often underdiagnosed, potentially due to difficulties associated with performing the gold standard polysomnography (PSG) in children. This scoping review aims to: 1) provide an overview of the studies reporting on safe, noncontact monitoring of respiration in young children; 2) describe the accuracy of these techniques, and 3) highlight their respective advantages and limitations. PubMed and EMBASE were searched for studies researching techniques in children <12 years old. Both quantitative data and the quality of the studies were analyzed. The evaluation of study quality was conducted using the QUADAS-2 tool. A total of 17 studies were included. Techniques could be grouped into bed-based methods, ultra-wideband (UWB) radar, Doppler radar, video, infrared (IR) cameras, garment-embedded sensors, and sound analysis. Most either measured respiratory rate (RR) or detected apneas; five aimed to do both. Noncontact sleep monitoring techniques are safe, but accuracy data of these techniques is limited, and large heterogeneity exists regarding study quality and stage. Motion artifacts affect accuracies of apnea detection. Sleep respiration analysis could benefit from sleep stage classification and breathing sound analysis. Further research is crucial to facilitate easily accessible and safe non-contact methods for respiration monitoring in a home setting.

Background Cystic fibrosis (CF) related diabetes affects up to half of all adults with CF and is associated with higher morbidity and mortality. Our aim is to co-design an ideal model of care that integrates diabetes technology and better meets the needs of adults living with the condition to improve attendance, engagement, service satisfaction and clinical outcomes. Methods Using qualitative research methods, we evaluated disease perceptions, barriers and enablers to optimal CF-related diabetes management and service delivery. Integration of continuous glucose monitoring (CGM) was also explored. An initial broad purposive consumer survey was followed by focus groups with end-users. Grounded theory approach was utilized with major problem-areas identified then explored, coded and grouped into requisites for an ‘ideal model of care’ for adults living with CF-related diabetes. Results Two key themes emerged i) CGM was acceptable for use in adults with CF-related diabetes with many perceived benefits and should be integrated into the model of care, ii) an ideal model of care consisted of a dual-specialty service co-led by endocrinology and CF physicians and supported by diabetes nurse educator and CF dietitian with a goal to provide consistent and personalized diabetes management. Barriers to optimizing glycaemic control included diet, finger-prick testing, reduced access to CGM and pulmonary exacerbations. End-user feedback on CGM was overwhelmingly positive with regards to user operability. CGM was also identified as a tool that could be used to engage, educate and empower adults living with CF-related diabetes and facilitate constructive and personalized clinical decision-making by healthcare providers. Conclusion For adults living with CF, a diagnosis of diabetes is associated with increased treatment burden. End-users agreed CGM had many benefits and should be integrated into an ‘ideal model of care’ for CF-related diabetes that was co-led by endocrinology services integrated within a pre-existing CF service.

Title: Determinants of school absences due to respiratory tract infections among children during COVID-19 pandemic: a cross-sectional study of the Sentinel Schools Network.Antoni Soriano-Arandes1,●,○, Andreu Colom-Cadena2,3,●, Anna Bordas2,3, Fabiana Ganem2,3,4, Lucia Alonso3,5, Marcos Montoro2,3, Mireia Gascon6,7,8, Maria Subirana6,7,8, Ariadna Mas9, Jordi Sunyer6,7,8, Pere Soler-Palacin1, Jordi Casabona2,3,4,8 ; on behalf of Sentinel School Network of Catalonia*Paediatric Infectious Diseases and Immunodeficiencies Unit. Children’s Hospital. Vall d’Hebron Barcelona Hospital Campus, Barcelona, Catalonia, Spain.Centre d’Estudis Epidemiològics sobre les ITS i Sida de Catalunya (CEEISCAT). Badalona, Spain.Institut d’Investigació Germans Trias i Pujol (IGTP), Badalona, Spain.Departament de Pediatria, d’Obstetrícia i Ginecologia i de Medicina Preventiva i de Salut Publica, Universitat Autònoma de Barcelona, Bellaterra, Spain.Fundació Lluita contra les infeccions, Badalona, Spain.ISGlobal, Barcelona. Spain.Universitat Pompeu Fabra (UPF), Barcelona, SpainCentro de Investigación Biomédica en Red de Epidemiología y Salud Pública (CIBERESP) Instituto de Salud Carlos III, Madrid, Spain.Direcció Assistencial d’Atenció Primària i Comunitària, Institut Català de la Salut, Barcelona, Catalonia, Spain● Contributed equally to this work with: Antoni Soriano-Arandes, Andreu Colom-Cadena○ Corresponding author: Antoni Soriano-Arandes,[email protected]. ORCID: 0000-0001-9613-7228Keywords: COVID-19, respiratory infections, ventilation, school, CO2, Indoor air qualityTo the editor:Different public health measures were implemented during the COVID-19 pandemic to maintain the schools open1,2. Regarding COVID-19 vaccination, at the beginning of this study, more than 92% and 40% of population older than 12 years and children aged 5-11 years in Spain was fully vaccinated against COVID-19, respectively3.Ventilation was proposed as one of the main strategies to reduce aerosol transmission for SARS-CoV-2 during the pandemic. A study from Germany assessed the efficiency of air purifiers in reducing aerosols in high-school classrooms4. However, no clinical endpoints were investigated and, therefore, a very low certainty of evidence was graded by a Cochrane review5. In an Italian study, the authors concluded that ventilation reduced the likelihood of SARS-CoV-2 infection in classrooms by 80%6. Moreover, a survey study from the US reported that elementary schools with face masks and ventilation strategies in place had lower SARS-CoV-2 incidence rates7. In a Lancet Task Force Commission review8, the authors concluded that improving building ventilation systems may carry benefits beyond protection from COVID-19. However, there is scarce evidence that demonstrate the association of epidemiological and environmental risk factors, including CO2 concentrations, with the incidence of respiratory tract infections (RTI) in children who are attending schools.The present study is part of COVID-19 Sentinel Schools Network of Catalonia (CSSNC), a project including 23 schools and 5687 students, teachers and other school staff with the aim to monitor SARS-CoV-2 and other respiratory viruses, their determinants and preventive measures in Catalonia9. The objectives of this study are to assess the potential association of demographic and epidemiological factors, including the indoor environmental conditions (CO2levels) in the classrooms, with students’ school absence due to RTI.We conducted a cross-sectional study in two different periods, the first from April 19 to June 21, 2022, and the second from November 8 to December 21, 2022. The main outcome of the study was the absence of the child from attending the school in person due to RTI or other non-respiratory medical causes. The study population was composed of 253 students (4-11 years) belonging to 20 classrooms, 11 in spring 2022 and 9 in autumn 2022, attending 4 and 16 classrooms of preschool (4 to 5 years-old) and primary school (6 to 11 years-old) stages, respectively. A written informed consent was previously obtained from parents or guardians of children.Absences from school attendance were notified by the tutor/teacher to the study researchers. We proceed to collect epidemiological and clinical/diagnostic data within the first 48 hours through a case report form deposited on the digital platform REDCap©. All the absences (cases) were followed-up through telephone calls made by health professionals until their return to the school. RTI was registered according to the symptoms described during the telephonic interview and confirmed through the computerised health record programme of the Health Ministry (eCAP).Sensors to monitor the CO2 concentrations (DIOXCARE DX700 PDF, Smartcare Services, Spain) were installed in classrooms, recording data every 10 minutes. For the analysis we only used the measurements taken during the time in which the students were in the classroom. We obtained a median [IQR] of CO2 levels which was used to conduct our analyses. All the participating schools received an operational protocol to install the sensor correctly and download data weekly.We calculated the RTI incidence per week and for the total study period. We performed a descriptive analysis of all survey variables, stratifying them by respiratory-related absence or absence due to other causes. Additionally, we ran a univariate logistic model to study the association between the type of absence and each described variable to obtain the corresponding odds ratios, 95% confidence intervals and p-values.Furthermore, we conducted a Latent Class Analysis (LCA) to explore potential groups of students with similar symptomatology. We started exploring the optimal number of latent classes, trying two to five classes, and we selected the best model using the entropy criterion, which indicates the accuracy of the latent classes, combined with other goodness of fit criteria such as BIC, cAIC and likelihood ratio. All these measures suggested that the optimal number of classes was two. However, we also examined the rest of number of classes to see if their classification had more clinical significance. All statistical analyses were performed in R (version 4.2.2).We registered one-hundred and five school absences during the study, 43 in the first study period (spring 2022), and 62 in the last one (autumn 2022) (Figure 1S ). Among these, we could obtain data related to the diagnosis in 98 (93%) cases, the rest of them were lost during the follow-up of the absence. Seventy-one absences were respiratory-related and 27 were due to other causes, mainly with gastrointestinal symptoms (66.7%). Among the absences due to RTI, the most were upper RTI (56/71, 78.9%), and only six (8.5%) were confirmed infections, 2 caused by influenza virus and 4 due to SARS-CoV-2 infection. These results represent a RTI incidence of 15.9 and 33.1 cases per 100 population in the first and second study period, respectively. The maximum weekly RTI incidence was of 10.2 cases per 100 population in December 12-18, 2022.The clinical, epidemiological and environmental characteristics of study sample are summarized in table 1 , categorized by type of absence (respiratory versus non-respiratory). We found a statistically significant association with absences due to RTI when someone else at home had respiratory symptoms (OR=9.12, CI 95%=2.54-33.39). We found a positive association between higher median levels of CO2at class and respiratory-related absences (OR=1.2, CI 95%=0.98-1.46). Moreover, there were more respiratory absences in autumn (OR=2.4, CI 95%=0.97-5.94). However, these last two associations did not reach statistical significance at a level of 0.05; although their p-values were lower than 0.1.No other epidemiological risk factors were associated with RTI incidence, such as household floor level, number of people living at home, living with smokers, having any comorbidity or being vaccinated against COVID-19.Finally, in Figure 1 , we present the symptomatology of absences belonging to each of the two latent classes. We can see that in the first cluster, the most frequent symptoms were cough, nasal congestion and fever, whereas in the second one, fever, gastrointestinal symptoms, and fatigue predominated.Our findings confirm that the RTI incidence during the study period was very high in children attending in the CNSSC schools, and the main medical cause of school absence. Although differences on school absence due to RTI were observed between spring and autumn, they were not statistically significant and in any case they may be due to the respiratory viruses’ seasonal pattern. In fact, the most important and significantly associated risk factor for RTI was the presence of someone else at home with respiratory symptoms, suggesting that households could be the main setting for initiating of the transmission of RTI.We observed a slightly association between RTI and median of CO2 levels in classrooms (p=0.07), which is an indicator of the degree of ventilation. However, we cannot exclude other potential factors such as rainfall, ambient temperature, or air pollutants (e.g. PM2.5, NO2, etc.) influencing on this outcome, as suggested by other authors10. To our knowledge, previous studies assessed the CO2concentration as a proxy of ventilation to evaluate the risk transmission of SARS-CoV-2 in schools11,12, but they did not analyse the association between CO2 median values and RTI incidence.Finally, we studied the symptomatology associated with the school absences through a LCA. The best approach to differentiate RTI from other causes was using two latent classes, and the most frequent symptoms were cough, nasal congestion and fever.The major strength of this study is our extensive data collection on clinical, epidemiological and environmental factors related to the school and also to the households of the participants. However, there were limitations such as possible incomplete reporting of RTI or insufficient sample size to determine small effect sizes. CO2 concentration was only measured in a selection of classrooms per school, so it may not be representative for the entire study period and school.In conclusion, RTI incidence was very high during the study period being the most important and significantly associated factor with RTI to have anyone else at home with respiratory symptoms. This suggests that households and not schools could be the key epidemiological factor for initiating the transmission of RTI to the children. Improving household preventive measures could reduce childhood RTI. In the LCA, the most frequent symptoms associated with RTI were cough, nasal congestion and fever. Although we found a slightly association between RTI and reduced ventilation we cannot exclude other potential factors influencing on this outcome. The study has been crucial to assess the feasibility and potential utility of collecting both school absence and morbidity data for further developing a systematic monitoring system.

Introduction. Respiratory syncytial virus infection is the leading cause of lower respiratory infection globally. Recently, nirsevimab has been approved to prevent RSV infection. This study explores the economically justifiable price of nirsevimab for preventing RSV infection in Colombia’s children under one year of age. Materials and methods. A static model was developed using the decision tree microsimulation to estimate the quality-adjusted costs and life years of two interventions: a single intramuscular dose of nirsevimab versus not applying nirsevimab. This analysis was made during a time horizon of 1 year and from a societal perspective. Results The annual savings in Colombia associated with this cost per dose ranged from U$ 2.5 to 4.1 million. Based on thresholds of U$4828, U$ 5128, and U$19 992 per QALY evaluated in this study, we established economically justifiable drug acquisition prices of U$ 21.88, U$ 25.04, and U$ 44.02 per dose of nirsevimab. Conclusion the economically justifiable cost for nirsevimab in Colombia is between U$21 to U$44 per dose, depending on the WTP used to decide its implementation. This result should encourage more studies in the region that optimize decision-making processes when incorporating this drug into the health plans of each country.

Ventilator associated respiratory tract infections (VARTI) are among the most common indications for hospitalization among children with chronic respiratory failure requiring at-home ventilation. This review aims to provide an overview of the key clinical features, diagnostic approaches, and management strategies for home VARTIs while highlighting the challenges in diagnosis and management.

A document by Miles Weinberger. Click on the document to view its contents.

Paediatric home invasive mechanical ventilation patients are a small but resource intensive cohort, requiring close monitoring and multidisciplinary care. Patients are often dependent on their ventilator for life support, with any significant complications such as equipment failure, tracheostomy blockage, or accidental decannulation becoming potentially life threatening, if not identified quickly. This review discusses the indications and variations in practice worldwide, in terms of models of care, including home care provision, choice of equipment and monitoring. With advances in technology, optimal monitoring strategies for home, continue to be debated: In-built ventilator alarms are often inadequately sensitive for paediatric patients, necessitating additional external monitoring devices to minimise risk. Pulse oximetry has been the preferred monitoring modality at home, though in some special circumstances such as congenital central hypoventilation syndrome, home carbon dioxide monitoring may be important to consider. Children should be under regular follow up at specialist respiratory centres where clinical evaluation, nocturnal oximetry and capnography monitoring and/or poly(somno)graphy and analysis of ventilator download data can be performed regularly to monitor progress. Recent exciting advances in technology, particularly in telemonitoring, which have potential to hugely benefit this complex group of patients are also discussed.

A Pediatric Case of Neuromyelitis Optica and Pulmonary Inflammatory Myofibroblastic TumorAlyson Win, BS ([email protected])a : Conceptualization; writing- original draft; writing- review and editing.Jesper Jiang, BS ([email protected])a:Conceptualization; writing- original draft. John Fitzwater, MD ([email protected])b : writing- review and editing; supervision. Edwin Hernandez Caro, MD ([email protected])c : writing- review and editing. Amy Cruickshank, DO ([email protected])d : writing- review and editing. Duriel Hardy, MD ([email protected])e : writing- review and editing. Ydamis Estrella Perez, MD ([email protected])f : writing- review and editing. Michele Prater, PNP ([email protected])c: writing- review and editing. Malvika Sagar, MD ([email protected])c : Conceptualization; writing- review and editing; supervision.a: Texas A&M Health Science Center School of Medicine, Temple, TXb: Department of Pediatric Surgery, Baylor Scott and White McClane Children’s Medical Center, Temple, TXc: Department of Pediatric Pulmonology, Baylor Scott and White McClane Children’s Medical Center, Temple, TXd: Department of Pediatric Hematology/Oncology, Baylor Scott and White McClane Children’s Medical Center, Temple, TXe: Department of Pediatric Neurology, Dell Children’s Medical Center, Austin, TXf: Department of Pathology, Baylor Scott and White Medical Center, Temple, TX

As our understanding of the uncommon group of disorders called plastic bronchitis has evolved, so too has the classification of these disorders.

Background: Globally, observational studies have demonstrated an association between high levels of air pollution and asthma attacks in children. It remains unclear whether and to what extent exposure may be associated with increased near fatal and fatal attacks. Objective: To systematically review the evidence for an association between ambient outdoor air pollution and fatal and/or near fatal asthma (NFA). Methods: Following Cochrane methodology, we searched MEDLINE, EMBASE, Web of Science, Scopus and Open Grey electronic databases for studies reporting the association of fatal/NFA and air pollution (Particulate Matter (PM), sulphur dioxide, nitrogen dioxide, black carbon and ozone) in children. NFA was defined as requiring intensive care (ICU) management. Results: Two reviewers independently screened 1,358 papers. 276 studies identified asthma attacks related to air pollution, 272 did not meet inclusion criteria after full text review. Four observational studies described fatal/NFA, of which 3 addressed NFA. PM2.5 and ozone (22ppb) were associated with NFA in one study (RR 1.26 CI (1.10-1.44)). PM10 was associated with ICU admission in the context of thunderstorm asthma. Elemental carbon was associated equally with NFA that did not require an ICU admission (p=0.67). Studies of fatal asthma including children did not demarcate age within analysis. Conclusions: Ozone and PM2.5 have been associated with NFA in children but synthesis is limited by the paucity of studies and methodological heterogeneity. Poor reporting of severities of asthma attack does not enable assessment of whether outdoor air pollution is associated with an increased number of NFA and fatal attacks in children.

Advanced cystic fibrosis liver disease (aCFLD) with portal hypertension develops in a minority of people (5-7.5%) with Cystic Fibrosis (CF), necessitating surgical interventions, including portosystemic shunting, gastrostomy tube placement, and in some cases, liver transplantation. This manuscript also aims to explore the different endoscopic, endovascular and surgical procedures needed to diagnose and manage aCFLD, including liver biopsy, endoscopic retrograde cholangiopancreatography, intraoperative cholangiograms, hepatic venous pressure gradient measurement and portosystemic shunt procedures in patients with aCFLD. By utilizing advanced diagnostic and surgical techniques, healthcare professionals can effectively manage aCFLD and improve patient outcomes.

Tracheostomies are indicated in children to facilitate long-term ventilatory support, aid in the management of secretions, or to manage upper airway obstruction. Children with tracheostomies often experience ongoing airway complications, of which respiratory tract infections are common. They subsequently receive frequent courses of broad spectrum antimicrobials for the prevention or treatment of respiratory tract infections. However, there is little consensus in practice with regard to the indication for treatment/ prophylactic antimicrobial use, choice of antimicrobial, route of administration, or duration of treatment between different centres. Routine antibiotic use is associated with adverse effects and an increased risk of antimicrobial resistance. Tracheal cultures are commonly obtained from paediatric tracheostomy patients, with the aim of helping guide antimicrobial therapy choice. However, a positive culture alone is not diagnostic of infection and the role of routine surveillance cultures remains contentious. Inhaled antimicrobial use is also widespread in the management of tracheostomy associated infections; this is largely based upon theoretical benefits of higher airway antibiotic concentrations. The role of prophylactic inhaled antimicrobial use for tracheostomy associated infections remains largely unproven. This systematic review summarises the current evidence base for antimicrobial selection, duration, and administration route in paediatric tracheostomy associated infections. It also highlights significant variation in practice between centres and the urgent need for further prospective evidence to guide the management of these vulnerable patients.

Background: CFTR-Related Metabolic Syndrome/ Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CRMS/CFSPID) is the diagnosis in infants who have a positive Cystic Fibrosis (CF) newborn screen (NBS), 2 CF-causing mutations, and borderline or normal sweat test. NY State implemented a new CF NBS algorithm (IRT-DNA-SEQ) in December 2017 with significant improvement in positive predictive value. This algorithm also resulted in detection of more CRMS cases. For these infants repeat sweat testing is recommended at 6, 12, and 18 months to monitor for risk of rising sweat chloride over time and 6-48% of infants with CRMS develop clinical features suggestive of CF. Infants with CRMS and sweat test results in the normal range are often lost to follow and parents were unwilling to return for recommended repeat sweat testing during the statewide lockdown during the peak of COVID-19 pandemic. We recognized the practice gap exacerbated during the pandemic and underscore the importance of establishing a medical home in a CF Center for longitudinal care. Methods: Retrospective analysis of infants with CRMS from December 2017 to December 2020 were collected by 10 NY CF Centers and the NBS program with NYU as the data collection and statistical analysis site. Infants with CRMS without repeat sweat chloride testing at 6 months of age were considered lost to follow up, and their parents were contacted via mail or telephone. Families completed a questionnaire that was developed with the assistance of CF Voice to evaluate parental understanding of CRMS and the recommendation for repeat sweat chloride testing. Primary care providers (PCPs) caring for infants with CRMS were also contacted and provided educational materials about CRMS. A subcommittee of CF Center Directors met to develop a statewide approach for the management of infants with CRMS. Results: Of 350 infants diagnosed with CRMS, 179 (51.1%) infants were lost to follow up. As an outcome of this QI effort 31 (17.3%) were scheduled for repeat sweat tests and follow up at CF Centers. This QI effort explored the knowledge and practice gap among PCPs with limited understanding of the implications of a CRMS diagnosis. CF Center Directors subcommittee issued a consensus statement regarding evaluation and follow up for infants with CRMS in NY. Conclusions: This QI effort effectively recaptured infants with CRMS previously lost to follow up. Consensus recommendations for CRMS include annual visits until 2-6 years of age for repeat sweat testing and in adolescence to educate the patient about clinical and reproductive implications of CRMS.

Pathogenic variants in the Surfactant Protein C gene ( SFTPC) result in fibrotic childhood interstitial lung disease (chILD). We previously reported three children with SFTPC pathogenic variants with respiratory failure who were supported by chronic invasive ventilation via tracheostomy as an alternative to lung transplantation or comfort care [(1)](#ref-0001). We present two children with SFTPC pathogenic variants treated with non-invasive ventilation (NIV) (Figure 1).

In recent decades, the patient survival is increased due to the advances in intensive care units and development of modern mechanic ventilators. Unfortunately, it is not always possible to wean these children from mechanical ventilation. Recently, after placement a tracheostomy tube, they can support at home with non-invasive or invasive mechanical ventilation. Most of the children who need ventilation support at home have neurological impairment. The nutritional issues and gastrointestinal complications are well defined in critically ill patients, but there are very limited studies on the children with tracheostomy. Considering that majority of the patients has neuromuscular disorders, the nutritional and gastrointestinal problems of the children with tracheostomy are discussed, in the light of the knowledge on critically ill patients.

We are presenting a teenage girl with a recent history of virilism who presented with tension pneumothorax. Subsequent contrast-enhanced chest CT revealed multiple bilateral irregular thin-walled cysts of varying sizes with numerous solid noncalcified pulmonary nodules and pulmonary artery pseudoaneurysms. Lung biopsy revealed trophoblastic neoplasm with metastasis to both lungs. We discussed the differential diagnosis of diffuse cystic lung diseases. While diffuse cystic lung diseases in children are rarely due to malignancy, we emphasize that clinicians be aware of the possibility of metastatic lung diseases and their radiologic features when encountering diffuse cystic lung diseases.

Introduction: Elective flexible bronchoscopy (FB) is now widely available and standard practice for a variety of indications in children with respiratory conditions. However, there is limited evidence regarding the utility of elective FB in children. This systematic review aimed to determine the utility of FB on its impact in clinical decision making and patient-reported outcome measures (PROMs). Methods: We searched Pubmed, Cochrane central register of controlled trials, Embase, World Health Organisation Clinical Trials Registry Platform and Cochrane database of systematic reviews from inception to 20 th April 2023. We included systematic reviews (SRs) and randomised controlled trials (RCTs) that used parallel group design (comparing use of elective FB versus no FB, or a wait-list approach [early FB versus usual wait FB]) in children aged ≤ 18 years. Our protocol was prospectively registered and used Cochrane methodology for systemic reviews of interventions. Results: Our search identified 859 articles; 102 duplicates were removed, and 753 articles were excluded by title and abstract. Four full text articles were reviewed and subsequently excluded, as none met the inclusion criteria outlined in our PICO framework. Conclusions: Given the paucity of data, there is need for high-quality evidence to support the routine use of elective FB in children with respiratory conditions. We recommend clinicians adhere to current international guidelines until such evidence emerges. Registration: PROSPERO CRD42021291305.